title: Progressive left ventricular remodeling in patients with hypertrophic cardiomyopathy and severe left ventricular hypertrophy.
abstract: OBJECTIVE
  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.
authors.affiliation: The Heart Hospital, University College London, London, United Kingdom.
authors.forename: Rajesh
authors.initials: R
authors.lastname: Thaman
authors.forename: Juan R
authors.initials: JR
authors.lastname: Gimeno
authors.forename: Sebastian
authors.initials: S
authors.lastname: Reith
authors.forename: Maria T Tome
authors.initials: MT
authors.lastname: Esteban
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Limongelli
authors.forename: Ross T
authors.initials: RT
authors.lastname: Murphy
authors.forename: Bryan
authors.initials: B
authors.lastname: Mist
authors.forename: William J
authors.initials: WJ
authors.lastname: McKenna
authors.forename: Perry M
authors.initials: PM
authors.lastname: Elliott
mesh_terms: D000328:Adult; D000367:Age Factors; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications* / Q000401:mortality / Q000503:physiopathology; D016757:Death, Sudden, Cardiac; D018450:Disease Progression; D004452:Echocardiography; D005260:Female; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology*; D008297:Male; D012307:Risk Factors; D015996:Survival Rate; D020257:Ventricular Remodeling