Cardiovascular involvement in osteogenesis imperfecta

J. Hortop, P. Tsipouras, J. A. Hanley, B. J. Maron, Jay Shapiro

Research output: Contribution to journalArticle

Abstract

While aortic root dilatation and valvular dysfunction have been well-documented in osteogenesis imperfecta (OI), the nature and extent of cardiovascular involvement in OI have not been clearly delineated. A clinical and echocardiographic survey involving 109 individuals with various nonlethal OI syndromes from 66 separate families was undertaken. Clinically discernible valvular dysfunction was encountered in only four of the 109 individuals (aortic regurgitation in two, aortic stenosis in one, and mitral valve prolapse in one), none of whom were related. Aortic root dilatation was recognized echocardiographically in eight (12.1%) of 66 individuals comprising a subset of the sample in which each family was represented by a single individual. The extent of the aortic root dilatation was mild (the largest dimension measuring 4.3 cm) and was unrelated to the age of the individual. Dilatation was seen in each of the different OI syndromes but was strikingly segregated within certain families (p

Original languageEnglish (US)
Pages (from-to)54-61
Number of pages8
JournalCirculation
Volume73
Issue number1
StatePublished - 1986
Externally publishedYes

Fingerprint

Osteogenesis Imperfecta
Dilatation
Mitral Valve Prolapse
Aortic Valve Insufficiency
Aortic Valve Stenosis

ASJC Scopus subject areas

  • Physiology
  • Cardiology and Cardiovascular Medicine

Cite this

Hortop, J., Tsipouras, P., Hanley, J. A., Maron, B. J., & Shapiro, J. (1986). Cardiovascular involvement in osteogenesis imperfecta. Circulation, 73(1), 54-61.

Cardiovascular involvement in osteogenesis imperfecta. / Hortop, J.; Tsipouras, P.; Hanley, J. A.; Maron, B. J.; Shapiro, Jay.

In: Circulation, Vol. 73, No. 1, 1986, p. 54-61.

Research output: Contribution to journalArticle

Hortop, J, Tsipouras, P, Hanley, JA, Maron, BJ & Shapiro, J 1986, 'Cardiovascular involvement in osteogenesis imperfecta', Circulation, vol. 73, no. 1, pp. 54-61.
Hortop J, Tsipouras P, Hanley JA, Maron BJ, Shapiro J. Cardiovascular involvement in osteogenesis imperfecta. Circulation. 1986;73(1):54-61.
Hortop, J. ; Tsipouras, P. ; Hanley, J. A. ; Maron, B. J. ; Shapiro, Jay. / Cardiovascular involvement in osteogenesis imperfecta. In: Circulation. 1986 ; Vol. 73, No. 1. pp. 54-61.
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