Key result
Isolated left ventricular apical hypoplasia manifests as a truncated, spherical LV with an elongated RV.
Why the study?
Isolated left ventricular apical hypoplasia is an extremely rare congenital cardiomyopathy with limited reported cases and clinical recognition.
Isolated left ventricular apical hypoplasia is an extremely rare congenital cardiomyopathy characterized by a truncated, spherical left ventricle and an elongated right ventricle.
Isolated left ventricular apical hypoplasia (ILVAH) is an extremely rare congenital cardiomyopathy first described in 2004.1 It is characterized by a truncated, spherical left ventricle (LV) with a deficient apex and an elongated right ventricle (RV), which wraps around the LV and forms the cardiac apex, producing the characteristic “banana-shaped” RV configuration. Approximately 37 cases have been reported worldwide,2 most diagnosed in adolescence or adulthood.
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Aly et al. (2026) conducted a review in Isolated left ventricular apical hypoplasia (ILVAH). Isolated left ventricular apical hypoplasia is an extremely rare congenital cardiomyopathy characterized by a truncated, spherical left ventricle and an elongated right ventricle.
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