OBJECTIVE: To analysing the so far 37 reported ILVAH cases worldwide.
METHODS: The electronic databases PubMed and Scopus were investigated from their establishment up to December 13, 2022.
RESULTS: The majority of cases reported occurred in males (52.7%). Mean age at diagnosis was 26.1 ± 19.6 years. More than a third of the patients were asymptomatic (35.1%). The most usual clinical presentation was breathlessness (40.5%). The most commonly detected electrocardiogram changes were T wave abnormalities (29.7%) and right axis deviation with poor R wave progression (24.3%). Atrial fibrillation/flutter was detected in 24.3%. Echocardiography was performed in 97.3% of cases and cardiac MRI in 91.9% of cases. Ejection fraction was reduced in more than a half of patients (56.7%). An associated congenital heart disease was found in 16.2%. Heart failure therapy was administered in 35.1% of patients. The outcome was favorable in the vast majority of patients, with just one death.
CONCLUSIONS: ILVAH is a multifaceted entity with a so far unpredictable course, ranging from benign until the elderly to sudden death during adolescence.
目的:分析迄今为止全球37例报告的ILVAH病例。
方法:电子数据库PubMed和Scopus从建立到2022年12月13日进行了调查。
结果:报告的大多数病例发生在男性(52.7%)。诊断时的平均年龄为26.1±19.6岁。超过三分之一的患者无症状(35.1%)。最常见的临床表现是呼吸困难(40.5%)。最常见的心电图改变是T波异常(29.7%)和右轴偏离,R波进展不良(24.3%)。房颤/扑动的检出率为24.3%。在97.3%的病例中进行了超声心动图检查,在91.9%的病例中进行了心脏MRI检查。超过一半的患者的射血分数降低(56.7%)。16.2%的人发现了相关的先天性心脏病。35.1%的患者接受了心力衰竭治疗。绝大多数患者的预后良好,只有一个死亡。
结论:ILVAH是一个多方面的实体,到目前为止其过程是不可预测的,从良性到老年到青春期猝死。