Upfront Combination Therapy for Pulmonary Arterial Hypertension: Time to Be More Ambitious than AMBITION

Cascino, Thomas; McLaughlin, Vallerie V.

doi:10.1164/rccm.202107-1625ed

Cited by 10 publications

(2 citation statements)

References 12 publications

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“…The most recent society-endorsed treatment algorithm utilizes multiparametric risk stratification and proposes upfront triple combination therapy, including a parenteral prostacyclin, in high-risk patients [ 15 ]. Further, recent observational studies suggest that early triple therapy in intermediate-risk patients may be beneficial as well, providing a possible mortality benefit as well as the best chance at reverse RV remodeling [ 16 , 17 , 18 , 19 ]. Although the current treatment algorithm also recommends continuing existing treatment with structured follow-up, even when treatment results in low-risk status, many patients favor transitioning to oral regimens.…”

Section: Discussionmentioning

confidence: 99%

Transitioning Stable Patients with Pulmonary Arterial Hypertension from Parenteral Prostanoids to Oral Selexipag

et al. 2022

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Introduction: Parenteral prostanoids are the most potent therapies for pulmonary arterial hypertension (PAH) but are associated with complications and lifestyle limitations. Carefully selected stable patients may be considered for a transition from parenteral prostanoids to a more convenient oral regimen. We present our experience transitioning patients on parenteral prostanoids to selexipag on an outpatient basis. Methods: This was a retrospective cohort study of all group 1 PAH patients on parenteral prostanoids who transitioned to selexipag using a standardized outpatient-based protocol. Hospitalization and routine prognostic data were recorded. Results: Fourteen patients were followed for a median of 1,240 (1,052–1,528) days; all were functional class (FC) II (n = 9) or III (n = 5). Thirteen patients completed the transition, including 11 who underwent catheterization 376 (321–735) days after discontinuing parenteral therapy. Three patients had unfavorable transitions requiring reinitiation of parenteral treatment. Overall, pulmonary vascular resistance increased (3.3–4.5 WU, p = 0.01), cardiac index fell (4.0–2.8 L/min/m2, p = 0.01), N-terminal pro-hormone of brain natriuretic peptide worsened (111–205 pg/dL, p = 0.03), but PAH-related hospitalizations improved (27–8, p = 0.02). Cardiac imaging, FC, and 6-min walk distance (6MWD) were unchanged. Patients who failed were older (64 vs. 56 years old) with shorter 6MWD (274 vs. 392 m) and higher REVEAL 2.0 scores (11 vs. 3). Conclusions: Transition from parenteral prostanoids to oral selexipag in carefully selected low-risk patients was well-tolerated in many patients, with up to 5 years of follow-up. Overall, the hemodynamic response to transition is unpredictable and close monitoring, particularly in the first year of follow-up, is recommended. Additional evaluation of potential predictors of success is necessary.

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Section: Discussionmentioning

confidence: 99%

Transitioning Stable Patients with Pulmonary Arterial Hypertension from Parenteral Prostanoids to Oral Selexipag

et al. 2022

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“…In the developing interim from RESPITE to REPLACE to the present, a greater emphasis is now placed on offering dual -or even triple -upfront therapy to patients with PAH. [39][40][41] Riociguat clearly has a place in this conversation especially as half of patients in PATENT-1 and 82% of patients enrolled in RESPITE were on background therapy. Recent studies indicate that the inclusion of riociguat in upfront combinations of PAH medications with sister drug classes is an appropriate treatment strategy.…”

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confidence: 99%

Evaluating Riociguat in the Treatment of Pulmonary Arterial Hypertension: A Real-World Perspective

Mihalek¹,

Scott²,

Mazimba³

2022

VHRM

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Pulmonary hypertension (PH) is a broad term describing the mean pulmonary artery pressure, as measured by right heart catheterization, exceeds 20mmHg. Pulmonary arterial hypertension (PAH) exists when PH is accompanied by a normal wedge pressure and elevated pulmonary vascular resistance. PAH is typified by dysmorphic and dysfunctional pulmonary arterial vasculature. Attempting to restore the functionality of the pulmonary artery is a hallmark of care to the PAH patient. Riociguat is a powerful stimulator of soluble guanylate cyclase and increases blood flow through the pulmonary arteries by dilating vascular smooth muscle cells. This review examines the pharmacology of riociguat, the fundamental clinical trials applying it to PAH patients, practical aspects when selecting its use, and future directions for its utilization.

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Nanomedicine-based treatment: An emerging therapeutical strategy for pulmonary hypertension

et al. 2023

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Upfront Combination Therapy for Pulmonary Arterial Hypertension: Time to Be More Ambitious than AMBITION

Cited by 10 publications

References 12 publications

Transitioning Stable Patients with Pulmonary Arterial Hypertension from Parenteral Prostanoids to Oral Selexipag

Transitioning Stable Patients with Pulmonary Arterial Hypertension from Parenteral Prostanoids to Oral Selexipag

Evaluating Riociguat in the Treatment of Pulmonary Arterial Hypertension: A Real-World Perspective

Nanomedicine-based treatment: An emerging therapeutical strategy for pulmonary hypertension

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