Aortic displacement and hemodynamics are abnormal in patients with Marfan syndrome: A combined four-dimensional

D Bosshardt1, R Merton2, A J Nederveen2

  • 1Radiology and Nuclear Medicine, Amsterdam University Medical Center, Amsterdam, the Netherlands; Amsterdam Cardiovascular Sciences, Amsterdam, the Netherlands; Cardiology, Amsterdam University Medical Center, Amsterdam, the Netherlands.

Abstract

Insights

Marfan syndrome patients with aortic root surgery show abnormal aortic displacement and wall shear stress, particularly in the proximal descending aorta, increasing dissection risk. These findings highlight distinct aortic changes in ARS MFS patients.

Area of Science:

  • Cardiovascular Imaging
  • Biomedical Engineering
  • Medical Physics

Background:

  • Marfan syndrome (MFS) patients with prior aortic root surgery (ARS) face elevated risks of type B aortic dissection.
  • This increased risk may stem from noncompliant aortic grafts failing to absorb systolic forces, leading to undampened flow in the proximal descending aorta (pDAo).

Purpose of the Study:

  • To investigate abnormal aortic displacement and wall shear stress (WSS) in MFS patients with and without ARS using cardiac magnetic resonance imaging (CMR).
  • To identify the magnitude and location of these hemodynamic abnormalities, particularly in regions susceptible to dissection.

Main Methods:

  • Eighty-two MFS patients (32 with ARS) and 45 controls underwent 4D high-resolution CINE and flow CMR.
  • Automated segmentation was used to calculate peak displacement and WSS, with heatmaps visualizing abnormal regions.
  • Surface areas of abnormal displacement and WSS were quantified in four aortic regions.

Main Results:

  • ARS MFS patients exhibited higher WSS in the proximal and distal ascending aorta compared to native MFS patients.
  • Significantly larger areas of decreased displacement and increased WSS were observed in the proximal ascending aorta of ARS MFS patients.
  • A trend towards increased displacement in the pDAo of ARS MFS patients was noted, correlating positively with pDAo WSS.

Conclusions:

  • Marfan syndrome patients, with or without ARS, display abnormal aortic displacement and flow dynamics.
  • ARS MFS patients show unique hemodynamic features in the ascending aorta (AAo) and descending aorta (DAo), especially the pDAo.
  • These distinct pDAo abnormalities in ARS MFS patients may predispose them to type B aortic dissections.