Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital/Stanford University, Stanford, California.
Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital/Stanford University, Stanford, California.
Ann Thorac Surg. 2014 Mar;97(3):909-15; discussion 914-5. doi: 10.1016/j.athoracsur.2013.10.115. Epub 2014 Jan 28.
Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is a complex and diverse form of congenital heart defect. Although most patients with PA/VSD/MAPCAs can wait until they are 3 to 6 months of age to undergo surgical reconstruction, there are three specific criteria that merit an earlier repair. These 3 criteria are (1) unremitting heart failure; (2) a ductus to one lung and MAPCAs to the other; and (3) hemitruncus to one lung and MAPCAs to the other. The purpose of this study was to evaluate our surgical experience with early complete repair of PA/VSD/MAPCAs.
This was a retrospective review of patients undergoing complete repair of PA/VSD/MAPCAs within the first 60 days of life. Twenty-seven patients were identified in our database (2002 to 2013) who met these criteria. Fifteen had congestive heart failure, 9 had a ductus plus MAPCAs, and 3 had hemitruncus plus MAPCAs. The median age at surgery was 5 weeks.
There was no operative mortality in this cohort of 27 patients. Hemodynamics at the conclusion of the complete repair demonstrated an average right ventricular peak systolic pressure of 32 ± 5 mm Hg and an average right ventricle to aortic pressure ratio of 0.36 ± 0.06. The median length of hospital stay was 26 days. There have been 2 subsequent mortalities (7%), with a median follow-up duration of 4 years. Eight of the 27 patients have subsequently undergone conduit replacements at our institution. The hemodynamics at the conclusion of the conduit change were statistically unchanged compared with the hemodynamics after complete repair.
The data demonstrate that early complete repair of PA/VSD/MAPCAs can be accomplished with low mortality and excellent postoperative hemodynamics. These early hemodynamic results are maintained at medium-term follow-up. We conclude that early complete repair is an appropriate choice for this highly select subgroup of patients.
肺动脉瓣闭锁伴室间隔缺损和大型体肺侧支循环（PA/VSD/MAPCAs）是一种复杂多样的先天性心脏病。虽然大多数 PA/VSD/MAPCAs 患者可以等到 3 至 6 个月大时进行手术重建，但有三个特定的标准需要更早的修复。这三个标准是：（1）持续的心衰；（2）导管仅通向一叶肺，而 MAPCAs 则通向另一叶肺；（3）单干畸形仅通向一叶肺，而 MAPCAs 则通向另一叶肺。本研究旨在评估我们对 PA/VSD/MAPCAs 早期完全修复的手术经验。
这是一项对在生命的前 60 天内接受 PA/VSD/MAPCAs 完全修复的患者进行的回顾性研究。我们的数据库中确定了 27 例符合这些标准的患者（2002 年至 2013 年）。15 例有充血性心力衰竭，9 例有导管加 MAPCAs，3 例有单干畸形加 MAPCAs。手术时的中位年龄为 5 周。
这 27 例患者的手术死亡率为零。完全修复结束时的血液动力学显示平均右心室收缩峰压为 32 ± 5mmHg，平均右心室与主动脉压比值为 0.36 ± 0.06。中位住院时间为 26 天。此后有 2 例死亡（7%），中位随访时间为 4 年。27 例患者中有 8 例随后在我院进行了管道置换。在管道更换结束时的血液动力学与完全修复后的血液动力学相比没有统计学上的变化。
数据表明，PA/VSD/MAPCAs 的早期完全修复可以实现低死亡率和出色的术后血液动力学。这些早期的血液动力学结果在中期随访中得到维持。我们的结论是，早期完全修复是这个高度选择的患者亚组的合适选择。
