Cardiac rehabilitation improved exercise capacity in 42.8% of Marfan syndrome patients, with ≥50% adherence linked to VO₂ increase ≥1 ml/kg/min.
Does a personalized cardiac rehabilitation program improve exercise capacity and cardiocirculatory parameters in adult patients with Marfan syndrome?
A personalized cardiac rehabilitation program is feasible and safe in patients with Marfan syndrome, leading to improvements in exercise capacity that are associated with adherence to the prescribed regimen.
Absolute Event Rate: 0% vs 0%
Abstract Introduction Marfan syndrome (MFS) is a disease with multisystemic involvement (cardiovascular, pulmonary, and musculoskeletal), with reduction in exercise capacity (EC). Although the benefits of cardiac rehabilitation programs (CRP) are widely demonstrated in different cardiovascular diseases, the beneficial effect of CRP In patients with MFS is unknown. Purpose to evaluate, through cardiopulmonary exercise testing (CPET), the change in EC and cardiocirculatory parameters in patients with MFS enrolled in a CRP. Methods Patients with MFS, aged ≥18 years, aortic root (AR) diameter ≤45 mm, no prior history of acute aortic syndrome, and no more than moderate aortic or mitral valve disease were included. A basal functional assessment by CPET was performed (ramp cycle ergometer protocol, 15W/minute increase). A personalized training program with 54 sessions (3 sessions/week) of both aerobic endurance and strength training of respiratory and peripheral muscles exercises was prescribed. After completing CRP, a second CPET as per protocol was performed. Reduced EC was defined as a pVO280%, the pattern of this reduction (cardiocirculatory, ventilatory, peripheric or deconditioning) was evaluated. Furthermore, basal and post-CRP echocardiogram was performed and adherence to de CPR was assessed. Results A total of 14 patients were included, 11 (78.5%) women, with a mean age of 45.5 ± 11.4 years. 4 patients (28%) with previous AR replacement (3 David, 1 Bentall). 2 patients were treated with beta-blockers (BB), 8 with ARBs, 2 with both BB and ARBs, and 2 with no ARB nor BB. Both the basal and post-CRP LVEF was within normal range, with no statistically significant differences between the two measurements (59.9±5.2% and 58.4±4.0%, p=0.154). No statistically significant differences were observed in aortic diameter between baseline and post-CRP echocardiograms (37.8±6.1mm and 38.0±5.4mm, p=0.871). 7 patients (50%) exhibited reduced EC: 3 (42.9%) cardiocirculatory pattern, 2(28.6%) a mixed pattern, 1 (14.2%) respiratory pattern, 1(14.2%) deconditioning pattern. In this group, 4 patients (42.8%) showed an improvement in EC after CRP: 2 patients with a normalization of EC, 1 patient with a normalization of cardiocirculatory parameters (VO2VT1 and OUES) and an improvement of VO2 (2.6ml/Kg/min increase) but persisting with decreased EC with deconditioning pattern, and 1 patient presented an increase of ≥1ml/Kg/min of VO2. Improvement in EC (VO₂ increase ≥1 ml/kg/min) was associated with and adherence of at least 50% of the prescribed exercise program, including both aerobic and strength exercises. (Table 1, Figure 1). Conclusions Exercise capacity impairment is frequent in MFS, predominantly attributed to a cardiocirculatory inefficiency despite the absence of severe ventricular or valvular disfunction. A cardiac rehabilitation program is feasible and safe in these patients, showing improvements in EC, marked by the adherence to the exercise prescribed Figure 1
Molins et al. (Sat,) reported a other. Cardiac rehabilitation improved exercise capacity in 42.8% of Marfan syndrome patients, with ≥50% adherence linked to VO₂ increase ≥1 ml/kg/min.