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Letter to the Editor
Partial Anomalous Pulmonary Venous Connection With Pulmonary Hypertension
Conexión venosa pulmonar anómala parcial con hipertensión pulmonar
Julio Hernández Vázqueza,
Corresponding author
, Javier de Miguel Díezb, Cristina de Cortina Camareroc
a Servicio de Neumología, Hospital Infanta Leonor, Madrid, Spain
b Servicio de Neumología, Hospital General Universitario Gregorio Marañón, Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Universidad Complutense de Madrid, Madrid, Spain
c Servicio de Cardiología, Hospital Infanta Leonor, Madrid, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Partial anomalous pulmonary venous connection &#40;PAPVC&#41; encompasses a group of cardiovascular anomalies that are caused by the abnormal return of one or more of the pulmonary veins directly to the right atrium or indirectly through a variety of venous connections from the anomalous pulmonary veins&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of a 51-year-old man&#44; a former smoker&#44; with a history of hypertension&#44; diabetes&#44; dyslipidemia and peripheral artery disease&#44; who reported dyspnea on moderate exertion lasting several months&#46; Laboratory test results &#40;including serologic studies for hepatotropic viruses&#41; were normal&#46; Spirometry tests showed mild obstruction&#44; and plethysmography and diffusing capacity measurements were normal&#46; Chest radiography revealed enlargement of both hila and their vascular markings&#44; with a double density sign on the left paratracheal area &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>A&#41;&#46; The echocardiogram showed&#58; normal left ventricle&#44; right ventricle severely dilated&#44; and indirect signs of pulmonary hypertension &#40;PH&#41;&#46; Right heart catheterization showed slight PH &#40;mean PAP 29<span class="elsevierStyleHsp" style=""></span>mmHg&#44; total PVR 254&#46;84<span class="elsevierStyleHsp" style=""></span>dyn&#46;s&#46;cm<span class="elsevierStyleSup">&#8722;5</span>&#41;&#46; On computed tomography &#40;CT&#41; scan&#44; an anomalous venous structure was seen in which the left pulmonary veins converged&#44; draining into the brachiocephalic vein &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>B&#41;&#46; Based on these findings&#44; the patient underwent surgery &#40;anastomosis of the defective left vein to the left atrium&#41; and was discharged with antiplatelet therapy in addition to his usual medication&#46; This improved his dyspnea&#46; The follow-up echocardiogram at 3 years showed&#58; normal left and right ventricle&#44; no PH&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">There are 2 types of PAPVC&#58; partial anomalous pulmonary venous connections and partial anomalous pulmonary venous drainage&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> In the first&#44; an anomalous vein connects with a systemic vein&#44; so that oxygenated blood from the pulmonary circulation mixes with the systemic venous blood&#44; before draining into the right atrium&#59; the most common form is drainage of the left upper pulmonary vein into the brachiocephalic vein&#46; In the second&#44; the pulmonary veins are correctly connected to the left atrium&#44; but venous pulmonary blood flows into the right atrium or into both atria through a cardiac defect&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Although PAPVC can present as an isolated structural abnormality&#44; it is often associated with other heart defects&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> The severity of symptoms depends on the extent of the left-right short circuit<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> and the presence of other cardiac or pulmonary abnormalities&#46; A preliminary diagnosis can be made with echocardiography and then confirmed with CT&#44; magnetic resonance imaging or heart catheterization&#46; Pulmonary angiography gives a more detailed anatomical image&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">PAPVC must be treated surgically&#44; although this can be avoided in asymptomatic patients with a minor short circuit&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> In some cases&#44; even occlusion by catheterization can be considered&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> Prognosis for PAPVC patients is generally good&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Although these are congenital malformations&#44; they can go unnoticed until adulthood&#46; Given that up to 42&#46;8&#37; of PAPVC patients can present PH&#44;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> clinicians should aware of the danger of diagnosing PAPVC patients with primary PH&#44; since the therapeutic approach is very different in both cases&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Hern&#225;ndez V&#225;zquez J&#44; de Miguel D&#237;ez J&#44; de Cortina Camarero C&#46; Conexi&#243;n venosa pulmonar an&#243;mala parcial con hipertensi&#243;n pulmonar&#46; Arch Bronconeumol&#46; 2015&#59;51&#58;153&#8211;154&#46;</p>"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; Chest radiograph&#44; posteroanterior view&#58; double density sign on the left mediastinal border &#40;arrows&#41; caused by the ascending path of the defective venous vessel&#46; &#40;B&#41; Contrast-enhanced computed tomography&#44; axial plane&#58; anomalous venous vessel &#40;arrowhead&#41; causing the double density&#46;</p>"
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Article information
ISSN: 15792129
Original language: English
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