Left ventricular noncompaction—a rare cause of triad: heart failure, ventricular arrhythmias, and systemic embolic events: a case report 

Abstract

Background Left ventricular noncompaction is a rare cardiomyopathy characterized by a thin, compacted epicardial layer and a noncompacted endocardial layer, with trabeculations and recesses that communicate with the left ventricular cavity. In the advanced stage of the disease, the classical triad of heart failure, ventricular arrhythmia, and systemic embolization is common. Segments involved are the apex and mid inferior and lateral walls. The right ventricular apex may be affected as well. Case presentation A 29-year-old Caucasian male was hospitalized with dyspnea and fatigue at minimal exertion during the last months before admission. He also described a history of edema of the legs and abdominal pain in the last weeks. Physical examination revealed dyspnea, pulmonary rales, cardiomegaly, hepatomegaly, and splenomegaly. Electrocardiography showed sinus rhythm with nonspecific repolarization changes. Twenty-four-hour Holter monitoring identified ventricular tachycardia episodes with right bundle branch block morphology. Transthoracic echocardiography at admission revealed dilated left ventricle with trabeculations located predominantly at the apex but also in the apical and mid portion of lateral and inferior wall; end-systolic ratio of noncompacted to compacted layers > 2; moderate mitral regurgitation; and reduced left ventricular ejection fraction. Between apical trabeculations, multiple thrombi were found. The right ventricle had normal morphology and function. Speckle-tracking echocardiography also revealed systolic left ventricle dysfunction and solid body rotation. Abdominal echocardiography showed hepatomegaly and splenomegaly. Abdominal computed tomography was suggestive for hepatic and renal infarctions. Laboratory tests revealed high levels of N-terminal pro-brain natriuretic peptide and liver enzymes. Cardiac magnetic resonance evaluation at 1 month after discharge confirmed the diagnosis. The patient received anticoagulants, antiarrhythmics, and heart failure treatment. After 2 months, before device implantation, he presented clinical improvement, and echocardiographic evaluation did not detect thrombi in the left ventricle. Coronary angiography was within normal range. A cardioverter defibrillator was implanted for prevention of sudden cardiac death. Conclusions Left ventricular noncompaction is rare cardiomyopathy, but it should always be considered as a possible diagnosis in a patient hospitalized with heart failure, ventricular arrhythmias, and systemic embolic events. Echocardiography and cardiac magnetic resonance are essential imaging tools for diagnosis and follow-up.

Isolated Left Ventricular Noncompaction Presenting With Heart Failure With Reduced Ejection Fraction and Intrahospital Cardiac Arrest: A Case Report and Literature Review

“…There is a mismatch between myocardial mass and the number of capillaries, leading to hypoperfusion of the endocardium, despite normal epicardial coronary arteries, with hypokinetic areas observed both in compacted and noncompacted segments [ 9 ]. Also, trabeculations diminish ventricular compliance, promoting diastolic dysfunction; systolic dysfunction is attributed to hypoperfusion secondary to subendocardial microvascular abnormalities and by LV dyssynchrony between the compacted and noncompacted myocardial layers [ 10 ].…”

Left Ventricular Noncompaction as a Rare Cause of Syncope

“…The prevalence of LVNC has been estimated to vary from 0.01% to 1.3% in patients referred for echocardiography. However, recent studies show that it may be seen in as high as 3%-4% of patients with heart failure [3][4][5].…”

“…This is thought to result from premature cessation of the compaction of the loosely interwoven meshwork of myocardial fibers during endomyocardial morphogenesis, a process that occurs between 12-18 weeks of fetal life, beginning at the base of the left ventricle and progressing toward the apex. This process of trabeculation appears to involve the release of neuregulin growth factors from the endocardium and may also involve angiogenetic factors, such as vascular endothelial growth factor and angiopoietin-1 [4][5][6][7][8].…”

“…However, they are thought to be significantly more common than in the general population, particularly in those who have already developed heart failure (a potent trigger for malignant arrhythmias). Furthermore, recurrent syncope in LVNC may be predictive for eventual SCD [1,[4][5][6][7]12].…”

“…MRI also has the added ability to clearly visualize the apex, which has been a limitation of echocardiography. LVNC has also been identified by high-resolution computed tomography and positron emission tomography [ 2 , 4 , 6 - 8 , 13 ].…”