Document Detail

Impairment of diastolic function in adult patients affected by osteogenesis imperfecta clinically asymptomatic for cardiac disease: casuality or causality?
MedLine Citation:
PMID:  18207586     Owner:  NLM     Status:  MEDLINE    
Osteogenesis imperfecta (OI) is a rare inherited connective disorder causing increased bone fragility and low bone mass. OI includes severe bone fragility, impaired dentinogenesis, with less common alterations in the joints, blood vessels, heart valves, skin. Interestingly, description of left ventricular rupture, aortic dissection and heart valves incompetence has been previously described. Death may occur in OI patients for cardiac disease in asyntomatic subjects. Aim of our study has been to evaluate the presence of potential subclinical cardiac disorders and to characterize cardiac functional parameters by echocardiography in adults with OI in absence of cardiac symptoms. Forty patients (21 females and 19 males) affected by type I, III, IV OI and 40 control subjects (20 females and 20 males) were evaluated in the study. Patients and controls underwent clinical examination, screening for endocrine and metabolic disorders, 12-lead electrocardiogram and echocardiogram. In particular, all subjects were evaluated by two-dimensional echocardiography with continuous- and pulse-wave Doppler. Patients and controls belonged to NYHA class I and no significant electrocardiographic alteration was documented in both groups. Thirty-eight patients (95%) showed valvular regurgitation compared to one control subject (2.5%; P<0.001). As regards the diastolic function parameters, in OI patients E wave velocity was reduced by 23% (95% CI: 9% to 29%; P<0.001), E/A ratio was reduced by 17% (95% CI: 15% to 26%; P<0.001) while isovolumetric relaxation time (IRT) was increased by 47% (95% CI: 26% to 53%; P<0.001) and E wave deceleration time (DT) was increased by 18% (95% CI: 13% to 26%; P<0.001) compared to controls. In conclusion, our data indicate that adult patients affected by OI have an altered diastolic function in absence of other metabolic alterations. These diastolic echocardiographic parameters might worsen over time, especially if other cardiovascular risk factors (e.g., smoking, hypertension, metabolic and endocrine alterations) are not carefully checked, monitored and treated. In the context of a multidisciplinary evaluation of OI patients, our data suggest that a careful cardiological evaluation of these patients is indicated beside skeletal evaluation and therapeutical skeletal options.
Silvia Migliaccio; Giuseppe Barbaro; Rachele Fornari; Gabriella Di Lorenzo; Mauro Celli; Carla Lubrano; Stefania Falcone; Elisa Fabbrini; Emanuela Greco; Anna Zambrano; Marina Brama; Giancarlo Prossomariti; Sara Marzano; Mario Marini; Francesco Conti; Patrizia D'Eufemia; Giovanni Spera
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Publication Detail:
Type:  Comparative Study; Journal Article     Date:  2008-01-18
Journal Detail:
Title:  International journal of cardiology     Volume:  131     ISSN:  1874-1754     ISO Abbreviation:  Int. J. Cardiol.     Publication Date:  2009 Jan 
Date Detail:
Created Date:  2008-12-22     Completed Date:  2009-09-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8200291     Medline TA:  Int J Cardiol     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  200-3     Citation Subset:  IM    
Dipartimento di Fisiopatologia Medica,Cattedra di Medicina Interna, Università degli Studi di Roma La Sapienza, Italy.
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MeSH Terms
Age Factors
Diastole / physiology*
Heart Diseases / complications*,  physiopathology*
Osteogenesis Imperfecta / complications*,  physiopathology*

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