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孤立性部分性肺静脉异位连接伴症状性右心室衰竭和肺动脉高压的医学管理

Medical Management of Isolated Partial Anomalous Pulmonary Venous Connection With Symptomatic Right Ventricular Failure and Pulmonary Hypertension.

作者信息

Choi Myoung Hyun, Greathouse Frances, Darki Amir

机构信息

Internal Medicine-Pediatrics, Loyola University Medical Center, Maywood, USA.

Internal Medicine, Loyola University Medical Center, Maywood, USA.

出版信息

Cureus. 2024 Jun 20;16(6):e62779. doi: 10.7759/cureus.62779. eCollection 2024 Jun.

DOI:10.7759/cureus.62779
PMID:39036244
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11260183/
Abstract

Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital heart disease in which one or more pulmonary veins drain into the systemic venous circulation. The abnormal connection between the pulmonary vein and the right atrium can result in a right-sided volume overload due to a left-to-right shunt, followed by eventual right-sided pressure overload and right ventricular failure. PAPVC is usually associated with an atrial septal defect but can present as an isolated finding. We present a case of isolated PAPVC resulting in right heart failure and predominantly pre-capillary pulmonary hypertension. We discuss the challenges in the diagnosis and medical management of isolated PAPVC and highlight the clinical and hemodynamic indications for pulmonary vasodilators and diuretics.

摘要

部分性肺静脉异位连接（PAPVC）是一种罕见的先天性心脏病，其中一条或多条肺静脉引流至体循环静脉系统。肺静脉与右心房之间的异常连接可因左向右分流导致右侧容量负荷过重，继而最终出现右侧压力负荷过重和右心室衰竭。PAPVC通常与房间隔缺损相关，但也可单独出现。我们报告一例孤立性PAPVC导致右心衰竭并主要表现为毛细血管前性肺动脉高压的病例。我们讨论了孤立性PAPVC诊断和药物治疗中的挑战，并强调了肺血管扩张剂和利尿剂的临床及血流动力学指征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3739/11260183/5e09f9269899/cureus-0016-00000062779-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3739/11260183/a231521bc6bc/cureus-0016-00000062779-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3739/11260183/1249c903c59b/cureus-0016-00000062779-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3739/11260183/5b84228d568a/cureus-0016-00000062779-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3739/11260183/5e09f9269899/cureus-0016-00000062779-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3739/11260183/a231521bc6bc/cureus-0016-00000062779-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3739/11260183/1249c903c59b/cureus-0016-00000062779-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3739/11260183/5b84228d568a/cureus-0016-00000062779-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3739/11260183/5e09f9269899/cureus-0016-00000062779-i04.jpg

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本文引用的文献

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Evaluation and Management of Pulmonary Hypertension in Noncardiac Surgery: A Scientific Statement From the American Heart Association.非心脏手术相关肺动脉高压的评估与管理：美国心脏协会科学声明
Circulation. 2023 Apr 25;147(17):1317-1343. doi: 10.1161/CIR.0000000000001136. Epub 2023 Mar 16.
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Cryoballoon ablation of atrial fibrillation in a patient with partial anomalous pulmonary vein drainage in the superior vena cava.
上腔静脉部分肺静脉异位引流患者的房颤冷冻球囊消融术
HeartRhythm Case Rep. 2021 Nov 24;8(2):119-121. doi: 10.1016/j.hrcr.2021.11.009. eCollection 2022 Feb.
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Clinical Significance of Partial Anomalous Pulmonary Venous Connections (Isolated and Atrial Septal Defect Associated) Determined by Cardiovascular Magnetic Resonance.心血管磁共振对部分肺静脉异常连接（孤立性和房间隔缺损相关）的临床意义。
Circ Cardiovasc Imaging. 2021 Aug;14(8):e012371. doi: 10.1161/CIRCIMAGING.120.012371. Epub 2021 Aug 13.
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Partial Anomalous Pulmonary Venous Return Presenting in Adults: A Case Series With Review of Literature.成人部分性肺静脉异位引流：文献复习的病例系列报道
Cureus. 2020 Jun 1;12(6):e8388. doi: 10.7759/cureus.8388.
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Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension.部分性肺静脉异位连接的临床意义：重度肺动脉高压的罕见病因
Pulm Circ. 2020 Mar 26;10(1):2045894019885352. doi: 10.1177/2045894019885352. eCollection 2020 Jan-Mar.
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Partial anomalous pulmonary venous return: A case series with management approach.部分性肺静脉异位引流：病例系列及处理方法
Respir Med Case Rep. 2019 Apr 3;27:100833. doi: 10.1016/j.rmcr.2019.100833. eCollection 2019.
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2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.2018年美国心脏协会/美国心脏病学会成人先天性心脏病管理指南：执行摘要：美国心脏病学会/美国心脏协会临床实践指南工作组报告
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