Cardiovascular Involvement in Children with Osteogenesis Imperfecta

Homa  Ilkhanipoor; Hamdollah Karamifar; Gholamhossein Ajami; Zohreh Karamizadeh; Gholamhossein Amirhakimi; Ali-Mohammad Shakiba

Innovative Journal of Pediatrics

The Scientific Journal of Growth & Development Research Center

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Cardiovascular Involvement in Children with Osteogenesis Imperfecta

Author(s):

Homa Ilkhanipoor^1,*,

Hamdollah Karamifar²,

Gholamhossein Ajami³,

Zohreh Karamizadeh⁴,

Gholamhossein Amirhakimi⁵,

Ali-Mohammad Shakiba⁶

Innovative Journal of Pediatrics:Vol. 23, issue 5; 513-518

Published online:Jul 14, 2013

Article type:Research Article

Received:Dec 15, 2012

Accepted:Jun 04, 2013

How to Cite:Ilkhanipoor H, Karamifar H, Ajami G, Karamizadeh Z, Amirhakimi G, et al. Cardiovascular Involvement in Children with Osteogenesis Imperfecta. Inn J Pediatr. 2013;23(5):. doi:

Abstract

Objective: Osteogenesis imperfecta is a hereditary disease resulting from mutation in type I procollagen genes. One of the extra skeletal manifestations of this disease is cardiac involvement. The prevalence of cardiac involvement is still unknown in the children with osteogenesis imperfecta. The present study aimed to investigate the prevalence of cardiovascular abnormalities in these patients. Methods: 24 children with osteogenesis imperfecta and 24 normal children who were matched with the patients regarding sex and age were studied. In both groups, standard echocardiography was performed, and heart valves were investigated. Dimensions of left ventricle, aorta annulus, sinotubular junction, ascending and descending aorta were measured and compared between the two groups. Findings: The results revealed no significant difference between the two groups regarding age, sex, ejection fraction, shortening fraction, mean of aorta annulus, sinotubular junction, ascending and descending aorta, but after correction based on the body surface area, dimensions of aorta annulus, sinotubular junction, ascending and descending aorta in the patients were significantly higher than those in the control group (P<0.05). Two (8.3%) patients had aortic insufficiency and five (20%) patients had tricuspid regurgitation, three of whom had gradient >25 mmHg and one patient had pulmonary insufficiency with indirect evidence of pulmonary hypertension. According to Z scores of aorta annulus, sinotubular junction and ascending aorta, 5, 3, and 1 out of 24 patients had Z scores >2 respectively. Conclusion: The prevalence of valvular heart diseases and aortic root dilation was higher in children with osteogenesis imperfecta. In conclusion, cardiovascular investigation is recommended in these children.

Keywords

Osteogenesis Imperfecta

Cardiovascular Abnormalities

Heart Valve Diseases

Echocardiography

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Author(s):

Homa Ilkhanipoor:[PubMed][Scholar]
Hamdollah Karamifar:[PubMed][Scholar]
Gholamhossein Ajami:[PubMed][Scholar]
Zohreh Karamizadeh:[PubMed][Scholar]
Gholamhossein Amirhakimi:[PubMed][Scholar]
Ali-Mohammad Shakiba:[PubMed][Scholar]

The Scientific Journal of Growth & Development Research Center

Outlines

Cardiovascular Involvement in Children with Osteogenesis Imperfecta

Abstract

Fulltext

References

Last Update: 1 month ago

Last Update: 1 month ago

Last Update: 1 month ago

Last Update: 6 days ago