HYPERTROPHIC CARDIOMYOPATHY IN GREECE - CLINICAL COURSE AND OUTCOME

Objective: Evaluation of clinical course and outcome of hypertrophic c ardiomyopathy in a representative Greek population. Background: Hypert rophic cardiomyopathy is characterized by unexplained left ventricular hypertrophy and varied clinical expression. Recent studies suggest et hnic differences. Materials and methods: One hundred seventy-four cons ecutive Greek patients (117 male, 57 female, age 47 +/- 16 years) from 143 different families were assessed at the Department of Cardiology of the University of Athens, Greece, and the State Cardiac Department, Hippokration Hospital, both located in Athens, Greece. To reduce sele ction bias, referral was based on disease diagnosis irrespective of cl inical status or treatment needs. All patients were examined clinicall y, echocardiographically, and by ECG ambulatory monitoring at 6-month intervals for a period of 74 +/- 22 months (range, 8 to 108 months). R esults: Most patients (n = 156, 89.7%) were in New York Heart Associat ion (NYHA) functional class I or II. The disease was familial (at leas t one affected first-degree relative) in 81 of the 143 families (56.6% ), and in 19 of these (13.3%) there was familial history of sudden car diac death. At initial examination, intraventricular septal thickness was 17.3 +/- 4.1 mm and posterior wall thickness was 13.7 +/- 3.8 mm a nd a left ventricular outflow gradient >30 mm Hg was present in 58 pat ients (33.3%). Similar were the findings during the last examination ( 17.5 +/- 4.3 mm, 13.5 +/- 4.4 mm, and 56 (32.2%, respectively, p = not significant). Episodes of nonsustained ventricular tachycardia were n oted in 15 patients (8.6%). There were eight deaths during follow-up: four sudden deaths and four from intractable heart failure. Syncope wa s reported by all patients who died. The annual mortality in this stud y was 1%. Syncope and NYHA class were the only predictors of outcome. Conclusions: In this representative Greek patient cohort with hypertro phic cardiomyopathy, the arrhythmogenic substrate was modest and the c linical course benign. Sudden cardiac death was infrequent and syncope , functional class, and ventricular arrhythmias were the only predicto rs of a poor outcome.