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Purpose: The aim of our study was to examine the dimensions of the right ventricle (RV) by echocardiography in adults with osteogenesis imperfecta (OI), compare to the controls and investigate if pulmonary function might have an influence on the RV dimensions.Methods: The present echocardiographic survey included 99 adults with OI divided in three clinical types, I, III and IV, and 52 controls. RV cavity diastolic dimensions at base (RVD1) and from base to apex (RVD3), distal RV outflow tract (RVOT) and pulmonary artery (PA) diameters were measured and indexed for body surface area (BSA). Forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and FEV1/FVC ratio were measured in 75 patients.Results: The OI group had significantly lower BSA than the control individuals, 1.7 ± 0.3 vs. 1.9 ± 0.2 m2 (p<0.05). All RV dimensions were significantly larger in the OI group when compared to the controls, RVD1, 1.9±.5 vs. 1.7 ±.3 cm/m2, (p<0.05), RVD3, 4.3 ±1.1 vs. 4.0 ±.5 cm/m2, (p <0.05) and RVOT, 1.2 ±.3 vs. 1.1 ±.2cm/m2, (p<0.05). Furthermore PA, 1.2 ±.3 vs. 1.0 ±.2 cm/m2, (p<0.05) were also significantly larger in OI group. Besides RVD1, RVD3, RVOT and PA were significantly larger in OI type III as compared OI type I and IV, RVD1, 2.7 ±.4 cm/m2, (p<0.05), RVD3, 6.7 ±1.4cm/m2 (p<0.05), RVOT, 1.7 ±.3, (p<0.05), and PA, 1.6 ±.3cm/m2, (p<0.05). In the OI group, mild restrictive pulmonary pattern was recognized in 12 and mild obstructive pulmonary pattern in 5 patients. There were no differences in RV, RVOT or PA dimensions between the patients with restrictive ventilatory pattern and patients with normal ventilatory pattern.Conclusion: Increased RV and PA dimensions were found in the OI patients in comparison with the control group. Patients with OI type III seemed to have greater RV and PA dimensions in relation to both controls and other two OI types. Ventilatory pattern did not influence RV dimensions in OI patients.