A. Callegari, J. Grynblat, M. Meot, I. Szezepanski, S. Malekzadeh-Milani, D. Bonnet
{"title":"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","authors":"A. Callegari, J. Grynblat, M. Meot, I. Szezepanski, S. Malekzadeh-Milani, D. Bonnet","doi":"10.1016/j.acvd.2024.07.002","DOIUrl":null,"url":null,"abstract":"<div><h3>Introduction</h3><p>Some patients develop pulmonary hypertension (PH) in the first months of life.</p></div><div><h3>Objective</h3><p>Knowledge gaps remain on their characteristics and long-term outcome.</p></div><div><h3>Methods</h3><p>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.</p></div><div><h3>Results</h3><p>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.m<sup>2</sup>. Genetic disease was found in 122 (19%).</p><p>Most frequent indications for cath were PH on TTE (220, 39%), shunt calculation/closure (256, 45%), or suspected pulmonary veins anomaly (51, 9%).</p><p>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.</p><p>In open shunt patients PVRi was<!--> <!--><<!--> <!-->3<!--> <!-->WU.m<sup>2</sup> 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 (<em>P</em> <!--><<!--> <!-->0.001) and survival (<em>P</em> <!--><<!--> <!-->0.001, <span><span>Fig. 1</span></span>).</p><p>Overall PH at follow-up was found in 84 (15%) and independent risk factors for persistence of PH were lung disease (<em>P</em> <!--><<!--> <!-->0.001), arterial switch for TGA (<em>P</em> <!--><<!--> <!-->0.008), PVRi<!--> <!-->><!--> <!-->6<!--> <!-->WU.m<sup>2</sup> (<em>P</em> <!--><<!--> <!-->0.001), idiopathic PH (<em>P</em> <!-->=<!--> <!-->0.009), and low pulmonary vein saturation (<em>P</em> <!-->=<!--> <!-->0.05).</p><p>Mortality was 10% and survival was significantly reduced in patients with concomitant lung disease (<em>P</em> <!-->=<!--> <!-->0.001, <span><span>Fig. 2</span></span>), normal cardiac anatomy (<em>P</em> <!-->=<!--> <!-->0.001), and cath for suspected PH at TTE (<em>P</em> <!-->=<!--> <!-->0.014).</p></div><div><h3>Conclusion</h3><p>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.</p></div>","PeriodicalId":55472,"journal":{"name":"Archives of Cardiovascular Diseases","volume":null,"pages":null},"PeriodicalIF":2.3000,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Pulmonary hypertension (PH) in the first twelve-months of live: Long-term outcome in relation to diagnosis and invasive hemodynamic\",\"authors\":\"A. Callegari, J. Grynblat, M. Meot, I. Szezepanski, S. Malekzadeh-Milani, D. Bonnet\",\"doi\":\"10.1016/j.acvd.2024.07.002\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Introduction</h3><p>Some patients develop pulmonary hypertension (PH) in the first months of life.</p></div><div><h3>Objective</h3><p>Knowledge gaps remain on their characteristics and long-term outcome.</p></div><div><h3>Methods</h3><p>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.</p></div><div><h3>Results</h3><p>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.m<sup>2</sup>. Genetic disease was found in 122 (19%).</p><p>Most frequent indications for cath were PH on TTE (220, 39%), shunt calculation/closure (256, 45%), or suspected pulmonary veins anomaly (51, 9%).</p><p>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.</p><p>In open shunt patients PVRi was<!--> <!--><<!--> <!-->3<!--> <!-->WU.m<sup>2</sup> 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 (<em>P</em> <!--><<!--> <!-->0.001) and survival (<em>P</em> <!--><<!--> <!-->0.001, <span><span>Fig. 1</span></span>).</p><p>Overall PH at follow-up was found in 84 (15%) and independent risk factors for persistence of PH were lung disease (<em>P</em> <!--><<!--> <!-->0.001), arterial switch for TGA (<em>P</em> <!--><<!--> <!-->0.008), PVRi<!--> <!-->><!--> <!-->6<!--> <!-->WU.m<sup>2</sup> (<em>P</em> <!--><<!--> <!-->0.001), idiopathic PH (<em>P</em> <!-->=<!--> <!-->0.009), and low pulmonary vein saturation (<em>P</em> <!-->=<!--> <!-->0.05).</p><p>Mortality was 10% and survival was significantly reduced in patients with concomitant lung disease (<em>P</em> <!-->=<!--> <!-->0.001, <span><span>Fig. 2</span></span>), normal cardiac anatomy (<em>P</em> <!-->=<!--> <!-->0.001), and cath for suspected PH at TTE (<em>P</em> <!-->=<!--> <!-->0.014).</p></div><div><h3>Conclusion</h3><p>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.</p></div>\",\"PeriodicalId\":55472,\"journal\":{\"name\":\"Archives of Cardiovascular Diseases\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":2.3000,\"publicationDate\":\"2024-08-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Archives of Cardiovascular Diseases\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S1875213624002237\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"CARDIAC & CARDIOVASCULAR SYSTEMS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Archives of Cardiovascular Diseases","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S1875213624002237","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
Pulmonary hypertension (PH) in the first twelve-months of live: Long-term outcome in relation to diagnosis and invasive hemodynamic
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.
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.
求助内容：
应助结果提醒方式：
京公网安备 11010802042870号
