A 23-year-old man presented with worsening dyspnea and peripheral edema for several weeks. He had been diagnosed with non-compaction cardiomyopathy 2 years earlier and was receiving standard heart failure medications. An implantable cardiac defibrillator had been implanted 1 year earlier. A transthoracic echocardiographic exam showed dilatation of all chambers with biventricular failure. There was also characteristic thinning of the compacted epicardial layer and thickened endocardial layer with prominent trabeculation (Figure 1A-B, Videos 1-2) and deep perfused recesses (Figure 1C, Video 3) that particularly involved inferior and lateral walls. Additionally, multiple mobile thrombi were visualized between trabeculations (Figure 1B and D, Videos 2 and 4). The patient was started on systemic anticoagulation.
Figure 1
Figure 1
Video 1
Video 2
Video 3
Video 4
When would you consider systemic anticoagulation for a patient with non-compaction cardiomyopathy?
Show Answer
The correct answer is: E. All of the above
Non-compaction cardiomyopathy is a rare cardiomyopathy causing progressive heart failure, arrhythmias, and thromboembolic events though presentation can be highly variable. Thrombi that form within intertrabecular recesses may lead to systemic embolization.1 Although treatment approaches to this condition are similar to those of other cardiomyopathies, controversy remains on the subject of systemic prophylactic anticoagulation.2 Studies have supported systemic anticoagulation in cases of systolic dysfunction or atrial arrhythmia.3 Current evidence justifies systemic anticoagulation for patients with cardiac thrombus, history of thromboembolism, atrial fibrillation, and ejection fraction less than 40%.2
References
Gurunathan S, Senior R. Catastrophic stroke in a patient with left ventricular non-compaction. Echo Res Pract 2018;5:K59-K62.
Kido K, Guglin M. Anticoagulation Therapy in Specific Cardiomyopathies: Isolated Left Ventricular Noncompaction and Peripartum Cardiomyopathy. J Cardiovasc Pharmacol Ther 2018 Jan 1:1074248418783745:[Epub ahead of print].
Stöllberger C, Blazek G, Dobias C, Hanafin A, Wegner C, Finsterer J. Frequency of stroke and embolism in left ventricular hypertrabeculation/noncompaction. Am J Cardiol 2011;108:1021-3.
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