B50-16 Trapped in the Trabeculae: A Rare Case of Noncompaction Cardiomyopathy

Noncompaction cardiomyopathy (NCCM) is a rare congenital disorder arising from arrested myocardial development, characterized by excessive trabeculations and deep intertrabecular recesses. It predisposes patients to heart failure, arrhythmias, and thromboembolic events. This case highlights new-onset heart failure in a young adult ultimately diagnosed with NCCM. 34-year-old male with no medical history presented with progressive dyspnea on exertion, orthopnea, and bilateral lower extremity edema for two weeks. He endorsed a single episode of chest pain but denied cough, fever, or recent illness.Vital signs were only remarkable for a heart rate of 106. On examination, he was tachycardic with 3+ pitting edema and a systolic murmur at the apex. Lungs were clear. Labs revealed elevated troponin (753 ng/L), elevated proBNP (3626 pg/mL), and elevated creatinine (2.0 mg/dL) which was his baseline. ECG showed sinus tachycardia and T-wave inversions in II, III, aVF, and V5-V6. Transthoracic Echocardiogram was done which revealed severely reduced ejection fraction (10-15%) with global hypokinesis and prominent apical trabeculations. Fibrinous stranding and multiple mural thrombi, including a 2 cm mobile apical thrombus, were noted. He was started on IV furosemide and heparin given the multiple mural thrombi. Cardiac catheterization was later preformed which showed normal coronaries and cardiac output of 2.99 L/min. He was eventually transitioned to oral diuretics and apixaban. Guideline-directed medical therapy (GDMT) was initiated with Entresto, carvedilol, spironolactone, and empagliflozin. A LifeVest was provided due to persistent low EF. Chronic kidney disease stage 3b was identified, attributed to cardiorenal syndrome type 2. Cardiology referred the patient to electrophysiology for an implantable cardiac defibrillator evaluation and advanced heart failure for transplant consideration. Education on medication adherence, diet, and device use was reinforced prior to discharge. This case illustrates the initial presentation of NCCM as decompensated heart failure in a previously healthy adult. Classic echocardiographic features of deep apical trabeculations and multiple ventricular thrombi had guided diagnosis. The presence of mural thrombi highlighted the thromboembolic risk inherent to NCCM and reinforced the need for early anticoagulation. Management focused on stabilizing volume status, initiating GDMT, and evaluating for advanced therapies. The patient’s rapid clinical decline highlights the importance of early recognition and multidisciplinary coordination, including heart failure, electrophysiology, and transplant services. NCCM should be considered in young patients presenting with new-onset heart failure and reduced ejection fraction. Prompt echocardiographic evaluation and initiation of GDMT, anticoagulation, and multidisciplinary care are essential to improving outcomes in this high-risk population. This abstract is funded by: none