{"title":"镰状细胞病长期生存的心肺血流动力学决定因素:来自ETENDARD研究的见解","authors":"Laurent Savale,Thomas D'humières,Athénaïs Boucly,Anoosha Habibi,François Lionnet,Gylna Loko,Jocelyn Inamo,Gonzalo De Luna,Salma Al Khaf,Christelle Chantalat-Auger,Bernard Maitre,Xavier Jaïs,David Montani,Olivier Sitbon,Florence Parent,Gérald Simonneau,Frederic Galacteros,Etienne Audureau,Pablo Bartolucci,Marc Humbert","doi":"10.1164/rccm.202502-0353oc","DOIUrl":null,"url":null,"abstract":"RATIONALE\r\nPulmonary hypertension (PH) is a serious cardiopulmonary complication of sickle cell disease (SCD), but the prognostic impact of hemodynamic parameters remains poorly defined.\r\n\r\nOBJECTIVES\r\nThis study aimed to assess the clinical and long-term prognostic relevance of hemodynamic parameters in SCD.\r\n\r\nMETHODS\r\nData were analyzed from the French Etendard cohort. All 398 participants underwent echocardiography; those with tricuspid regurgitation velocity (TRV) ≥ 2.5 m/s proceeded to right heart catheterization. Over a minimum 10-year follow-up, we examined the clinical and prognostic significance of hemodynamic variables.\r\n\r\nMEASUREMENTS AND MAIN RESULTS\r\nPH defined by a mean pulmonary arterial pressure (mPAP) > 20 mmHg was identified in 44 patients (11%). Among them, 26 (6.5%) had a pulmonary arterial wedge pressure (PAWP) ≤ 5 mmHg, and 18 (4.5%) had a PAWP>15 mmHg. Mortality during follow-up was 11.1%. A significant association was found between pulmonary vascular resistance (PVR) levels and mortality risk, with a threshold identified at 1.5 Wood units through ROC analyses. In patients with mPAP > 20 mmHg and PVR ≥ 1.5 WU, the adjusted hazard ratio (HR) for mortality was 4.27 (95% CI: 1.88-9.74; p < 0.001). A hemolytic phenotype and the presence of systemic complications, including hypertension, left ventricular diastolic dysfunction, renal impairment, and leg ulcers, were associated with elevated PVR and increased mortality risk.\r\n\r\nCONCLUSION\r\nA PVR threshold of 1.5 WU emerges as a key predictor of mortality in patients with a mPAP above 20 mmHg, particularly when assessed in conjunction with markers of hemolysis and systemic complications.","PeriodicalId":7664,"journal":{"name":"American journal of respiratory and critical care medicine","volume":"35 1","pages":""},"PeriodicalIF":19.4000,"publicationDate":"2025-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Cardiopulmonary Hemodynamic Determinants of Long-Term Survival in Sickle Cell Disease: Insights from the ETENDARD Study.\",\"authors\":\"Laurent Savale,Thomas D'humières,Athénaïs Boucly,Anoosha Habibi,François Lionnet,Gylna Loko,Jocelyn Inamo,Gonzalo De Luna,Salma Al Khaf,Christelle Chantalat-Auger,Bernard Maitre,Xavier Jaïs,David Montani,Olivier Sitbon,Florence Parent,Gérald Simonneau,Frederic Galacteros,Etienne Audureau,Pablo Bartolucci,Marc Humbert\",\"doi\":\"10.1164/rccm.202502-0353oc\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"RATIONALE\\r\\nPulmonary hypertension (PH) is a serious cardiopulmonary complication of sickle cell disease (SCD), but the prognostic impact of hemodynamic parameters remains poorly defined.\\r\\n\\r\\nOBJECTIVES\\r\\nThis study aimed to assess the clinical and long-term prognostic relevance of hemodynamic parameters in SCD.\\r\\n\\r\\nMETHODS\\r\\nData were analyzed from the French Etendard cohort. All 398 participants underwent echocardiography; those with tricuspid regurgitation velocity (TRV) ≥ 2.5 m/s proceeded to right heart catheterization. Over a minimum 10-year follow-up, we examined the clinical and prognostic significance of hemodynamic variables.\\r\\n\\r\\nMEASUREMENTS AND MAIN RESULTS\\r\\nPH defined by a mean pulmonary arterial pressure (mPAP) > 20 mmHg was identified in 44 patients (11%). Among them, 26 (6.5%) had a pulmonary arterial wedge pressure (PAWP) ≤ 5 mmHg, and 18 (4.5%) had a PAWP>15 mmHg. Mortality during follow-up was 11.1%. A significant association was found between pulmonary vascular resistance (PVR) levels and mortality risk, with a threshold identified at 1.5 Wood units through ROC analyses. In patients with mPAP > 20 mmHg and PVR ≥ 1.5 WU, the adjusted hazard ratio (HR) for mortality was 4.27 (95% CI: 1.88-9.74; p < 0.001). A hemolytic phenotype and the presence of systemic complications, including hypertension, left ventricular diastolic dysfunction, renal impairment, and leg ulcers, were associated with elevated PVR and increased mortality risk.\\r\\n\\r\\nCONCLUSION\\r\\nA PVR threshold of 1.5 WU emerges as a key predictor of mortality in patients with a mPAP above 20 mmHg, particularly when assessed in conjunction with markers of hemolysis and systemic complications.\",\"PeriodicalId\":7664,\"journal\":{\"name\":\"American journal of respiratory and critical care medicine\",\"volume\":\"35 1\",\"pages\":\"\"},\"PeriodicalIF\":19.4000,\"publicationDate\":\"2025-09-12\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"American journal of respiratory and critical care medicine\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1164/rccm.202502-0353oc\",\"RegionNum\":1,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"CRITICAL CARE MEDICINE\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"American journal of respiratory and critical care medicine","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1164/rccm.202502-0353oc","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CRITICAL CARE MEDICINE","Score":null,"Total":0}
Cardiopulmonary Hemodynamic Determinants of Long-Term Survival in Sickle Cell Disease: Insights from the ETENDARD Study.
RATIONALE
Pulmonary hypertension (PH) is a serious cardiopulmonary complication of sickle cell disease (SCD), but the prognostic impact of hemodynamic parameters remains poorly defined.
OBJECTIVES
This study aimed to assess the clinical and long-term prognostic relevance of hemodynamic parameters in SCD.
METHODS
Data were analyzed from the French Etendard cohort. All 398 participants underwent echocardiography; those with tricuspid regurgitation velocity (TRV) ≥ 2.5 m/s proceeded to right heart catheterization. Over a minimum 10-year follow-up, we examined the clinical and prognostic significance of hemodynamic variables.
MEASUREMENTS AND MAIN RESULTS
PH defined by a mean pulmonary arterial pressure (mPAP) > 20 mmHg was identified in 44 patients (11%). Among them, 26 (6.5%) had a pulmonary arterial wedge pressure (PAWP) ≤ 5 mmHg, and 18 (4.5%) had a PAWP>15 mmHg. Mortality during follow-up was 11.1%. A significant association was found between pulmonary vascular resistance (PVR) levels and mortality risk, with a threshold identified at 1.5 Wood units through ROC analyses. In patients with mPAP > 20 mmHg and PVR ≥ 1.5 WU, the adjusted hazard ratio (HR) for mortality was 4.27 (95% CI: 1.88-9.74; p < 0.001). A hemolytic phenotype and the presence of systemic complications, including hypertension, left ventricular diastolic dysfunction, renal impairment, and leg ulcers, were associated with elevated PVR and increased mortality risk.
CONCLUSION
A PVR threshold of 1.5 WU emerges as a key predictor of mortality in patients with a mPAP above 20 mmHg, particularly when assessed in conjunction with markers of hemolysis and systemic complications.
期刊介绍:
The American Journal of Respiratory and Critical Care Medicine focuses on human biology and disease, as well as animal studies that contribute to the understanding of pathophysiology and treatment of diseases that affect the respiratory system and critically ill patients. Papers that are solely or predominantly based in cell and molecular biology are published in the companion journal, the American Journal of Respiratory Cell and Molecular Biology. The Journal also seeks to publish clinical trials and outstanding review articles on areas of interest in several forms. The State-of-the-Art review is a treatise usually covering a broad field that brings bench research to the bedside. Shorter reviews are published as Critical Care Perspectives or Pulmonary Perspectives. These are generally focused on a more limited area and advance a concerted opinion about care for a specific process. Concise Clinical Reviews provide an evidence-based synthesis of the literature pertaining to topics of fundamental importance to the practice of pulmonary, critical care, and sleep medicine. Images providing advances or unusual contributions to the field are published as Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences.
A recent trend and future direction of the Journal has been to include debates of a topical nature on issues of importance in pulmonary and critical care medicine and to the membership of the American Thoracic Society. Other recent changes have included encompassing works from the field of critical care medicine and the extension of the editorial governing of journal policy to colleagues outside of the United States of America. The focus and direction of the Journal is to establish an international forum for state-of-the-art respiratory and critical care medicine.