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Title
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Progressive pulmonary artery dilatation is associated with type B aortic dissection in patients with Marfan syndrome
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Author
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Abstract
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| 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. |
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Language
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English
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Source (journal)
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JOURNAL OF CLINICAL MEDICINE
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Publication
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2019
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DOI
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10.3390/JCM8111848
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Volume/pages
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8
:11
(2019)
, 9 p.
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Article Reference
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1848
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ISI
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000502294400091
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Pubmed ID
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31684091
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Medium
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E-only publicatie
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Full text (Publisher's DOI)
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Full text (open access)
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