Beyer J, Brunner L, Klinner W
Thoraxchir Vask Chir. 1976 Oct;24(5):431-7. doi: 10.1055/s-0028-1095959.
Among 843 patients with Tetralogy of Fallot, 10 showed unilateral absence of a pulmonary artery. In 7 cases the cause was congenital aplasia, whereas thrombosis of one of the pulmonary arteries following systemic-pulmonary anastomosis was the cause in the remaining 3 patients. According to the reports of other authors and to our own experiences, this rare malformation is treated best with palliative operations, the technique depending on the patient's age and on the individual anatomical condition. Only in very few cases with an acceptable pathologic anatomy there will be a reasonable chance for successful repair with reconstruction of the continuity from the right ventricle to the affected lung. However, if complete repair is performed, reconstruction of the absent pulmonary artery usually will be necessary for avoidance of pulmonary hypertension of the contralateral lung.
在843例法洛四联症患者中，10例显示一侧肺动脉缺如。7例病因是先天性发育不全，其余3例患者的病因是体肺分流术后一侧肺动脉血栓形成。根据其他作者的报告以及我们自己的经验，这种罕见畸形最好采用姑息性手术治疗，技术取决于患者年龄和个体解剖状况。只有在极少数病理解剖可接受的情况下，才有合理机会通过重建从右心室到患侧肺的连续性来成功修复。然而，如果进行完全修复，通常需要重建缺如的肺动脉以避免对侧肺的肺动脉高压。
