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Dilated-Hypokinetic Evolution of Hypertrophic Cardiomyopathy

Prevalence, Incidence, Risk Factors, and Prognostic Implications in Pediatric and Adult Patients

Elena Biagini, MD^_*, Fabio Coccolo, MD^_*, Marinella Ferlito, MD^_*, Enrica Perugini, MD^_*, Guido Rocchi, MD^_*, Letizia Bacchi-Reggiani, BSc^_*, Carla Lofiego, MD^_*, Giuseppe Boriani, MD^_*, Daniela Prandstraller, MD, Fernando M. Picchio, MD, Angelo Branzi, MD^_* and Claudio Rapezzi, MD^_*,_*

^_* Institute of Cardiology, University of Bologna, Bologna, Italy
Pediatric Cardiology and Adult Congenital Unit, University of Bologna, Bologna, Italy

View larger version (119K): [in a new window]   Figure 1 A representative example of evolution to dilated-hypokinetic hypertrophic cardiomyopathy (HCM) in a female pediatric patient. (A) The basal echocardiogram (at age 13 years) shows HCM with massive left ventricular (LV) hypertrophy involving the intraventricular septum and the left posterior wall, accompanied by diminutive LV cavity size. (B) Three years later (at age 16 years), the LV cavity has become enlarged and the walls have thinned in the context of severe heart failure, requiring heart transplantation.

View larger version (25K): [in a new window]   Figure 2 Changes in left ventricular (LV) maximal wall thickness (A), LV end-diastolic diameter (B), and LV ejection fraction (C) in the 12 hypertrophic cardiomyopathy (HCM) patients who underwent dilated-hypokinetic evolution during the study period. SD = standard deviation.

View larger version (14K): [in a new window]   Figure 3 Actuarial incidence curve of dilated-hypokinetic evolution among 210 patients with classic hypertrophic cardiomyopathy at first evaluation.

View larger version (17K): [in a new window]   Figure 4 Kaplan-Meier estimates of cardiovascular death-free survival of patients with and without prevalent/incident dilated-hypokinetic hypertrophic cardiomyopathy (HCM).