scholarly journals Long‐Term Outcomes in Patients With Connective Tissue Disease–Associated Pulmonary Arterial Hypertension in the Modern Treatment Era: Meta‐Analyses of Randomized, Controlled Trials and Observational Registries

2021 ◽  
Author(s):  
Dinesh Khanna ◽  
Carol Zhao ◽  
Rajan Saggar ◽  
Stephen C. Mathai ◽  
Lorinda Chung ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Controlled Trials ◽  
Long Term Outcomes ◽  
Randomized Controlled ◽  
Pulmonary Arterial ◽  
Modern Treatment ◽  
Meta Analyses

Long-term outcomes of domiciliary intravenous iloprost in idiopathic and connective tissue disease-associated pulmonary arterial hypertension

Respirology ◽  
2016 ◽  
Vol 22 (2) ◽  
pp. 372-377 ◽  
Author(s):  
Sheila Ramjug ◽  
Nehal Hussain ◽  
Judith Hurdman ◽  
Charlie A. Elliot ◽  
Ian Sabroe ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Long Term Outcomes ◽  
Pulmonary Arterial

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 Occult catheter rupture causing episodic symptoms in a patient treated with epoprostenol

2017 ◽  
Vol 8 (1) ◽  
pp. 204589321774805 ◽  
Author(s):  
Barbara L. LeVarge ◽  
Anica C. Law ◽  
Blanche Murphy
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Adverse Events ◽  
Arterial Hypertension ◽  
Long Term Outcomes ◽  
Pulmonary Arterial

Infection, thrombosis, and catheter dislodgment are well-recognized potential complications of chronic intravenous prostanoid therapy for pulmonary arterial hypertension. As long-term outcomes of pulmonary hypertension patients improve, novel adverse events are likely to arise. We describe the sudden development of unexplained hypotension and lightheadedness in a patient receiving intravenous epoprostenol for several years, ultimately determined to be due to an unusual catheter complication, not previously described in this population.

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