{Reference Type}: Journal Article
{Title}: Isolated left ventricular apical hypoplasia: Systematic review and analysis of the 37 cases reported so far.
{Author}: Bassareo PP;Duignan S;James A;Dunne E;McMahon CJ;Walsh KP;
{Journal}: World J Clin Cases
{Volume}: 11
{Issue}: 23
{Year}: 2023 Aug 16
{Factor}: 1.534
{DOI}: 10.12998/wjcc.v11.i23.5494
{Abstract}: <B>BACKGROUND: </B>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.<BR><B>OBJECTIVE: </B>To analysing the so far 37 reported ILVAH cases worldwide.<BR><B>METHODS: </B>The electronic databases PubMed and Scopus were investigated from their establishment up to December 13, 2022.<BR><B>RESULTS: </B>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.<BR><B>CONCLUSIONS: </B>ILVAH is a multifaceted entity with a so far unpredictable course, ranging from benign until the elderly to sudden death during adolescence.