[Sudden death and the congenital long QT syndrome (Romano, Ward and Barlow)].
We present four cases, a mother and three sons with Romano, Ward and Barlow syndrome (long QT without deaf-muteness) controlled over the past 20 years. The mother died suddenly at the age of 42, as did two sons at 24 and 17 years. A retrospective study was conducted comprising the three generations prior to the mother, and a high rate of sudden death was found. Possible causes were analyzed with a view to implementing proper prophylaxis treatment for ventricular tachycardia or fibrillation as causes for syncopal patterns and sudden death. Therefore, digital, left stellectomy and betablocker effects were analyzed. The anatomical study of the patient who died at 17 years is presented, and finally the new perspectives of the syndrome in terms of possible recognition of the responsible gene, as well as the convenience of implanting new defibrillating cardioverters, were evaluated.
Casellas Bernat
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Departamento de Cardiología y Cirugía Cardíaca, Hospital de la Santa Creu i Sant Pau de Barcelona.
D000293:Adolescent; D000328:Adult; D002648:Child; D016757:Death, Sudden, Cardiac / Q000473:pathology; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008133:Long QT Syndrome / Q000151:congenital* / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D008297:Male; D009206:Myocardium / Q000473:pathology; D010375:Pedigree
Muerte súbita y síndrome congénito de QT largo (Romano, Ward y Barlow).