Beitzke A
Helv Paediatr Acta. 1978 Dec;33(6):567-76.
A 2-day-old female infant was presented with severe cyanosis without cardiac failure. The ECG revealed a normal axis and left ventricular hypertrophy. Chest X-ray examination showed a slightly but uncharacteristically enlarged heart. Cardiac catheterization and angiography showed isolated right ventricular hypoplasia with atrial septal defect. A large right to left shunt at atrial level produced marked systemic arterial desaturation. Up to the age of now nine months the patient remained in good health without cardiac failure. The clinical findings and surgical treatment of this rare malformation are discussed. Differential diagnosis with clinically similar malformations such as tricuspid atresia type 1 B or pulmonary atresia type 1 (with intact ventricular septum) is impossible without angiography.
一名2日龄女婴出现严重青紫但无心力衰竭。心电图显示电轴正常及左心室肥厚。胸部X线检查显示心脏轻度但不典型增大。心导管检查及血管造影显示孤立性右心室发育不全伴房间隔缺损。心房水平大量右向左分流导致明显的体循环动脉血氧饱和度降低。直至9个月龄，该患者一直健康无心力衰竭。讨论了这种罕见畸形的临床发现及外科治疗。若无血管造影，与临床类似畸形如1B型三尖瓣闭锁或1型肺动脉闭锁（室间隔完整）进行鉴别诊断是不可能的。
