Care of Patients with Congenital Heart Defects Right pulmonary artery agenesis with patent ductus arteriosus and Eisenmenger syndrome: A rare case diagnosed during the postpartum period Mehtap Beker-Acay a, * , Gulay Ozkececi b , Ebru Unlu a , Elif Hocaoglu c , Emre Kacar a , Ersel Onrat b a Department of Radiology, Faculty of Medicine, Afyon Kocatepe University, 03200 Afyonkarahisar, Turkey b Department of Cardiology, Faculty of Medicine, Afyon Kocatepe University, 03200 Afyonkarahisar, Turkey c Department of Radiology, Bakirkoy Dr. Sadi Konuk Training and Research Hospital, 34140 Istanbul, Turkey article info Article history: Received 14 January 2014 Received in revised form 27 March 2014 Accepted 12 April 2014 Available online 22 May 2014 Keywords: Pulmonary artery agenesis Multidetector computed tomography Eisenmenger syndrome Patent ductus arteriosus abstract Unilateral absence of a pulmonary artery a very rare congenital disorder. We here present a case of a 22- year-old female patient with agenesis of the right pulmonary artery accompanying patent ductus arte- riosus and Eisenmenger syndrome, diagnosed by chest X-ray and multidetector computed tomography 5 days after giving birth. Ó 2014 Elsevier Inc. All rights reserved. Introduction Unilateral absence of a pulmonary artery (UAPA) is a rare congenital abnormality with an estimated prevalence of 1/200,000 adults. 1 The median age at UAPA diagnosis is 14 years (range, 0.1e58 years). 2 The most common symptoms include frequent pulmonary infections (37%), dyspnea or limited exercise tolerance (40%) and hemoptysis (20%). Moreover, pulmonary hypertension is reportedly present in 44% of patients. Approximately 17% of patients undergo surgical pro- cedures, and the overall mortality rate is reported to be 7%. 2 Very few patients remain asymptomatic until adulthood. Among the 108 cases described between 1978 and 2000, only 14 were asymptomatic. 3 In a retrospective cohort study analyzing patients with UAPA reported in the literature between 1990 and 2009, 92 patients were identified. According to the study, 78 of these had isolated UAPA and 14 had accompanying patent ductus arteriosus (PDA). Compared with isolated UAPA, UAPAwith PDAwas associated with an earlier diagnosis (median age, 20 vs. 0 years), a higher prevalence of pulmonary hypertension (22% vs. 86%), and a higher mortality rate (4% vs. 21%). 4 Here we discuss the case of a 22-year-old woman who had undergone a normal delivery in spite of having Eisenmenger syndrome. In this case, we achieved the exact diag- nosis with the aid of chest X-ray, computerized tomography (CT) angiography, echocardiography, and angiography. Case report A 22-year-old woman who had given birth 5 days earlier, was admitted to the emergency department of our hospital because of dyspnea and palpitations. The baby was healthy and weighed 2500 g. Childbearing process was on its natural course without any complications. Past medical history was remarkable for exertional dyspnea continuing for 3 years, cyanosis of the fingertips and the tips of the toes, lassitude, and occasional palpitations. The symptoms progressed after the delivery. Her physical development was normal. On physical examination, perioral cyanosis, clubbing of the toes and fingers, cachexia, and dyspnea were noted. Chest X-ray revealed decreased volume of the right lung, a rightward shift of the mediastinum, right heart enlargement, and elevation of the right hemidiaphragm (Fig. 1). Electrocardiography demonstrated a normal sinus rhythm pattern, with right axis deviation, inverted T waves at D1, aVL, V1-5 * Corresponding author. Tel.: þ272 2463303; fax: þ272 2463300. E-mail addresses: mehtapacay@gmail.com, mehtapbekeracay@gmail.com (M. Beker-Acay). Contents lists available at ScienceDirect Heart & Lung journal homepage: www.heartandlung.org 0147-9563/$ e see front matter Ó 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.hrtlng.2014.04.009 Heart & Lung 43 (2014) 363e366