Ashish Kulhari1, Nikhil Kalra1, Cathy Sila2. 1. Department of Neurology, University Hospitals Case Medical Center, Cleveland, Ohio. 2. Department of Neurology, University Hospitals Case Medical Center, Cleveland, Ohio. Electronic address: cathy.sila@UHhospitals.org.
Abstract
BACKGROUND: To describe a rare case of isolated noncompaction cardiomyopathy and stroke and to review the medical literature on noncompaction cardiomyopathy. METHODS: Retrospective chart review of the case was performed. Extensive literature review on etiology, clinical presentation, diagnosis, and management of noncompaction cardiomyopathy was also performed. RESULTS: Our patient is a healthy 20-year-old woman who presented with sudden onset left face and arm weakness and hypesthesia. Magnetic resonance imaging (MRI) brain showed right middle cerebral artery (MCA) infarct. Magnetic resonance angiography head showed right MCA artery (M2) cutoff. MRI neck was nonsignificant. Echocardiogram was suggestive of noncompaction of left ventricle. Cardiac MRI confirmed the noncompaction of the left ventricle myocardium, which was thought to be the etiology of stroke. Patient was started on anticoagulation for secondary stroke prevention. CONCLUSIONS: Isolated left ventricular noncompaction cardiomyopathy (LVNC) is a rare form of primary genetic cardiomyopathy, which occurs because of the arrest of the process of compaction of ventricular myocardium during embryogenesis. Noncompaction cardiomyopathy is usually associated with other primary cardiac structural abnormalities like dysfunctional cardiac valves. In isolated noncompaction cardiomyopathy, there are no other primary cardiac structural abnormalities. The most common clinical features seen in LVNC include left ventricular systolic dysfunction, congestive heart failure, arrhythmias, and cardiac embolic events theorized to result from thrombus formation within the intertrabecular recesses. As it is a rare disease, evidence-based recommendations for preventing thromboembolic events in isolated left ventricular noncompaction have not been established.
BACKGROUND: To describe a rare case of isolated noncompaction cardiomyopathy and stroke and to review the medical literature on noncompaction cardiomyopathy. METHODS: Retrospective chart review of the case was performed. Extensive literature review on etiology, clinical presentation, diagnosis, and management of noncompaction cardiomyopathy was also performed. RESULTS: Our patient is a healthy 20-year-old woman who presented with sudden onset left face and arm weakness and hypesthesia. Magnetic resonance imaging (MRI) brain showed right middle cerebral artery (MCA) infarct. Magnetic resonance angiography head showed right MCA artery (M2) cutoff. MRI neck was nonsignificant. Echocardiogram was suggestive of noncompaction of left ventricle. Cardiac MRI confirmed the noncompaction of the left ventricle myocardium, which was thought to be the etiology of stroke. Patient was started on anticoagulation for secondary stroke prevention. CONCLUSIONS: Isolated left ventricular noncompaction cardiomyopathy (LVNC) is a rare form of primary genetic cardiomyopathy, which occurs because of the arrest of the process of compaction of ventricular myocardium during embryogenesis. Noncompaction cardiomyopathy is usually associated with other primary cardiac structural abnormalities like dysfunctional cardiac valves. In isolated noncompaction cardiomyopathy, there are no other primary cardiac structural abnormalities. The most common clinical features seen in LVNC include left ventricular systolic dysfunction, congestive heart failure, arrhythmias, and cardiac embolic events theorized to result from thrombus formation within the intertrabecular recesses. As it is a rare disease, evidence-based recommendations for preventing thromboembolic events in isolated left ventricular noncompaction have not been established.
Authors: Cristina Chimenti; Carlo Lavalle; Michele Magnocavallo; Maria Alfarano; Marco Valerio Mariani; Federico Bernardini; Domenico Giovanni Della Rocca; Gioacchino Galardo; Paolo Severino; Luca Di Lullo; Fabio Miraldi; Francesco Fedele; Andrea Frustaci Journal: ESC Heart Fail Date: 2021-12-16