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ECC: enfermedad cardiaca congénita; ECG: electrocardiograma; ETC: enfermedades del tejido conectivo; ETT: ecocardiografía transtorácica; EVOP: enfermedad venooclusiva pulmonar; HAP: hipertensión arterial pulmonar; HCP: hemangiomatosis capilar pulmonar; HP: hipertensión pulmonar; HPTEC: hipertensión pulmonar tromboembólica crónica; PAPm: presión arterial pulmonar media; PAWP: presión de oclusión arterial pulmonar; PFR: pruebas funcionales respiratorias; RVP: resistencia vascular pulmonar; Rx: radiografía; TC: tomografía computarizada; UW: unidades Wood; V-Q: ventilación-perfusión; VD: ventrículo derecho; VIH: virus de la inmunodeficiencia humana.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Joan Albert Barberà, Antonio Román, Miguel Ángel Gómez-Sánchez, Isabel Blanco, Remedios Otero, Raquel López-Reyes, Isabel Otero, Gregorio Pérez-Peñate, Ernest Sala, Pilar Escribano" "autores" => array:10 [ 0 => array:2 [ "nombre" => "Joan Albert" "apellidos" => "Barberà" ] 1 => array:2 [ "nombre" => "Antonio" "apellidos" => "Román" ] 2 => array:2 [ "nombre" => "Miguel Ángel" "apellidos" => "Gómez-Sánchez" ] 3 => array:2 [ "nombre" => "Isabel" "apellidos" => "Blanco" ] 4 => array:2 [ "nombre" => "Remedios" "apellidos" => "Otero" ] 5 => array:2 [ "nombre" => "Raquel" "apellidos" => "López-Reyes" ] 6 => array:2 [ "nombre" => "Isabel" "apellidos" => "Otero" ] 7 => array:2 [ "nombre" => "Gregorio" "apellidos" => "Pérez-Peñate" ] 8 => array:2 [ "nombre" => "Ernest" "apellidos" => "Sala" ] 9 => array:2 [ "nombre" => "Pilar" "apellidos" => "Escribano" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S157921291830051X" "doi" => "10.1016/j.arbr.2017.11.017" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S157921291830051X?idApp=UINPBA00003Z" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0300289617304362?idApp=UINPBA00003Z" "url" => "/03002896/0000005400000004/v2_201804150418/S0300289617304362/v2_201804150418/es/main.assets" ] ] "itemSiguiente" => array:19 [ "pii" => "S157921291830048X" "issn" => "15792129" "doi" => "10.1016/j.arbr.2017.07.021" "estado" => "S300" "fechaPublicacion" => "2018-04-01" "aid" => "1695" "copyright" => "SEPAR" "documento" => "article" "crossmark" => 1 "subdocumento" => "sco" "cita" => "Arch Bronconeumol. 2018;54:216" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:2 [ "total" => 1272 "formatos" => array:3 [ "EPUB" => 132 "HTML" => 784 "PDF" => 356 ] ] "en" => array:11 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Clinical image</span>" "titulo" => "Tuberculoma Cavitation: A Rare Aspect of a Common Disease Clinical Image" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:1 [ "paginaInicial" => "216" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Tuberculoma cavitado: un patrón inusual en la imagen clínica de una enfermedad común" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1000 "Ancho" => 1538 "Tamanyo" => 174084 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Chest CT image obtained with the mediastinal window setting (A) shows a nodular soft-tissue lesion with internal calcification located in the apicoposterior segment of the left upper lobe (tuberculoma). 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Hipertensión Pulmonar, Servicio de Cardiología, Hospital 12 de Octubre, Madrid, Spain" "etiqueta" => "d" "identificador" => "aff0020" ] 4 => array:3 [ "entidad" => "Unidad Médico-Quirúrgica de Enfermedades Respiratorias, Hospital Universitario Virgen del Rocío, Instituto de Biomedicina de Sevilla (IBiS), Sevilla, Spain" "etiqueta" => "e" "identificador" => "aff0025" ] 5 => array:3 [ "entidad" => "Servicio de Neumología, Hospital Universitari i Politècnic La Fe, Valencia, Spain" "etiqueta" => "f" "identificador" => "aff0030" ] 6 => array:3 [ "entidad" => "Servicio de Neumología, Complexo Hospitalario Universitario, A Coruña, Spain" "etiqueta" => "g" "identificador" => "aff0035" ] 7 => array:3 [ "entidad" => "Unidad Multidisciplinar de Circulación Pulmonar, Servicio de Neumología, Hospital Universitario de Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria, Spain" "etiqueta" => "h" "identificador" => "aff0040" ] 8 => array:3 [ "entidad" => "Servicio de Neumología, Hospital Son Espases, Palma de Mallorca, Spain" "etiqueta" => "i" "identificador" => "aff0045" ] 9 => array:3 [ "entidad" => "Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Instituto de Salud Carlos III, Madrid, Spain" "etiqueta" => "j" "identificador" => "aff0050" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Guía de diagnóstico y tratamiento de la hipertensión pulmonar: resumen de recomendaciones" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 2019 "Ancho" => 2917 "Tamanyo" => 255774 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Therapeutic algorithm for pulmonary arterial hypertension.</p> <p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">CCB: calcium channel blockers; FC: functional class.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">This document is a summary of the recommendations of the Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension prepared by the Spanish Society of Pulmonology and Thoracic Surgery,<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">1</span></a> that was drawn from the clinical practice guidelines of the European Society of Cardiology and the European Respiratory Society.<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">2</span></a> For more details, please refer to the original guidelines,<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">1</span></a> (<a id="intr9070" class="elsevierStyleInterRef" href="https://issuu.com/separ/docs/normativa_70?e=3049452/44188557">https://issuu.com/separ/docs/normativa_70?e=3049452/44188557</a>). The levels of evidence and class of recommendation used are set out in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Definition and Classification</span><p id="par0010" class="elsevierStylePara elsevierViewall">Pulmonary hypertension (PH) is a hemodynamic, pathophysiological disorder defined by elevated mean pulmonary arterial pressure (mPAP) ≥25<span class="elsevierStyleHsp" style=""></span>mmHg, measured by right heart catheterization (RHC).<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">2</span></a> PH can occur in various clinical processes, that can be classified into 5 groups (<a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>).</p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Diagnosis of Pulmonary Hypertension</span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Detection</span><p id="par0015" class="elsevierStylePara elsevierViewall">Transthoracic echocardiography (TTE) is the main tool for the early detection and screening of PH. The probability of PH according to TTE findings is shown in <a class="elsevierStyleCrossRef" href="#tbl0015">Table 3</a>.</p><elsevierMultimedia ident="tbl0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">TTE screening for PH is recommended in asymptomatic subjects in the following risk groups:<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">–</span><p id="par0025" class="elsevierStylePara elsevierViewall">Patients with systemic sclerosis [I, B].</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">–</span><p id="par0030" class="elsevierStylePara elsevierViewall">First-degree relatives of patients with a diagnosis of hereditary pulmonary arterial hypertension (PAH) [I, C].</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">–</span><p id="par0035" class="elsevierStylePara elsevierViewall">Patients with portal hypertension who are candidates for liver transplantation [I, B].</p></li></ul></p><p id="par0040" class="elsevierStylePara elsevierViewall">In other cases, TTE will be performed on the basis of clinical suspicion.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">General Approach to Diagnosis</span><p id="par0045" class="elsevierStylePara elsevierViewall">The diagnostic algorithm of PH is shown in <a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>. TTE will be performed if PH is suspected. If the probability of PH is intermediate or high, left heart disease (PH group 2) and chronic respiratory disease (PH group 3) will be ruled out. Patients in these PH groups or those with severe right ventricular dysfunction will be referred to an expert in PH<span class="elsevierStyleSup">2</span> [IIa, C]. When PH has been ruled out in groups 2 and 3, ventilation–perfusion lung scintigraphy will be used to rule out thromboembolic disease. If perfusion defects are observed on the ventilation–perfusion scintigraphy, a study for probable chronic thromboembolic pulmonary hypertension will be performed. Hemodynamic diagnosis with RHC will be carried out in an expert PH unit [I, C]. If PAH is confirmed, the subtype should be identified.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Pulmonary Arterial Hypertension</span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Evaluation</span><p id="par0050" class="elsevierStylePara elsevierViewall">In patients with idiopathic, hereditary, or drug-related PAH, a vasodilator test with inhaled nitric oxide or iv epoprostenol will be performed during the RHC diagnostic procedure [I, C]. The test is positive if mPAP drops ≥10<span class="elsevierStyleHsp" style=""></span>mmHg to reach a value ≤40<span class="elsevierStyleHsp" style=""></span>mmHg, with no reduction in cardiac output [I, C]. The subtype will be identified by contrast echocardiography, autoimmunity testing, hepatotropic virus serology, and HIV serology (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). If there is a family history of PH, or if it is suspected, a study to identify BMPR2 gene mutations is advisable.<a class="elsevierStyleCrossRefs" href="#bib0245"><span class="elsevierStyleSup">3–5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The diagnosis of pulmonary veno-occlusive disease (PVOD) or pulmonary capillary hemangiomatosis (PCH) is based on clinical data, very low carbon dioxide diffusing capacity, severe hypoxemia, and consistent findings on high-resolution computed tomography (HRCT).<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">6</span></a> It can also be diagnosed from the presence of EIF2AK4 gene mutations.<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">6</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">A set of variables associated with survival are used for evaluating prognosis<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">2</span></a> (<a class="elsevierStyleCrossRef" href="#tbl0020">Table 4</a>). For monitoring, clinical parameters and more easily performed tests (functional class [FC], 6-minute walk test, ECG, clinical laboratory tests) should be evaluated every 3–6 months, while the more complex procedures should be performed every 6–12 months,<a class="elsevierStyleCrossRef" href="#bib0265"><span class="elsevierStyleSup">7</span></a> or in case of clinical deterioration [I, C].</p><elsevierMultimedia ident="tbl0020"></elsevierMultimedia></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Treatment</span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">General Measures and Support</span><p id="par0065" class="elsevierStylePara elsevierViewall">General therapeutic measures for PAH are listed in <a class="elsevierStyleCrossRef" href="#tbl0025">Table 5</a>. Diuretics are indicated in patients with right ventricular failure and water retention [I, C]. Loop diuretics or aldosterone antagonists should be used.<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">2</span></a> Anticoagulation with vitamin K antagonists is recommended in idiopathic and hereditary PAH, and PAH caused by anorectics [IIb, C]. Oxygen therapy is recommended if PaO<span class="elsevierStyleInf">2</span> is <60<span class="elsevierStyleHsp" style=""></span>mmHg [I, C]. It may also be considered as an option for correcting desaturation during exercise.<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">2</span></a> Regular monitoring of iron levels is recommended, and supplements should be administered if necessary.</p><elsevierMultimedia ident="tbl0025"></elsevierMultimedia></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Specific Treatment</span><p id="par0070" class="elsevierStylePara elsevierViewall">Specific drugs for the treatment of PAH include (<a class="elsevierStyleCrossRef" href="#tbl0030">Table 6</a>):<ul class="elsevierStyleList" id="lis0010"><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">–</span><p id="par0075" class="elsevierStylePara elsevierViewall">Calcium channel blockers: indicated for use in patients with idiopathic PAH and positive vasodilator test [I, C]. High-dose nifedipine, diltiazem and amlodipine are recommended.<a class="elsevierStyleCrossRef" href="#bib0270"><span class="elsevierStyleSup">8</span></a></p></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">–</span><p id="par0080" class="elsevierStylePara elsevierViewall">Endothelin receptor antagonists, including ambrisentan, bosentan, and macitentan. Ambrisentan and bosentan can cause liver toxicity, so monthly monitoring of liver enzymes is required. Macitentan carries a risk of anemia, and regular monitoring of hemoglobin levels is recommended.</p></li><li class="elsevierStyleListItem" id="lsti0030"><span class="elsevierStyleLabel">–</span><p id="par0085" class="elsevierStylePara elsevierViewall">Phosphodiesterase type 5 (PDE5) inhibitors and soluble guanylate cyclase (sGC) stimulators: available PDE5 inhibitors are sildenafil and tadalafil, and the only available sGC stimulator is riociguat. The concomitant administration of PDE5 inhibitors and sGC stimulators is contraindicated.</p></li><li class="elsevierStyleListItem" id="lsti0035"><span class="elsevierStyleLabel">–</span><p id="par0090" class="elsevierStylePara elsevierViewall">Prostacyclin analogs and prostacyclin receptor agonists: available prostacyclin analogs include epoprostenol, administered via continuous iv infusion; iloprost, administered by inhalation; and treprostinil, administered in a continuous subcutaneous microinfusion pump. Inhaled treprostinil has also been shown to be beneficial<a class="elsevierStyleCrossRef" href="#bib0275"><span class="elsevierStyleSup">9</span></a> (<a class="elsevierStyleCrossRef" href="#tbl0020">Table 4</a>). Selexipag is a prostacyclin receptor agonist that is administered orally.<a class="elsevierStyleCrossRef" href="#bib0280"><span class="elsevierStyleSup">10</span></a></p></li></ul></p><elsevierMultimedia ident="tbl0030"></elsevierMultimedia></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Invasive Treatments</span><p id="par0095" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0015"><li class="elsevierStyleListItem" id="lsti0040"><span class="elsevierStyleLabel">–</span><p id="par0100" class="elsevierStylePara elsevierViewall">Atrial septostomy: indicated in patients with FC IV, with right ventricular failure,<a class="elsevierStyleCrossRef" href="#bib0285"><span class="elsevierStyleSup">11</span></a> or as a bridge treatment in patients waitlisted for transplantation [IIb, C]. This procedure can be performed in hospitals with experience. It should be avoided in patients with right atrial pressure >20<span class="elsevierStyleHsp" style=""></span>mmHg or SaO<span class="elsevierStyleInf">2</span> <85% breathing room air.</p></li><li class="elsevierStyleListItem" id="lsti0045"><span class="elsevierStyleLabel">–</span><p id="par0105" class="elsevierStylePara elsevierViewall">Lung transplantation: the most common procedure is double lung transplantation. This is indicated in young patients without associated comorbidity who do not respond fully to medical treatment<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">2</span></a> [I, C]. This is the treatment of choice in PVOD and PCH.</p></li></ul></p></span><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Therapeutic Strategy</span><p id="par0110" class="elsevierStylePara elsevierViewall">The therapeutic strategy in PAH is based on 4 components (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>):</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0115" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Establish therapeutic objectives:</span> the main aim is to ensure that the patient's risk of death is low (<a class="elsevierStyleCrossRef" href="#tbl0020">Table 4</a>) [I, C]. Characteristics of a low-risk profile include good tolerance to exertion, quality of life, and right ventricular function. The risk will be defined at the beginning, before starting treatment, and in the periodic monitoring visits [I, C].</p><p id="par0120" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Initial approach:</span> this includes general measures (<a class="elsevierStyleCrossRef" href="#tbl0025">Table 5</a>) and supportive therapy. Hemodynamic diagnosis with vasodilator test should be performed in an expert PH unit [I, B], since the result will help define the risk profile and establish the course of treatment.</p><p id="par0125" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Initial treatment</span>: in patients with positive vasodilator response, treatment will begin with high-dose calcium channel blockers. If clinical response at 3 months is inadequate, other specific drugs will be used.</p><p id="par0130" class="elsevierStylePara elsevierViewall">Patients with a low or intermediate risk with a negative vasodilator response will begin treatment with specific drugs in monotherapy or combination (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Endothelin receptor antagonists, PDE5 inhibitors and sGC stimulators in monotherapy have been effective in patients with FC II and III. Prostanoids have been evaluated in patients with FC III. The choice of drug is based on the route of administration, safety profile, possible interactions with other drugs, comorbidities, the amount and quality of the available evidence, patient preferences, experience of the physician, and cost.</p><p id="par0135" class="elsevierStylePara elsevierViewall">If combined treatment is selected from the outset, the only combination that has shown superiority to single-agent therapy is ambrisentan plus tadalafil<a class="elsevierStyleCrossRef" href="#bib0290"><span class="elsevierStyleSup">12</span></a> [I, B].</p><p id="par0140" class="elsevierStylePara elsevierViewall">In patients with a high-risk profile or FC IV, the treatment of choice is intravenous epoprostenol<a class="elsevierStyleCrossRef" href="#bib0295"><span class="elsevierStyleSup">13</span></a> [I, A]. Evidence shows that combined initial treatment with epoprostenol plus 1 or 2 drugs is effective<a class="elsevierStyleCrossRef" href="#bib0300"><span class="elsevierStyleSup">14</span></a> [IIa, C].</p><p id="par0145" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Response assessment</span>: response to treatment at 3–4 months will be evaluated [I, C]. If response is unsatisfactory, a second or third drug will be added, and possible referral of the patient for lung transplant evaluation will be considered.<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">2</span></a> All patients should be followed periodically in an expert PH unit. Visit intervals will be established on the basis of disease severity, but should never be longer than 6 months, even in patients with satisfactory clinical response.<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">2</span></a></p></span></span><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Subtype Considerations</span><span id="sec0070" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Congenital Heart Disease</span><p id="par0150" class="elsevierStylePara elsevierViewall">Congenital heart diseases are included in PH groups 1, 2, 3, and 5, depending on the underlying mechanism. <a class="elsevierStyleCrossRef" href="#tbl0035">Table 7</a> lists the PAH classifications associated with congenital heart disease and <a class="elsevierStyleCrossRef" href="#tbl0040">Table 8</a> summarizes recommendations for pharmacological treatment. The following limits are proposed for systemic-pulmonary shunt ligation [IIa, C]: it is indicated if pulmonary vascular resistance is <4<span class="elsevierStyleHsp" style=""></span>Wood<span class="elsevierStyleHsp" style=""></span>units·m<span class="elsevierStyleSup">2</span> and contraindicated for >8<span class="elsevierStyleHsp" style=""></span>Wood<span class="elsevierStyleHsp" style=""></span>units·m<span class="elsevierStyleSup">2</span>. Intermediate situations will be assessed individually.<a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">15</span></a></p><elsevierMultimedia ident="tbl0035"></elsevierMultimedia><elsevierMultimedia ident="tbl0040"></elsevierMultimedia></span><span id="sec0075" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Connective Tissue Diseases</span><p id="par0155" class="elsevierStylePara elsevierViewall">PAH associated with systemic sclerosis is the most common presentation.<a class="elsevierStyleCrossRef" href="#bib0310"><span class="elsevierStyleSup">16</span></a> Annual screening with TTE and carbon dioxide diffusing capacity is recommended in patients with systemic sclerosis<a class="elsevierStyleCrossRef" href="#bib0315"><span class="elsevierStyleSup">17</span></a> [I, C]. In other connective tissue diseases, TTE is recommended in symptomatic individuals. Chest HRCT is useful for evaluating the presence of interstitial lung disease and PVOD.<a class="elsevierStyleCrossRef" href="#bib0320"><span class="elsevierStyleSup">18</span></a> RHC is recommended whenever PAH is suspected [I, C]. Patients with scleroderma and mPAP 21–24<span class="elsevierStyleHsp" style=""></span>mmHg should be monitored due to their high risk of developing PAH.<a class="elsevierStyleCrossRef" href="#bib0325"><span class="elsevierStyleSup">19</span></a></p><p id="par0160" class="elsevierStylePara elsevierViewall">Patients with connective tissue diseases and PAH should be treated according to the general PAH algorithm [I, C]. Oral anticoagulation is associated with a worse prognosis,<a class="elsevierStyleCrossRef" href="#bib0330"><span class="elsevierStyleSup">20</span></a> so it should only be used in patients prone to thrombophilia (antiphospholipid antibodies)<a class="elsevierStyleCrossRef" href="#bib0335"><span class="elsevierStyleSup">21</span></a> [IIb, C]. Immunosuppressive therapy may benefit patients with PAH associated with systemic lupus erythematosus or mixed connective tissue disease.<a class="elsevierStyleCrossRef" href="#bib0340"><span class="elsevierStyleSup">22</span></a></p></span><span id="sec0080" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Portopulmonary Hypertension</span><p id="par0165" class="elsevierStylePara elsevierViewall">Portopulmonary hypertension is defined as combined portal and pulmonary hypertension. Patients with portopulmonary hypertension have higher mortality than patients with idiopathic PAH,<a class="elsevierStyleCrossRefs" href="#bib0345"><span class="elsevierStyleSup">23,24</span></a> so referral to expert centers is recommended [I, C]. The use of anticoagulants [III, C] and beta-blockers is not recommended.<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">25</span></a> Portopulmonary hypertension is a major risk factor in liver transplantation,<a class="elsevierStyleCrossRef" href="#bib0360"><span class="elsevierStyleSup">26</span></a> so it must be ruled out by TTE in all transplant candidates [I, B] and these results must be confirmed with a hemodynamic study. If mPAP is <35<span class="elsevierStyleHsp" style=""></span>mmHg, transplantation can be considered<a class="elsevierStyleCrossRef" href="#bib0360"><span class="elsevierStyleSup">26</span></a> [IIb, C]. If mPAP is ≥35<span class="elsevierStyleHsp" style=""></span>mmHg, specific therapy with reevaluation at 3 months is recommended. If PAP remains high despite treatment, liver transplantation is contraindicated [III, C].</p></span><span id="sec0085" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">HIV Infection</span><p id="par0170" class="elsevierStylePara elsevierViewall">TTE to detect PAH should be performed in cases of unexplained dyspnea [III, C]. Anticoagulation is not recommended because of the risk of bleeding and possible drug interactions [III, C]. Account should be taken of interactions between PDE5 inhibitors and some antiretroviral drugs.</p></span><span id="sec0090" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">Pulmonary Veno-occlusive Disease and Pulmonary Capillary Hemangiomatosis</span><p id="par0175" class="elsevierStylePara elsevierViewall">PVOD and PCH share clinical, pathological, and genetic characteristics, and treatment is the same.<a class="elsevierStyleCrossRef" href="#bib0365"><span class="elsevierStyleSup">27</span></a> PVOD may be associated with systemic sclerosis, HIV infection, or drugs. The familial form is caused by mutations in the biallelic EIF2AK4 gene.<a class="elsevierStyleCrossRef" href="#bib0370"><span class="elsevierStyleSup">28</span></a></p><p id="par0180" class="elsevierStylePara elsevierViewall">Diagnosis is established by clinical criteria, physical examination, bronchoscopy, and radiological tests [I, C], or identification of the EIF2AK4 gene mutation [I, B].</p><p id="par0185" class="elsevierStylePara elsevierViewall">Vasodilators can cause pulmonary edema in PVOD/PCH. Lung transplantation is the treatment of choice, so patients should be referred to a lung transplantation unit after diagnosis [I, C].</p></span></span><span id="sec0095" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0115">Special Situations</span><p id="par0190" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Pregnancy and birth control:</span> due to the high risk of mortality, patients with PAH should avoid pregnancy [I, C]. The combined use of 2 contraceptive methods is advisable. Progestins are preferable to estrogens. If pregnancy occurs, patients should be informed of the risk and termination should be proposed. Patients who decide to take the risk and continue the pregnancy should be monitored closely in a center with expertise in PH and high-risk pregnancies.</p><p id="par0195" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Surgery:</span> surgery confers a high risk of morbidity and mortality, especially if it is unscheduled,<a class="elsevierStyleCrossRef" href="#bib0375"><span class="elsevierStyleSup">29</span></a> and should be performed in PH reference centers. Epidural anesthesia is preferable to general anesthesia.<a class="elsevierStyleCrossRef" href="#bib0375"><span class="elsevierStyleSup">29</span></a></p><p id="par0200" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Right heart failure:</span> diuretics provide symptomatic benefits. In situations requiring ICU admission, the patient's water balance must be optimized with intravenous diuretics, right ventricular overload must be minimized (generally with intravenous prostanoids), cardiac output must be optimized (preferably with dobutamine). Intubation, which frequently produces hemodynamic collapse, should be avoided. Extracorporeal membrane oxygenation (ECMO) and other devices should be considered in selected patients.<a class="elsevierStyleCrossRef" href="#bib0380"><span class="elsevierStyleSup">30</span></a></p></span></span><span id="sec0100" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0120">Pulmonary Hypertension due to Left Heart Disease</span><p id="par0205" class="elsevierStylePara elsevierViewall">PH associated with left heart disease is classified as post-capillary. Two types have been identified: Isolated post-capillary PH and combined pre-capillary and post-capillary PH, depending on diastolic pressure gradient values and pulmonary vascular resistance (<a class="elsevierStyleCrossRef" href="#tbl0045">Table 9</a>).</p><elsevierMultimedia ident="tbl0045"></elsevierMultimedia><p id="par0210" class="elsevierStylePara elsevierViewall">Differential diagnosis between PAH and group 2 PH can be complex, particularly in patients with PH and heart failure with preserved ejection fraction. Attention will be given to the characteristics indicated in <a class="elsevierStyleCrossRef" href="#tbl0050">Table 10</a>.<a class="elsevierStyleCrossRef" href="#bib0385"><span class="elsevierStyleSup">31</span></a></p><elsevierMultimedia ident="tbl0050"></elsevierMultimedia><p id="par0215" class="elsevierStylePara elsevierViewall">The approach focuses on optimizing heart failure treatment [I, B]. Patients with severe combined post-capillary and pre-capillary PH should be referred to expert centers for inclusion in clinical trials and/or individualized treatment [IIa, C]. The use of drugs indicated for PAH is not recommended [III, C].</p></span><span id="sec0105" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0125">Pulmonary Hypertension due to Respiratory Disease</span><p id="par0220" class="elsevierStylePara elsevierViewall">Respiratory diseases most commonly associated with PH are COPD, interstitial lung diseases, and the combination of pulmonary fibrosis and emphysema. <a class="elsevierStyleCrossRef" href="#tbl0055">Table 11</a> shows the hemodynamic classification of PH in this group. PH is usually mild or moderate.<a class="elsevierStyleCrossRef" href="#bib0390"><span class="elsevierStyleSup">32</span></a> Severe PH is most often seen in the combination of pulmonary fibrosis and emphysema (CPFE) and is often associated with a disproportionately reduced carbon dioxide diffusing capacity and low PaCO<span class="elsevierStyleInf">2</span>.<a class="elsevierStyleCrossRef" href="#bib0395"><span class="elsevierStyleSup">33</span></a></p><elsevierMultimedia ident="tbl0055"></elsevierMultimedia><p id="par0225" class="elsevierStylePara elsevierViewall">TTE is the examination of choice for the detection of PH [I, C], although its accuracy in patients with advanced respiratory disease is low. This procedure is indicated if significant PH is suspected or to rule out left heart disease.</p><p id="par0230" class="elsevierStylePara elsevierViewall">The definitive diagnosis of PH is established with RHC. Indications include: 1) correct diagnosis or exclusion of PH in candidates for surgery (transplantation, lung volume reduction); 2) suspected concomitant PAH or chronic thromboembolic pulmonary hypertension (CTEPH); 3) repeated episodes of right heart failure, and 4) inconclusive TTE in cases with high suspicion of PH.<a class="elsevierStyleCrossRef" href="#bib0400"><span class="elsevierStyleSup">34</span></a></p><p id="par0235" class="elsevierStylePara elsevierViewall">The treatment of choice in patients with COPD and hypoxemic PH is continuous home oxygen therapy [I, C]. The role of oxygen therapy is less clear in interstitial disease.</p><p id="par0240" class="elsevierStylePara elsevierViewall">Conventional vasodilators or specific PAH drugs are not recommended in COPD patients with mild-moderate PH [III, C].<a class="elsevierStyleCrossRefs" href="#bib0240"><span class="elsevierStyleSup">2,35</span></a> The use of ambrisentan and riociguat is contraindicated in idiopathic pulmonary fibrosis [III, A]. Patients with respiratory disease and severe PH should be referred for individualized treatment in a hospital specializing in both conditions [I, C].</p></span><span id="sec0110" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0130">Chronic Thromboembolic Pulmonary Hypertension</span><p id="par0245" class="elsevierStylePara elsevierViewall">A diagnosis of CTEPH is established by the presence of pulmonary thrombosis and pre-capillary PH, after more than 3 months of appropriate anticoagulation.</p><p id="par0250" class="elsevierStylePara elsevierViewall">The diagnostic algorithm for CTEPH (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>) has 2 components: hemodynamic diagnosis by RHC and localization of thrombotic lesions using imaging techniques (angio-CT and selective pulmonary digital subtraction angiography).</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0255" class="elsevierStylePara elsevierViewall">There are 3 treatment options in CTEPH (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>):<ul class="elsevierStyleList" id="lis0020"><li class="elsevierStyleListItem" id="lsti0050"><span class="elsevierStyleLabel">1.</span><p id="par0260" class="elsevierStylePara elsevierViewall">Surgery</p></li></ul></p><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0265" class="elsevierStylePara elsevierViewall">Pulmonary endarterectomy (PEA) is the treatment of choice [I, C]. This intervention can achieve cure of CTEPH and is appropriate in more than 60% of cases. All patients diagnosed with CTEPH should be evaluated for possible PEA by a multidisciplinary team that includes a specialized surgeon in a hospital with experience in this type of surgery [I, C]. There are 2 accredited centers in Spain.<a class="elsevierStyleCrossRefs" href="#bib0410"><span class="elsevierStyleSup">36–38</span></a><ul class="elsevierStyleList" id="lis0025"><li class="elsevierStyleListItem" id="lsti0055"><span class="elsevierStyleLabel">2.</span><p id="par0270" class="elsevierStylePara elsevierViewall">Medical intervention</p></li></ul></p><p id="par0275" class="elsevierStylePara elsevierViewall">Patients with CTEPH should receive chronic anticoagulation, even after PEA [I, C]. Vitamin K antagonists are recommended, since there is no evidence to support the use of the new oral anticoagulants. Currently, the only drug specifically indicated for CTEPH is riociguat<a class="elsevierStyleCrossRef" href="#bib0425"><span class="elsevierStyleSup">39</span></a> [I, B]. Beneficial effects have been demonstrated with macitentan,<a class="elsevierStyleCrossRef" href="#bib0430"><span class="elsevierStyleSup">40</span></a> and to some extent with bosentan.<a class="elsevierStyleCrossRef" href="#bib0435"><span class="elsevierStyleSup">41</span></a> Pharmacological treatment is indicated in patients in whom surgery has been ruled out by an expert multidisciplinary committee in PEA and if PH persists after PEA [I, B].<ul class="elsevierStyleList" id="lis0030"><li class="elsevierStyleListItem" id="lsti0060"><span class="elsevierStyleLabel">3.</span><p id="par0280" class="elsevierStylePara elsevierViewall">Pulmonary angioplasty</p></li></ul></p><p id="par0285" class="elsevierStylePara elsevierViewall">Pulmonary balloon angioplasty is a new procedure that has provided good outcomes in patients with obstructive lesions that cannot be accessed with PEA,<a class="elsevierStyleCrossRefs" href="#bib0440"><span class="elsevierStyleSup">42–44</span></a> although the available evidence is still scant.<a class="elsevierStyleCrossRef" href="#bib0455"><span class="elsevierStyleSup">45</span></a> This procedure should only be performed in hospitals with extensive experience in CTEPH, after PEA has been ruled out.<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">2</span></a></p></span><span id="sec0115" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0135">Pulmonary Hypertension With Unclear or Multifactorial Mechanisms</span><p id="par0290" class="elsevierStylePara elsevierViewall">This group includes various etiological processes: hematologic diseases, systemic diseases, metabolic disorders, and a miscellaneous group of disorders (<a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>). Diagnosis is difficult, so management in hospitals with experience in PH is advisable. Currently there is no specific treatment for this group.</p></span><span id="sec0120" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0140">Healthcare Organization</span><p id="par0295" class="elsevierStylePara elsevierViewall">Primary forms of PH (groups 1, 4, and 5) (<a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>) are rare serious diseases that require complex procedures for diagnosis and treatment. The broad consensus is that patients with diseases of these characteristics should be seen in specialized referral units with experience in the disease.<a class="elsevierStyleCrossRefs" href="#bib0240"><span class="elsevierStyleSup">2,46</span></a> In 2008, the Spanish Society of Pulmonology and Thoracic Surgery and the Spanish Society of Cardiology prepared a consensus document in which they proposed a healthcare organization for the care of PH patients in Spain based on expert PH units that interact in a network with hospitals at a local level.<a class="elsevierStyleCrossRef" href="#bib0460"><span class="elsevierStyleSup">46</span></a> The criteria that expert PH units must meet, as established in the clinical guidelines of the European Society of Cardiology-European Respiratory Society,<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">2</span></a> are shown in <a class="elsevierStyleCrossRef" href="#tbl0060">Table 12</a>. Three CSURs (centers, services or units of reference) for complex PH cases, appointed by the Ministry of Health, have been operating in Spain since 2015.</p><elsevierMultimedia ident="tbl0060"></elsevierMultimedia><p id="par0300" class="elsevierStylePara elsevierViewall">Given the organizational structure of the Spanish healthcare system, care in the area of PH must be set up a network of networks, with expert PH units on an autonomous community level, which interact with associated sites within the autonomous community itself. CSURs, which can deliver PEA programs and care in more complex patients and situations, must operate on a national level.</p><p id="par0305" class="elsevierStylePara elsevierViewall">Expert PH units must set up protocols for consultation circuits and referral for specific diseases and clinical situations: CTEPH (PEA, pulmonary angioplasty), lung transplantation, portopulmonary hypertension, congenital heart disease, connective tissue diseases, genetic studies, elective surgery and care of the pregnant patient.</p></span><span id="sec0125" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0145">Conflict of Interests</span><p id="par0310" class="elsevierStylePara elsevierViewall">Dr. Barberà has received fees from Actelion, Bayer, GlaxoSmithKline, Merck Sharp & Dohme, and Pfizer, and has received funding from Actelion, Bayer, GlaxoSmithKline and Pfizer, not related with this publication. Dr. Blanco has received fees from Merck Sharp & Dohme, not related with this publication. Dr. Otero Candelera has received fees from Actelion, Bayer, Rovi, Leo Pharma, and Merck Sharp & Dohme, and has received funding from Bayer and Leo Pharma, not related with this publication. Dr. Lopez-Reyes has received fees from Actelion, and funding from GlaxoSmithKline, Ferrer, and Actelion, not related with this publication. Dr. Otero has received fees from Actelion, Bayer, Glaxo-SmithKline, and Ferrer, not related with this publication. Dr. Pérez-Peñate has received fees from Actelion, Bayer, and Merck Sharp & Dohme, not related with this publication. Dr. Sala declares no conflict of interests. Dr. Escribiano has received fees from Actelion, Bayer, GlaxoSmithKline, and Merck Sharp & Dohme, and has received funding from Actelion, Bayer, GlaxoSmithKline and Ferrer, not related with this publication.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:16 [ 0 => array:3 [ "identificador" => "xres1015602" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec974214" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1015601" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec974215" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Definition and Classification" ] 6 => array:3 [ "identificador" => "sec0015" "titulo" => "Diagnosis of Pulmonary Hypertension" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0020" "titulo" => "Detection" ] 1 => array:2 [ "identificador" => "sec0025" "titulo" => "General Approach to Diagnosis" ] ] ] 7 => array:3 [ "identificador" => "sec0030" "titulo" => "Pulmonary Arterial Hypertension" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "sec0035" "titulo" => "Evaluation" ] 1 => array:3 [ "identificador" => "sec0040" "titulo" => "Treatment" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "sec0045" "titulo" => "General Measures and Support" ] 1 => array:2 [ "identificador" => "sec0050" "titulo" => "Specific Treatment" ] 2 => array:2 [ "identificador" => "sec0055" "titulo" => "Invasive Treatments" ] 3 => array:2 [ "identificador" => "sec0060" "titulo" => "Therapeutic Strategy" ] ] ] 2 => array:3 [ "identificador" => "sec0065" "titulo" => "Subtype Considerations" "secciones" => array:5 [ 0 => array:2 [ "identificador" => "sec0070" "titulo" => "Congenital Heart Disease" ] 1 => array:2 [ "identificador" => "sec0075" "titulo" => "Connective Tissue Diseases" ] 2 => array:2 [ "identificador" => "sec0080" "titulo" => "Portopulmonary Hypertension" ] 3 => array:2 [ "identificador" => "sec0085" "titulo" => "HIV Infection" ] 4 => array:2 [ "identificador" => "sec0090" "titulo" => "Pulmonary Veno-occlusive Disease and Pulmonary Capillary Hemangiomatosis" ] ] ] 3 => array:2 [ "identificador" => "sec0095" "titulo" => "Special Situations" ] ] ] 8 => array:2 [ "identificador" => "sec0100" "titulo" => "Pulmonary Hypertension due to Left Heart Disease" ] 9 => array:2 [ "identificador" => "sec0105" "titulo" => "Pulmonary Hypertension due to Respiratory Disease" ] 10 => array:2 [ "identificador" => "sec0110" "titulo" => "Chronic Thromboembolic Pulmonary Hypertension" ] 11 => array:2 [ "identificador" => "sec0115" "titulo" => "Pulmonary Hypertension With Unclear or Multifactorial Mechanisms" ] 12 => array:2 [ "identificador" => "sec0120" "titulo" => "Healthcare Organization" ] 13 => array:2 [ "identificador" => "sec0125" "titulo" => "Conflict of Interests" ] 14 => array:2 [ "identificador" => "xack342745" "titulo" => "Acknowledgements" ] 15 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2017-10-22" "fechaAceptado" => "2017-11-27" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec974214" "palabras" => array:5 [ 0 => "Pulmonary hypertension" 1 => "Pulmonary arterial hypertension" 2 => "Chronic thromboembolic pulmonary hypertension" 3 => "Pulmonary circulation" 4 => "Clinical guidelines" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec974215" "palabras" => array:5 [ 0 => "Hipertensión pulmonar" 1 => "Hipertensión arterial pulmonar" 2 => "Hipertensión pulmonar tromboembólica crónica" 3 => "Circulación pulmonar" 4 => "Guía clínica" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Pulmonary hypertension is a hemodynamic disorder defined by abnormally high pulmonary artery pressure that can occur in numerous diseases and clinical situations. The causes of pulmonary hypertension are classified into 5 major groups: arterial, due to left heart disease, due to lung disease and/or hypoxemia, chronic thromboembolic, with unclear and/or multifactorial mechanisms. This is a brief summary of the Guidelines on the Diagnostic and Treatment of Pulmonary Hypertension of the Spanish Society of Pulmonology and Thoracic Surgery. These guidelines describe the current recommendations for the diagnosis and treatment of the different pulmonary hypertension groups.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La hipertensión pulmonar es un trastorno hemodinámico definido por el aumento anómalo de la presión arterial pulmonar, que puede presentarse en numerosas enfermedades y situaciones clínicas. Las causas de hipertensión pulmonar se clasifican en 5 grandes grupos: arterial, debida a cardiopatía izquierda, debida a enfermedad pulmonar y/o hipoxemia, tromboembólica crónica y de mecanismo no establecido y/o multifactorial. El presente documento expone de forma resumida las recomendaciones de la Guía de Diagnóstico y Tratamiento de la Hipertensión Pulmonar de la Sociedad Española de Neumología y Cirugía Torácica. En dicha guía se presentan las pautas actuales de diagnóstico y tratamiento de los distintos grupos de hipertensión pulmonar.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0030">Please cite this article as: Barberà JA, Román A, Gómez-Sánchez MÁ, Blanco I, Otero R, López-Reyes R, et al. Guía de diagnóstico y tratamiento de la hipertensión pulmonar: resumen de recomendaciones. Arch Bronconeumol. 2018;54:205–215.</p>" ] ] "multimedia" => array:16 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 3962 "Ancho" => 2917 "Tamanyo" => 492776 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Diagnostic algorithm for pulmonary hypertension.</p> <p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">CHD: congenital heart disease; CT: computed tomography; CTD: connective tissue disease; CTEPH: chronic thromboembolic pulmonary hypertension; ECG, electrocardiogram; HIV: human immunodeficiency virus; HRCT: high-resolution computed tomography; mPAP: mean pulmonary arterial pressure; PAH: pulmonary arterial hypertension; PAWP: pulmonary arterial wedge pressure; PCH: pulmonary capillary hemangiomatosis; PH: pulmonary hypertension; PVOD: pulmonary veno-occlusive disease; PVR: pulmonary vascular resistance; RFT: respiratory function tests; RHC: right heart catheterization; RV: right ventricle; Rx: chest X-ray; TTE: transthoracic echocardiography; V-Q: ventilation–perfusion; WU: Wood units.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 2019 "Ancho" => 2917 "Tamanyo" => 255774 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Therapeutic algorithm for pulmonary arterial hypertension.</p> <p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">CCB: calcium channel blockers; FC: functional class.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Fig. 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1883 "Ancho" => 2667 "Tamanyo" => 235658 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Diagnostic algorithm for pulmonary hypertension.</p> <p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">CTEPH: chronic thromboembolic pulmonary hypertension; CT: computed tomography; PAH: pulmonary arterial hypertension; PH: pulmonary hypertension; V-Q: ventilation–perfusion.</p>" ] ] 3 => array:7 [ "identificador" => "fig0020" "etiqueta" => "Fig. 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 2948 "Ancho" => 2667 "Tamanyo" => 308689 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Therapeutic algorithm for chronic thromboembolic pulmonary hypertension.</p> <p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">PEA: pulmonary endarterectomy; PH: pulmonary hypertension.</p>" ] ] 4 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at1" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry " colspan="2" align="left" valign="top"><span class="elsevierStyleItalic">Levels of evidence</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>A \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Data derived from multiple randomized clinical trials or meta-analysis \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>B \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Data derived from a single randomized or large non-randomized studies \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Consensus of expert opinion, small or retrospective studies, or patient registries \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="2" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="2" align="left" valign="top"><span class="elsevierStyleItalic">Classes of recommendation</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Evidence and/or general agreement that a particular treatment or procedure is beneficial, useful, or effective \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>II \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Conflicting evidence and/or diverging opinions about the usefulness/effectiveness of a given treatment or procedure \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>IIa \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Evidence/opinion tends toward usefulness/effectiveness \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>IIb \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Usefulness/effectiveness is less supported by evidence/opinion \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>III \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Evidence or general agreement that a particular treatment or procedure is not useful/effective and, in some cases, may be harmful \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1720466.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Levels of Evidence and Class of Recommendation Used in the Guidelines.</p>" ] ] 5 => array:8 [ "identificador" => "tbl0010" "etiqueta" => "Table 2" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at2" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">1. Pulmonary arterial hypertension (PAH)</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">1.1. Idiopathic</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">1.2. Hereditary</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>1.2.1. BMPR2 mutation \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>1.2.2. Other mutations \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">1.3. Induced by drugs and toxins</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">1.4. Associated with:</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>1.4.1. Connective tissue disease \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>1.4.2. HIV infection \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>1.4.3. Portal hypertension \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>1.4.4. Congenital heart disease \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>1.4.5. Schistosomiasis \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">1′. Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">1′.1. Idiopathic</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">1′.2. Hereditary</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>1′.2.1. EIF2AK4 mutation \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>1′.2.2. Other mutations \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">1′.3. Induced by drugs, toxins, and radiation</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">1′.4. Associated with:</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>1′.4.1. Connective tissue disease \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>1′.4.2. HIV infection \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">1″. Persistent pulmonary hypertension of the newborn</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">2. Pulmonary hypertension due to left heart disease</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">2.1. Left ventricular systolic dysfunction</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">2.2. Left ventricular diastolic dysfunction</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">2.3. Valvular disease</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">2.4. Congenital/acquired obstruction of the left ventricular inflow/outflow tract and congenital cardiomyopathy</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">2.5. Congenital or acquired pulmonary vein stenosis</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">3. Pulmonary hypertension due to respiratory disease and/or hypoxemia</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">3.1. Chronic obstructive pulmonary disease</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">3.2. Diffuse interstitial lung disease</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">3.3. Other lung diseases with mixed restrictive and obstructive pattern</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">3.4. Sleep disordered breathing</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">3.5. Alveolar hypoventilation</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">3.6. Chronic exposure to high altitudes</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">3.7. Lung development disorders</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">4. Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">4.1. Chronic thromboembolic pulmonary hypertension</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">4.2. Other pulmonary artery obstructions</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>4.2.1. Angiosarcoma \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>4.2.2. Other intravascular tumors \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>4.2.3. Arteritis \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>4.2.4. Congenital pulmonary artery stenosis \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>4.2.5. Parasitosis (hydatid disease) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">5. Pulmonary hypertension with unclear and/or multifactorial mechanisms</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">5.1. Hematologic diseases: hemolytic anemia, myeloproliferative disorders, splenectomy</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">5.2. Systemic diseases: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis, neurofibromatosis</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">5.3. Metabolic disorders: glycogen storage disease, Gaucher's disease, thyroid disorders</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">5.4. Other: pulmonary tumor thrombotic microangiopathy, fibrosing mediastinitis, chronic renal failure (with/without dialysis), segmental pulmonary hypertension</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1720470.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Classification of Pulmonary Hypertension (European Society of Cardiology/European Respiratory Society, 2015).</p>" ] ] 6 => array:8 [ "identificador" => "tbl0015" "etiqueta" => "Table 3" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at3" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0070" class="elsevierStyleSimplePara elsevierViewall">PA: pulmonary artery; RV, right ventricle; LV, left ventricle; TRV: tricuspid regurgitation velocity.</p>" "tablatextoimagen" => array:2 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Low \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">TRV ≤2.8<span class="elsevierStyleHsp" style=""></span>m/s or not measurable \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Intermediate \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">TRV 2.9–3.4<span class="elsevierStyleHsp" style=""></span>m/s; or VRT ≤2.8<span class="elsevierStyleHsp" style=""></span>m/s or not measurable, in the presence of other ultrasonographic signs of pulmonary hypertension \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">High \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">TRV >3.4<span class="elsevierStyleHsp" style=""></span>m/s; or TRV 2.9–3.4<span class="elsevierStyleHsp" style=""></span>m/s, in the presence of other ultrasonographic signs of pulmonary hypertension \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1720475.png" ] ] 1 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">* Other echocardiographic signs that indicate pulmonary hypertension:</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">Ventricles</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>Basal RV/LV ratio >1.0 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>Flattening of interventricular septum (LV eccentricity index >1.1 in systole or diastole) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">Pulmonary artery</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>Doppler acceleration time of the RV outflow tract <105<span class="elsevierStyleHsp" style=""></span>ms and/or mesosystolic notch \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>Pulmonary regurgitation velocity in protodiastole >2.2<span class="elsevierStyleHsp" style=""></span>m/s \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>PA diameter >25<span class="elsevierStyleHsp" style=""></span>mm \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">Inferior vena cava and right atrium</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>Inferior vena cava diameter >21<span class="elsevierStyleHsp" style=""></span>mm with decreased inspiratory collapse (<50% in deep inspiration or <20% in normal inspiration) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>Right atrium area (end systolic) >18<span class="elsevierStyleHsp" style=""></span>cm<span class="elsevierStyleSup">2</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1720474.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Probability of Pulmonary Hypertension Based on Transthoracic Echocardiography Results.</p>" ] ] 7 => array:8 [ "identificador" => "tbl0020" "etiqueta" => "Table 4" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at4" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0080" class="elsevierStyleSimplePara elsevierViewall">6MWT: 6-minute walk test; BNP: brain natriuretic peptide; CI: cardiac index; MRI: magnetic resonance imaging; NT-proBNP: N-terminal prohormone brain natriuretic peptide; RA: right atrium; RAP: right atrial pressure; SvO<span class="elsevierStyleInf">2</span>: oxygen saturation of mixed venous blood; VE/VCO<span class="elsevierStyleInf">2</span>: ratio between minute ventilation and CO<span class="elsevierStyleInf">2</span> production; VO<span class="elsevierStyleInf">2</span>-peak: peak oxygen uptake.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td-with-role" title="table-head ; entry_with_role_rowhead " align="left" valign="top" scope="col">Prognostic Determinant \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " colspan="3" align="center" valign="top" scope="col" style="border-bottom: 2px solid black">Risk</th></tr><tr title="table-row"><th class="td" title="table-head " align="" valign="top" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Low \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Intermediate \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">High \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Clinical signs of right heart failure \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Absent \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Absent \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Present \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Progression of symptoms \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">No \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Slow \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Rapid \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Syncope \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">No \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Occasional \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Repeated \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Functional class \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I, II \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">III \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">IV \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Distance walked on 6MWT \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">>440<span class="elsevierStyleHsp" style=""></span>m \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">165–440<span class="elsevierStyleHsp" style=""></span>m \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top"><165<span class="elsevierStyleHsp" style=""></span>m \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Cardiopulmonary exertion test \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">VO<span class="elsevierStyleInf">2</span>-peak >15<span class="elsevierStyleHsp" style=""></span>mL/kg/min (>65% ref.)<br>VE/VCO<span class="elsevierStyleInf">2</span> <36 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">VO<span class="elsevierStyleInf">2</span>-peak 11–15<span class="elsevierStyleHsp" style=""></span>mL/kg/min (35–65% ref.)<br>VE/VCO<span class="elsevierStyleInf">2</span> 36–44.9 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">VO<span class="elsevierStyleInf">2</span>-peak <11<span class="elsevierStyleHsp" style=""></span>mL/kg/min (<35% ref.)<br>VE/VCO<span class="elsevierStyleInf">2</span> ≥45 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">BNP or NT-proBNP \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">BNP<50<span class="elsevierStyleHsp" style=""></span>ng/L<br>NT-proBNP <300<span class="elsevierStyleHsp" style=""></span>ng/L \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">BNP 50–300<span class="elsevierStyleHsp" style=""></span>ng/L<br>NT-proBNP 300–1400<span class="elsevierStyleHsp" style=""></span>ng/L \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">BNP >300<span class="elsevierStyleHsp" style=""></span>ng/L<br>NT-proBNP >1400<span class="elsevierStyleHsp" style=""></span>ng/L \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Imaging techniques (echocardiography, MRI) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">RA area <18<span class="elsevierStyleHsp" style=""></span>cm<span class="elsevierStyleSup">2</span><br>No pericardial effusion \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">RA area 18–26<span class="elsevierStyleHsp" style=""></span>cm<span class="elsevierStyleSup">2</span><br>Without or with minimal pericardial effusion \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">RA area >26<span class="elsevierStyleHsp" style=""></span>cm<span class="elsevierStyleSup">2</span><br>Pericardial effusion \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hemodynamics \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">RAP <8<span class="elsevierStyleHsp" style=""></span>mmHg<br>CI ≥2.5<span class="elsevierStyleHsp" style=""></span>L/min/m<span class="elsevierStyleSup">2</span><br>SvO<span class="elsevierStyleInf">2</span> >65% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">RAP 8–14<span class="elsevierStyleHsp" style=""></span>mmHg<br>CI 2.0–2.4<span class="elsevierStyleHsp" style=""></span>L/min/m<span class="elsevierStyleSup">2</span><br>SvO<span class="elsevierStyleInf">2</span> 60–65% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">RAP >14<span class="elsevierStyleHsp" style=""></span>mmHg<br>CI <2.0<span class="elsevierStyleHsp" style=""></span>L/min/m<span class="elsevierStyleSup">2</span><br>SvO<span class="elsevierStyleInf">2</span> <60% \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1720471.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0075" class="elsevierStyleSimplePara elsevierViewall">Prognostic Evaluation in Pulmonary Arterial Hypertension.</p>" ] ] 8 => array:8 [ "identificador" => "tbl0025" "etiqueta" => "Table 5" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at5" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0090" class="elsevierStyleSimplePara elsevierViewall">PVOD: pulmonary veno-occlusive disease; PAH: pulmonary arterial hypertension.</p><p id="spar0095" class="elsevierStyleSimplePara elsevierViewall">All recommendations have a level of evidence C unless otherwise indicated.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Recommended measures [I]</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Avoid pregnancy \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Prevention of infections \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Psychosocial support \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Measures that should be taken into consideration [class IIa]</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Supervised training [level of evidence B] \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Oxygen therapy during long flights \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Elective surgery should be carried out in centers with experience in pulmonary hypertension \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Measures that could be taken into consideration [class IIb]</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Genetic counseling in specialized units of patients or family members with mutations associated with PAH or PVOD \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Avoid drugs that can aggravate PH (nasal decongestants and beta-blockers) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Diet: advise a daily salt intake of <5<span class="elsevierStyleHsp" style=""></span>g (equivalent to 2<span class="elsevierStyleHsp" style=""></span>g sodium), particularly in patients with right heart failure. If RHF is severe or in case of hyponatremia, reduction of water intake to <1.5–2<span class="elsevierStyleHsp" style=""></span>L/day is also advisable. \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Inadvisable activities [III]</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Strenuous physical activity \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Being at altitudes above 1500–2000<span class="elsevierStyleHsp" style=""></span>m without supplemental oxygen \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1720476.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0085" class="elsevierStyleSimplePara elsevierViewall">General Therapeutic Measures in Pulmonary Arterial Hypertension.</p>" ] ] 9 => array:8 [ "identificador" => "tbl0030" "etiqueta" => "Table 6" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at6" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:3 [ "leyenda" => "<p id="spar0105" class="elsevierStyleSimplePara elsevierViewall">INH: inhaled; IV: intravenous; SC: subcutaneous; VO: oral.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head " align="" valign="top" scope="col"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="" valign="top" scope="col"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col">Route of Administration \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col">Dose \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " colspan="6" align="center" valign="top" scope="col" style="border-bottom: 2px solid black">Class of Recommendation/Level of Evidence<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a></th></tr><tr title="table-row"><th class="td" title="table-head " align="" valign="top" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="" valign="top" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="" valign="top" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="" valign="top" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " colspan="2" align="center" valign="top" scope="col" style="border-bottom: 2px solid black">FC II</th><th class="td" title="table-head " colspan="2" align="center" valign="top" scope="col" style="border-bottom: 2px solid black">FC III</th><th class="td" title="table-head " colspan="2" align="center" valign="top" scope="col" style="border-bottom: 2px solid black">FC IV</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry " colspan="2" align="left" valign="top">Calcium channel blockers (amlodipine, diltiazem, nifedipine)</td><td class="td" title="table-entry " align="left" valign="top">VO \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Nifedipine, 120–240<span class="elsevierStyleHsp" style=""></span>mg/day<br>Amlodipine, 20<span class="elsevierStyleHsp" style=""></span>mg/day<br>Diltiazem, 240–720<span class="elsevierStyleHsp" style=""></span>mg/day \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C<a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">b</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C<a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">b</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="10" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="3" align="left" valign="top">Endothelin receptor antagonists</td><td class="td" title="table-entry " align="left" valign="top">Ambrisentan \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">VO \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">5–10<span class="elsevierStyleHsp" style=""></span>mg/day \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">A \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">A \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">IIb \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Bosentan \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">VO \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">125<span class="elsevierStyleHsp" style=""></span>mg/12<span class="elsevierStyleHsp" style=""></span>h \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">A \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">A \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">IIb \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Macitentan<a class="elsevierStyleCrossRef" href="#tblfn0015"><span class="elsevierStyleSup">c</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">VO \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">10<span class="elsevierStyleHsp" style=""></span>mg/day \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">B \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">B \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">IIb \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="10" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="2" align="left" valign="top">Phosphodiesterase-5 inhibitors</td><td class="td" title="table-entry " align="left" valign="top">Sildenafil \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">VO \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">20<span class="elsevierStyleHsp" style=""></span>mg/8<span class="elsevierStyleHsp" style=""></span>h \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">A \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">A \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">IIb \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Tadalafil \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">VO \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">40<span class="elsevierStyleHsp" style=""></span>mg/day \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">B \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">B \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">IIb \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="10" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Soluble guanylate cyclase stimulators \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Riociguat \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">VO \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2.5<span class="elsevierStyleHsp" style=""></span>mg/8<span class="elsevierStyleHsp" style=""></span>h \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">B \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">B \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">IIb \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="10" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="4" align="left" valign="top">Prostacyclin analogs</td><td class="td" title="table-entry " align="left" valign="top">Epoprostenol<a class="elsevierStyleCrossRef" href="#tblfn0015"><span class="elsevierStyleSup">c</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">IV \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">20–40<span class="elsevierStyleHsp" style=""></span>ng/kg/min \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">A \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">A \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Iloprost \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">INH \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2.5–5<span class="elsevierStyleHsp" style=""></span>μg/3–4<span class="elsevierStyleHsp" style=""></span>h \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">B \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">IIb \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="2" align="left" valign="top">Treprostinil</td><td class="td" title="table-entry " align="left" valign="top">SC \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">20–80<span class="elsevierStyleHsp" style=""></span>ng/kg/min \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">B \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">IIb \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">INH \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">54<span class="elsevierStyleHsp" style=""></span>μg/6<span class="elsevierStyleHsp" style=""></span>h \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">B \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">IIb \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="10" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">IP prostacyclin receptor agonists \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Selexipag<a class="elsevierStyleCrossRef" href="#tblfn0015"><span class="elsevierStyleSup">c</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">VO \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1600<span class="elsevierStyleHsp" style=""></span>μg/12<span class="elsevierStyleHsp" style=""></span>h \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">B \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">B \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1720477.png" ] ] ] "notaPie" => array:3 [ 0 => array:3 [ "identificador" => "tblfn0005" "etiqueta" => "a" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">See <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>.</p>" ] 1 => array:3 [ "identificador" => "tblfn0010" "etiqueta" => "b" "nota" => "<p class="elsevierStyleNotepara" id="npar0010">Only for those patients with positive vasodilator test.</p>" ] 2 => array:3 [ "identificador" => "tblfn0015" "etiqueta" => "c" "nota" => "<p class="elsevierStyleNotepara" id="npar0015">Drug that has demonstrated delay in time to clinical deterioration as a primary objective in a clinical trial, or the reduction of all-cause death.</p>" ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0100" class="elsevierStyleSimplePara elsevierViewall">General Recommendations for Single-agent Treatment of Pulmonary Arterial Hypertension.</p>" ] ] 10 => array:8 [ "identificador" => "tbl0035" "etiqueta" => "Table 7" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at7" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:3 [ "leyenda" => "<p id="spar0115" class="elsevierStyleSimplePara elsevierViewall">CHD: congenital heart disease; PAH: pulmonary arterial hypertension; PVR: pulmonary vascular resistance.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">1. Eisenmenger's syndrome \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Includes large intra- and extracardiac defects which begin with systemic-to-pulmonary shunt and eventually progress to a severe elevated PVR and systemic-pulmonary shunt ligation or bidirectional shunt. Cyanosis, multiple organ failure and polycythemia tend to occur \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">2. PAH associated with prevalent systemic-pulmonary shunts \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Correctable<a class="elsevierStyleCrossRef" href="#tblfn0020"><span class="elsevierStyleSup">a</span></a><br>Not correctable<br>Includes moderate or large defects. PVR is slightly or moderately high and systemic-to-pulmonary shunt prevails. Cyanosis at rest is not characteristic \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">3. PAH with small defects or chance finding<a class="elsevierStyleCrossRef" href="#tblfn0025"><span class="elsevierStyleSup">b</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Marked elevation of PVR in presence of small cardiac defects (usually interventricular septal defects <1<span class="elsevierStyleHsp" style=""></span>cm or interatrial septal defects <2<span class="elsevierStyleHsp" style=""></span>cm in diameter, as assessed by echocardiography). The clinical picture is very similar to that of idiopathic PAH. Defect closure is contraindicated. \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">4. PAH associated with CHD with corrected heart defect \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">CHD is repaired but PAH persists immediately after correlation or recurs or develops months or years after the procedure. \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1720465.png" ] ] ] "notaPie" => array:2 [ 0 => array:3 [ "identificador" => "tblfn0020" "etiqueta" => "a" "nota" => "<p class="elsevierStyleNotepara" id="npar0020">With surgery or percutaneously.</p>" ] 1 => array:3 [ "identificador" => "tblfn0025" "etiqueta" => "b" "nota" => "<p class="elsevierStyleNotepara" id="npar0025">Size refers to adult patients. However, even in adults the diameter may be insufficient to define the hemodynamic significance of the defect or the pressure gradient, the directionality or size of the shunt, so pulmonary and systemic flow ratio must be taken into account.</p>" ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0110" class="elsevierStyleSimplePara elsevierViewall">Clinical Classification of Pulmonary Arterial Hypertension Associated With Congenital Heart Disease.</p>" ] ] 11 => array:8 [ "identificador" => "tbl0040" "etiqueta" => "Table 8" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at8" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0125" class="elsevierStyleSimplePara elsevierViewall">ERA: endothelin receptor antagonists; PDE5 inhibitors: phosphodiesterase type 5 inhibitors; SaO<span class="elsevierStyleInf">2</span>: oxygen saturation in arterial blood.</p><p id="spar0130" class="elsevierStyleSimplePara elsevierViewall">[Class of recommendation, level of evidence].</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">1. Anticoagulation is restricted to patients with atrial arrhythmias and/or thrombosis of the pulmonary arteries [IIb, C] \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">2. Supplemental oxygen is indicated if it improves clinical status and SaO<span class="elsevierStyleInf">2</span> [IIa, C] \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">3. Iron supplementation should be considered in the presence of iron deficiency [IIb, C] \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">4. Bosentan is the treatment of choice in patients with Eisenmenger's syndrome [I, B] \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">5. Combined treatment with ERA, PDE5 inhibitors, and/or prostanoids is indicated [IIa, C] \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">6. Lung transplantation with the closure of the defect is indicated in congenital heart disease, with heart-lung transplantation in complex cases. \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1720473.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0120" class="elsevierStyleSimplePara elsevierViewall">Pharmacological Treatment of Pulmonary Arterial Hypertension Associated With Congenital Heart Disease.</p>" ] ] 12 => array:8 [ "identificador" => "tbl0045" "etiqueta" => "Table 9" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at9" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0140" class="elsevierStyleSimplePara elsevierViewall">DPG: diastolic pressure gradient (diastolic PAP−PAWP); mPAP: mean pulmonary arterial hypertension; PAWP: pulmonary artery wedge pressure; PH: pulmonary hypertension; PVR: pulmonary vascular resistance; WU: Wood units (mmHg/L/min).</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Definition \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Characteristics \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Post-capillary PH \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">mPAP ≥25<span class="elsevierStyleHsp" style=""></span>mmHg<br>PAWP >15<span class="elsevierStyleHsp" style=""></span>mmHg \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Isolated post-capillary PH \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">DPG <7<span class="elsevierStyleHsp" style=""></span>mmHg and/or<br>PVR ≤3 WU \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Combined post-capillary and pre-capillary PH \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">DPG ≥7<span class="elsevierStyleHsp" style=""></span>mmHg and/or<br>PVR >3 WU \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1720472.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0135" class="elsevierStyleSimplePara elsevierViewall">Hemodynamic Classification of Post-capillary Pulmonary Hypertension.</p>" ] ] 13 => array:8 [ "identificador" => "tbl0050" "etiqueta" => "Table 10" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at10" "detalle" => "Table 1" "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Age >65 years \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Cardiovascular risk factors: diabetes mellitus, dyslipidemia, or systemic hypertension \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Coronary disease \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Atrial fibrillation \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Echocardiography: left atrium more dilated than right, left ventricular hypertrophy, interatrial septum bulging into the right atrium, diastolic dysfunction in mitral flow Doppler \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">ECG: presence of left ventricular hypertrophy and Q waves \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1720469.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0145" class="elsevierStyleSimplePara elsevierViewall">Data Indicative of Heart Failure With Preserved Ejection Fraction.</p>" ] ] 14 => array:8 [ "identificador" => "tbl0055" "etiqueta" => "Table 11" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at11" "detalle" => "Table 1" "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0155" class="elsevierStyleSimplePara elsevierViewall">CPFE: combination of pulmonary fibrosis and emphysema; CI: cardiac index; COPD: chronic obstructive pulmonary disease; IPF: idiopathic pulmonary fibrosis; mPAP: mean pulmonary arterial pressure; PH: pulmonary hypertension.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Terminology \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Hemodynamic characteristics \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">COPD/IPF/CPFE without PH \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">mPAP <25<span class="elsevierStyleHsp" style=""></span>mmHg \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">COPD/IPF/CPFE with PH \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">mPAP ≥25<span class="elsevierStyleHsp" style=""></span>mmHg \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">COPD/IPF/CPFE with severe PH \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">mPAP >35<span class="elsevierStyleHsp" style=""></span>mmHg or mPAP ≥25<span class="elsevierStyleHsp" style=""></span>mmHg in the presence of low cardiac output (CI <2.5<span class="elsevierStyleHsp" style=""></span>L/min, not explained by other causes) \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1720467.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0150" class="elsevierStyleSimplePara elsevierViewall">Hemodynamic Classification of Pulmonary Hypertension Associated With Respiratory Diseases.</p>" ] ] 15 => array:8 [ "identificador" => "tbl0060" "etiqueta" => "Table 12" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at12" "detalle" => "Table 1" "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0165" class="elsevierStyleSimplePara elsevierViewall">CTEPH: chronic thromboembolic pulmonary hypertension; PAH: pulmonary arterial hypertension.</p><p id="spar0170" class="elsevierStyleSimplePara elsevierViewall">[Class of recommendation, level of evidence].</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Multidisciplinary team of professionals [I, C] \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Monitoring >50 patients with PAH and CTEPH (ideally >200) [IIa, C] \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Receive >24 new cases per year with a diagnosis of PAH and CTEPH [IIa, C] \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Perform >20 right heart catheterizations with vasodilator test every year [IIa, C] \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1720468.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0160" class="elsevierStyleSimplePara elsevierViewall">Recommendations for Expert Pulmonary Hypertension Units.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:46 [ 0 => array:3 [ "identificador" => "bib0235" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Diagnóstico y tratamiento de la hipertensión pulmonar" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "J.A. 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Year/Month | Html | Total | |
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2024 November | 16 | 2 | 18 |
2024 October | 147 | 88 | 235 |
2024 September | 140 | 35 | 175 |
2024 August | 158 | 51 | 209 |
2024 July | 115 | 30 | 145 |
2024 June | 124 | 43 | 167 |
2024 May | 140 | 55 | 195 |
2024 April | 131 | 54 | 185 |
2024 March | 156 | 53 | 209 |
2024 February | 138 | 54 | 192 |
2024 January | 1 | 0 | 1 |
2023 March | 46 | 11 | 57 |
2023 February | 255 | 66 | 321 |
2023 January | 241 | 81 | 322 |
2022 December | 264 | 111 | 375 |
2022 November | 264 | 78 | 342 |
2022 October | 220 | 115 | 335 |
2022 September | 150 | 83 | 233 |
2022 August | 207 | 96 | 303 |
2022 July | 187 | 98 | 285 |
2022 June | 165 | 165 | 330 |
2022 May | 239 | 142 | 381 |
2022 April | 327 | 167 | 494 |
2022 March | 342 | 114 | 456 |
2022 February | 465 | 114 | 579 |
2022 January | 509 | 127 | 636 |
2021 December | 559 | 167 | 726 |
2021 November | 527 | 144 | 671 |
2021 October | 612 | 180 | 792 |
2021 September | 714 | 153 | 867 |
2021 August | 565 | 145 | 710 |
2021 July | 555 | 137 | 692 |
2021 June | 716 | 155 | 871 |
2021 May | 791 | 211 | 1002 |
2021 April | 2006 | 622 | 2628 |
2021 March | 1333 | 302 | 1635 |
2021 February | 888 | 193 | 1081 |
2021 January | 760 | 198 | 958 |
2020 December | 627 | 160 | 787 |
2020 November | 601 | 166 | 767 |
2020 October | 533 | 113 | 646 |
2020 September | 358 | 111 | 469 |
2020 August | 283 | 134 | 417 |
2020 July | 320 | 135 | 455 |
2020 June | 281 | 124 | 405 |
2020 May | 291 | 106 | 397 |
2020 April | 246 | 102 | 348 |
2020 March | 164 | 78 | 242 |
2020 February | 417 | 158 | 575 |
2020 January | 468 | 121 | 589 |
2019 December | 481 | 153 | 634 |
2019 November | 411 | 187 | 598 |
2019 October | 445 | 155 | 600 |
2019 September | 628 | 189 | 817 |
2019 August | 275 | 121 | 396 |
2019 July | 426 | 98 | 524 |
2019 June | 411 | 114 | 525 |
2019 May | 262 | 114 | 376 |
2019 April | 428 | 192 | 620 |
2019 March | 336 | 153 | 489 |
2019 February | 316 | 124 | 440 |
2019 January | 954 | 120 | 1074 |
2018 December | 524 | 152 | 676 |
2018 November | 329 | 103 | 432 |
2018 October | 413 | 92 | 505 |
2018 September | 11 | 3 | 14 |
2018 April | 3 | 3 | 6 |