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Progressive Pulmonary Artery Dilatation is Associated with Type B Aortic Dissection in Patients with Marfan Syndrome

Christel Brouwer1, Haldun Bulut2, Willemijn van Gemert3

  • 1Department of Radiology and Nucleair Imaging, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands. brouwer.christel@gmail.com.

Journal of Clinical Medicine
|November 6, 2019
PubMed
Abstract

Insights

Progressive pulmonary artery (PA) dilatation is associated with type B aortic dissection in Marfan syndrome (MFS) patients. This finding highlights the importance of monitoring PA dimensions in MFS for cardiovascular risk assessment.

Area of Science:

  • Cardiovascular Medicine
  • Genetics and Inherited Diseases
  • Radiology and Imaging

Background:

  • Marfan syndrome (MFS) is a genetic connective tissue disorder.
  • MFS significantly increases cardiovascular morbidity and mortality, particularly due to aortic complications.
  • The relationship between aortic complications and pulmonary artery (PA) dilatation in MFS is not well understood.

Purpose of the Study:

  • To investigate the association between progressive pulmonary artery (PA) dilatation and aortic complications in patients with Marfan syndrome.
  • To determine if PA dilatation is a predictor of dissection in MFS.
  • To assess the clinical significance of PA dimensions in MFS management.

Main Methods:

  • Retrospective analysis of magnetic resonance imaging (MRI) data from 162 Marfan syndrome patients.
  • Measurement of PA diameter at baseline and during follow-up (mean 8.6 years).
  • Definition of PA dilatation as a ≥2 mm increase in diameter; correlation with dissection events.

Main Results:

  • 44% (71/162) of patients exhibited progressive PA dilatation.
  • A significantly higher prevalence of PA dilatation was observed in patients with aortic dissection (64%) compared to those without (39%).
  • PA dilatation was independently associated with type B aortic dissection (OR 1.85; p=0.02), while descending aorta diameter was not.

Conclusions:

  • Progressive pulmonary artery dilatation is associated with type B aortic dissection in Marfan syndrome.
  • These findings suggest that PA dimensions should be closely monitored in MFS patients.
  • Further research into the role of PA dimensions in MFS cardiovascular risk stratification is warranted.
Keywords:
Marfanaortadiagnosisdissection

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