Isolated left ventricular apical hypoplasia:Systematic review and analysis of the 37 cases reported so far  

作　　者：Pier Paolo Bassareo Sophie Duignan Adam James Esme Dunne Colin J McMahon Kevin P Walsh 

机构地区：[1]University College of Dublin,School of Medicine and Department of Cardiology at Mater Misericordiae University Hospital,Dublin D07 R2WY,Ireland [2]Department of Cardiology,Children's Health Crumlin,Dublin D12 N512,Ireland [3]University College of Dublin,School of Medicine and Department of Cardiology,Children's Health Ireland,Dublin D12 N512,Ireland

出　　处：《World Journal of Clinical Cases》2023年第23期5494-5503,共10页世界临床病例杂志

摘　　要：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.AIM 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.CONCLUSION ILVAH is a multifaceted entity with a so far unpredictable course,ranging from benign until the elderly to sudden death during adolescence.

关 键 词：Isolated left ventricular apical hypoplasia Truncated left ventricle ELECTROCARDIOGRAPHY ECHOCARDIOGRAPHY Cardiac magnetic resonance imaging Heart failure 

分 类 号：R542.2[医药卫生—心血管疾病]