2 ADULT CASES OF UNILATERAL ABSENCE OF THE RIGHT PULMONARY-ARTERY WITH MARKEDLY DIFFERENT CLINICAL PRESENTATIONS

Two adult cases of unilateral absence of the right pulmonary artery wi th markedly different clinical presentations are reported. One patient was a 21-year-old female without any known history of a coexisting co ngenital anomaly. An abnormal chest roentgenogram (small right hemitho rax, deviation of the mediastinum toward the right side and a dilated left pulmonary artery) was noted and prompted further evaluation. No p ulmonary hypertension was noted and the patient remained asymptomatic. The other patient was a 42-year-old male who had unilateral absence o f the right pulmonary artery and a peripheral stenosis of the left pul monary artery. The clinical course of this patient had been complicate d by impaired exercise tolerance and occasional hemoptysis since adole scence. At the age of 29 years, a cardiac catheterization revealed pul monary hypertension, but no left-to-right shunt. Progressive respirato ry failure resulted in a premature death at the age of 42 years. The p rognosis of patients with unilateral absence of the pulmonary artery l argely depends on the coexisting cardiac anomaly (left-to-right shunt) and pulmonary hypertension. A combination of unilateral absence of th e pulmonary artery and contralateral peripheral pulmonary arterial ste nosis is very rare, but is an important cause of pulmonary hypertensio n and gives a worse prognosis for this entity.