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Congenital heart disease · May 2012
Case ReportsCongenital right pulmonary artery agenesis with atrial septal defect and pulmonary hypertension.
- Utku Arman Orun, Osman Yilmaz, Meki Bilici, Selmin Karademir, Cigdem Uner, Filiz Senocak, and Vehbi Dogan.
- Department of Pediatric Cardiology, Dr. Sami Ulus Pediatric Research and Training Hospital, Ankara, Turkey.
- Congenit Heart Dis. 2012 May 1; 7 (3): E6-9.
AbstractUnilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. A 7-year-old girl was admitted to our clinic with the complaint of shortness of breath upon exertion. Chest radiography revealed a hypoplastic right lung. Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.© 2011 Wiley Periodicals, Inc.
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