儿童和青少年肥厚性心肌病的性别差异

Gabrielle Norrish PhD , Kimberley Hall BSc , Ella Field MSc , Elena Cervi MD , Olga Boleti MD , Lidia Ziółkowska MD , Iacopo Olivotto MD , Sylvia Passantino MD , Diala Khraiche MD , Giuseppe Limongelli MD , Robert G. Weintraub MD , Aris Anastasakis MD , Elena Biagini MD , Luca Ragni MD , Georgia Sarquella-Brugada MD , Sergi Cesar MD , Terrence Prendiville MD , Karen McLeod MD , Maria Ilina MD , Anwar Baban MD , Juan Pablo Kaski MD
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引用次数: 0

摘要

成人肥厚性心肌病(HCM)的性别差异已被描述,但尚不清楚在儿童期发病的疾病中是否存在类似的差异。目的探讨生理性别对HCM患儿临床特征及预后的影响。方法对诊断为非综合征性HCM≤16年的患者进行国际回顾性队列研究。研究了基线特征和临床结果的性别差异。主要结局是全因死亡率或心脏移植。次要结局包括主要心律失常事件和心力衰竭事件。结果1433例中位年龄为11岁(IQR: 6-14)的患者中,471例(33.0%)为女性。虽然青春期前患者(12岁)在表型上没有性别差异,但青春期女性患者更容易出现心力衰竭症状(n = 53 [31.9%] vs n = 86 [22.5%];P = 0.019)。青少年女性患者左心房尺寸较大(1.4 z-score[±2.3]vs 2.1 z-score[±2.5]);P = 0.0056),但肥大程度和梗阻性疾病的比例无差异。中位随访5.3年(IQR: 2.9, 8.0),全因死亡率或心脏移植、主要心律失常或心力衰竭事件的年发生率无性别差异。结论年轻女性HCM患者更容易出现心衰症状,超声心动图表现为舒张功能损害。尽管在表型上存在差异,但在儿童期和青年期的结果并没有什么不同。需要进一步的研究来探索这些观察到的差异的潜在机制。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Sex Differences in Children and Adolescents With Hypertrophic Cardiomyopathy

Background

Sex differences have been described in adults with hypertrophic cardiomyopathy (HCM), but it is unknown if similar differences exist in childhood-onset disease.

Objectives

This study aimed to investigate the influence of biological sex on the clinical characteristics and outcomes of children with HCM.

Methods

An international retrospective cohort of patients diagnosed with nonsyndromic HCM ≤16 years was formed. Sex differences in baseline characteristics and clinical outcomes were investigated. Primary outcome was all-cause mortality or cardiac transplantation. Secondary outcomes include major arrhythmic cardiac event and heart failure event.

Results

Of 1,433 patients diagnosed at a median age of 11 years (IQR: 6-14), 471 (33.0%) were female. Although there were no sex differences in phenotype in preadolescent patients (<12 years), adolescent female patients were more likely to have heart failure symptoms (n = 53 [31.9%] vs n = 86 [22.5%]; P = 0.019). Adolescent female patients had larger left atrial size (1.4 z-score [±2.3] vs 2.1 z-score [±2.5]; P = 0.0056) but there was no difference in degree of hypertrophy or proportion with obstructive disease. Over a median follow-up of 5.3 years (IQR: 2.9, 8.0) annual incidence of all-cause mortality or cardiac transplantation, major arrhythmic cardiac event or heart failure events did not vary by sex.

Conclusions

Young female patients with HCM are more likely to experience heart failure symptoms and have echocardiographic features of diastolic impairment. Despite differences in phenotype, outcomes during childhood and young adulthood are not different. Further studies are required to explore the underlying mechanisms for these observed differences.
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JACC advances
JACC advances Cardiology and Cardiovascular Medicine
CiteScore
1.90
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