To determine the efficacy of losartan vs. atenolol in aortic dilation progression in Marfan syndrome (MFS) patients. A phase IIIb, randomized, parallel, double-blind study was conducted in 140 MFS patients, age range: 5–60 years, with maximum aortic diameter <45 mm who received losartan (n = 70) or atenolol (n = 70). Doses were raised to a maximum of 1.4 mg/kg/day or 100 mg/day. The primary end-point was the change in aortic root and ascending aorta maximum diameter indexed by body surface area on magnetic resonance imaging after 36 months of treatment. No serious drug-related adverse effects were observed. Five patients presented aortic events during a follow-up (one in the losartan and four in the atenolol groups, P = 0.366). After 3 years of follow-up, aortic root diameter increased significantly in both groups: 1.1 mm (95% CI 0.6–1.6) in the losartan and 1.4 mm (95% CI 0.9–1.9) in the atenolol group, with aortic dilatation progression being similar in both groups: absolute difference between losartan and atenolol −0.3 mm (95% CI −1.1 to 0.4, P = 0.382) and indexed by BSA −0.5 mm/m² (95% CI −1.2 to 0.1, P = 0.092). Similarly, no significant differences were found in indexed ascending aorta diameter changes between the losartan and atenolol groups: −0.3 mm/m² (95% CI −0.8 to 0.3, P = 0.326). Among patients with MFS, the use of losartan compared with atenolol did not result in significant differences in the progression of aortic root and ascending aorta diameters over 3 years of follow-up.

Background: Pulmonary hypertension (PH) leads to exercise capacity impairment, but limited data are available on the objective evaluation of physical activity (PA) levels in these patients. Methods: We assessed PA levels using triaxial accelerometry in Spanish PH patients (n=75, 48±14 years, 65% female) and gender/age matched controls (n=107), and examined potential associations of meeting minimum international recommendations [moderate-vigorous PA (MVPA) ≥150 min/week] with survival predictors. Results: With the exception of vigorous PA (with very low values in both groups), all accelerometry data showed significant differences between patients and controls, with lower PA levels and proportion of individuals meeting minimum PA guidelines, but higher inactivity time, in the former. Notably, the odds ratio (OR) of having a “low-risk” value of 6-minute walking distance (≥464 m) or ventilatory equivalent for carbon dioxide (≤39) was higher in patients following MVPA guidelines than in their less active peers [OR =4.3, 95% confidence interval (CI), 1.6–11.6, P=0.005, and OR =4.5, 95% CI, 0.9–21.1, P=0.054]. Conclusions: Daily PA is reduced in patients with PH, often to a level that may decrease their odds of survival. Efforts should be made to promote the implementation of healthy PA habits in this patient population.

Purpose: We determined the effects of an innovative 8-wk exercise intervention (aerobic, resistance, and inspiratory muscle training) for patients with mitochondrial disease.Methods: Several end points were assessed in 12 patients (19–59 yr, 4 women) at pretraining, posttraining, and after 4-wk detraining: aerobic power, muscle strength/power and maximal inspiratory pressure (main end points), ability to perform activities of daily living, body composition, quality of life, and blood myokines (secondary end points).Results: The program was safe, with patients’ adherence being 94% ± 5%. A significant time effect was found for virtually all main end points (P≤ 0.004), indicating a training improvement. Similar findings (P≤ 0.003) were found for activities of daily living tests, total/trunk/leg lean mass, total fat mass, femoral fracture risk, and general health perception. No differences were found for blood myokines, except for an acute exertional increase in interleukin 8 at posttraining/detraining (P= 0.002) and in fatty acid binding protein 3 at detraining (P= 0.002).Conclusions: An intervention including novel exercises for mitochondrial disease patients (e.g., inspiratory muscle training) produced benefits in numerous indicators of physical capacity and induced a previously unreported shift toward a healthier body composition phenotype.