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Isolated left ventricular apical hypoplasia: case report.

Ricardo Román¹, Henry Anchante¹, Katia Menacho²

¹Division of Cardiology, Cayetano Heredia Hospital, San Martin de Porres, Lima 15102, Perú.

European Heart Journal. Case Reports

|February 27, 2023

Summary

Isolated left ventricular apical hypoplasia (ILVAH) is a rare congenital heart condition. This case report details a severe presentation in Peru, emphasizing diagnostic imaging and management of heart failure and atrial fibrillation.

Keywords:

Atrial fibrillation Cardiac magnetic resonance Case report Echocardiography Heart failure Isolated left ventricular apical hypoplasia

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Area of Science:

Cardiology
Congenital Heart Disease
Medical Imaging

Background:

Isolated left ventricular apical hypoplasia (ILVAH) is a rare congenital cardiac condition.
While often asymptomatic, severe cases necessitate focused diagnosis and treatment.
This report presents the first severe case of ILVAH in Peru and Latin America.

Observation:

A 24-year-old male with substance use history presented with heart failure (HF) and atrial fibrillation (AF).
Echocardiography and cardiac MRI revealed biventricular dysfunction, a spherical left ventricle, abnormal papillary muscle insertion, and right ventricle elongation.
Subepicardial fatty replacement at the left ventricular apex was noted.

Findings:

Diagnosis of ILVAH was confirmed via multimodality non-invasive cardiovascular imaging.
The patient was treated with carvedilol, enalapril, digoxin, and warfarin.
After 18 months, the patient remained mildly symptomatic (NYHA class II) without HF progression or thromboembolism.

Implications:

Multimodality non-invasive imaging is crucial for accurate ILVAH diagnosis.
Close follow-up and management of complications like HF and AF are essential.
This case expands the understanding of ILVAH presentation and management in Latin America.