Hirata K, Kyushima M, Asato H
Division of Cardiology, Okinawa Chubu Hospital, Japan.
Jpn Circ J. 1995 Aug;59(8):574-8. doi: 10.1253/jcj.59.574.
Two adult cases of unilateral absence of the right pulmonary artery with markedly different clinical presentations are reported. One patient was a 21-year-old female without any known history of a coexisting congenital anomaly. An abnormal chest roentgenogram (small right hemithorax, deviation of the mediastinum toward the right side and a dilated left pulmonary artery) was noted and prompted further evaluation. No pulmonary hypertension was noted and the patient remained asymptomatic. The other patient was a 42-year-old male who had unilateral absence of the right pulmonary artery and a peripheral stenosis of the left pulmonary artery. The clinical course of this patient had been complicated by impaired exercise tolerance and occasional hemoptysis since adolescence. At the age of 29 years, a cardiac catheterization revealed pulmonary hypertension, but no left-to-right shunt. Progressive respiratory failure resulted in a premature death at the age of 42 years. The prognosis of patients with unilateral absence of the pulmonary artery largely depends on the coexisting cardiac anomaly (left-to-right shunt) and pulmonary hypertension. A combination of unilateral absence of the pulmonary artery and contralateral peripheral pulmonary arterial stenosis is very rare, but is an important cause of pulmonary hypertension and gives a worse prognosis for this entity.
本文报告了两例临床表现明显不同的成人右肺动脉单侧缺如病例。其中一例患者为21岁女性，无已知先天性并存畸形病史。胸部X线片显示异常（右半胸小、纵隔向右侧偏移及左肺动脉扩张），促使进一步评估。未发现肺动脉高压，患者无症状。另一例患者为42岁男性，患有右肺动脉单侧缺如及左肺动脉外周狭窄。自青春期以来，该患者的临床病程因运动耐量受损和偶尔咯血而复杂化。29岁时，心脏导管检查显示有肺动脉高压，但无左向右分流。进行性呼吸衰竭导致患者在42岁时过早死亡。肺动脉单侧缺如患者的预后很大程度上取决于并存的心脏异常（左向右分流）和肺动脉高压。肺动脉单侧缺如与对侧外周肺动脉狭窄并存的情况非常罕见，但却是肺动脉高压的重要原因，且该疾病的预后较差。
