ARRHYTHMOGENIC RIGHT-VENTRICULAR CARDIOMY OPATHY, ANOMALOUS ORIGIN OFTHE RIGHT CORONARY-ARTERY AND SUDDEN-DEATH IN A 36 YEAR-OLD MALE - COINCIDENCE OR CLUE TO THE ETIOLOGIC MECHANISM OF STRUCTURAL-CHANGES OF THE MYOCARDIUM

A metal worker who suffered sudden death (SD) at age 36, presented at autopsy with an as yet unknown combination of structural changes of th e heart, both an arrhythmogenic right ventricular cardiomyopathy (RVCM ) and an anomalous right coronary artery originating from the left aor tic sinus (AORC). Clinically the progressive RVCM (over 9 years) was c haracterized by ventricular arrhythmias, mainly in form of increased P VC-activity (5000/24h). Post mortem a chronic RV-insufficiency was dia gnosed, which had failed to be recognized, either clinically or sympto matically: Also at his last outpatient visit (1/2 year prior SD) the p atient was asymptomatic, clinically unimpaired as always, and the PVC rate proved unchanged. Theoretically, AORC and RVCM, either one alone could be held responsible for the SD of this patient. Although progres sive RV-cardiomyopathy (interpreted as fatty degeneration of myocardiu m due to chronic RV ischemia) is the most obvious plausible cause for an arrhythmia-induced fatal event here, the additional partaking of is chemic events, produced by the compressing mechanism of the AORC (betw een aorta and a. pulmonalis) cannot completely be excluded. The combin ed occurrence of the two pathological entities (RVCM plus AORC) is rat ed coincidental, but never before reported.