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Open AccessArticle

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

1
Department of Radiology and Nucleair Imaging, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands
2
Department of Cardiology, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands
3
Department of Clinical Genetics, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands
4
Center for Medical Genetics, Antwerp University Hospital & University of Antwerp, Antwerp 2000, Belgium
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work.
J. Clin. Med. 2019, 8(11), 1848; https://doi.org/10.3390/jcm8111848
Received: 22 September 2019 / Revised: 24 October 2019 / Accepted: 28 October 2019 / Published: 2 November 2019
Objective: Marfan syndrome (MFS) is a connective tissue disorder associated with severe cardiovascular morbidity and mortality. It is unknown if aorta complications in MFS are associated with progressive pulmonary artery (PA) dilatation. Methods: We measured the PA diameter on routine magnetic resonance imaging in a population of MFS patients seen in our specialised centre with follow up of diameters as well as the outcome. Results: PA dilatation was defined as an increase in diameter of 2 mm or more, and 71 patients (44%) of our total cohort (n = 162) met this criterion; mean follow up between two scans was 8.6 years (standard deviation (SD) ± 2.7 years). Furthermore, 28 patients suffered from dissections, of which 14 had a type A dissection, 10 had a type B dissection, and 4 patients suffered from both. Of those who suffered from dissection, 64% (18 out of 28) had a dilatation of the PA, versus 39% (53 out of 134) in the patient group without a dissection (p < 0.05). There was a significant association between type B dissection and descending aorta diameter (OR 1.14; 95% CI 1.05–1.24 p < 0.01) and PA dilatation (OR 1.69; 95% CI 1.03–2.77 p = 0.04). In the multivariable analysis the final model for type B dissection, only systolic blood pressure (OR 1.06; 95% CI 1.01–1.11 p = 0.02) and PA dilatation were statistically significant (OR 1.85; 95% CI 1.10–3.12 p = 0.02) while descending aorta diameter was not. Conclusions: We report an association between progressive PA dilatation and type B dissection. Our findings encourage a renewed interest in PA dimensions in MFS. View Full-Text
Keywords: aorta; Marfan; dissection; diagnosis aorta; Marfan; dissection; diagnosis
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Brouwer, C.; Bulut, H.; van Gemert, W.; Staal, A.H.; Cortenbach, K.; Snoeren, M.; Nijveldt, R.; Duijnhouwer, A.; Loeys, B.L.; van Royen, N.; Timmermans, J.; van Kimmenade, R.R. Progressive Pulmonary Artery Dilatation is Associated with Type B Aortic Dissection in Patients with Marfan Syndrome. J. Clin. Med. 2019, 8, 1848.

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