Impact of Parenteral Prostanoids in Pulmonary Arterial Hypertension: The Relevance of Timing (original) (raw)
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CHEST Journal, 2011
BACKGROUND: Oral drugs have made the treatment of pulmonary hypertension (PH) feasible in nonexpert centers, which could delay patient access to prostanoid therapy. METHODS: Fifty-seven consecutive patients with precapillary PH received a prostanoid in our center. Data at prostanoid initiation included modality of center referral, medical history, New York Heart Association [NYHA] class, exercise capacity, echocardiographic parameters, and hemodynamics. RESULTS: Overall survival at 1, 2, and 3 years was 85%, 69%, 55%, respectively. Non-survivors had worse NYHA class III/IV (17/12) than survivors (27/1; p Ͻ 0.01) and exercise capacity on 6-minutewalk distance (254 Ϯ 114 vs 354 Ϯ 91 meters; p Ͻ 0.01). Non-survivors were more frequently referred on oral therapy (83% vs 36%; p Ͻ 0.01) and had a higher rate of urgent prostanoid treatment (69% vs 17%; p Ͻ 0.0001). Multivariate analysis (hazard ratio [95% confidence interval]) found the independent prognostic factors were urgent prostanoid therapy (2.0 [1.1-3.9]) and NYHA class (3.5 [1.5-8.2]). Survivors had a significant response to prostanoid, improving NYHA class from 2.8 Ϯ 0.4 to 2.3 Ϯ 0.5 (p ϭ 0.002), 6-minute walk distance from 354 Ϯ 91 to 426 Ϯ 82 meters (p ϭ 0.0001), and pulmonary hemodynamics (pulmonary artery pressure from 56 Ϯ 13 to 44 Ϯ 18 mm Hg [p Ͻ 0.05]; cardiac index from 2.0 Ϯ 1.2 to 3.1 Ϯ 1.2 liters/min/m 2 [p ϭ 0.002], and pulmonary vascular resistance from 17 Ϯ 10 to 8 Ϯ 6 WU [p ϭ 0.001]). CONCLUSIONS: Referral of patients on oral treatment to a tertiary PH center is delayed and significantly affects prognosis. J Heart Lung Transplant 2012;xx:xxx
Parenteral Prostanoids in Pediatric Pulmonary Arterial Hypertension: Start Early, Dose High, Combine
Annals of the American Thoracic Society, 2022
Rationale: There are currently no data supporting specific dosing and weaning strategies for parenteral prostanoid therapy in children with pulmonary arterial hypertension (PAH). Objectives: To describe the clinical practice of intravenous (IV) or subcutaneous (SC) prostanoid therapy in pediatric PAH and identify dosing strategies associated with favorable outcome. Methods: From an international multicenter cohort of 275 children with PAH, 98 patients who received IV/SC prostanoid therapy were retrospectively analyzed. Results: IV/SC prostanoids were given as monotherapy (20%) or combined with other PAH-targeted drugs as dual (46%) or triple therapy (34%). The median time-averaged dose was 37 ng/kg/min, ranging 2-136 ng/kg/min. During follow-up, IV/SC prostanoids were discontinued and transitioned to oral or inhaled PAH-targeted therapies in 29 patients. Time-dependent receiver operating characteristic analyses showed specific hemodynamic criteria at discontinuation of IV/SC prostanoids (mean pulmonary arterial pressure , 35 mm Hg and/or pulmonary vascular resistance index , 4.4 Wood units [WU]Ám 2) identified children with favorable long-term outcome after IV/SC prostanoid discontinuation, compared with patients who do not meet those criteria (P = 0.027). In the children who continued IV/SC prostanoids until the end of follow-up, higher dose (.25 ng/kg/ min), early start after diagnosis, and combination with other PAHtargeted drugs were associated with better transplant-free survival. Conclusions: Early initiation of IV/SC prostanoids, higher doses of IV/SC prostanoids, and combination with additional PAH-targeted therapy were associated with favorable outcome. Transition from IV/SC prostanoid therapy to oral or inhaled therapies is safe in the long term in selected children, identified by reaching hemodynamic criteria for durable IV/SC prostanoid discontinuation while on IV/SC prostanoid therapy.
The American journal of managed care, 2016
Prostacyclins for the treatment of pulmonary arterial hypertension (PAH) have historically been covered under the insurance medical benefit because they require durable medical equipment and are administered by an intravenous, subcutaneous, or inhalation route. However, more treatment options that target the prostacyclin pathway have become available. As the number and type of options expand, an improved understanding of these drugs will aid managed care decision makers in evaluating new treatment options and making clinically sound and cost-effective treatment decisions. PAH is a progressive disease of pulmonary vascular remodeling that increases pulmonary vascular resistance and often results in right-side heart failure and death if left untreated. Adverse event profiles, the complexity of administration modalities, and potential complications must be considered when administering prostacyclin therapy. Traditional modes of administration, with their potential challenges and compli...
Pulmonary circulation, 2013
Transition from prostacyclin analogue infusion to oral therapy in patients with pulmonary arterial hypertension (PAH) is possible with acceptable short-and midterm results. However, there is a paucity of data on long-term outcomes after successful transition. Using a predefined protocol, transition to oral therapy was attempted in 22 patients with clinically stable PAH. Clinical and hemodynamic data were retrospectively collected at baseline as well as during and after transition. Parameters for successful versus nonsuccessful transition were also evaluated. All patients had severe PAH at baseline and showed clinical and hemodynamic improvement with prostacyclin analogue infusion. Initial oral agents used for transition were bosentan (63.6%), sildenafil (31.8%), and tadalafil (4.5%). Combination therapy was used in 68% of the patients. Successful transition was achieved in 11 patients (50%) with a mean transition duration of 16 months. After successful transition, clinical and hemodynamic parameters remained stable at midterm (mean, 18 months) and long-term (mean, 60 months) follow-up. Compared with the successful transition group, patients who experienced failure were older, had a higher frequency of idiopathic PAH, and had worse hemodynamic parameters during treatment with prostacyclin analogue alone, as well as during the transition period. In conclusion, successful transition from prostacyclin analogue infusion to oral therapy can be achieved in a significant proportion of patients with clinically stable PAH. After an initial successful transition, patients were able to maintain clinical and hemodynamic stability at the mid-and long-term follow-up.
Journal of the American College of Cardiology, 2013
Transition from prostacyclin analogue infusion to oral therapy in patients with pulmonary arterial hypertension (PAH) is possible with acceptable short-and midterm results. However, there is a paucity of data on long-term outcomes after successful transition. Using a predefined protocol, transition to oral therapy was attempted in 22 patients with clinically stable PAH. Clinical and hemodynamic data were retrospectively collected at baseline as well as during and after transition. Parameters for successful versus nonsuccessful transition were also evaluated. All patients had severe PAH at baseline and showed clinical and hemodynamic improvement with prostacyclin analogue infusion. Initial oral agents used for transition were bosentan (63.6%), sildenafil (31.8%), and tadalafil (4.5%). Combination therapy was used in 68% of the patients. Successful transition was achieved in 11 patients (50%) with a mean transition duration of 16 months. After successful transition, clinical and hemodynamic parameters remained stable at midterm (mean, 18 months) and long-term (mean, 60 months) follow-up. Compared with the successful transition group, patients who experienced failure were older, had a higher frequency of idiopathic PAH, and had worse hemodynamic parameters during treatment with prostacyclin analogue alone, as well as during the transition period. In conclusion, successful transition from prostacyclin analogue infusion to oral therapy can be achieved in a significant proportion of patients with clinically stable PAH. After an initial successful transition, patients were able to maintain clinical and hemodynamic stability at the mid-and long-term follow-up.
Objective: Limited data exists regarding Prostanoid (PGI2) use in critically ill patients with pulmonary hypertension. (PH) in the pediatric cardiac intensive care unit (CICU) setting. Materials and Methods: Single center, retrospective study of patients with diagnosis of PH who received continuous PGI2 and were admitted to CICU from January/2015 to April/2022. Data collected included patient demographics and clinical characteristics including diagnosis, etiology of PH, vasoactive and ventilatory support, length of stay, and survival. Type, initial, maximum, and final dose of PGI2 as well as hemodynamic data was obtained. Data reported as mean ± standard deviation. Significance taken p-value <0.05. Results 24 patients received PGI2 therapy at a mean age of 3.1 years, range (0-16.6 years). PGI2 was in the form of IV epoprostenol in 12 patients, IV treprostinil in 6, and SQ treprostinil in 6 patients. Mean initial dose was 2.79 ng/kg/min, max dose 18.75 ng/kg/min, and average durat...
Journal of managed care & specialty pharmacy, 2017
Prostacyclins play an important role in the management of pulmonary arterial hypertension (PAH). Intravenous prostacyclin was the first disease-specific treatment for patients with PAH. Subcutaneous and nonparenteral (oral or inhaled) formulations have subsequently become available. However, data are lacking on how these different prostacyclin formulations are being used in clinical practice. To (a) conduct retrospective analyses of a large U.S. health care claims database to describe the characteristics of patients with PAH initiating prostacyclin therapy, and (b) evaluate their treatment patterns, health care resource use, and associated costs. Truven Commercial and Medicare databases were used to define annual cohorts of adults with PAH between January 1, 2010, and October 31, 2015. These patients were identified based on claims with ICD-9-CM diagnoses indicative of PAH (codes 416.0 or 416.8) and claims for PAH-specific medications and PAH-related procedures. Patients with eviden...