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Ageing in Marfan syndrome

A Hasan1, J Poloniecki, A Child

  • 1Department of Cardiological Sciences, St. George's, University of London, London, UK.

International Journal of Clinical Practice
|July 14, 2007
PubMed
Abstract

Insights

Older adults with Marfan syndrome (MFS) face increased risks of eye and heart complications, including retinal detachment and cardiovascular issues. Early medical intervention is crucial due to the condition's degenerative nature.

Area of Science:

  • Genetics
  • Connective Tissue Disorders
  • Geriatric Medicine

Background:

  • Marfan syndrome (MFS) is a genetic disorder affecting connective tissue.
  • Historically, research focused on younger MFS patients due to reduced lifespan.
  • Older MFS survivors present unique challenges and require specific medical attention.

Purpose of the Study:

  • To investigate the spectrum of medical complications in older adults (50+ years) with Marfan syndrome.
  • To identify ocular, cardiovascular, and musculoskeletal issues in aging MFS patients.
  • To compare the health status of older MFS patients with age- and sex-matched controls.

Main Methods:

  • Analysis of 156 questionnaires from patients aged 50+ in the National Marfan Syndrome Clinical Database.
  • Inclusion of 60 eligible MFS patients (median age 57) and 56 controls.
  • Collection of data on medical history, including eye, heart, and musculoskeletal conditions, via patient and control questionnaires.

Main Results:

  • Patients over 50 with MFS showed a higher risk of retinal detachment (24%) and cardiovascular abnormalities (42% post-aortic surgery, 53% palpitations).
  • Cataract was a notable complication, reported by 27% of MFS patients.
  • The study highlights lesser-known complications in older MFS populations.

Conclusions:

  • Medical practitioners must recognize the diverse complications in aging MFS patients beyond typical aging.
  • Proactive investigation and tailored therapies are essential for MFS patients over 50.
  • The degenerative nature of Marfan syndrome necessitates earlier intervention compared to the general aging population.

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