Long-Term Follow-Up With Cardiovascular Magnetic Resonance in 2 Patients With Isolated Left Ventricular Apical...

Isolated left ventricular apical hypoplasia is a rare congenital cardiac anomaly characterized by a truncated, spherical left ventricle, apical fatty replacement, anomalous origin of the papillary muscles, and variable right-ventricular involvement with a right ventricle that wraps around the left ventricle apex. Most published reports are isolated cases with limited follow-up, leaving long-term outcomes unclear.

We describe 2 patients with isolated left ventricular apical hypoplasia who underwent serial cardiovascular magnetic resonance imaging over more than a decade, from childhood into early adulthood.

Both patients demonstrated stable ventricular morphology and myocardial tissue characteristics during follow-up, with preserved or only mildly reduced biventricular systolic function and no evidence of adverse remodeling or clinical deterioration.

Isolated left ventricular apical hypoplasia can remain structurally and functionally stable for more than a decade. Cardiovascular magnetic resonance is essential for diagnosis, long-term surveillance, and risk stratification of this congenital anomaly.