Open Access

ARTICLE

The Clinical Application Value of Selective Unifocalization in the Treatment of Severe Pulmonary Artery Atresia with Ventricular Septal Defect

Jinyang Liu^1,#, Bo Peng^1,#, Xianchao Jiang^1,#, Simeng Zhang¹, Jiachen Li¹, Lizhi Lv¹, Qiang Wang^1,2,*, Xiang Li^3,*

1 Pediatric Cardiac Surgery Center, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
2 Department of Cardiac Surgery, Yunnan Fuwai Cardiovascular Hospital, Kunming, China
3 Medical Nutrition Center, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China

* Corresponding Authors: Qiang Wang. Email: ; Xiang Li. Email:

^# All three authors contributed equally to this article

Congenital Heart Disease 2021, 16(1), 65-72. https://doi.org/10.32604/CHD.2021.013462

Received 07 August 2020; Accepted 29 October 2020; Issue published 23 December 2020

Abstract

Background: This study aims to explore the efficacy of selective unifocalization (UF) for major aortopulmonary collateral arteries (MAPCAs) unifocalization in children with pulmonary atresia with ventricular septal defect (PA/VSD). Methods: A retrospective analysis of 13 patients with PA/VSD/MAPCAs who underwent surgery from June 2017 to December 2019. Sex, age, preoperative cardiovascular angiography test results and McGoon ratio were collected. The properties of the collateral arteries were evaluated by angiography, and selective UF for the “dendritic” MAPCAs and ligation of MAPCAs demonstrating distortion and resistance. Results: A total of 13 severe patients underwent one-stage repair, of which 1 case underwent ventricular septal fenestration and died after 2 weeks of ECMO support. The median age was 37 months, and a median weight was 14.0 kg. A right ventricular to aortic systolic pressure ratio (pRV/pAo) of no more than 0.5 was achieved in 12 living patients. Conclusion: Selective unifocalization based on MAPCAs morphology can achieved a good outcome at the early stage. This surgical concept might be provided a novel insight into treatment for some of the subgroups presenting with this complex form of PA/VSD/MAPCAs.

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Keywords

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