The Clinical Trajectory of NYHA Functional Class I Patients With Obstructive Hypertrophic Cardiomyopathy.

IF 10.3 1区医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS JACC. Heart failure Pub Date : 2024-10-25 DOI:10.1016/j.jchf.2024.09.008

Monica Ahluwalia, Jiankang Liu, Iacopo Olivotto, Victoria Parikh, Euan A Ashley, Michelle Michels, Jodie Ingles, Rachel Lampert, John C Stendahl, Steven D Colan, Dominic Abrams, Alexandre C Pereira, Joseph W Rossano, Thomas D Ryan, Anjali T Owens, James S Ware, Sara Saberi, Adam S Helms, Sharlene Day, Brian Claggett, Carolyn Y Ho, Neal K Lakdawala

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Abstract

Background: An improved understanding of the natural history in NYHA functional class I patients with obstructive hypertrophic cardiomyopathy (oHCM) is needed.

Objectives: Using a multicenter registry (SHaRe [Sarcomeric Human Cardiomyopathy Registry]), this study described the natural history in patients with oHCM who were classified as NYHA functional class I at the initial visit compared with patients classified as NYHA functional class II and reported baseline characteristics associated with incident clinical events.

Methods: Incident events assessed included a composite of NYHA functional class III to IV symptoms, left ventricular ejection fraction <50%, atrial fibrillation, stroke, ventricular arrhythmias, septal reduction therapy, ventricular assist device or transplantation, or death. Factors associated with incident events were determined using Kaplan-Meier, Cox proportional hazards, and restricted cubic spline models.

Results: Of 7,964 patients with HCM in SHaRe, 1,239 patients with oHCM met inclusion criteria; 598 were in NYHA functional class I at the initial visit (age 48 ± 17 years; 31.1% female; peak gradient, 75 ± 40 mm Hg). At 5-year follow-up, the composite event rate of NYHA functional class I patients was 28% compared with 44% (P < 0.001) in 641 NYHA functional class II patients with oHCM (age 54 ± 16 years; 46.5% female; peak gradient, 83 ± 39 mm Hg). Left atrial (LA) diameter ≥45 mm (HR: 1.56 [95% CI: 1.14-2.12]; P = 0.005), female sex (HR: 1.61 [95% CI: 1.16-2.24]; P = 0.003), and older age (HR: 1.21 per 10 years [95% CI: 1.09-1.34]; P < 0.001), but not the magnitude of left ventricular outflow tract obstruction, were associated with a higher risk of the composite outcome in NYHA functional class I patients.

Conclusions: Although NYHA functional class I patients with oHCM fared better than NYHA functional class II patients, more than one-fourth experienced adverse events over 5-year follow-up, especially if they were older, female, and/or had LA enlargement. Strategies to reduce the rate of clinical outcomes in NYHA functional class I patients warrant further study.

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NYHA 功能分级 I 级阻塞性肥厚型心肌病患者的临床轨迹。

背景：需要进一步了解 NYHA 功能分级 I 型阻塞性肥厚型心肌病（oHCM）患者的自然病史：需要进一步了解 NYHA 功能分级为 I 级的阻塞性肥厚型心肌病（oHCM）患者的自然病史：本研究通过多中心登记（SHaRe [Sarcomeric Human Cardiomyopathy Registry]），描述了初次就诊时被归为 NYHA 功能分级 I 级的阻塞性肥厚型心肌病患者与被归为 NYHA 功能分级 II 级的患者的自然病史，并报告了与临床事件相关的基线特征：评估的事件包括NYHA功能分级III级至IV级症状、左室射血分数结果：在 SHaRe 的 7964 名 HCM 患者中，1239 名 oHCM 患者符合纳入标准；598 名患者在首次就诊时处于 NYHA 功能分级 I 级（年龄 48 ± 17 岁；31.1% 为女性；峰值梯度 75 ± 40 mm Hg）。随访5年时，NYHA功能分级I级患者的综合事件发生率为28%，而641名NYHA功能分级II级的oHCM患者（年龄为54±16岁；46.5%为女性；峰值梯度为83±39毫米汞柱）的综合事件发生率为44%（P<0.001）。左心房（LA）直径≥45 mm（HR：1.56 [95% CI：1.14-2.12]；P = 0.005）、性别为女性（HR：1.61 [95% CI：1.16-2.24]；P = 0.003）、年龄较大（HR：每 10 年 1.21 [95% CI：1.09-1.34]；P < 0.003）。34]；P < 0.001），但与左心室流出道梗阻的程度无关，与 NYHA 功能分级 I 患者较高的综合结局风险相关：尽管NYHA功能分级I级的oHCM患者比NYHA功能分级II级的患者情况要好，但超过四分之一的患者在5年的随访中出现了不良事件，尤其是年龄较大、女性和/或LA增大的患者。降低 NYHA 功能分级 I 患者临床结局发生率的策略值得进一步研究。

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来源期刊

JACC. Heart failure CARDIAC & CARDIOVASCULAR SYSTEMS-

CiteScore

21.20

自引率

2.30%

发文量

164

期刊介绍： JACC: Heart Failure publishes crucial findings on the pathophysiology, diagnosis, treatment, and care of heart failure patients. The goal is to enhance understanding through timely scientific communication on disease, clinical trials, outcomes, and therapeutic advances. The Journal fosters interdisciplinary connections with neuroscience, pulmonary medicine, nephrology, electrophysiology, and surgery related to heart failure. It also covers articles on pharmacogenetics, biomarkers, and metabolomics.