Marfan's syndrome: a family affair

David Strider, Terri Moore, Jane Guarini, Beth Fallin, Jan Ivey, Irving Kron

Research output: Contribution to journalArticle

3 Scopus citations

Abstract

Marfan's syndrome (MFS), a heritable connective tissue disorder, may result in cardiac valvular insufficiency, aortic aneurysm or dissection, dislocated lens, and musculoskeletal abnormalities. During a 20-month period (1994-96), an interdisciplinary health care team at a central Virginia medical center evaluated the histories of 112 persons from 15 different families for the presence of MFS-related traits. Seventy-five had at least one MFS-related trait, and 27 subjects underwent echocardiography to evaluate for aortic root dilatation and valvular lesions. Forty-three patients (57.3%) in the above cohort demonstrated significant cardiovascular lesions, with 20 undergoing cardiac surgery. Thirty-one patients (41.3%) were initially seen with significant ocular lesions, and 38 (50.7%) displayed orthopedic deformities. The health care team developed strategies for long-term management of persons with MFS, including antihypertensive therapy, periodic testing, risk-factor modification, genetic counseling, and surgery for appropriate patients. Proactive, consistent management of MFS families will improve long-term health outcomes for this patient population.

Original languageEnglish (US)
Pages (from-to)91-98
Number of pages8
JournalJournal of Vascular Nursing
Volume14
Issue number4
DOIs
StatePublished - Dec 1 1996
Externally publishedYes

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ASJC Scopus subject areas

  • Medical–Surgical

Cite this

Strider, D., Moore, T., Guarini, J., Fallin, B., Ivey, J., & Kron, I. (1996). Marfan's syndrome: a family affair. Journal of Vascular Nursing, 14(4), 91-98. https://doi.org/10.1016/S1062-0303(96)80024-0