Congenital Right Pulmonary Artery Agenesis with Atrial Septal Defect and Pulmonary Hypertension
Congenital Heart Disease, cilt.7, sa.3, 2012 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 7 Sayı: 3
- Basım Tarihi: 2012
- Doi Numarası: 10.1111/j.1747-0803.2011.00552.x
- Dergi Adı: Congenital Heart Disease
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
- Anahtar Kelimeler: Unilateral Pulmonary Artery Agenesis, Atrial Septal Defect, Pulmonary Hypertension, Bosentan
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
Unilateral 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.