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Scientific Letter
Incidental Finding of Right Pulmonary Artery Agenesis in an Adult
Hallazgo incidental de agenesia de la arteria pulmonar derecha en el adulto
Eva Cabrera César
Corresponding author
evacabreracesar@gmail.com

Corresponding author.
, M. Carmen Fernández Aguirre, Lorena Piñel Jiménez
Servicio de Neumología, Hospital Universitario Virgen de la Victoria, Málaga, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Pulmonary artery agenesis is a rare disease with a prevalence of 1 in 200<span class="elsevierStyleHsp" style=""></span>000 adults&#46; It is associated with cardiovascular abnormalities&#44; and is diagnosed in childhood&#46; Patients can sometimes remain asymptomatic until adulthood without experiencing other complications&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a 47-year-old man&#44; referred to the pulmonology clinic after observation of elevated right hemidiaphragm on a routine chest X-ray &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The patient was a former smoker with no other history of interest&#46; He did not report any symptoms&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Thoracic ultrasound was requested&#44; which revealed an elevated right hemidiaphragm&#44; with no signs of paradoxical movement&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Chest computed tomography &#40;CT&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; showed findings consistent with right pulmonary artery agenesis&#44; and an underdeveloped right lung with compensatory eventration of the contralateral lung&#46; The examination was completed with cardiac magnetic resonance imaging &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#44; which showed right pulmonary artery agenesis and partial agenesis of the right lung&#46; The right upper lobe was draining via a single right pulmonary vein to the left atrium&#44; with no evidence of an artery feeding that lobe&#44; nor of shunts or other anomalies&#46; Pulmonary hypertension was ruled out by echocardiogram&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Since the patient was asymptomatic&#44; we decided to treat him conservatively with clinical follow-up in the pulmonology clinic&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Unilateral pulmonary artery agenesis is a rare congenital anomaly&#44; caused by an anomaly in the rotation and migration of the sixth primitive aortic arch in the fourth or fifth week of embryonic development&#46; Absent pulmonary artery is more frequent in the right branch than in the left&#46; It is classified into three groups&#44; depending on the clinical presentation<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a>&#58; in group I&#44; a left-to-right shunt develops and is diagnosed in childhood&#46; In group II&#44; severe pulmonary hypertension occurs and these patients generally die in the first few months of life&#46; Group III consists of adults with few manifestations&#46; The most common symptoms in adults are dyspnea&#44; chest pain&#44; hemoptysis&#44; and recurrent infections&#44; and 44&#37; of patients develop pulmonary hypertension&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">This disease is generally diagnosed in childhood&#44; in association with other cardiovascular malformations&#46; A diagnostic suspicion in adulthood can be prompted by an abnormal chest X-ray&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> The definitive diagnostic test is CT with contrast medium and&#47;or magnetic resonance imaging and&#47;or angiographic studies&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">There is no consensus on treatment&#46; Some authors have recommended the use of repeated echocardiograms to monitor the development of pulmonary hypertension&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> In these cases&#44; treatment with specific vasodilators or revascularization of the affected lung can improve patient progress&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> When lung infections are recurrent or severe hemoptysis develops&#44; lobectomy or pneumonectomy is indicated&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Pulmonary artery agenesis&#44; though rare&#44; should be considered in the differential diagnosis of adults with recurrent lung infections or hemoptysis&#46; Chest X-ray is a useful tool for the initial diagnosis&#44; which should be confirmed by CT scans with contrast medium or magnetic resonance imaging&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; C&#233;sar EC&#44; Aguirre MCF&#44; Jim&#233;nez LP&#46; Hallazgo incidental de agenesia de la arteria pulmonar derecha en el adulto&#46; Arch Bronconeumol&#46; 2017&#59;53&#58;693&#8211;694&#46;</p>"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; Chest radiograph showing elevation of the right hemidiaphragm&#46; &#40;B&#41; CT reconstruction showing absence of the right pulmonary artery&#46; &#40;C&#41; Cardiac magnetic resonance imaging slice with right pulmonary artery agenesis and partial agenesis of the right lung&#46;</p>"
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        "titulo" => "Acknowledgements"
        "texto" => "<p id="par0050" class="elsevierStylePara elsevierViewall">We thank the Respiratory Medicine Department of the Hospital Universitario Virgen de la Victoria&#44; and&#44; in particular&#44; Dr&#46; Juan Antonio Romero Arias&#46;</p>"
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Article information
ISSN: 15792129
Original language: English
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