Why the study?
Arrhythmogenic cardiomyopathies are an under-investigated common cause for myocardial infarction with no obstructive coronary artery disease.
Recurrent episodes of MINOCA with non-obstructive coronary artery disease and specific LV fibrosis patterns on CMRI should raise suspicion for desmoplakin cardiomyopathy.
Introduction: Arrhythmogenic cardiomyopathies are an under-investigated common cause for myocardial infarction with no obstructive coronary artery disease. Case description: A 56-year-old male presented with myocardial infarction with no obstructive coronary artery disease in three different occasions. His transthoracic echocardiography showed dropped left ventricle function in the last presentation. This was further investigated by cardiac magnetic resonance imaging which showed distinguishable pattern of myocardial fibrosis. He had primary prevention with an implantable cardioverter-defibrillator. He tested positive for pathological variant for the desmoplakin (DSP) gene. Conclusion: Recurrent myocardial injury left ventricular fibrosis, and frequent premature ventricular contractions should always raise the suspicion of DSP cardiomyopathy. LEARNING POINTS: Recurrent episodes of myocardial infarction with non-obstructive coronary artery disease should raise the suspicion of desmoplakin cardiomyopathy.Cardiac magnetic resonance imaging is the gold standard imaging tool for diagnosis.No consensus regarding risk assessment however primary prevention towards ventricular arrhythmias needs to be considered.
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Mahdy et al. (2026) studied this question.
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