G30(P) Atypical presentation of ARVC mimicking acute coronary syndrome

Introduction Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is an autosomal dominant inherited disease of the heart muscle. It is characterised by replacement of cardiomyocytes with fibro-fatty tissue, predominantly affecting the right ventricle. This process leads to thinning of the ventricular wall and progression to heart failure. However, its major immediate complications are the increased risk of ventricular arrhythmia and sudden cardiac death (SCD). Case Report We report a 15-year-old, who was under surveillance due to a family history of suspected ARVC in his mother, presented with excruciating chest pain which started while watching a football match. Significantly raised troponin-I level of >3000 (later found to be due to auto-antibodies), and a CK of 175 prompted to initially treat him for suspected acute coronary syndrome. Subsequent ECGs showed epsilon waves and inverted T waves in leads V1-V5, whereas echocardiography and cardiac CT angiogram were found normal. But cardiac MRI demonstrated fatty infiltration in the inferolateral wall of the RV and LV myocardium, along with mild RV dilation and dysfunction. Hence an early and severe onset of ARVC was confirmed according to Task Force Major Criteria of 2010. The presence of polymorphic ventricular ectopics along with short runs of 4 beats of non-sustained VT resulted in an indication for an early ICD implantation. Discussion This is a rare case of ARVC that mimics acute coronary syndrome, due to a severe onset of fatty myocardium infiltration resulting in myocardial cell injury and elevated troponin levels. ARVC is generally asymptomatic, being diagnosed in young males (especially athletes) who present with ventricular arrhythmias, syncope or SCD. Diagnosis of ARVC is very difficult due to its variable penetration and incomplete expression. Conclusion Chest pain with high elevation of troponin level is a rare presentation of ARVC. We highlight the importance of considering ARVC as a differential diagnosis in young patients presenting with chest pain and elevated troponin, especially in the presence of ECG findings of epsilon waves or repolarisation abnormalities in the right precordial leads. An early diagnosis is crucial in this disease to implement strategies to prevent and/or treat ventricular arrhythmias and SCD, including lifestyle restrictions and placement of an ICD.