Noncompaction Cardiomyopathy and Stroke: Case Report and Literature Review
Ashish Kulhari1, Nikhil Kalra1, Cathy Sila1
1Department of Neurology, University Hospitals Case Medical Center, Cleveland, Ohio.
Insights
A rare case of isolated left ventricular noncompaction cardiomyopathy (LVNC) in a young woman led to stroke. This condition, a genetic heart muscle disorder, requires further research for effective prevention of embolic events.
Area of Science:
- Cardiology
- Neurology
- Genetics
Background:
- Isolated left ventricular noncompaction cardiomyopathy (LVNC) is a rare genetic cardiomyopathy.
- It arises from incomplete compaction of the ventricular myocardium during embryogenesis.
- LVNC typically lacks other primary cardiac structural abnormalities.
Observation:
- A healthy 20-year-old woman presented with acute stroke symptoms (hemiparesis, hypesthesia).
- Brain imaging revealed a right middle cerebral artery (MCA) infarct.
- Cardiac imaging confirmed LVNC as the suspected etiology of the embolic event.
Findings:
- The patient's stroke was attributed to LVNC, a rare cardiac condition.
- Echocardiogram and cardiac MRI confirmed left ventricular noncompaction.
- Anticoagulation was initiated for secondary stroke prevention.
Implications:
- LVNC can manifest as embolic events, including stroke, even in young, otherwise healthy individuals.
- Further research is needed to establish evidence-based guidelines for preventing thromboembolic events in isolated LVNC.
- Understanding the link between LVNC and stroke is crucial for early diagnosis and management.
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.
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