Pulmonary hypertension (PH) in the first twelve-months of live: Long-term outcome in relation to diagnosis and invasive hemodynamic

IF 2.3 3区医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS

Archives of Cardiovascular Diseases Pub Date : 2024-08-01 DOI:10.1016/j.acvd.2024.07.002

A. Callegari, J. Grynblat, M. Meot, I. Szezepanski, S. Malekzadeh-Milani, D. Bonnet

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引用次数: 0

Abstract

Introduction

Some patients develop pulmonary hypertension (PH) in the first months of life.

Objective

Knowledge gaps remain on their characteristics and long-term outcome.

Methods

In total, 563 consecutive patients (mean follow-up 4-years) with mPAP > 20 mmHg between 1–12 months of life were included and their history examined.

Results

Age was mean (SD) 154 (95) days, mPAP 40 (14) mmHg, transpulmonary gradient 31 (14) mmHg, pulmonary vascular resistance index (PVRi) 4.4 (3.9) WU.m². Genetic disease was found in 122 (19%).

Most frequent indications for cath were PH on TTE (220, 39%), shunt calculation/closure (256, 45%), or suspected pulmonary veins anomaly (51, 9%).

Majority of patients (404, 72%) had an open shunt, 37 (6%) repaired CHD, 57 (10%) postcapillary PH and 66 (12%) normal cardiac anatomy but lung development disease (36/66), severe systemic disease (9/66), drug induced (16/66), or heritable (4/66) PH.

In open shunt patients PVRi was < 3 WU.m² in 99 (41%), 3–6 in 89 (37%), > 6 in 52 (22%). Thirteen (3%) patients were considered not operable due to high PVRi (3/13), combined lung diseases (5/13), and/or severe chromosomal abnormalities (6/13). PH related to open shunt was protective for persistent PH at follow-up (P < 0.001) and survival (P < 0.001, Fig. 1).

Overall PH at follow-up was found in 84 (15%) and independent risk factors for persistence of PH were lung disease (P < 0.001), arterial switch for TGA (P < 0.008), PVRi > 6 WU.m² (P < 0.001), idiopathic PH (P = 0.009), and low pulmonary vein saturation (P = 0.05).

Mortality was 10% and survival was significantly reduced in patients with concomitant lung disease (P = 0.001, Fig. 2), normal cardiac anatomy (P = 0.001), and cath for suspected PH at TTE (P = 0.014).

Conclusion

Open shunt normally causes PH in early life, but hardly impacts operability and isolated open shunt rarely results in PH or reduced survival at follow-up. Patients with normal cardiac anatomy and lung disease have higher PH-related mortality and prevalence of PH at follow-up.

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肺动脉高压（PH）的头十二个月寿命：与诊断和侵入性血液动力学相关的长期结果

导言：有些患者在出生后的头几个月就会出现肺动脉高压（PH）。结果平均年龄（标清）154（95）天，mPAP 40（14）mmHg，跨肺梯度 31（14）mmHg，肺血管阻力指数（PVRi）4.4（3.9）WU.m2。最常见的介入治疗指征是 TTE 显示 PH（220 人，占 39%）、分流计算/关闭（256 人，占 45%）或怀疑肺静脉异常（51 人，占 9%）。大多数患者（404 人，72%）有开放性分流，37 人（6%）有修复过的 CHD，57 人（10%）有毛细血管后 PH，66 人（12%）有正常的心脏解剖结构，但有肺发育疾病（36/66）、严重的全身性疾病（9/66）、药物诱发（16/66）或遗传性 PH（4/66）。在开放分流患者中，99 人（41%）的 PVRi 为 3 WU.m2，89 人（37%）的 PVRi 为 3-6 WU.m2，52 人（22%）的 PVRi 为 6 WU.m2。13例（3%）患者因PVRi过高（3/13）、合并肺部疾病（5/13）和/或严重染色体异常（6/13）而被认为无法手术。与开放分流有关的 PH 对随访时的持续 PH（P < 0.001）和存活率（P < 0.001，图 1）具有保护作用。84 例（15%）患者在随访时发现有持续 PH，肺部疾病（P < 0.001）、TGA的动脉转换（P < 0.008）、PVRi > 6 WU.m2（P < 0.001）、特发性PH（P = 0.009）和低肺静脉饱和度（P = 0.图 2）、心脏解剖结构正常（P = 0.001）、TTE 时因怀疑 PH 而行腔内介入治疗（P = 0.014）的患者死亡率为 10%，生存率明显降低。有正常心脏解剖结构和肺部疾病的患者与PH相关的死亡率和随访时的PH患病率较高。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Archives of Cardiovascular Diseases 医学-心血管系统

CiteScore

4.40

自引率

6.70%

发文量

审稿时长

34 days

期刊介绍： The Journal publishes original peer-reviewed clinical and research articles, epidemiological studies, new methodological clinical approaches, review articles and editorials. Topics covered include coronary artery and valve diseases, interventional and pediatric cardiology, cardiovascular surgery, cardiomyopathy and heart failure, arrhythmias and stimulation, cardiovascular imaging, vascular medicine and hypertension, epidemiology and risk factors, and large multicenter studies. Archives of Cardiovascular Diseases also publishes abstracts of papers presented at the annual sessions of the Journées Européennes de la Société Française de Cardiologie and the guidelines edited by the French Society of Cardiology.