Supplemental material for Riociguat treatment for portopulmonary hypertension: a subgroup analysis from the PATENT-1/-2 studies

<p>Supplemental material for Riociguat treatment for portopulmonary hypertension: a subgroup analysis from the PATENT-1/-2 studies by Rodrigo Cartin-Ceba, Michael Halank, Hossein-Ardeschir Ghofrani, Marc Humbert, John Mattson, Arno Fritsch and Michael Krowka in Pulmonary Circulation</p

Additional file 1: Figure S1. of Survival with sildenafil and inhaled iloprost in a cohort with pulmonary hypertension: an observational study

Kaplan–Meier plots of cumulative transplant-free survival in patients with pulmonary arterial hypertension associated with collagen-vascular disease, idiopathic pulmonary arterial hypertension, and pulmonary arterial hypertension associated with systemic-to-pulmonary shunt. Data are shown for patients who were treated with iloprost followed by addition of sildenafil (iloprost/sildenafil) or sildenafil followed by addition of iloprost (sildenafil/iloprost). (PDF 853 kb

Association between six-minute walk distance and long-term outcomes in patients with pulmonary arterial hypertension: Data from the randomized SERAPHIN trial

<div><p>Background</p><p>Patients with pulmonary arterial hypertension who achieve a six-minute walk distance of 380–440 m may have improved prognosis. Using the randomized controlled trial of macitentan in pulmonary arterial hypertension (SERAPHIN), the association between six-minute walk distance and long-term outcomes was explored.</p><p>Methods</p><p>Patients with six-minute walk distance data at Month 6 were dichotomized as above or below the median six-minute walk distance (400 m) and assessed for future risk of pulmonary arterial hypertension-related death or hospitalization and all-cause death. Additionally, six-minute walk distance values at baseline, Month 6 and the change from baseline to Month 6 were categorized by quartiles. All associations were analyzed by the Kaplan–Meier method using a log-rank test and Cox regression models.</p><p>Results</p><p>Patients with a six-minute walk distance >400 m vs. ≤400 m at Month 6 have a reduced risk of pulmonary arterial hypertension-related death or hospitalization (hazard ratio 0.48; 95% confidence interval 0.33–0.69). The risk was also lower for patients with higher quartiles of six-minute walk distance at baseline or Month 6 (baseline: hazard ratio [Q4 (>430 m) vs. Q1 (≤300 m)] 0.23; 95% confidence interval 0.15–0.36; Month 6: hazard ratio [Q4 (>455 m) vs. Q1 (≤348 m)] 0.33; 95% confidence interval 0.19–0.55). In contrast, six-minute walk distance changes at Month 6 were not associated with the risk of pulmonary arterial hypertension-related death or hospitalization (<i>p</i> = 0.477). These findings were consistent when adjusted for known confounders. Similar results were observed for the risk of all-cause death up to end of study.</p><p>Conclusions</p><p>Patients with pulmonary arterial hypertension walking >400 m had better long-term prognosis. Although changes in six-minute walk distance were not associated with long-term outcomes, assessing absolute six-minute walk distance values remains important in the clinical management of patients with pulmonary arterial hypertension.</p></div

Association between 6MWD and time to all-cause death up to end of study.

<p>Association between 6MWD and time to all-cause death up to end of study.</p

Association between change in 6MWD from baseline to Month 6 (by quartiles) and long-term outcomes in patients with PAH: A. PAH-related death or hospitalization up to end of treatment; B. All-cause death up to end of study.

<p>6MWD, six-minute walk distance; K-M, Kaplan–Meier; PAH, pulmonary arterial hypertension.</p

Association between absolute 6MWD quartiles at baseline and long-term outcomes in patients with PAH: A. PAH-related death or hospitalization up to end of treatment; B. All-cause death up to end of study.

<p>6MWD, six-minute walk distance; K-M, Kaplan–Meier; PAH, pulmonary arterial hypertension.</p

Association between absolute 6MWD quartiles at Month 6 and long-term outcomes in patients with PAH: A. PAH-related death or hospitalization up to end of treatment; B. All-cause death up to end of study.

<p>6MWD, six-minute walk distance; K-M, Kaplan–Meier; PAH, pulmonary arterial hypertension.</p

Association between absolute 6MWD applying a cut-off of 400 m at Month 6 and long-term outcomes in patients with PAH: A. PAH-related death or hospitalization up to end of treatment; B. All-cause death up to end of study.

<p>6MWD, six-minute walk distance; K-M, Kaplan–Meier; PAH, pulmonary arterial hypertension.</p