Prognostic determinants in hypertrophic cardiomyopathy. Prospective evaluation of a therapeutic strategy based on clinical, Holter, hemodynamic, and electrophysiological findings.

Abstract

Background. Patients with hypertrophic cardiomyopathy (HCM) frequently have arrhythmias and hemodynamic abnormalities and are prone to sudden death and syncope. An important need exists for improved risk stratification and definition of appropriate investigation and therapy.Methods and Results. The relation of 31 clinical, Holter, cardiac catheterization, and electrophysiological (EP) variables to subsequent cardiac events in 230 HCM patients was examined by multivariate analysis. Studies were for cardiac arrest (n=32), syncope (n=80), presyncope (n=52), ventricular tachycardia (VT) on Holter (n=36), a strong family history of sudden death (n=9), and palpitations (n=21). Nonsustained VT on Holter was present in 115 patients (50%o). Sustained ventricular arrhythmia was induced in 82 patients (36%). Seventeen cardiac events (eight sudden deaths, one cardiac arrest, and eight syncope with defibrillator discharges) occurred during a follow-up of 28±19 months. The 1-year and 5-year event-free rates were 99%, and 79%o, respectively. Two variables were significant independent predictors of subsequent

Electrophysiological abnormalities and arrhythmias in alpha MHC mutant familial hypertrophic cardiomyopathy mice.

“…The clinical course of FHC is highly variable, with a spectrum at presentation that ranges from the asymptomatic carrier state to premature sudden death in childhood. The risk factors for sudden death in FHC include young age at presentation, syncope, myocardial ischemia, inducible sustained ventricular tachycardia during EP study, and a malignant family history (21)(22)(23)(24). In FHC patients who have survived cardiac arrest, electrophysiologic testing has demonstrated that sinus node dysfunction, electrogram fractionation, and inducible ventricular arrhythmias are common (16,25,26).…”

Noncardiac surgery and the risk of death and other cardiovascular events in patients with hypertrophic cardiomyopathy

“…1 Although the majority of patients remain asymptomatic, some exhibit severe limiting symptoms of dyspnea, angina, or syncope, and some may even die suddenly from cardiac causes. Multiple studies have shown that patients with HCM are at higher risk for sudden cardiac death, 2-4 stroke, 2 atrial fibrillation, 2,5,6 atrial reentrant tachycardias, 6 syncope, 5,6 and congestive heart failure (CHF). 3 The mechanisms underlying the morbid consequences of HCM include dynamic left ventricular (LV) outflow tract obstruction, mitral regurgitation, diastolic dysfunction, myocardial ischemia, and cardiac arrhythmias.…”

Avaliação da função autonômica na cardiomiopatia hipertrófica

“…Relevant clinical and echocardiographic data are shown in Table II. Comparison of intergroup HRV analysis at each stage - Table III 28 . ** Gradient on left ventricle outfl ow higher than 30mmHg at rest 29 -either in the time domain or in the frequency domain (Table IV and V).…”