Arrhythmogenic cardiomyopathy causing sudden death in Spain. Report of 21 cases

Introduction and objectives. Arrhythmogenic cardiomyopathy is a myocardial disease of unknown origin characterized by progressive replacement of right and/or left ventricular myocardium by fibrofatty tissue. Young adult people are more frequently affected and symptoms include palpitations, syncopes or sudden death. The objective of this study is to assess the frequency and characteristics of arrhythmogenic cardiomyopathy as a cause of sudden death in a series of cases in Spain, Material and methods. Among the sudden death cases studied at the Toxicology Institute of Madrid between 1991 and 1997, those with arrhythmogenic cardiomyopathy diagnosis mere analysed. Results. 21 cases mere diagnosed (0.62% of all sudden deaths and 6.8% of sudden deaths under 35 years). There were 20 males and one female (mean age 24.5 +/- 9.37 years); eleven (52.3%) died during sport activity and 8 (38%) had previous exercise related symptoms. In only two cases other, members of the family had died suddenly and only one had been diagnosed in life. Biventricular involvement was observed in 13 cases; isolated involvement of the left ventricle in 4, and isolated involvement of the right ventricle in 4. Conclusions. Arrhythmogenic cardiomyopathy is a relatively frequent cause of sudden death in the young population in Spain. It is very often the first manifestation of the disease. The myocardial involvement is more frequently;ly biventricular than isolated in the right ventricle The diagnosis could be difficult for cardiologists, pathologists and forensic doctors.