The effect of beta-blockers therapy on progression of thoracic aortic dilatation in the young Marfan syndrome patients: Difference between subtypes of FBN1 gene mutation - 14/08/18
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Résumé |
Background |
Mutation in the FBN1 gene was common in the Marfan syndrome (MFS), and caused aortic dilatation that could be delayed by beta-blocker therapy.
Aims |
This study evaluated the effect of beta-blockers therapy on aortic dilatation in the MFS patients with subtypes of FBN1 gene mutation.
Methods |
A single center observational prospective study included 53 MFS patients less than 20-years-old with predicting-happlo insufficiency (PTC) or dominant-negative (DN) mutation of FBN1 gene. Among 41 patients used beta-blockers therapy, aortic diameter and aortic dilatation rate were evaluated by echocardiography.
Results |
At the baseline, dimension of aortic sinus of Valsalva of PTC-MFS patients and DN-MFS patients were in the upper bound of normal range (Z-score: 1.76±1.25 vs. 1.69±0.87, P=0.621, respectively). While, dimension of ascending aorta was in the normal range. After a mean follow-up 1.5±0.71 years with beta-blockers therapy, the dilatation rate at aortic sinus of Valsalva decreased [PTC-MFS: −0.28±0.85mm/m2.year (95% CI: −0.82; 0.26) vs. DN-MFS: −0.32±1.34mm/m2.year (95% CI: −0.91; 1.34), P=0.069]. The dilatation rate at ascending aorta decreased in PTC-MFS patients [−0.18±1.19mm/m2.year (95% CI: −0.96; 0.61)] and increased in DN-MFS patients [0.4±1.82mm/m2.year (95% CI: −0.43; 1.23)], P=0.554.
Conclusion |
Among the FBN1-MFS patients, beta-blockers therapy was effective to reduce the aortic dilatation rate, of which difference between PTC-MFS patients and DN-MFS patients was not significant.
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Vol 10 - N° 3-4
P. 287-288 - septembre 2018 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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