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Aortic Branch Aneurysms and Vascular Risk in Patients With Marfan Syndrome

Angela Lopez-Sainz1, Laia Mila2, Jose Rodriguez-Palomares2

  • 1Department of Cardiology, Vall d'Hebron Hospital Universitari, Barcelona, Spain; Grupo de Enfermedades Cardiovasculares, Vall d'Hebron Institut de Recerca (VHIR), Barcelona, Spain.

Journal of the American College of Cardiology
|June 18, 2021
PubMed
Abstract

Insights

Aortic branch aneurysms affect over a quarter of Marfan syndrome patients and are linked to age and aortic dilation. These aneurysms independently predict the need for aortic surgery in MFS patients.

Area of Science:

  • Cardiovascular Medicine
  • Genetics
  • Radiology

Background:

  • Aortic branch aneurysms are not currently diagnostic criteria for Marfan syndrome (MFS).
  • The prevalence and prognostic significance of these aneurysms in MFS are largely unknown.

Purpose of the Study:

  • To determine the prevalence of aortic branch aneurysms in patients with Marfan syndrome.
  • To investigate the relationship between aortic branch aneurysms and aortic prognosis in MFS.

Main Methods:

  • Included 187 MFS patients with a pathogenic FBN1 variant and vascular imaging of aortic branches.
  • Recorded aortic events and aneurysm complication-related events during follow-up.
  • Utilized multivariable Cox analysis for prognostic assessment.

Main Results:

  • Aortic branch aneurysms were found in 26.7% of MFS patients (104 aneurysms in 50 individuals).
  • Patients with aneurysms were older, had a more dilated aortic root, and higher rates of dyslipidemia.
  • Aneurysms independently predicted the need for aortic surgery (HR: 3.4; 95% CI: 1.1-10.3).

Conclusions:

  • Aortic branch aneurysms are common in MFS and associated with increased aortic surgery risk.
  • Whole-body vascular assessment is recommended for MFS patients to identify at-risk individuals and aggressive disease patterns.
Keywords:
Marfan syndromeaneurysmaortaaortic branches

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