Takayasu's arteritis mimicking unilateral pulmonary artery agenesis in a child with severe pulmonary hypertension and right heart failure: a diagnostic dilemma.

Affliction of the pulmonary arteries in Takayasu's arteritis is uncommon. Moreover the incidence of pulmonary artery involvement in this condition is often underestimated because of asymptomatic nature in most patients. Severe involvement may however present with pulmonary artery hypertension and hemoptysis, which may prove to be fatal. This case report describes a 9-year-old girl with severe pulmonary hypertension and right heart failure secondary to total occlusion of the right pulmonary artery. Detailed clinical examination and computed tomography (CT) angiography confirmed this diagnosis.