CONGRESS ABSTRACT

INTRODUCTION Becker Muscular Dystrophy (BMD) is a genetic disorder, caused by mutations in the dystrophin gene. Cardiac involvement is a common feature in this condition, often manifesting as dilated cardiomyopathy (DCM) or arrhythmias. In patients with BMD, ventricular dysfunction is frequently attributed to non-ischemic mechanisms, given the known pathophysiology of dystrophinopathy. However, overlooking coexisting ischemic heart disease, particularly in older patients with cardiovascular risk factors, can result in suboptimal management. CASE REPORT A 57-year-old patient, hypertensive, with high cholesterol and with a family history of idiopathic dilated cardiomyopathy, affected by BMD, presented for a cardiological evaluation as part of follow-up for his dystrophinopathy, asymptomatic for cardiac symptoms. The echocardiogram showed a worsening of left ventricular contractile function compared to the previous year’s check-up left ventricular ejection fraction (LVEF) was 42% vs 55%, with hypokinesia of the mid-basal lateral wall. Considering these findings, cardiac magnetic resonance (MRI) was recommended. The MRI confirmed left ventricular contractile dysfunction: LVEF 36%, with intramural late gadolinium enhancement (LGE) in the basal lateral, inferior, and septal segments, as well as the mid-inferolateral segment. Due to these findings, and despite the highly likely nature of the LV dysfunction related to the known muscular dystrophy, the patient underwent CT angiography to rule out coronary disease. The coronary CT angiography revealed bivasal coronary artery disease. Therefore, coronary angiography was performed and percutaneous coronary intervention (PCI) was performed on proximal and mid-RCA, distal LAD, and proximal D1 that had critical stenoses. DISCUSSION This case demonstrates that cardiomyopathy secondary to coronary artery disease can coexist with dystrophin-related cardiomyopathy, especially in older patients with traditional cardiovascular risk factors. This case underscores the need to maintain a broad differential diagnosis when evaluating left ventricular dysfunction in BMD patients. The findings highlight the need for a comprehensive cardiovascular assessment. The importance of multimodal imaging: LGE patterns in cardiac MRI, while suggestive of fibrosis, may overlap with ischemic scarring, necessitating further evaluation with coronary imaging.