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An update of medical care in Marfan syndrome

Hsin-Hui Chiu1

  • 1Department of Pediatrics, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei, Taiwan.

Tzu Chi Medical Journal
|March 2, 2022
PubMed

Insights

Marfan syndrome (MFS) treatment focuses on managing aortic root dilatation. Current medical therapies significantly improve life expectancy for MFS patients, bringing it closer to the general population.

Area of Science:

  • Cardiovascular Medicine
  • Genetics
  • Rheumatology

Background:

  • Marfan syndrome (MFS) is a genetic connective tissue disorder caused by fibrillin-1 gene mutations.
  • It follows an autosomal dominant inheritance pattern, affecting multiple body systems.
  • Aortic root aneurysm and dissection are the primary causes of mortality in MFS patients.

Purpose of the Study:

  • To review and update current medical treatments for Marfan syndrome.
  • To discuss therapeutic strategies aimed at reducing aortic wall structural changes.
  • To explore treatments that slow aortic root dilatation in MFS patients.

Main Methods:

  • Review of current medical literature on Marfan syndrome treatments.
  • Analysis of studies investigating various pharmacological interventions.
  • Synthesis of data on the efficacy of different drug classes.

Main Results:

  • Several medications, including ARBs, beta-blockers, ACE inhibitors, calcium channel blockers, tetracyclines, and statins, have been investigated.
  • Medical advancements offer new insights into MFS pathogenesis and treatment options.
  • Proper treatment has led to significant improvements in life expectancy for MFS patients.

Conclusions:

  • Medical treatments for Marfan syndrome have advanced significantly.
  • Current therapeutic approaches improve patient outcomes and life expectancy.
  • Continued research offers hope for further advancements in MFS management.
Keywords:
Angiotensin II receptor blockerAngiotensin-converting enzyme inhibitorAortic dissectionBeta-blockersMarfan syndrome

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