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Hypertrophic cardiomyopathy in Greece: clinical course and outcome
M Kyriakidis1, F Triposkiadis, A Anastasakis
1Department of Cardiology, Hippokration Hospital, Athens, Greece.
Insights
This study on Greek patients with hypertrophic cardiomyopathy found a generally benign clinical course with infrequent sudden cardiac death. Syncope and functional class predicted poorer outcomes in this cohort.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) presents with unexplained left ventricular hypertrophy and diverse clinical manifestations.
- Emerging evidence suggests potential ethnic variations in HCM presentation and progression.
Purpose of the Study:
- To evaluate the clinical course and outcomes of hypertrophic cardiomyopathy within a representative Greek population.
- To identify predictors of adverse events in Greek patients diagnosed with HCM.
Main Methods:
- A cohort of 174 consecutive Greek patients with HCM from 143 families were prospectively followed for an average of 74 months.
- Clinical assessment, echocardiography, and 24-hour ECG monitoring were performed at 6-month intervals.
- Referral criteria minimized selection bias, including patients regardless of clinical status or treatment needs.
Main Results:
- The majority of patients (89.7%) were in New York Heart Association (NYHA) functional class I or II.
- Familial disease was present in 56.6% of families, with a history of sudden cardiac death in 13.3% of these.
- Annual mortality was 1%, with syncope and NYHA class identified as the sole predictors of poor outcome.
Conclusions:
- The Greek patient cohort with hypertrophic cardiomyopathy exhibited a modest arrhythmogenic substrate and a predominantly benign clinical course.
- Sudden cardiac death was infrequent, and syncope, functional class, and ventricular arrhythmias were key predictors of adverse outcomes.
Objective:
Evaluation of clinical course and outcome of hypertrophic cardiomyopathy in a representative Greek population.
Background:
Hypertrophic cardiomyopathy is characterized by unexplained left ventricular hypertrophy and varied clinical expression. Recent studies suggest ethnic differences.
Materials And Methods:
One hundred seventy-four consecutive 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 selection bias, referral was based on disease diagnosis irrespective of clinical status or treatment needs. All patients were examined clinically, echocardiographically, and by ECG ambulatory monitoring at 6-month intervals for a period of 74+/-22 months (range, 8 to 108 months).
Results:
Most patients (n = 156, 89.7%) were in New York Heart Association (NYHA) functional class I or II. The disease was familial (at least 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 cardiac death. At initial examination, intraventricular septal thickness was 17.3+/-4.1 mm and posterior wall thickness was 13.7+/-3.8 mm and a left ventricular outflow gradient >30 mm Hg was present in 58 patients (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 noted in 15 patients (8.6%). There were eight deaths during follow-up: four sudden deaths and four from intractable heart failure. Syncope was reported by all patients who died. The annual mortality in this study was 1%. Syncope and NYHA class were the only predictors of outcome.
Conclusions:
In this representative Greek patient cohort with hypertrophic cardiomyopathy, the arrhythmogenic substrate was modest and the clinical course benign. Sudden cardiac death was infrequent and syncope, functional class, and ventricular arrhythmias were the only predictors of a poor outcome.