Left Ventricular Noncompaction Syndrome: A Rare Congenital Cardiomyopathy

Authors

Ashwini V. Mallad, MBBS, Marshall University School of MedicineFollow
Waseem Ahmed, MD, Marshall University School of MedicineFollow
Madhulika Urella, MD, Marshall University School of MedicineFollow
Dr. Mehiar El-Hamdani, MD, FACC, FSCAI, Marshall University School of MedicineFollow

Keywords

noncompaction, cardiomyopathy, congenital, heart failure

Disciplines

Cardiology | Internal Medicine | Medical Pathology

Abstract

Isolated left ventricular noncompaction cardiomyopathy (LVNC) is a rare congenital condition occurring due to arrest of myocardial compaction in the first trimester, resulting in a thin layer of compacted epicardium and thick hypertrabeculated myocardium containing deep recesses. This article presents a 44-year-old female with progressive dyspnea, orthopnea, paroxysmal nocturnal dyspnea, and pleuritic chest pain. Examination revealed elevated jugular venous distention, lower extremity edema, and bibasilar crackles on lung auscultation, while the electrocardiogram (EKG) exhibited left bundle branch block. Two-dimensional echocardiography (2D-Echo) showed a dilated left ventricle (LV) with ejection fraction (EF) of 25% and severe diffuse hypokinesia. Cardiac magnetic resonance imaging (MRI) confirmed the diagnosis of LVNC. Thereafter, she developed atrial fibrillation with rapid ventricular rate. Conservative treatment was initiated with Tikosyn, Xarelto, Lasix, Toprol XL, Lisinopril, statin and life vest. Eventually, a cardiac resynchronization therapy defibrillator (CRT-D) was implanted to prevent sudden cardiac death and reduce heart failure complications.

Recommended Citation

Mallad, MBBS AV, Ahmed, MD W, Urella, MD M, El-Hamdani, MD, FACC, FSCAI D. Left Ventricular Noncompaction Syndrome: A Rare Congenital Cardiomyopathy. Marshall J Med. 2016; 2(3): 47 DOI: