Journal of clinical medicine · 2019
Progressive Pulmonary Artery Dilatation is Associated with Type B Aortic Dissection in Patients with Marfan Syndrome.
СтатьяНазвание и аннотация: Перевод в очереди
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Brouwer Christel, Bulut Haldun, van Gemert Willemijn, Staal Alexander Hj, Cortenbach Kim, Snoeren Miranda, Nijveldt Robin, Duijnhouwer Anthonie, Loeys Bart L, van Royen Niels, Timmermans Janneke, van Kimmenade Roland Rj
Аннотация
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.
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.