Unilateral Agenesis of Pulmonary Artery

Abstract

Isolated unilateral agenesis of pulmonary artery was first defined at 1868. Although the exact prevalance is unknown, in a study it is estimated as 1/200,000. Congenital unilateral agenesis of pulmonary artery is a rare anomaly which is frequently associated with other congenital cardiovascular anomalies such as tetralogy of Fallot or septal defects. In 37% of cases left pulmonary artery is absent while in 63% the right one is absent. Nearly 30% of patients are asymptomatic. When symptomatic, patients may experience chest pain, pleural effusion, recurrent pulmonary infections, dyspnea or limited exertion or hemoptysis. Several methods are used in diagnosis such as computerized tomography, bronchography, perfusion scintigraphy, echocardiography and cardiac catheterisation. Massive hemoptysis, cardiac insufficiency, bronchectasis and necrotizing bronchopnemonia may be included among the complications. The following case report presents a patient who was not diagnosed until adult ages.




J Med Cases. 2013;4(10):696-698
doi: http://dx.doi.org/10.4021/jmc1428w