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

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

巴西人群肥厚性心肌病的临床和超声心动图特征及其对预后的影响。
背景:肥厚性心肌病(HCM)表现为超声心动图异常,对诊断和预后具有重要意义。在巴西文献中数据很少。目的:评估巴西HCM患者的临床和超声心动图特征和疾病进展。方法:该回顾性队列包括年龄≥18岁的HCM患者。中度或重度主动脉狭窄的患者和接受间隔缩小术的患者被排除在外。统计学分析采用显著性水平5%。结果:本研究纳入1244例患者,随访时间为2010 - 2022年,平均随访时间7.7±4.5年;53.6%的患者为男性。平均年龄54.6±16.5岁,平均左室射血分数(LVEF)为65.8%±7.6。我们观察到5.8%的患者LVEF≤50%,88.7%的患者为不对称型,85.4%的患者为间隔肥大。我们发现30.1%的患者出现二尖瓣收缩前运动,30.7%的患者出现左心室流出道梗阻,7.2%的患者出现中隔≥28 mm。仅有1例患者有室性动脉瘤。9.6%的患者发生心房颤动/扑动。总死亡率为232例(1.3%/年)。b型利钠肽(BNP) > 200 pg/ml、左心房≥45 mm、LVEF≤50%的患者死亡率较高(p < 0.001)。年龄和心房颤动/扑动也与死亡率相关。结论:大多数患者LVEF bbbb50 %,不对称肥厚,室间隔优势。BNP、LA直径、LVEF≤50%、年龄、心房颤动/扑动与预后差相关。
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