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成人主动脉缩窄合并肺动脉高压的临床特征、血液动力学和结局。

Clinical features, hemodynamics, and outcomes of pulmonary hypertension in adults with coarctation of aorta.

机构信息

Department of Cardiovascular Medicine, Mayo Clinic Rochester, MN 55905, USA.

Department of Cardiovascular Medicine, Mayo Clinic Rochester, MN 55905, USA.

出版信息

Int J Cardiol. 2025 Jan 1;418:132634. doi: 10.1016/j.ijcard.2024.132634. Epub 2024 Oct 11.

DOI:10.1016/j.ijcard.2024.132634
PMID:39395716
Abstract

BACKGROUND

Pulmonary hypertension (pH) and secondary right ventricle dysfunction is present in 20 % of adults with coarctation of aorta (COA) based on echocardiographic studies. There are limited data about invasive hemodynamic characterization of PH in COA. The purpose of this study was to delineate the clinical features, hemodynamics, and outcomes of PH in COA.

METHOD

Retrospective cohort study of adults with repaired COA that underwent right heart catheterization (RHC). PH was defined as pulmonary artery (PA) mean pressure > 20 mmHg, and PH was classified as isolated precapillary PH and combined pre/postcapillary PH.

RESULTS

Of 99 COA patients that underwent RHC, 57 (58 %) had PH. Of the patients with PH, 14 (25 %) had isolated precapillary PH while 43 (75 %) had postcapillary PH with or without precapillary disease. The correlates of PH were PA compliance (adjusted OR 0.79, 95 % CI 0.71-0.86 per 1 ml/mmHg), left atrial reservoir strain (adjusted OR 0.95, 95 % CI 0921-0.98 per 1 %), and atrial fibrillation (adjusted OR 2.18, 95 % CI 1.20-13.5). Higher PA mean pressure was associated with risk of cardiovascular events (adjusted HR 1.04, 95 % CI 1.02-1.06 per 1 mmHg) and all-cause mortality (adjusted HR 1.05, 95 % CI 1.02-1.08 per 1 mmHg).

CONCLUSIONS

PH was present in over half of adults with COA referred for RHC, and one-quarter of the patients with PH presented with isolated precapillary PH suggesting an underlying PA vascular dysfunction as a contributing mechanism. Further studies are required to determine optimal therapies and strategies for prevention and treatment of PH in this population.

CLINICAL SUMMARY

Of 99 adults with repaired coarctation of aorta (COA) that underwent right heart catheterization, 57 % had pulmonary hypertension (pH). Of the patients with PH, 25 % had isolated precapillary PH while 75 % had combined pre/postcapillary PH. The correlates of PH were pulmonary artery (PA) compliance, left atrial reservoir strain and atrial fibrillation. PH was associated with cardiovascular events and all-cause mortality. These data suggest PA vascular dysfunction in addition to left-sided heart disease as potential etiologies for PH in this population. Further studies are required to determine optimal therapies and strategies for prevention and treatment of PH in this population.

摘要

背景

基于超声心动图研究,20%的成人主动脉缩窄(COA)患者存在肺动脉高压(pH)和继发性右心室功能障碍。关于 COA 中 pH 的侵入性血流动力学特征的资料有限。本研究的目的是描述 COA 中 pH 的临床特征、血流动力学和结局。

方法

对接受右心导管检查(RHC)的成人 COA 患者进行回顾性队列研究。pH 定义为肺动脉(PA)平均压>20mmHg,pH 分为单纯毛细血管前性 pH 和合并毛细血管前/后性 pH。

结果

在 99 例接受 RHC 的 COA 患者中,57 例(58%)存在 pH。在 pH 患者中,14 例(25%)存在单纯毛细血管前性 pH,43 例(75%)存在合并毛细血管前/后性 pH,伴有或不伴有毛细血管前疾病。pH 的相关因素为肺动脉顺应性(每增加 1ml/mmHg,调整后的 OR 0.79,95%CI 0.71-0.86)、左心房储备应变(每增加 1%,调整后的 OR 0.95,95%CI 0.921-0.98)和心房颤动(调整后的 OR 2.18,95%CI 1.20-13.5)。较高的 PA 平均压与心血管事件风险(每增加 1mmHg,调整后的 HR 1.04,95%CI 1.02-1.06)和全因死亡率(每增加 1mmHg,调整后的 HR 1.05,95%CI 1.02-1.08)相关。

结论

在接受 RHC 检查的 COA 成人中,超过一半存在 pH,其中四分之一的 pH 患者存在单纯毛细血管前性 pH,提示潜在的 PA 血管功能障碍是其发病机制之一。需要进一步研究以确定该人群中预防和治疗 pH 的最佳治疗方法和策略。

临床概述

在 99 例接受右心导管检查的成人修复性主动脉缩窄(COA)患者中,57%存在肺动脉高压(pH)。在 pH 患者中,25%存在单纯毛细血管前性 pH,75%存在合并毛细血管前/后性 pH。pH 的相关因素为肺动脉(PA)顺应性、左心房储备应变和心房颤动。pH 与心血管事件和全因死亡率相关。这些数据表明,除了左心疾病外,PA 血管功能障碍也可能是该人群中 pH 的潜在病因。需要进一步研究以确定该人群中预防和治疗 pH 的最佳治疗方法和策略。

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