RESUMEN
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.
Asunto(s)
Muerte Súbita Cardíaca , Síndrome de QT Prolongado/congénito , Adolescente , Adulto , Niño , Muerte Súbita Cardíaca/patología , Electrocardiografía , Femenino , Humanos , Síndrome de QT Prolongado/diagnóstico , Síndrome de QT Prolongado/genética , Síndrome de QT Prolongado/patología , Masculino , Miocardio/patología , LinajeRESUMEN
Endocardial monophasic active potentials (M.A.P.) recorded by suction --tip intracardiac catheters constitute a new research-- method in cardiac arrythmias, electrophysiology and pharmacology. The characteristics of MAP at auricular and ventricular levels are analyzed. The limitations of the method in its incapacity to record the MAP of conduction tissues is discussed. This method of recording MAP is evaluated with antiarrythmic drugs of tipes I, II, III and IV. The results are identical to recordings performed with intracellular microelectrodes.