Asunto(s)
Desfibriladores Implantables , Cardioversión Eléctrica/instrumentación , Técnicas Electrofisiológicas Cardíacas/tendencias , Pautas de la Práctica en Medicina/tendencias , Fibrilación Ventricular/terapia , Cardioversión Eléctrica/efectos adversos , Análisis de Falla de Equipo , Seguridad de Equipos , Encuestas de Atención de la Salud , Humanos , Italia , Valor Predictivo de las Pruebas , Diseño de Prótesis , Falla de Prótesis , Factores de Tiempo , Procedimientos Innecesarios/tendencias , Fibrilación Ventricular/diagnóstico , Fibrilación Ventricular/fisiopatologíaRESUMEN
BACKGROUND: The role of implantable cardioverter-defibrillator (ICD) in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation (VF) or sustained ventricular tachycardia is an unsolved issue. METHODS AND RESULTS: We studied 106 consecutive patients (62 men and 44 women; age, 35.6±18 years) with arrhythmogenic right ventricular cardiomyopathy/dysplasia who received an ICD based on 1 or more arrhythmic risk factors such as syncope, nonsustained ventricular tachycardia, familial sudden death, and inducibility at programmed ventricular stimulation. During follow-up of 58±35 months, 25 patients (24%) had appropriate ICD interventions and 17 (16%) had shocks for life-threatening VF or ventricular flutter. At 48 months, the actual survival rate was 100% compared with the VF/ventricular flutter-free survival rate of 77% (log-rank P=0.01). Syncope significantly predicted any appropriate ICD interventions (hazard ratio, 2.94; 95% confidence interval, 1.83 to 4.67; P=0.013) and shocks for VF/ventricular flutter (hazard ratio, 3.16; 95% confidence interval, 1.39 to 5.63; P=0.005). The positive predictive value of programmed ventricular stimulation was 35% for any appropriate ICD intervention and 20% for shocks for VF/ventricular flutter, with a negative predictive value of 70% and 74%. None of the 27 asymptomatic patients with isolated familial sudden death had appropriate ICD therapy. Twenty patients (19%) had inappropriate ICD interventions, and 18 (17%) had device-related complications. CONCLUSIONS: One fourth of patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior sustained ventricular tachycardia or VF had appropriate ICD interventions. Syncope was an important predictor of life-saving ICD intervention and is an indication for ICD. Prophylactic ICD may not be indicated in asymptomatic patients because of their low arrhythmic risk regardless of familial sudden death and programmed ventricular stimulation findings. Programmed ventricular stimulation had a low predictive accuracy for ICD therapy.
Asunto(s)
Displasia Ventricular Derecha Arritmogénica/fisiopatología , Displasia Ventricular Derecha Arritmogénica/terapia , Desfibriladores Implantables , Taquicardia Ventricular/fisiopatología , Fibrilación Ventricular/fisiopatología , Adolescente , Adulto , Displasia Ventricular Derecha Arritmogénica/mortalidad , Técnicas Electrofisiológicas Cardíacas , Femenino , Estudios de Seguimiento , Humanos , Cooperación Internacional , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del Tratamiento , Adulto JovenRESUMEN
This case refers to a 39-year-old woman with hypertrophic cardiomyopathy (HCM) and family history of sudden death (SD). In 1985, high rate atrial stimulation induced VF. In 1996 an ICD was implanted and she remained without arrhythmic events until November 2000 when the device reported one episode of atrial fibrillation degenerating into VF and terminated by the ICD. The VF induction mechanism recorded by the ICD was similar to that observed in 1985. The high incidence of atrial tachyarrhythmias in HCM renders cases like this at higher risk of SD. The predictive role of incremental atrial stimulation merits highlighting in future studies.