Left Ventricular Noncompaction: An Unusual Cause of Heart Failure

Abstract

Left ventricular noncompaction (LVNC) is a cardiomyopathy characterized by prominent trabeculae and deep intertrabecular recesses in communication with the ventricular cavity. LVNC affects up to 1.3% of the population and is responsible for 3-4% of all heart failure syndromes. CASE PRESENTATION: A 76-year-old male presented to the emergency department due to increasing dyspnea. He endorsed lower extremity swelling and orthopnea. He was tachypneic and hypoxemic. Lung auscultation revealed bibasilar crackles. Bilateral pitting pedal edema was noted. BNP was elevated at 861 pg/mL and troponins were elevated at 0.6 ng/L. Electrocardiogram demonstrated normal sinus rhythm with T-wave inversions in the inferolateral leads. Chest radiograph showed cardiomegaly, vascular congestion and bilateral pleural effusions suggestive of congestive heart failure syndrome. Transthoracic echocardiogram revealed left ventricular ejection fraction of 25-30%. Prominent trabeculae and deep interventricular recesses were noted, concerning for LVNC. Coronary angiography showed moderate nonobstructive coronary artery disease without need for percutaneous coronary intervention. His clinical condition improved with diuretic therapy, blood pressure control, and initiation of guideline-directed medical therapy. During his hospitalization, telemetry captured 6 beats of non-sustained ventricular tachycardia. Lifevest was placed to evaluate need for ICD implantation after optimization of medical therapy. He was scheduled for follow up echocardiogram in 3 months and counseled on the need for family screening.