Cardiological assessment of first-degree relatives in sudden arrhythmic death syndrome.
4.1% of sudden cardiac deaths in the 16-64 age-group are unexplained. In this group, cardiac pathological findings are normal and toxicological tests are negative; termed sudden arrhythmic death syndrome (SADS). We searched for evidence of inherited cardiac disease in cases of SADS. Of 147 first-degree relatives of 32 people who died of SADS, 109 (74%) underwent cardiological assessment. Seven (22%) of the 32 families were diagnosed with inherited cardiac disease: four with long QT syndrome; one with non-structural cardiac electrophysiological disease; one with myotonic dystrophy; and one with hypertrophic cardiomyopathy. Families of people who die of SADS should be offered assessment in centres with experience of inherited cardiac disease.
Behr
E
E
St George's Hospital Medical School, University of London, London, UK.
Wood
D A
DA
Wright
M
M
Syrris
P
P
Sheppard
M N
MN
Casey
A
A
Davies
M J
MJ
McKenna
W
W
D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000453:epidemiology / Q000235:genetics / Q000401:mortality*; D002648:Child; D015897:Comorbidity; D016757:Death, Sudden, Cardiac / Q000453:epidemiology* / Q000209:etiology; D004452:Echocardiography; D004562:Electrocardiography; D004739:England / Q000453:epidemiology; D005080:Exercise Test; D005190:Family; D005260:Female; D006331:Heart Diseases / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics*; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D010808:Physical Examination