A 29‐year‐old man with no medical history presented with increasing shortness of breath and orthopnea for ten days. He denied alcohol and drug abuse. Family history was negative for any cardiac disease. Examination revealed distended neck veins, bibasilar crackles, and S3. EKG showed normal sinus rhythm with QRS duration of 130 ms. An echocardiogram demonstrated noncompacted ventricles, global left ventricular hypokinesia (ejection fraction, 17%), with left ventricular thrombus. Cardiac catheterization showed clean coronaries with normal hemodynamics. He was started on standard heart failure regimen and anticoagulation. Follow‐up echocardiogram after 3 months was unchanged. He underwent cardiac resynchronization therapy with anticipation of heart transplant in the future.
Although a rare cardiomyopathy, NCCM is being recognized more frequently than before due to increased awareness about its natural history, clinical manifestations, and improved modalities of cardiac imaging. It usually presents with left heart failure. If not recognized early it can present with fatal arrhythmias, sudden cardiac death and systemic embolism which can lead to significant morbidity and mortality. Echocardiography is diagnostic and reveals trabeculated myocardium with thick noncompacted layer/compacted layer ratio > 2. Familial occurrence is frequent with autosomal dominant and X‐linked transmissions. Early cardiology referral and genetic testing of the first‐degree relatives are important. Cardiac transplantation is the treatment of choice
Left ventricular noncompaction (LVNC) is a rare disorder caused by the arrest of myocardial compaction during embryogenesis, leading to a noncompacted endocardial layer with marked trabeculations. The diagnosis is primarily based on echocardiographic demonstration of a spongy myocardium Learning points: (1) Despite being a rare condition, left ventricular noncompaction is being recognized more frequently than before due to increased awareness about its natural history, clinical manifestations, and improved modalities of cardiac imaging. (2) If not recognized early, LVNC can present with fatal arrhythmias, sudden cardiac death, and systemic embolism, which can lead to significant morbidity and mortality. It is important for physicians to recognize this condition in anticipation of successful treatment. (3) Because of strong familial occurrence, all first‐degree relatives are recommended to have screening echocardiography.
To cite this abstract:Badal M, Aryal M. Left Ventricular Noncompaction Presenting As Systolic Heart Failure with Intraventricular Thrombus. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 322. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/left-ventricular-noncompaction-presenting-as-systolic-heart-failure-with-intraventricular-thrombus/. Accessed April 2, 2020.