Sudden death due to arrhythmogenic right ventricular dysplasia: A medico-legal case report.

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is characterized by progressive fibrofatty replacement of the myocardium that predisposes to ventricular tachycardia and sudden death in young individuals and athletes. The prevalence of ARVD/C is estimated as 1:1000 to 1:1250 in the general population. Although it is a relatively uncommon cause of sudden cardiac death, it accounts for up to one fifth of sudden cardiac death in people less than 35 years of age. Clinical presentation of ARVD/C usually consists of arrhythmias of right ventricular origin that include premature ventricular beats, sustained ventricular tachycardia and ventricular fibrillation that can lead to sudden death. The authors present a case of a 26 year old young male, carpenter by occupation, previously healthy, with sudden death. The internal and external postmortem findings were normal except for mild right ventricular hypertrophy. But histo-pathological examination of heart tissues revealed replacement of myocardial tissue with mature fibrofatty tissue. The following medico-legal autopsy case is being reported for its rarity and the importance of histopathology to find out the cause of death.

Comparison of Uhl’s anomaly, right ventricular outflow tract ventricular tachycardia (RVOT VT) & arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) with an insight into genetics of ARVD/C.

Among the right ventricular conditions, Uhl’s anomaly, arrhythmogenic right ventricular dysplasia / cardiomyopathy (ARVD/C) and right ventricular outflow tract ventricular tachycardia (RVOT VT) are disorders that exhibit pathogenic changes involving the right ventricular (RV) myocardium, and are expected to be severe or milder forms of the same condition. The review focuses on the aspect whether the three RV disorders are a spectrum of the same disease. ARVD/C is the only condition among these to be genetically well characterized. Also, variations in the clinical expression of ARVD/C due to the genetic heterogeneity are examined. Based on clinical manifestations, age at onset, gender ratio and the possible molecular mechanisms implicated, Uhl’s anomaly, ARVD/C and RVOT VT may be considered as separate entities. Further, to differentiate between the three RV disorders, the molecular studies on ARVD/C might be helpful. An attempt was made to differentiate between the eleven different types of ARVD/Cs based on clinical symptoms presented including the progression of the disease to the left ventricle, ventricular arrhythmias and clinical characteristics like ECG, SAECG, ECHO and histopathological studies.