Pulmonary artery agenesis is a rare congenital abnormality in which atresia was encountered in the short segment of the right or left pulmonary arteries. It can be isolated or associated with cardiac abnormalities such as tetralogy of Fallot, septal defects or pulmonary stenosis. The majority of cases are diagnosed in childhood whereas some cases yield no symptoms until adulthood. We evaluated retrospectively 5 pulmonary artery agenesis cases diagnosed in our clinics between 1998-2010 with respect to the literature.
Keywords: Angiography, echocardiography, pulmonary artery agenesis, vascular abnormalities
INTRODUCTIONIsolated one-sided pulmonary artery agenesis is a rare congenital anomaly in the proximal short segment of the right or left pulmonary artery. Generally, the distal parts of the artery at the hilus are protected (1). It can occur with Fallot tetralogy, septal defects, or pulmonary stenosis as well as by itself. It can often be identified during childhood years, but it can hide symptoms until the late adult ages. When it presents, the symptoms are recurrent infections, hemoptysis, shortness of breath, restricted exercise, and chest pain are observed. The diagnosis is hard because of the lack of specific symptoms and rarity of the disease. It is important to diagnose this disease at early ages because it causes serious complications such as massive hemoptysis, pulmonary hypertension, respiratory failure, and bronchiectasis (2).The mortality rate of this rare anomaly is high because of its complications. We retrospectively analyzed five cases of pulmonary artery agenesis at older ages in accordance with the literature knowledge.
CASE PRESENTATIONS
Case1A 37-year-old male patient was admitted to our emergency department with complaints of fever, shortness of breath, yellow phlegm, and hemoptysis occurring occasionally. It was learned from his history that he had been treated for bronchiectasis. Because a left pulmonary artery shadow was not seen on posteroanterior (PA) chest X-ray (Figure 1a), echocardiography (ECO) was performed for vein malformation. A mild expansion in the right atrium and right ventricle and a systolic pulmonary artery pressure (PAPs) of 50 mmHg were observed. Chest computerized tomography (CT) (Figure 1b) revealed varicose and cystic bronchiectasis in the left lung, nearly total volume loss, rising in the left diaphragm, and replacement toward the left in mediastinal structures. In the spirometric examination, mild restrictive and moderate obstructive [FVC: 3.29 L (72% of expected), FEV 1 : 1.90 L (51% of expected), FEV 1 /FVC: 57.7% (not expected)] respiratory dysfunction was detected. In the bronchoscopic analysis of the patient, the mouth of the left upper lobe was found to be narrowed in the shape of a lip. The left lung was totally hypoplasic and hypoperfused on ventilation perfusion (V/Q) scintigraphy Figure 1c). Invasive pulmonary angiography of the patient ( Figure 1d) revealed that the left pulmonary artery was closed starting from the ostium level and the patien...
