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Coronary Artery Aneurysms in Patients With Marfan Syndrome: Frequent, Progressive, and Relevant

Elisabetta Mariucci1, Lisa Bonori2, Luigi Lovato3

  • 1Marfan and Heritable Thoracic Aortic Disease Clinic, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy; Pediatric Cardiology and Adult Congenital Heart Disease Program, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.

The Canadian Journal of Cardiology
|March 12, 2021
PubMed

Insights

Coronary artery aneurysms (CAAs) are common in adult Marfan syndrome (MFS) patients, especially after aortic root replacement. Regular monitoring of coronary artery size is recommended for those with severe aortic disease.

Area of Science:

  • Cardiology
  • Genetics
  • Vascular Medicine

Background:

  • Limited data exists on coronary artery aneurysms (CAAs) prevalence and outcomes in adult Marfan syndrome (MFS).
  • Understanding CAA in MFS is crucial due to potential cardiovascular complications.

Purpose of the Study:

  • To determine the prevalence of CAAs in adult MFS patients with FBN1 gene variants.
  • To identify clinical factors associated with CAA development in this population.

Main Methods:

  • Retrospective cohort study of 109 adult MFS patients with pathogenic FBN1 variants.
  • Coronary artery diameters (LMCA, RCA) measured via computed tomography angiography.
  • Analysis of clinical data and follow-up for cardiovascular events.

Main Results:

  • Overall CAA prevalence was 46%, higher in patients with prior aortic root replacement (68%) versus native root (18%).
  • Factors associated with CAA included prior aortic dissection/intervention, longer time post-aortic root replacement, higher systemic score, mitral valve disease, and diffuse aortic disease.
  • Four patients developed pseudoaneurysms of coronary anastomoses requiring surgery during follow-up.

Conclusions:

  • CAAs are prevalent in adult MFS patients and linked to a more severe aortic phenotype.
  • Regular monitoring of coronary artery size is advised, particularly post-aortic root replacement and in severe phenotypes.
  • Further multicenter studies are needed to establish optimal surveillance strategies.
Abstract

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