A Review of the Management of Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries.


Journal

The Annals of thoracic surgery
ISSN: 1552-6259
Titre abrégé: Ann Thorac Surg
Pays: Netherlands
ID NLM: 15030100R

Informations de publication

Date de publication:
08 2019
Historique:
received: 13 05 2018
revised: 13 01 2019
accepted: 15 01 2019
pubmed: 5 3 2019
medline: 22 11 2019
entrez: 5 3 2019
Statut: ppublish

Résumé

The management of pulmonary atresia with ventricular septal defect (PA/VSD) and major aortopulmonary collateral arteries (MAPCAs) has significantly changed over the past 20 years. Unifocalization and rehabilitation have been described as diametrically opposed strategies. An updated review focused on the management of this complex and rare condition is needed. Articles related to PA/VSD/MAPCAs issued until December 2017 were screened. Twelve main studies published in the modern era (since 2000) were selected and analyzed. Unifocalization and rehabilitation respectively focus on the mobilization of collateral arteries and the growth of native pulmonary vessels. A third strategy, called "combined strategy," was distinguished from the review of the literature. Surgical cohorts and methods of data reporting were found to be heterogenous. Outcomes, regardless of the strategy, have transformed the natural history of the condition, with a complete repair rate of approximately 80% and low rates of early and late mortality. Patients with the most unfavorable anatomy (absent central pulmonary arteries and hypoplastic MAPCAs) remain a challenge and are still left palliated. Variable surgical strategies are used in the management of PA/VSD/MAPCAs. Most teams report a repair rate of 70% to 80% and a mortality rate lower than 10%. Standardization in data reporting is necessary to better compare the existing strategies.

Sections du résumé

BACKGROUND
The management of pulmonary atresia with ventricular septal defect (PA/VSD) and major aortopulmonary collateral arteries (MAPCAs) has significantly changed over the past 20 years. Unifocalization and rehabilitation have been described as diametrically opposed strategies. An updated review focused on the management of this complex and rare condition is needed.
METHODS
Articles related to PA/VSD/MAPCAs issued until December 2017 were screened. Twelve main studies published in the modern era (since 2000) were selected and analyzed.
RESULTS
Unifocalization and rehabilitation respectively focus on the mobilization of collateral arteries and the growth of native pulmonary vessels. A third strategy, called "combined strategy," was distinguished from the review of the literature. Surgical cohorts and methods of data reporting were found to be heterogenous. Outcomes, regardless of the strategy, have transformed the natural history of the condition, with a complete repair rate of approximately 80% and low rates of early and late mortality. Patients with the most unfavorable anatomy (absent central pulmonary arteries and hypoplastic MAPCAs) remain a challenge and are still left palliated.
CONCLUSIONS
Variable surgical strategies are used in the management of PA/VSD/MAPCAs. Most teams report a repair rate of 70% to 80% and a mortality rate lower than 10%. Standardization in data reporting is necessary to better compare the existing strategies.

Identifiants

pubmed: 30831109
pii: S0003-4975(19)30242-5
doi: 10.1016/j.athoracsur.2019.01.046
pii:
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

601-612

Informations de copyright

Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Auteurs

Jerome Soquet (J)

Department of Cardiac Surgery, Royal Children's Hospital, Parkville, Victoria, Australia.

David J Barron (DJ)

Department of Cardiac Surgery, Birmingham Children's Hospital, Birmingham, United Kingdom.

Yves d'Udekem (Y)

Department of Cardiac Surgery, Royal Children's Hospital, Parkville, Victoria, Australia; Department of Paediatrics, University of Melbourne, Victoria, Australia; Murdoch Childrens Research Institute, Parkville, Victoria, Australia. Electronic address: yves.dudekem@rch.org.au.

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Classifications MeSH