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Progressive left ventricular remodeling in patients with hypertrophic cardiomyopathy and severe left ventricular
Rajesh Thaman1, Juan R Gimeno, Sebastian Reith
1The Heart Hospital, University College London, London, United Kingdom.
Insights
Left ventricular remodeling is common in patients with hypertrophic cardiomyopathy (HCM) and severe left ventricular hypertrophy (LVH). This cardiac remodeling contributes to the lower prevalence of severe LVH observed in older individuals.
Area of Science:
- Cardiology
- Cardiovascular Medicine
- Medical Research
Background:
- Severe left ventricular hypertrophy (LVH) is frequently observed in younger patients with hypertrophic cardiomyopathy (HCM), but is rare in the elderly.
- This age-related difference in prevalence is partly attributed to a high incidence of sudden cardiac death in younger individuals.
- A hypothesis suggests that left ventricular wall thinning over time may explain the decreased prevalence of severe LVH in older HCM patients.
Purpose of the Study:
- To investigate the natural history of patients diagnosed with hypertrophic cardiomyopathy (HCM) and severe left ventricular hypertrophy (LVH), defined as maximal left ventricular wall thickness (MLVWT) ≥30 mm.
- To determine if changes in cardiac morphology, specifically left ventricular wall thinning, influence the disease's progression and clinical outcomes.
Main Methods:
- A cohort of 106 consecutive patients with severe LVH underwent comprehensive clinical assessments, including ECG, echocardiography, and exercise testing.
- Survival data were meticulously collected through clinic visits and communication with healthcare providers.
- Serial echocardiographic assessments over at least one year were performed on 71 patients to evaluate morphologic and functional changes.
Main Results:
- The majority of the 106 patients (71.6%) were under 40 years old.
- During a median follow-up of 92 months, 17% of patients experienced death or heart transplantation, with sudden cardiac death being the most common cause.
- Serial evaluations revealed an average reduction in MLVWT of 0.6 mm/year, with significant wall thinning (≥5 mm) observed in 57.7% of patients. Follow-up duration was the sole predictor of wall thinning.
Conclusions:
- Left ventricular remodeling, characterized by wall thinning, is a common phenomenon in patients with severe LVH.
- This remodeling process contributes to the observed decrease in the prevalence of severe LVH in middle-aged and elderly individuals with HCM.
- Understanding these remodeling patterns is crucial for managing HCM and predicting long-term outcomes.
Objectives:
The aim of this study was to determine the natural history of patients with hypertrophic cardiomyopathy (HCM) and severe left ventricular hypertrophy (LVH) (i.e., maximal left ventricular wall thickness [MLVWT] >/=30 mm) and whether changes in cardiac morphology influence the course of the disease.
Background:
Severe LVH is common in young and rare among elderly patients with HCM. This has been explained by a high incidence of sudden death. We hypothesized that this age-related difference might be explained by left ventricular wall thinning.
Methods:
A total of 106 (age 33 +/- 15 years; 71 males) consecutive patients with severe LVH underwent history taking, examination, electrocardiography, echocardiography, cardiopulmonary exercise testing, and Holter analysis. Survival data were collected at subsequent clinic visits or by communication with patients and their general practioners. In order to assess morphologic and functional changes, 71 (67.0%) patients (mean age 31 +/- 15 years; 47 males) followed at our institution underwent serial (>/=1 year) assessment.
Results:
Of the 106 patients, the majority (78 [71.6%]) were <40 years of age. During follow-up (92 +/- 50 months [range 1 to 169]), 18 (17.0%) patients died or underwent heart transplantation (13 sudden cardiac deaths, 2 heart failure deaths, 1 heart transplantation, 1 stroke, 1 postoperative death). Five-year survival from sudden death was 90.1% (95% confidence interval [CI] 84.0% to 96.3%), and that from heart failure death or transplantation was 97.7% (95% CI 94.5 to 100). In patients serially evaluated over 85 +/- 51 months, there was an overall reduction in MLVWT of 0.6 mm/year (95% CI 0.31 to 0.81, p = 0.00004). Wall thinning >/=5 mm was observed in 41 patients (57.7%; age 35 +/- 13 years; 28 males). On multivariate analysis, the follow-up duration only predicted wall thinning (0.6 mm/year, 95% CI 0.38 to 0.85, p < 0.00001).
Conclusions:
Left ventricular remodeling is common in patients with severe LVH and contributes to the low prevalence of severe LVH seen in middle age and beyond.
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