The effect of losartan therapy on ventricular function in Marfan patients with haploinsufficient or dominant negative FBN1 mutations
Autor: | Aeilko H. Zwinderman, V. de Waard, Anje M. Spijkerboer, Janneke Timmermans, M. P. Van Den Berg, M. Groenink, Arthur J.H.A. Scholte, A.W. den Hartog, Gerard Pals, Romy Franken, B.J.M. Mulder |
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Přispěvatelé: | Graduate School, Cardiology, APH - Amsterdam Public Health, Epidemiology and Data Science, ACS - Amsterdam Cardiovascular Sciences, Medical Biochemistry, Other Research, Radiology and Nuclear Medicine, Cardiovascular Centre (CVC), Surgery, RS: FHML non-thematic output, MUMC+: MA Heelkunde (9) |
Jazyk: | angličtina |
Rok vydání: | 2016 |
Předmět: |
0301 basic medicine
Marfan syndrome musculoskeletal diseases medicine.medical_specialty DIMENSIONS congenital hereditary and neonatal diseases and abnormalities Ventricular volumes Vascular damage Radboud Institute for Health Sciences [Radboudumc 16] Cardiomyopathy 030204 cardiovascular system & hematology PRESSURE Asymptomatic Losartan 03 medical and health sciences 0302 clinical medicine Cardiac magnetic resonance imaging Fibrosis Internal medicine Original Article – Point of View medicine FIBROSIS cardiovascular diseases skin and connective tissue diseases Ventricular function CARDIOMYOPATHY medicine.diagnostic_test business.industry ADULTS medicine.disease DYSFUNCTION CLINICAL PHENOTYPES MICE 030104 developmental biology Cardiology cardiovascular system TRIAL FBN1 mutation classes medicine.symptom Cardiology and Cardiovascular Medicine business Haploinsufficiency medicine.drug |
Zdroj: | Netherlands heart journal, 24(11), 675-681. Bohn Stafleu van Loghum Netherlands Heart Journal, 24(11), 675-681 Netherlands Heart Journal, 24(11), 675-681. Bohn, Stafleu, Van Loghum Netherlands Heart Journal, 24(11), 675-681. Bohn Stafleu van Loghum Netherlands Heart Journal Netherlands Heart Journal, 24, 11, pp. 675-681 Netherlands Heart Journal, 24, 675-681 |
ISSN: | 1568-5888 |
Popis: | Contains fulltext : 171721.pdf (Publisher’s version ) (Open Access) BACKGROUND: Mild biventricular dysfunction is often present in patients with Marfan syndrome. Losartan has been shown to reduce aortic dilatation in patients with Marfan syndrome. This study assesses the effect of losartan on ventricular volume and function in genetically classified subgroups of asymptomatic Marfan patients without significant valvular regurgitation. METHODS: In this predefined substudy of the COMPARE study, Marfan patients were classified based on the effect of their FBN1 mutation on fibrillin-1 protein, categorised as haploinsufficient or dominant negative. Patients were randomised to a daily dose of losartan 100 mg or no additional treatment. Ventricular volumes and function were measured by magnetic resonance imaging at baseline and after 3 years of follow-up. RESULTS: Changes in biventricular dimensions were assessed in 163 Marfan patients (48 % female; mean age 38 +/- 13 years). In patients with a haploinsufficient FBN1 mutation (n = 43), losartan therapy (n = 19) increased both biventricular end diastolic volume (EDV) and stroke volume (SV) when compared with no additional losartan (n = 24): left ventricular EDV: 9 +/- 26 ml vs. -8 +/- 24 ml, p = 0.035 and right ventricular EDV 12 +/- 23 ml vs. -18 +/- 24 ml; p < 0.001 and for left ventricle SV: 6 +/- 16 ml vs. -8 +/- 17 ml; p = 0.009 and right ventricle SV: 8 +/- 16 ml vs. -7 +/- 19 ml; p = 0.009, respectively. No effect was observed in patients with a dominant negative FBN1 mutation (n = 92), or without an FBN1 mutation (n = 28). CONCLUSION: Losartan therapy in haploinsufficient Marfan patients increases biventricular end diastolic volume and stroke volume, furthermore, losartan also appears to ameliorate biventricular filling properties. |
Databáze: | OpenAIRE |
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