PDF(Pubmed)
Abstract：
BACKGROUND: Isolated left ventricular apical hypoplasia (ILVAH), also known as truncated left ventricle (LV), is a very unusual cardiomyopathy. It is characterised by a truncated, spherical, and non-apex forming LV. The true apex is occupied by the right ventricle. Due to the rarity of the disease, just a few case reports and limited case series have been published in the field.
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.
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.
摘要：
背景：孤立的左心室心尖发育不全（ILVAH），也称为左心室截断（LV），是一种非常不寻常的心肌病。它的特点是截断的，球形,和非顶点形成LV。真正的心尖被右心室占据。由于这种疾病的稀有性,该领域仅发布了一些病例报告和有限的病例系列。
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。
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。
