Valvular heart complications in children with osteogenesis imperfecta

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Abstract Purpose: The aim of this study was to determine the cardiovascular function and prevalence of valvular diseases in children with osteogenesis imperfecta (OI). Methods: Twenty-eight OI children (mean age: 9.9±6.0 years) underwent echocardiography. Heart function, heart dimensions, and valvular complications were assessed calculating standard deviation (SD) scores referring to body surface area, and compared retrospectively in clinical types according to Sillence’s classification and with height SD scores. Results: Of the 28 OI children, 17 (61%) were complicated with valvular diseases. The mean height SD scores was lower in more severe clinical types (p<0.01). Tricuspid regurgitation was detected in 54%, mitral regurgitation occurred in 21%, and aortic regurgitation was observed in 4% of the patients. Any valvular disease was detected in 86% (6/7), 46% (5/11), and 60% (6/10) of the patients with OI type 1, 4, and 3, respectively. A negative correlation was detected between aortic root diameters and height SD scores (r=-0.55). Interventricular septum (IVS) thicknesses were within ±2 SD in all cases, but those in 79% of the patients were indicated above the mean value of normal population. The mean IVS SD scores was +0.62±0.72. All measured values, including ventricular diastolic dimensions and ejection fraction, were within normal range and not different between the clinical types. Conclusions: Valvular dysfunction is a frequent complication of OI patients, even in childhood. Aortic root enlargement and hypertrophic IVS appeared to be negatively impacted by the disease. Thus, careful echocardiographic follow-up during childhood, especially for patients with severe short stature, should be performed.

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