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CLINICAL RESEARCH: HYPERTROPHIC CARDIOMYOPATHY

Progressive left ventricular remodeling in patients with hypertrophic cardiomyopathy and severe left ventricular hypertrophy

Rajesh Thaman, MRCP, MD^*, Juan R. Gimeno, MD^*, Sebastian Reith, MD^*, Maria T. Tome Esteban, MD^*, Giuseppe Limongelli, MD^*, Ross T. Murphy, MRCPI, MD^*, Bryan Mist, PhD^*, William J. McKenna, MD, FRCP, FESC, FACC^* and Perry M. Elliott, MD, MRCP, FACC^,*

^* Department of Cardiological Sciences, St. George's Hospital Medical School, London, United Kingdom
The Heart Hospital, University College London, London, United Kingdom

Manuscript received July 24, 2003; revised manuscript received January 21, 2004, accepted January 27, 2004.

^* Reprint requests and correspondence: Dr. Perry M. Elliott, The Heart Hospital, 16-18 Westmoreland Street, London W1G 8PH, United Kingdom.
perry.elliott{at}uclh.org

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.

Abbreviations and Acronyms   FS = fractional shortening   HCM = hypertrophic cardiomyopathy   ICD = implantable cardioverter-defibrillator   LV = left ventricular   LVEDD = left ventricular end-diastolic diameter   LVESD = left ventricular end-systolic diameter   LVH = left ventricular hypertrophy   LVOTG = left ventricular outflow tract gradient   MLVWT = maximal left ventricular wall thickness   NSVT = nonsustained ventricular tachycardia

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