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Isolated Left Ventricular Apical Hypoplasia Presenting as Vasovagal Syncope in a Middle-Aged Male Patient

Year: 2025

Type: article

Abstract: BACKGROUND: Isolated left ventricular apical hypoplasia (ILVAH) is a rare congenital cardiomyopathy characterized by a truncated and spherical left ventricle with compensatory right ventricle elongation. Clinical presentation is variable and often delayed. CASE SUMMARY: We report a 40-year-old man with no comorbidities who presented with a vasovagal episode. Electrocardiogram showed sinus rhythm with incomplete left bundle branch block. Echocardiography suggested dilated cardiomyopathy; however, cardiac magnetic resonance imaging (CMR) revealed the classic features of isolated left ventricular apical hypoplasia, including a truncated, spherical left ventricle with fatty apical infiltration, elongated right ventricle encasing the apex, and papillary muscle abnormalities. Despite preserved ejection fraction (53%), we initiated medical therapy with angiotensin receptor neprilysin inhibitor, beta-blockers, SGLT2i, spironolactone, and aspirin. The patient declined coronary angiography and is under follow-up. DISCUSSION: ILVAH can mimic dilated cardiomyopathy on echocardiography. CMR is key to accurate diagnosis and management. TAKE HOME MESSAGES: Suspect ILVAH in a globular left ventricle with apical truncation. It may mimic dilated cardiomyopathy and requires CMR for confirmation.

Source: JACC Case Reports

DOI: 10.1016/j.jaccas.2025.105600

Authors: Lakshmi Siddhi Kolaparthi, Rajesh Thachathodiyl, Ramiah Rajesh Kannan

Institutions: Amrita Institute of Medical Sciences and Research Centre, Amrita Vishwa Vidyapeetham

Language: English

Topic: Cardiomyopathy and Myosin Studies

Subfield: Cardiology and Cardiovascular Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $850