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Long-term outcome in patients with apical hypertrophic cardiomyopathy
Maria J Eriksson1, Brian Sonnenberg, Anna Woo
1Division of Cardiology, University Hospital Network, Toronto General Hospital, University of Toronto, Toronto, Canada.
Insights
Apical hypertrophic cardiomyopathy (ApHCM) in North American patients shows a benign prognosis with low cardiovascular mortality. However, one-third experience major complications like atrial fibrillation and myocardial infarction, influencing patient management.
Area of Science:
- Cardiology
- Internal Medicine
- Genetics
Background:
- Apical hypertrophic cardiomyopathy (ApHCM) is a rare variant of hypertrophic cardiomyopathy (HCM).
- Early studies suggested a generally benign clinical course for ApHCM.
- This study investigates long-term outcomes in a North American cohort.
Purpose of the Study:
- To describe the long-term outcomes of patients diagnosed with ApHCM.
- To analyze mortality and cardiovascular morbidity in ApHCM patients.
- To identify predictors of adverse events in ApHCM.
Main Methods:
- Retrospective analysis of 105 ApHCM patients.
- Data collected from 1975 to 2000 at a tertiary referral center.
- Evaluation of symptoms, clinical findings, mortality, and cardiovascular morbidity.
Main Results:
- Mean follow-up was 13.6 years; overall survival was 95% at 15 years.
- Cardiovascular mortality was low (1.9%), but 30% experienced major morbid events (e.g., atrial fibrillation, myocardial infarction).
- Predictors of morbidity included younger age at presentation, left atrial enlargement, and NYHA class II+.
Conclusions:
- ApHCM in North American patients has a favorable prognosis regarding cardiovascular mortality and sudden cardiac death.
- Significant cardiovascular complications occur, necessitating careful patient counseling and management.
- Despite a benign overall outlook, monitoring for arrhythmias and infarction is crucial.
Objectives:
The aim of this study was to describe long-term outcome in patients with apical hypertrophic cardiomyopathy (ApHCM) followed in a tertiary referral center.
Background:
Apical hypertrophic cardiomyopathy is a relatively rare form of hypertrophic cardiomyopathy (HCM), first described in Japan. Initial reports, based on a limited number of patients, emphasized the benign nature of this condition.
Methods:
A retrospective study of 105 patients with ApHCM diagnosed at the Toronto General Hospital from 1975 to 2000 was performed. Symptoms, clinical findings, mortality and cardiovascular morbidity were analyzed.
Results:
The mean age at presentation was 41.4 +/- 14.5 years. During a mean follow-up of 13.6 +/- 8.3 years from presentation, cardiovascular mortality was 1.9% (2/105) and annual cardiovascular mortality was 0.1%. Overall survival was 95% at 15 years. Thirty-two patients (30%) had one or more major morbid events, the most frequent being atrial fibrillation (12%) and myocardial infarction (10%). Probability of survival without morbid events was 74% at 15 years. Three predictors of cardiovascular morbidity were identified: age at presentation <41 years, left atrial enlargement, and New York Heart Association (NYHA) class > or = II at baseline. Forty-four percent of the patients were asymptomatic at the time of last follow-up.
Conclusions:
Apical hypertrophic cardiomyopathy in North American patients is not associated with sudden cardiac death and has a benign prognosis in terms of cardiovascular mortality. Nevertheless, one third of these patients experience serious cardiovascular complications, such as myocardial infarction and arrhythmias. These data are likely to influence the counseling and management of patients with ApHCM.