Sudden cardiac death due to arrhythmogenic right ventricular cardiomyopathy and cystic tumor of the AV node
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CASE REPORT
Sudden cardiac death due to arrhythmogenic right ventricular cardiomyopathy and cystic tumor of the AV node John Cavanaugh • Joseph A. Prahlow
Accepted: 14 March 2013 / Published online: 6 April 2013 Ó Springer Science+Business Media New York 2013
Abstract An 18-year-old white male collapsed suddenly in his home and died. At autopsy, the right ventricle of the decedent was noted to be dilated with marked thinning of the wall focally. Microscopically, the myocardium of the right ventricle was noted to be significantly thinned focally, where transmural infiltration with fibroadipose tissue was noted. Depending on the criteria utilized to render such a diagnosis, these findings were consistent with arrhythmogenic right ventricular cardiomyopathy (ARVC). Subsequent microscopic examination of the SA and AV node, however, revealed the presence of a cystic tumor of the AV node (CTAVN), a known cause of sudden death from arrhythmia. The case represents the first reported case of ARVC and CTAVN occurring together in the same individual. Keywords Sudden cardiac death Arrhythmogenic right ventricular cardiomyopathy AV node tumor Forensic pathology Autopsy Adolescent Pediatric
Introduction Sudden cardiac death (SCD) is usually defined as a death that results from cardiac causes without apparent antecedent symptoms or within the first hour after onset of symptoms [1]. Such cases present significant challenges for
J. Cavanaugh J. A. Prahlow (&) Indiana University School of Medicine-South Bend, South Bend, IN, USA e-mail: [email protected] J. A. Prahlow South Bend Medical Foundation, South Bend, IN, USA
cardiologists and forensic pathologists. For the cardiologist, current strategies and modalities that screen for many of the diseases that present frequently with SCD are either not cost effective or lack sensitivity. For the forensic pathologist, cases that show no obvious ischemic disease may necessitate various special tests, including possible genetic testing and dissection of the cardiac conduction system. However, there are an estimated 300,000 to 400,000 SCDs every year in the United States [1]. In persons under the age of 40 years who experience SCD and are subsequently autopsied, approximately 3 % of cases exhibit lethal abnormalities of the cardiac conduction system [2]. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by fatty or fibrofatty replacement of the myocardium of the right ventricle with associated right ventricular dilatation and increased incidence of cardiac arrhythmia. Fibrofatty replacement varies significantly in pattern and quantity, and it is not uncommon in routine forensic autopsy practice to encounter cases that are questionable as to whether or not the morphologic findings represent ARVC. Unfortunately there is currently no universally accepted autopsy criterion for diagnosis [3–5]. ARVC is a well-known cause of SCD and has a prevalence of roughly 1 in 5,000 in the general population [6]. SCD from ARVC occurs in both genders and all ages, but ten
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