Capuani A, Del Torso S, Lincoln J C
G Ital Cardiol. 1982;12(5):381-3.
This report describes a combination of a ventricular septal defect and partial anomalous pulmonary venous drainage in an infant with right lung agenesis. Unilateral agenesis of the lung has been rarely described. Although this condition is compatible with long survival, morbidity and mortality are increased by the associations with other malformations both intra- and extracardiac. In unilateral lung agenesis although there is a compensatory increase in volume of the single lung, there is a reduction in the number of branches of the pulmonary artery and the whole right ventricular output passes through a reduced vascular bed. In isolated unilateral agenesis of the lung, pulmonary hypertension is present in about 19% of cases, while in patients with additional left to right shunt the incidence is 88%. The basic condition of increased pulmonary blood flow (single lung) and the undiagnosed partial anomalous pulmonary venous drainage may explain why the early closure of the ventricular septal defect did not prevent the progressive pulmonary vascular disease. We discuss shortly Tolazoline as pulmonary vasodilator.
本报告描述了一名右肺发育不全婴儿合并室间隔缺损和部分肺静脉异位引流的情况。单侧肺发育不全鲜有报道。尽管这种情况可长期存活，但合并心内和心外其他畸形会增加发病率和死亡率。在单侧肺发育不全中，虽然单肺体积会代偿性增大，但肺动脉分支数量减少，整个右心室输出量通过减少的血管床。在孤立性单侧肺发育不全中，约19%的病例存在肺动脉高压，而在合并左向右分流的患者中，发病率为88%。肺血流量增加（单肺）这一基本情况以及未被诊断出的部分肺静脉异位引流，可能解释了为什么室间隔缺损的早期闭合未能阻止进行性肺血管疾病。我们简要讨论了妥拉唑啉作为肺血管扩张剂的情况。
