Left ventricular noncompaction—a rare cause of triad: heart failure, ventricular arrhythmias, and systemic embolic events: a case report
Open Access
- 8 June 2021
- journal article
- research article
- Published by Springer Science and Business Media LLC in Journal of Medical Case Reports
- Vol. 15 (1), 1-9
- https://doi.org/10.1186/s13256-021-02862-x
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.Keywords
This publication has 23 references indexed in Scilit:
- Left Ventricular Trabeculation and Noncompaction Cardiomyopathy: A Review.2019
- Genetics, Clinical Features, and Long-Term Outcome of Noncompaction CardiomyopathyJournal of the American College of Cardiology, 2018
- Recommendations for Cardiac Chamber Quantification by Echocardiography in Adults: An Update from the American Society of Echocardiography and the European Association of Cardiovascular ImagingJournal of the American Society of Echocardiography, 2015
- Predictors of Adverse Outcome in Adolescents and Adults With Isolated Left Ventricular NoncompactionThe American Journal of Cardiology, 2012
- Left ventricular solid body rotation in non‐compaction cardiomyopathy: A potential new objective and quantitative functional diagnostic criterion?European Journal of Heart Failure, 2008
- Classification of the cardiomyopathies: a position statement from the european society of cardiology working group on myocardial and pericardial diseasesEuropean Heart Journal, 2007
- Left Ventricular Non-Compaction: Insights From Cardiovascular Magnetic Resonance ImagingJournal of the American College of Cardiology, 2005
- Left ventricular hypertrabeculation/noncompactionJournal of the American Society of Echocardiography, 2004
- Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathyHeart, 2001
- Isolated noncompaction of left ventricular myocardium. A study of eight cases.Circulation, 1990