In Marfan patients, 50% had reduced exercise capacity (pVO2 <80%), mainly due to cardiocirculatory inefficiency with lower LVEF (56% vs 61%, p=0.0045).
In patients with Marfan syndrome, reduced exercise capacity is frequent and predominantly driven by cardiocirculatory inefficiency, which correlates with slightly lower left ventricular ejection fraction.
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Abstract Background Marfan syndrome (MFS) is a genetic disease with multisystemic involvement (cardiovascular, pulmonary, and musculoskeletal) affecting quality of life and limiting physical activity. However, knowledge on the decrease in exercise capacity (EC) in MFS as assessed by CPET is limited. Furthermore, the mechanism underlying EC decrease and the contribution of each component (cardiovascular, respiratory or muscular) is not well understood, with few studies quantifying or evaluating EC in these patients in an integrated manner through cardiopulmonary exercise testing (CPET). Purpose to describe the alterations in EC and their patterns through CPET in patients with MS. Methods Patients with MFS, aged ≥18 years, with an aortic root or ascending aorta diameter ≤45 mm, no prior history of acute aortic syndrome, and no more than moderate aortic or mitral valve disease were included. CPET was performed using a ramp cycle ergometer protocol, increasing 15W/minute. A reduced EC was defined as a pVO280%, the pattern of this reduction (cardiocirculatory, ventilatory, peripheric or deconditioning) was evaluated. A transthoracic echocardiogram was performed before the test. The International Physical Activity Questionnaire (IPAQ) was used to assess the level of physical activity performed before the inclusion. Results A total of 30 patients were included, 22 women (73.3%), with a mean age of 44.9 ± 11.8 years. 11 patients (36.6%) with previous aortic root replacement (9 David, 2 Bentall). Mean LVEF was 58.9% (range: 49.0–69.0%). 9 patients were treated with beta-blockers (BB), 11 with ARBs, 5 with both BB and ARBs, and 5 with no ARB nor BB. All patients achieved a maximal effort test (RER 1.1 at peak exertion or 1.09 at 2 minutes of recovery). 15 patients (50%) exhibited reduced EC (pVO₂ 80% of predicted) with a mean pVO2 of 20.2±5.6ml/min and 66.1±8.4% The mean VE/VCO₂ slope in this group was 30.9±5.6 with 8 patients with altered ventilatory class (7 (46%) in ventilatory class II and 1 (6%) in ventilatory class IV). Among the group of patients with reduced EC, 8 patients (53%) exhibited a cardiocirculatory pattern, 1 patient (6%) a respiratory pattern, 3 patients (20%) a mixed pattern, and 3 patients (20%) a deconditioning pattern. Those with a cardiocirculatory pattern had a mean LVEF of 54.0 ± 5.8%. Patients with reduced EC had a statistically lower LVEF compared to those with normal EC (56.0% vs 61.0%, p=0.0045). No differences were observed between groups regarding age, gender, baseline treatment, previous physical activity or history of aortic surgery (Table 1). Conclusions Exercise capacity impairment is frequent in Marfan Syndrome. In our study, this limitation is predominantly attributed to a cardiocirculatory inefficiency despite the absence of severe ventricular or valvular disfunction. Ventilatory inefficiency is also frequent in our cohort. Larger studies are needed to confirm the findings observed in our cohort.Table 1
Molins et al. (Sat,) reported a other. In Marfan patients, 50% had reduced exercise capacity (pVO2 <80%), mainly due to cardiocirculatory inefficiency with lower LVEF (56% vs 61%, p=0.0045).