巴西人群肥厚型心肌病的临床和超声心动图特征及其预后影响。

Clinical and Echocardiographic Particularities of Hypertrophic Cardiomyopathy in a Brazilian Population and its Prognostic Impact.

作者信息

Luzuriaga Georgina Del Cisne Jadán, Arteaga-Fernandez Edmundo, Hotta Viviane Tiemi, Ianni Barbara, Nastari Luciano, Ramires Felix, Fonseca Guilherme Wesley Peixoto da, Mady Charles, Fernandes Fábio, Cardoso Juliano Novaes

机构信息

Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP - Brasil.

Escola de Educação Física e Desporto da Universidade de São Paulo, São Paulo, SP - Brasil.

出版信息

Arq Bras Cardiol. 2025 Apr;122(4):e20240640. doi: 10.36660/abc.20240640.

DOI:10.36660/abc.20240640

PMID:40367004

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12107843/

Abstract

BACKGROUND

Hypertrophic cardiomyopathy (HCM) presents echocardiographic abnormalities that are important for diagnosis and prognosis. Data are scarce in the Brazilian literature.

OBJECTIVE

To assess clinical and echocardiographic characteristics and disease progression in a Brazilian cohort of patients with HCM.

METHODS

This retrospective cohort included patients with HCM aged ≥ 18 years. Patients with moderate or severe aortic stenosis and those undergoing septal reduction were excluded. The significance level adopted in the statistical analysis was 5%.

RESULTS

The study included 1244 patients, between 2010 and 2022, with a mean follow-up time of 7.7 ± 4.5 years; 53.6% of patients were men. Mean age was 54.6 ± 16.5 years, and mean left ventricular ejection fraction (LVEF) was 65.8% ± 7.6. We observed LVEF ≤ 50% in 5.8% of patients, asymmetric form in 88.7%, and septal hypertrophy in 85.4%. We found systolic anterior motion of the mitral valve in 30.1% of patients, left ventricular outflow tract obstruction in 30.7%, and septum ≥ 28 mm in 7.2%. Only 1 patient had ventricular aneurysm. Atrial fibrillation/flutter occurred in 9.6% of patients. Overall mortality occurred in 232 patients (1.3%/year). Patients with B-type natriuretic peptide (BNP) > 200 pg/ml, left atrium ≥ 45 mm, and LVEF ≤ 50% had higher mortality (p < 0.001). Age and atrial fibrillation/flutter were also associated with mortality.

CONCLUSIONS

The majority of patients had LVEF > 50%, asymmetric hypertrophy, and septal predominance. BNP, LA diameter, LVEF ≤ 50%, age, and atrial fibrillation/flutter were associated with worse prognosis.

摘要

背景

肥厚型心肌病（HCM）存在对诊断和预后至关重要的超声心动图异常。巴西文献中的相关数据较少。

目的

评估巴西一组肥厚型心肌病患者的临床和超声心动图特征以及疾病进展情况。

方法

这项回顾性队列研究纳入了年龄≥18岁的肥厚型心肌病患者。排除中度或重度主动脉瓣狭窄患者以及接受室间隔减容术的患者。统计分析采用的显著性水平为5%。

结果

该研究纳入了2010年至2022年间的1244例患者，平均随访时间为7.7±4.5年；53.6%的患者为男性。平均年龄为54.6±16.5岁，平均左心室射血分数（LVEF）为65.8%±7.6。我们观察到5.8%的患者LVEF≤50%，88.7%为不对称型，85.4%有室间隔肥厚。30.1%的患者出现二尖瓣收缩期前向运动，30.7%有左心室流出道梗阻，7.2%的患者室间隔≥28mm。仅1例患者有室壁瘤。9.6%的患者发生心房颤动/扑动。232例患者发生全因死亡（1.3%/年）。B型利钠肽（BNP）>200pg/ml、左心房≥45mm以及LVEF≤50%的患者死亡率更高（p<0.001）。年龄和心房颤动/扑动也与死亡率相关。