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室间隔缺损合并主-肺动脉侧支动脉的肺动脉闭锁的中线单源化手术

Midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

作者信息

Mainwaring Richard D

机构信息

Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital/Stanford University, Stanford, CA, USA.

出版信息

J Thorac Dis. 2020 Mar;12(3):1263-1273. doi: 10.21037/jtd.2019.10.44.

DOI:10.21037/jtd.2019.10.44
PMID:32274207
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7139044/
Abstract

BACKGROUND

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a relatively rare and extremely heterogeneous form of congenital heart disease. Despite vast improvements in the surgical management of this disease, there is still an ongoing controversy regarding the optimal treatment. The purpose of this chapter is to summarize the surgical algorithm used at Stanford University with a special focus on midline unifocalization.

METHODS

We have an experience with well over 300 patients undergoing primary surgical treatment of PA/VSD/MAPCAs. There were three pathways employed to accommodate the various forms of this heart defect. The dominant pathway is midline unifocalization, which was utilized in 80% of all patients. Less frequently employed strategies included creation of an aortopulmonary window (15%), and other approaches (5%).

RESULTS

For the patients who underwent a midline unifocalization, 85% had a single-stage complete repair while 15% had a unifocalization and shunt. The operative mortality was 1.5% for the single-stage complete repair group 8% for the unifocalization/shunt group. Ultimately, 97% of the midline unifocalization patients were repaired. However, the mean right ventricle to aortic pressure ratio was lower in patients who had a single-stage complete repair compared with an initial unifocalization/shunt (0.36±0.09 0.42±0.07).

CONCLUSIONS

The surgical treatment of PA/VSD/MAPCAs is made more complicated due to heterogeneity of MAPCA and pulmonary artery anatomy. The algorithm utilized at Stanford takes into account the anatomic heterogeneity and the results demonstrate that a high percentage of patients achieve full repair.

摘要

背景

室间隔缺损合并主-肺动脉侧支动脉的肺动脉闭锁（PA/VSD/MAPCAs）是一种相对罕见且极为复杂的先天性心脏病形式。尽管该疾病的外科治疗有了很大改进，但关于最佳治疗方案仍存在争议。本章的目的是总结斯坦福大学使用的手术方案，特别关注中线单源化。

方法

我们有超过300例接受PA/VSD/MAPCAs一期手术治疗患者的经验。采用了三种途径来处理这种心脏缺损的各种形式。主要途径是中线单源化，在所有患者中80%采用此方法。较少使用的策略包括建立主-肺动脉窗（15%）和其他方法（5%）。

结果

接受中线单源化的患者中，85%进行了一期完全修复，15%进行了单源化和分流手术。一期完全修复组的手术死亡率为1.5%，单源化/分流组为8%。最终，97%的中线单源化患者得到修复。然而，与最初进行单源化/分流的患者相比，一期完全修复患者的平均右心室与主动脉压力比值更低（0.36±0.09对0.42±0.07）。

结论

由于MAPCA和肺动脉解剖结构的异质性，PA/VSD/MAPCAs的外科治疗更加复杂。斯坦福大学使用的方案考虑了解剖异质性，结果表明高比例患者实现了完全修复。

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