Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension.

BACKGROUND: Pulmonary hypertension (PH) is associated with restricted physical capacity, limited quality of life, and a poor prognosis because of right heart failure. The present study is the first prospective randomized study to evaluate the effects of exercise and respiratory training in patients with severe symptomatic PH. METHODS AND RESULTS: Thirty patients with PH (21 women; mean age, 50+/-13 years; mean pulmonary artery pressure, 50+/-15 mm Hg; mean World Health Organization [WHO] class, 2.9+/-0.5; pulmonary arterial hypertension, n=23; chronic thromboembolic PH, n=7) on stable disease-targeted medication were randomly assigned to a control (n=15) and a primary training (n=15) group. Medication remained unchanged during the study period. Primary end points were the changes from baseline to week 15 in the distance walked in 6 minutes and in scores of the Short Form Health Survey quality-of-life questionnaire. Changes in WHO functional class, Borg scale, and parameters of echocardiography and gas exchange also were assessed. At week 15, patients in the primary and secondary training groups had an improved 6-minute walking distance; the mean difference between the control and the primary training group was 111 m (95% confidence interval, 65 to 139 m; P<0.001). Exercise training was well tolerated and improved scores of quality of life, WHO functional class, peak oxygen consumption, oxygen consumption at the anaerobic threshold, and achieved workload. Systolic pulmonary artery pressure values at rest did not change significantly after 15 weeks of exercise and respiratory training (from 61+/-18 to 54+/-18 mm Hg) within the training group. CONCLUSIONS: This study indicates that respiratory and physical training could be a promising adjunct to medical treatment in severe PH. The effects add to the beneficial results of modern medical treatment.

Assessment of pulmonary artery systolic pressures by stress Doppler echocardiography after bilateral lung transplantation.

BACKGROUND: Even after successful single-lung transplantation (SLTx) or bilateral lung transplantation (BLTx), patients continue to have peripheral muscle weakness and exercise impairment. After SLTx, exercise limitation is also attributed to persistent or recurrent pulmonary vascular abnormalities with elevated pulmonary arterial pressures at rest or during exercise. Therefore, the aim of this study was to evaluate systolic pulmonary artery pressures (PASPs) at rest and during supine bicycle exercise, exercise capacity and cardiopulmonary function in post-BLTx patients. METHODS: Nine patients underwent BLTx due to end-stage pulmonary arterial hypertension (PAH) and 37 age- and gender-matched control subjects underwent a physical examination, electrocardiographic (ECG) test, a 6-minute walk test, a lung function test, a cardiopulmonary exercise test and echocardiographic assessment at rest and during exercise. RESULTS: Exercise capacity was significantly reduced in the BLTx group, with an impaired 6-minute walk test and maximal oxygen uptake and workload. Ventilatory factors did not appear to limit exercise capacity. Right and left ventricular size and pump function and PASP values at rest were normal in both groups, but exceeded 40 mm Hg in 3 of 9 BLTx recipients and in 1 of 37 controls during exercise at low workloads. Mean PASP during exercise was only slightly higher in the BLTx group (40 +/- 5 vs 36 +/- 4 mm Hg, p = not statistically significant). CONCLUSIONS: Reduced exercise capacity of patients after BLTx due to end-stage pulmonary hypertension is not attributed to persistent or recurrent manifest pulmonary hypertension or cardiopulmonary dysfunction. Nevertheless, latent pulmonary hypertension with exaggerated pulmonary artery pressures during exercise may occur in some patients.