SHM Converge Scientific Abstract ePoster Gallery-Left Ventricular Noncompaction

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This case report describes left ventricular non-compaction cardiomyopathy (LVNC), a rare congenital cardiomyopathy that hospitalists should consider in patients with unexplained heart failure or ventricular arrhythmias. A 61-year-old man presented with months of palpitations, exertional shortness of breath, and near-syncope. He arrived in sustained ventricular tachycardia storm and required multiple defibrillations despite treatment with lidocaine, amiodarone, and sotalol. Initial transthoracic echocardiography showed preserved left ventricular function and no significant valvular disease. Electrophysiologic testing suggested an epicardial focus for ventricular tachycardia, and an ablation attempt was unsuccessful, leading to dual-chamber ICD placement.<br /><br />One month later, he returned with incessant ventricular tachycardia and experienced 64 ICD shocks in one day despite antiarrhythmic therapy. Cardiac MRI then revealed reduced LVEF (36%), marked septal hypertrophy, and extensive left ventricular noncompaction with aneurysm formation, especially in the lateral wall. Based on these findings, LVNC was diagnosed. After medical therapy with ARNI, beta blocker, and spironolactone, he improved symptomatically at one-month follow-up.<br /><br />The report emphasizes that LVNC is characterized by a thin compacted epicardial layer and a thick non-compacted endocardial layer with prominent trabeculations and deep intertrabecular recesses. It may result from sarcomere gene mutations and can present with heart failure, ventricular arrhythmias, and embolic events. Diagnosis can be made with echocardiography, CT, ventriculography, or MRI; Jenni criteria are highlighted as a key diagnostic standard. Treatment focuses on heart failure management, antiarrhythmics, and anticoagulation in selected patients to reduce embolic risk.

Asset Subtitle

Mridula P. Talari

Meta Tag

Author List Camelia Chirculescu, Greg Miller, Haytham Adada, Mridula P. Talari

Category Clinical Vignettes

Concept Isolated Left Ventricular Noncompaction Cardiomyopathy

Concept Jenni Criteria

Concept Heart Failure

Concept Ventricular Arrhythmia

Concept Embolic Event

Distinguished Non-Finalist

Presenter Organization Johnston Memorial Hospital

Presenting Author Mridula P. Talari

Track Adult

Keywords

left ventricular non-compaction cardiomyopathy

LVNC

ventricular tachycardia

ICD shocks

cardiac MRI

heart failure

arrhythmia

echocardiography

Jenni criteria

antiarrhythmic therapy

Isolated Left Ventricular Noncompaction Cardiomyopathy

Jenni Criteria

Heart Failure

Ventricular Arrhythmia

Embolic Event