BACKGROUND: Cardiovascular involvement in Marfan syndrome is mainly characterized by progressive dilatation of the proximal aorta. Whether left ventricular dysfunction is present in these patients is not clear at present. OBJECTIVES: Assess left ventricular function in patients with Marfan syndrome, free of significant valvular heart disease, using a combination of MRI and Tissue Doppler imaging (TDI). METHODS AND RESULTS: A total of 26 Marfan patients (mean age=32.0+/-10.9, 12 men) without significant valvular heart disease, and 26 age- and sex-matched controls were studied. Left ventricular volumes and ejection fraction were measured with magnetic resonance imaging. Systolic and diastolic function parameters were assessed using conventional echocardiography and TDI. When compared to controls, Marfan patients showed impairment of left ventricular contractile function as expressed by a reduced ejection fraction (53.5+/-9.0% vs. 59.6+/-6.7%, p=0.009), an increased end-systolic volume (36.0+/-9.5 vs. 29.5+/-6.7 ml/m(2), p=0.007), and reduced peak systolic velocities at the basal septal and lateral myocardial wall (5.2+/-1.4 vs. 6.4+/-1.3 cm/s, p=0.003 and 6.0+/-2.2 vs. 7.5+/-2.3 cm/s, p=0.03, respectively). Diastolic function was impaired with an increased deceleration time of the E wave (171+/-41 ms vs. 141+/-36 ms, p=0.006). Peak early diastolic velocity at the mitral valve annulus was significantly lower (9.6+/-2.4 cm/s vs. 11.9+/-3.3 cm/s, p=0.006). CONCLUSION: These data provide evidence for mild, but significant impairment of left ventricular systolic and diastolic function in Marfan patients, not related to valvular heart disease.
BACKGROUND:Cardiovascular involvement in Marfan syndrome is mainly characterized by progressive dilatation of the proximal aorta. Whether left ventricular dysfunction is present in these patients is not clear at present. OBJECTIVES: Assess left ventricular function in patients with Marfan syndrome, free of significant valvular heart disease, using a combination of MRI and Tissue Doppler imaging (TDI). METHODS AND RESULTS: A total of 26 Marfan patients (mean age=32.0+/-10.9, 12 men) without significant valvular heart disease, and 26 age- and sex-matched controls were studied. Left ventricular volumes and ejection fraction were measured with magnetic resonance imaging. Systolic and diastolic function parameters were assessed using conventional echocardiography and TDI. When compared to controls, Marfan patients showed impairment of left ventricular contractile function as expressed by a reduced ejection fraction (53.5+/-9.0% vs. 59.6+/-6.7%, p=0.009), an increased end-systolic volume (36.0+/-9.5 vs. 29.5+/-6.7 ml/m(2), p=0.007), and reduced peak systolic velocities at the basal septal and lateral myocardial wall (5.2+/-1.4 vs. 6.4+/-1.3 cm/s, p=0.003 and 6.0+/-2.2 vs. 7.5+/-2.3 cm/s, p=0.03, respectively). Diastolic function was impaired with an increased deceleration time of the E wave (171+/-41 ms vs. 141+/-36 ms, p=0.006). Peak early diastolic velocity at the mitral valve annulus was significantly lower (9.6+/-2.4 cm/s vs. 11.9+/-3.3 cm/s, p=0.006). CONCLUSION: These data provide evidence for mild, but significant impairment of left ventricular systolic and diastolic function in Marfan patients, not related to valvular heart disease.
Authors: Helena Kuivaniemi; Natzi Sakalihasan; Frank A Lederle; Gregory T Jones; Jean-Olivier Defraigne; Nicos Labropoulos; Victor Legrand; Jean-Baptiste Michel; Christoph Nienaber; Marc A Radermecker; John A Elefteriades Journal: Aorta (Stamford) Date: 2013-06-01
Authors: Eusebio García-Izquierdo; Vanessa Moñivas-Palomero; Alberto Forteza; Carlos Martín-López; Mario Torres-Sanabria; Xabier Cia-Mendioroz; Consuelo Olivo-Rodríguez; Sara Navarro-Rico; Andrés Sánchez-Gómez; Jesús G Mirelis; Miguel A Cavero; Susana Mingo-Santos Journal: Int J Cardiovasc Imaging Date: 2021-04-19 Impact factor: 2.357
Authors: Laurence Campens; Marjolijn Renard; Bram Trachet; Patrick Segers; Laura Muino Mosquera; Johan De Sutter; Lynn Sakai; Anne De Paepe; Julie De Backer Journal: Pediatr Res Date: 2015-06-04 Impact factor: 3.756
Authors: Nicholas B Cavanaugh; Lan Qian; Nicole M Westergaard; William J Kutschke; Ella J Born; Joseph W Turek Journal: Ann Thorac Surg Date: 2017-03-27 Impact factor: 4.330
Authors: Yonghong Zhu; Anthony O Gramolini; Mark A Walsh; Yu-Qing Zhou; Cameron Slorach; Mark K Friedberg; Jun K Takeuchi; Hui Sun; R Mark Henkelman; Peter H Backx; Andrew N Redington; David H Maclennan; Benoit G Bruneau Journal: Proc Natl Acad Sci U S A Date: 2008-03-31 Impact factor: 11.205