Exercise and Quality-of-Life Outcomes of Two Versus Three Weekly Sessions of Pulmonary Rehabilitation.

PURPOSE: This study aimed to assess whether there are differences in exercise or health-related quality-of-life (HRQoL) outcomes following twice-weekly supervised sessions of pulmonary rehabilitation (PR) compared with three times weekly over an 8-wk program in patients with chronic obstructive pulmonary disease (COPD). METHODS: We conducted a quasi-experimental, single-center observational study using 198 subjects who completed two supervised PR sessions (intervention group) compared with 208 historical controls who completed three weekly sessions. We assessed between-group differences in outcomes after balancing groups using inverse probability of treatment weighting (IPTW) of propensity scores, followed by regression adjustment. RESULTS: Both groups achieved clinically and statistically significant improvements in exercise and HRQoL following the PR program. After IPTW and regression adjustment, the intervention group had a lower post-PR 6-min walk time by 1.2: 95% CI, -12.9 to 10.5 m ( P = .84), compared with the control group. Although post-PR COPD Assessment Test (CAT) scores decreased in both groups, the intervention group had a higher post-PR CAT score by 1.5: 95% CI, 0.37 to 2.66 a.u. ( P = .01), compared with the control group. All other HRQoL measures failed to reach statistical significance. None of the between-group differences reached minimal clinically important differences for COPD. CONCLUSIONS: Our findings support current international guidelines for twice-weekly supervised PR sessions combined with unsupervised home exercise sessions. We conclude there is no disadvantage in running a PR program for patients with COPD using twice-weekly supervised sessions compared with three times weekly supervised sessions.

Outcomes of pulmonary arterial hypertension therapy in Australia: is monotherapy adequate?

BACKGROUND: In Australia, government-subsidised treatment of pulmonary arterial hypertension (PAH) is limited to monotherapy. Recent international guidelines advocate that initial combination therapy be considered for all symptomatic PAH patients. AIM: To characterise 'real-life' outcomes in PAH patients initiated on monotherapy. METHODS: We performed a retrospective analysis of 100 consecutive PAH patients at a single centre who were commenced on monotherapy for PAH between 2004 and 2015. The composite clinical end-point of 'treatment failure' was prospectively defined as (i) >15% fall in 6-min walk distance (6MWD) on follow up, (ii) physician judgement of inadequate treatment response, (iii) adverse drug effect requiring cessation and (iv) death or transplantation. RESULTS: At initiation of therapy, mean age was 54 ± 18 years, and underlying diagnoses included idiopathic (36%), connective tissue disease-associated (37%) and congenital heart disease-associated-PAH (25%). Baseline 6MWD was 360 ± 140 m, and 75% were in either the New York Heart Association functional classes III or IV. Over a median follow up of 38 months (interquartile range 20-67), 62% of the subjects met the criteria for a clinical failure event. Median time to monotherapy failure was 24 months (95% confidence interval 14-34), with death or transplantation being the most common clinical failure event. Estimated 1-, 3- and 5-year survival rates from time of treatment initiation were 92, 75 and 66%. CONCLUSION: The majority of patients failed initial monotherapy therapy within 2 years of treatment initiation. Broader access to approved PAH agents is needed to enable combination therapy in line with evidence-based international guidelines.

Survival outcomes in severe congenital versus non-congenital pulmonary hypertension.

OBJECTIVE: Since 2006, our institution has cared for 70 patients with pulmonary arterial hypertension (PAH) and severe right ventricular (RV) hypertension; 44 with congenital heart disease (CHD) had Eisenmenger syndrome (ES) and 26 had PAH without CHD. We sought to determine and compare 'real-world' survival outcomes and cause of death in these two distinct groups. METHODS: An observational study of consecutive adult patients from our CHD or PAH clinics with RV systolic pressure ≥80 mm Hg (on echocardiography or right-heart catheter). Detailed patient demographics, investigations and outcomes at baseline and follow-up were reviewed. RESULTS: Patients with ES were younger than non-CHD group (39±9 vs 64±14 years, p<0.001) with a lower proportion of women (61% vs 85%; p=0.04), and higher RV systolic pressure. Estimated 1-year, 3-year and 7-year survival were not significantly different between the groups (98%, 95% and 74% ES; 100%, 92% and 63% non-CHD, p=0.52). In patients with non-CHD, poorer survival was associated with a diagnosis of connective tissue disease (HR 6.90, 95% CI 1.21 to 39.3) and number of PAH therapies (HR 2.8, 95% CI 1.03 to 7.59). Mortality was directly attributed to PAH in 75% of non-CHD group compared with 31% in patients with ES (p=0.049), many of whom died from infection or bleeding. CONCLUSIONS: We report favourable 7-year survival in adults with PAH and systemic RV pressures-equivalent in ES and non-CHD groups. Those with non-CHD predominately died of cardiac complications of PAH, as distinct from those with ES, many of whom died from complications of chronic cyanosis.