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与左向右分流型先天性心脏病相关的肺动脉高压：表型谱及管理方法

Pulmonary arterial hypertension related to congenital heart disease with a left-to-right shunt: phenotypic spectrum and approach to management.

作者信息

Ferrero Paolo, Constantine Andrew, Chessa Massimo, Dimopoulos Konstantinos

机构信息

ACHD Unit, IRCCS-Policlinico San Donato, Milan, Italy.

Adult Congenital Heart Disease Unit, Queen Elizabeth Hospital, Birmingham, United Kingdom.

出版信息

Front Cardiovasc Med. 2024 May 9;11:1360555. doi: 10.3389/fcvm.2024.1360555. eCollection 2024.

DOI:10.3389/fcvm.2024.1360555
PMID:38784170
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11111857/
Abstract

Patients with pulmonary hypertension associated with a left-right shunt include a wide spectrum of pathophysiological substrates, ranging from those characterized by pulmonary over-circulation to those with advanced pulmonary vascular disease. The former group may benefit from shunt repair in carefully selected cases but, when advanced pulmonary vascular disease has developed, defect closure should be avoided, and pulmonary vasodilators may be used to improve effort tolerance and hemodynamics. There is a paucity of evidence, however, to support decision-making in the care of these patients. We discuss the principles of management in patients with pulmonary hypertension and a predominant left-right shunt. The recommendations and statements made in this paper are based on pathophysiological considerations and expert opinion.

摘要

与左右分流相关的肺动脉高压患者包括广泛的病理生理基础，从以肺循环过度为特征的患者到患有晚期肺血管疾病的患者。前一组患者在经过精心挑选的病例中可能受益于分流修复，但当出现晚期肺血管疾病时，应避免封堵缺损，可使用肺血管扩张剂来改善运动耐力和血流动力学。然而，支持这些患者护理决策的证据很少。我们讨论了肺动脉高压且以左右分流为主的患者的管理原则。本文中的建议和陈述基于病理生理考虑和专家意见。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be25/11111857/72eaca018594/fcvm-11-1360555-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be25/11111857/2a35e99ca9d7/fcvm-11-1360555-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be25/11111857/72eaca018594/fcvm-11-1360555-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be25/11111857/2a35e99ca9d7/fcvm-11-1360555-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be25/11111857/72eaca018594/fcvm-11-1360555-g002.jpg

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Heart. 2024 Feb 12;110(5):346-352. doi: 10.1136/heartjnl-2023-323015.
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Clinical Implications of the Genetic Background in Pediatric Pulmonary Arterial Hypertension: Data from the Spanish REHIPED Registry.儿科肺动脉高压遗传背景的临床意义：来自西班牙 REHIPED 登记处的数据。
Int J Mol Sci. 2022 Sep 9;23(18):10433. doi: 10.3390/ijms231810433.
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Protective effects of calcyclin-binding protein against pulmonary vascular remodeling in flow-associated pulmonary arterial hypertension.
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Cardiovasc Diagn Ther. 2024 Dec 31;14(6):1048-1057. doi: 10.21037/cdt-24-288. Epub 2024 Dec 19.
钙调结合蛋白对血流相关性肺动脉高压肺血管重构的保护作用。
Respir Res. 2022 Aug 30;23(1):223. doi: 10.1186/s12931-022-02137-z.
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2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.2022年欧洲心脏病学会/欧洲呼吸学会肺动脉高压诊断和治疗指南。
Eur Heart J. 2022 Oct 11;43(38):3618-3731. doi: 10.1093/eurheartj/ehac237.
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