Association Between WHO Functional Class and Long-term Prognosis in Patients With Pulmonary Arterial Hypertension: Data From SERAPHIN, A Randomized Controlled Study of Macitentan

CHEST Journal ◽  
2013 ◽  
Vol 144 (4) ◽  
pp. 879A ◽  
Author(s):  
Rogùrio Souza ◽  
Richard Channick ◽  
Marion Delcroix ◽  
Nazzareno Galiè ◽  
Hossein Ghofrani ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
Randomized Controlled Study ◽  
Controlled Study ◽  
Randomized Controlled ◽  
Pulmonary Arterial ◽  
Long Term Prognosis

Longterm Survival Among Patients with Scleroderma-associated Pulmonary Arterial Hypertension Treated with Intravenous Epoprostenol

2009 ◽  
Vol 36 (10) ◽  
pp. 2244-2249 ◽  
Author(s):  
DAVID B. BADESCH ◽  
MICHAEL D. McGOON ◽  
ROBIN J. BARST ◽  
VICTOR F. TAPSON ◽  
LEWIS J. RUBIN ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Survival Rates ◽  
Randomized Controlled Study ◽  
Extension Study ◽  
Controlled Study ◽  
Open Label ◽  
Randomized Controlled ◽  
Pulmonary Arterial ◽  
Longterm Survival

Objective.Pulmonary arterial hypertension (PAH) remains challenging to treat, especially in association with scleroderma. We examined survival rates among patients with PAH in association with scleroderma who received epoprostenol (Flolan®) through continuous intravenous (IV) infusion in an uncontrolled open-label 3-year extension study following an initial randomized, controlled 12-week study.Methods.One hundred two patients diagnosed with PAH in association with scleroderma who received epoprostenol were included in the analyses. This included 51 PAH patients from a subject population of 56 who received epoprostenol in the randomized controlled study, and 46 patients from an initial population of 55 subjects on conventional therapy in the randomized controlled study, who received epoprostenol in the extension study. All patients in this extension study received open-label epoprostenol. Adverse events, survival, and dosing information were collected throughout the study.Results.The probabilities of survival during the first and second years for all subjects who received epoprostenol during the initial randomized controlled study or during the extension study were 0.71 and 0.52, respectively. This measure remained constant at 0.48 during the third and fourth years.Conclusion.This study reports longterm survival rates for patients with scleroderma-associated PAH treated with IV epoprostenol. Although comparisons to historical data should be made with caution, this study reports a better survival outcome than natural history data on patients with scleroderma-associated PAH.

scholarly journals Long-term outcomes in pulmonary arterial hypertension by functional class: a meta-analysis of randomized controlled trials and observational registries

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402093529
Author(s):  
Nick H. Kim ◽  
Micah Fisher ◽  
David Poch ◽  
Carol Zhao ◽  
Mehul Shah ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Randomized Controlled Trials ◽  
Controlled Trial ◽  
Functional Class ◽  
Class I ◽  
Controlled Trials ◽  
Randomized Controlled ◽  
Pulmonary Arterial

Limited data about the long-term prognosis and response to therapy in pulmonary arterial hypertension patients with World Health Organization functional class I/II symptoms are available. PubMed and Embase were searched for publications of observational registries and randomized, controlled trials in pulmonary arterial hypertension patients published between January 2001 and January 2018. Eligible registries enrolled pulmonary arterial hypertension patients ≥18 years, N > 30, and reported survival by functional class. Randomized, controlled trial inclusion criteria were pulmonary arterial hypertension patients ≥18 years, ≥6 months of treatment, and morbidity, mortality, or time to worsening as end points reported by functional class. The primary outcomes were survival for registries and clinical event rates for randomized, controlled trials. Separate random effects models were calculated for registries and randomized, controlled trials. Four randomized, controlled trials ( n = 2482) and 10 registries ( n = 6580) were included. Registries enrolled 9%–47% functional class I/II patients (the vast majority being functional class II) with various pulmonary arterial hypertension etiologies. Survival rates for functional class I/II patients at one, two, and three years were 93% (95% confidence interval (CI): 91%–95%), 86% (95% CI: 82%–89%), and 78% (95% CI: 73%–83%), respectively. The hazard ratio for the treatment effect in randomized, controlled trials overall was 0.61 (95% CI: 0.51–0.74) and 0.60 (95% CI: 0.44–0.82) for functional class I/II patients and 0.62 (95% CI: 0.49–0.78) for functional class III/IV. The calculated risk of death of 22% within three years for functional class I/II patients underlines the need for careful assessment and optimal treatment of patients with functional class I/II disease. The randomized, controlled trial analysis demonstrates that current medical therapies have a beneficial treatment effect in this population.

scholarly journals Riociguat for the treatment of pulmonary arterial hypertension: a long-term extension study (PATENT-2)

2015 ◽  
Vol 45 (5) ◽  
pp. 1303-1313 ◽  
Author(s):  
Lewis J. Rubin ◽  
Nazzareno Galiè ◽  
Friedrich Grimminger ◽  
Ekkehard Grünig ◽  
Marc Humbert ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Soluble Guanylate Cyclase ◽  
Primary Objective ◽  
Functional Class ◽  
World Health ◽  
Walking Distance ◽  
Open Label ◽  
Pulmonary Arterial

Riociguat is a soluble, guanylate cyclase stimulator, approved for pulmonary arterial hypertension. In the 12-week PATENT-1 study, riociguat was well tolerated and improved several clinically relevant end-points in patients with pulmonary arterial hypertension who were treatment naïve or had been pretreated with endothelin-receptor antagonists or prostanoids. The PATENT-2 open-label extension evaluated the long-term safety and efficacy of riociguat.Eligible patients from the PATENT-1 study received riociguat individually adjusted up to a maximum dose of 2.5 mg three times daily. The primary objective was to assess the safety and tolerability of riociguat; exploratory efficacy assessments included 6-min walking distance and World Health Organization (WHO) functional class.Overall, 396 patients entered the PATENT-2 study and 324 (82%) were ongoing at this interim analysis (March 2013). The safety profile of riociguat in PATENT-2 was similar to that observed in PATENT-1, with cases of haemoptysis and pulmonary haemorrhage also being observed in PATENT-2. Improvements in the patients', 6-min walking distance and WHO functional class observed in PATENT-1 persisted for up to 1 year in PATENT-2. In the observed population at the 1-year time point, mean±sd 6-min walking distance had changed by 51±74 m and WHO functional class had improved in 33%, stabilised in 61% and worsened in 6% of the patients versus the PATENT-1 baseline.Long-term riociguat was well tolerated in patients with pulmonary arterial hypertension, and led to sustained improvements in exercise capacity and functional capacity for up to 1 year.

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