Incidentally detected unilateral pulmonary artery agenesis with pulmonary hypoplasia in a 67 year old woman

摘要

Unilateral pulmonary artery agenesis is commonly seen associated with other congenital cardiovascular defects, when it is detected early in life, but isolated absence of the pulmonary artery is a rare entity, usually detected in adulthood. The latter patients are usually asymptomatic or might present with varied non-specific manifestations such as respiratory tract infections and hemoptysis. This report describes the imaging findings of a 67 year old female with absence of the right pulmonary artery. The embryology and clinical manifestations of the condition are reviewed. Keywords: Pulmonary artery agenesis, embryology, contracted hyperlucent hemithoraxCASE REPORTA 67 year old female presented to the out-patient department with non-exertional and intermittent chest pain for two months. Clinical examination of the respiratory and cardiovascular systems was normal. Electrocardiography was normal. Chest radiograph (Fig 1) showed a contracted right hemithorax with ipsilaterally shifted mediastinal structures and elevated right hemidiaphragm. The left pulmonary artery appeared prominent. With the differential diagnosis including lobar collapse, Swyer-James syndrome and right pulmonary artery hypoplasia/agenesis, computed tomography (CT) of the thorax with CT pulmonary angiography and Magnetic Resonance imaging (MRI) were performed. The right pulmonary artery was found to be absent, with the main pulmonary artery continuing as the left pulmonary artery (Fig 2) and the right heart chambers were seen to be of normal size (Fig 3), suggesting absence of pulmonary hypertension. Findings were confirmed on coronal reformats (Fig 4) and volume rendered images (Fig 5, ?,6).6). The pulmonary vasculature on the right side was grossly reduced while the left pulmonary veins and arteries were enlarged (Fig 5, ?,6).6). The right lung was hypoplastic with compensatory hyperinflation of the left lung (Fig 7, ?,8,8, ?,9).9). Prominent collaterals were seen in both modalities to arise from the descending thoracic aorta and supply the right lung (Fig 10). Transthoracic echocardiography confirmed absence of pulmonary hypertension, intracardiac shunts or structural heart defects. This is, as far as the authors’ knowledge, the oldest patient in whom this condition has been described. The patient’s symptoms resolved without treatment and she is presently healthy and on follow up. Open in a separate windowFigure 1 67 year old female with absent right pulmonary artery. Chest radiograph shows a contracted right hemithorax with diminished vasculature, mediastinum shifted to the right and an elevated right hemidiaphragm.