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Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy
- Source :
- Circulation. 114(3)
- Publication Year :
- 2006
-
Abstract
- Background— End stage (ES) is a recognized part of the hypertrophic cardiomyopathy (HCM) disease spectrum. Frequency, clinical profile and course, and treatment strategies in these patients remain incompletely defined. Methods and Results— Three HCM cohorts comprised 1259 patients, including 44 (3.5%) characterized as ES with systolic dysfunction (ejection fraction P =0.84). Appropriate defibrillator interventions were 10% per year in patients awaiting donor hearts. Most ES patients (n=23; 52%) showed substantial left ventricular (LV) remodeling with cavity dilatation. Less complete remodeling occurred in 21 patients (48%), including 5 with persistence of a nondilated and markedly hypertrophied LV. Pathology and magnetic resonance imaging showed extensive (transmural) fibrosis in 9 of 11 ES patients. At initial evaluation, patients who developed ES were younger with more severe symptoms, had a larger LV cavity, and more frequently had a family history of ES than other HCM patients. Conclusions— ES of nonobstructive HCM has an expanded and more diverse clinical expression than previously appreciated, including occurrence in young patients, heterogeneous patterns of remodeling, frequent association with atrial fibrillation, and impaired LV contractility that precedes cavity dilatation, wall thinning, and heart failure symptoms. ES is an unfavorable complication (mortality rate 11% per year) and a sudden death risk factor; it requires vigilance to permit timely recognition and the necessity for defibrillator implantation and heart transplantation.
- Subjects :
- Adult
Male
medicine.medical_specialty
Time Factors
Heart disease
Adolescent
medicine.medical_treatment
Cardiomyopathy
Sudden death
Cohort Studies
Physiology (medical)
Internal medicine
medicine
Prevalence
Humans
Child
Retrospective Studies
Heart transplantation
Ejection fraction
Ventricular Remodeling
business.industry
Hypertrophic cardiomyopathy
Cardiomyopathy, Hypertrophic
medicine.disease
Magnetic Resonance Imaging
United States
Transplantation
Echocardiography
Heart failure
Cardiology
Female
Cardiology and Cardiovascular Medicine
business
Follow-Up Studies
Subjects
Details
- ISSN :
- 15244539
- Volume :
- 114
- Issue :
- 3
- Database :
- OpenAIRE
- Journal :
- Circulation
- Accession number :
- edsair.doi.dedup.....8318e343a09e5c434b7d21f439cee01b