Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital/Stanford University, Stanford, California 94305, USA.
Ann Thorac Surg. 2013 Apr;95(4):1397-402. doi: 10.1016/j.athoracsur.2012.12.066. Epub 2013 Mar 1.
Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is a complex form of congenital heart defect. There are limited data regarding late hemodynamics of patients after repair of PA/VSD/MAPCAs. This study evaluated the hemodynamics of patients who underwent complete repair of PA/VSD/MAPCSs and subsequently returned for a conduit change.
This was a retrospective review of 80 children undergoing a right ventricle (RV)-to-pulmonary artery conduit replacement after complete repair of PA/VSD/MAPCAs. All patients underwent preoperative cardiac catheterization to define the cardiac physiology. Patients were an average age of 6.5±1.2 years, and the average interval between complete repair and conduit change was 4.5±1.1 years.
The preoperative cardiac catheterization demonstrated an average RV right peak systolic pressure of 70±22 mm Hg and pulmonary artery pressure of 38±14 mm Hg. This pressure gradient of 32 mm Hg reflects the presence of conduit obstruction. After conduit change, the intraoperative RV systolic pressure was 34±8 mm Hg, similar to 36±9 mm Hg at the conclusion of the previous complete repair. The corresponding RV/aortic pressure ratios were 0.36±0.07 and 0.39±0.09, respectively.
The data demonstrate that patients who underwent complete repair of PA/VSD/MAPCAs had nearly identical pulmonary artery pressures when they returned for conduit change some 4.5 years later. This finding indicates that the growth and development of the unifocalized pulmonary vascular bed is commensurate with visceral growth. We would hypothesize that complete repair, along with low RV pressures, will confer a long-term survival advantage.
肺动脉瓣闭锁伴室间隔缺损和主-肺动脉侧支循环（PA/VSD/MAPCAs）是一种复杂的先天性心脏病。关于 PA/VSD/MAPCAs 修复后患者的晚期血流动力学数据有限。本研究评估了完全修复 PA/VSD/MAPCS 后返回更换管道的患者的血流动力学。
这是一项对 80 名接受右心室（RV）-肺动脉导管置换术的患者进行的回顾性研究，这些患者在完全修复 PA/VSD/MAPCAs 后进行了该手术。所有患者均在术前进行了心脏导管检查以确定心脏生理学。患者的平均年龄为 6.5±1.2 岁，完全修复和更换管道之间的平均间隔为 4.5±1.1 年。
术前心脏导管检查显示 RV 收缩压平均为 70±22mmHg，肺动脉压为 38±14mmHg。32mmHg 的压力梯度反映了管道阻塞的存在。更换管道后，术中 RV 收缩压为 34±8mmHg，与上次完全修复结束时的 36±9mmHg 相似。相应的 RV/主动脉压力比分别为 0.36±0.07 和 0.39±0.09。
数据表明，在大约 4.5 年后因管道更换返回的完全修复 PA/VSD/MAPCAs 的患者的肺动脉压力几乎相同。这一发现表明，单发性肺血管床的生长和发育与内脏生长成正比。我们假设完全修复和低 RV 压力将带来长期生存优势。
