Isolated unilateral absence of pulmonary artery: a case report and review of the literature

Objective To highlight the clinical manifestation,pathophysiology,diagnostic and therapeutic method of isolated unilateral absence of pulmonary artery(UAPA).Methods The clinical,auxiliary examinational and pathological data of a patient with isolated UAPA from our hospital was presented,and relevant literatures from multiple databases of Chinese Jorunals were retrospectively reviewed.Results The median age of the 44 patients identified was 31.5 years (range,0.25 to 69 years).The main symptoms were frequent pulmonary infections (47.7％),hemoptisis (52.6％),dyspnea or shortness of breath after the event (57.9％),chest tightness (31.6％),palpitations (18.4％),chest pain (7.9％),and,1 was asymptomatic.Pulmonary hypertension was present in 43.2％ (19/44) of the patients.CTPA,MRI,EBCT and DSA had diagnostic value.Surgical procedures were performed in 20.5％ of the patients.Conclusions Isolated UAPA is a rare congenital pulmonary vessel malformation,the symptoms no specific,and was easy to be misdiagnosed.The disease can be diagnosed by CTPA,MRI,EBCT and DSA,and the best surgical approach is revascularization which can not be implemented in our country. Key words: Unilateral absence of pulmonary artery;  Pulmonary artery hypoplasia;  Pulmonary artery agenesis;  Pulmonary artery malformation ;  Pulmonary hypertension ;  Treatment