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Increased visceral arterial tortuosity in Marfan syndrome

Bence Ágg1,2,3, Bálint Szilveszter4, Noémi Daradics5,6

  • 1Heart and Vascular Center, Semmelweis University, Városmajor u. 68, Budapest, H-1122, Hungary. agg.bence@med.semmelweis-univ.hu.

Orphanet Journal of Rare Diseases
|April 16, 2020
PubMed
Abstract

Insights

Visceral arterial tortuosity is increased in Marfan syndrome (MFS) patients. This finding suggests that tortuosity of splenic and renal arteries may serve as a novel predictor for serious MFS complications.

Area of Science:

  • Cardiovascular Imaging and Diagnostics
  • Genetics and Rare Diseases
  • Vascular Biology

Background:

  • Clinical risk stratification for aortic dissection in Marfan syndrome (MFS) requires improvement.
  • Current methods for assessing MFS complications lack sufficient reliability.

Purpose of the Study:

  • To investigate the potential role of visceral arterial tortuosity in MFS risk stratification.
  • To explore visceral artery changes as novel biomarkers for MFS severity.

Main Methods:

  • CT angiography was used to segment splenic and renal arteries in 37 MFS patients and 74 controls.
  • Tortuosity was quantified using distance metric (DM), sum of angles metric (SOAM), and inflection count metric (ICM).
  • The ratio of ICM to SOAM (ICM/SOAM) was calculated to characterize tortuosity patterns.

Main Results:

  • MFS patients exhibited significantly higher DM in splenic, right renal, and left renal arteries compared to controls.
  • The ICM/SOAM ratio was significantly elevated in MFS patients across all three visceral arteries.
  • Increased renal artery DM and ICM/SOAM were observed in MFS patients who underwent aortic root replacement.

Conclusions:

  • This study provides the first evidence of increased visceral arterial tortuosity in MFS.
  • Visceral arterial tortuosity, characterized by lower frequency, higher amplitude curves, may be a new predictor for severe MFS manifestations.
  • Arterial tortuosity measurements offer a potential new avenue for MFS risk stratification and management.
Keywords:
Arterial tortuosityMarfan syndromeRisk stratificationVisceral arteries

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