First nationwide study of sudden cardiac death due to arrhythmogenic right ventricular cardiomyopathy in the young; fifty percent have symptoms prior to death

Abstract

Purpose: The aim of this nationwide study was to investigate symptoms prior to death in all persons ≤35 years who died suddenly from arrhythmogenic right ventricular cardiomyopathy (ARVC) in Denmark between 2000-2006. Methods: We included all deaths (6,629) in persons 1-35 years in Denmark between 2000-2006. Thereby we identified 314 autopsied sudden cardiac deaths (SCD) from which 16 died due to ARVC. Using the National Patient Registry which is a database containing ICD-10 codes and dates of admission and discharge from hospitals of all Danish citizens, we could identify the time, place and diagnosis given at any admission to a hospital, as well as the patient's general practitioner (GP). Hospitals and GP'swere contacted by letter to retrieve all medical files. A control group of 74 persons in the same age group, who died in accidents in the same time interval were used. Results: In total eight of the 16 ARVC cases had antecedent cardiac symptoms (syncope, palpitations, angina, dyspnoea and fatigue) prior to death, and seven of them sought medical attention. The symptoms in the case group were significantly higher than in the control group, (1/74 -p<0,001, Fisher exact test). Four of the patients experienced prodromal symptoms (angina, dyspnoea, syncope) two of which also had antecedent symptoms, making a total of 10 patients (63%) experiencing cardiac symptoms before death. None of the SCD cases were diagnosed with ARVC pre-mortem. One was diagnosed with myocarditis and three were diagnosed with asthma. From the seven patients who sought medical attention due to symptoms, six had an ECG performed of which two showed changes characteristic of ARVC, and three had further cardiac examination than an ECG In total six of the patients died during strenuous physical activity and four of them where athletes, making these four deaths sports related sudden cardiac deaths Five died during everyday activities. Conclusion: This is the first nationwide study to report antecedent and prodromal symptoms in young SCD by ARVC victims. Half of the cases had antecedent cardiac symptoms prior to death. This was significantly higher than in the control group. All but one of the symptomatic cases sought medical attention, but none were diagnosed before death. A high percentage of the patients were athletes and an even higher percentage died during strenuous physical activity. We recommend a high degree of attention towards conducting sufficiently thorough cardiac investigation in young patients with cardiac symptoms.