Safety and Tolerability of a Rapid Transition From Intravenous Treprostinil to Oral Selexipag in Three Adolescent Patients With Pulmonary Arterial Hypertension

Colglazier, Elizabeth; Ng, Angelica J; Parker, Claire E; Nawaytou, Hythem; Fineman, Jeffrey R.

doi:10.5863/1551-6776-26.5.512

Cited by 4 publications

(7 citation statements)

References 19 publications

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“…One patient underwent a similar protocol as an inpatient, and one underwent a previously described rapid transition from IV treprostinil to selexipag over 5–7 days while in the hospital. 21 …”

Section: Methodsmentioning

confidence: 99%

“…Nine of our 11 total patients were transitioned from treprostinil to selexipag as outpatients with weekly increases in selexipag doses ranging from 50 to 200 mcg twice daily, while inhaled or parenteral treprostinil doses were simultaneously decreased. One patient underwent a similar protocol as an inpatient, and one underwent a previously described rapid transition from IV treprostinil to selexipag over 5–7 days while in the hospital 21 …”

Section: Methodsmentioning

confidence: 99%

“…One patient underwent a similar protocol as an inpatient, and one underwent a previously described rapid transition from IV treprostinil to selexipag over 5-7 days while in the hospital. 21 Baseline characteristics were described using mean and standard deviation. A variety of hemodynamic data (pulmonary vascular resistance indexed [PVRI], mean pulmonary arterial pressure [MPAP], transpulmonary gradient [TPG, the mean pulmonary arterial pressure minus the left atrial or pulmonary capillary wedge pressure], and cardiac index [CI]), B-type natriuretic factor (BNP), WHO functional class (FC), and 6-min walk test (6MWT), were compared by paired t-tests between the following time points: (a) before initiation of parenteral prostacyclin, (b) on parenteral prostacyclin therapy before transitioning to selexipag, and (c) on oral selexipag following a transition off parenteral prostacyclin therapy.…”

Section: Transitionsmentioning

confidence: 99%

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Hemodynamic assessment of transitioning from parenteral prostacyclin to selexipag in pediatric pulmonary hypertension

et al. 2022

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Despite the increase in therapeutic options, parenteral prostacyclins remain the cornerstone in the medical management of pulmonary arterial hypertension (PAH). While the use of parenteral prostacyclins in pediatric patients is well documented, less is known about alternative drug delivery methods such as enteral administration. Given that parenteral routes of prostacyclin administration (IV or SC) are invariably accompanied by complicated logistics and lifestyle compromises, enteral prostacyclin administration represents an attractive treatment option. Selexipag (Uptravi®) was approved for adults PAH in 2015. There is limited data on the hemodynamic efficacy of transitioning from parenteral prostacyclins to selexipag, particularly in the pediatric population. We report 11 pediatric PAH patients who underwent this transition, in which 10 had complete cardiac catheterization data before and following the transition to selexipag. All patients/families reported an improvement in quality of life, and the transitions occurred without adverse effects. However, 3 of the 11 (27%) did not tolerate the transition; two for worsening hemodynamics, and one for acute right ventricular failure in the setting of an intercurrent illness. In addition, the transition to selexipag was associated with a modest increase in pulmonary vascular resistance index (6/10) and decrease in cardiac index (6/10) in some patients. Selexipag use in pediatric PAH represents a significant addition to our therapeutic arsenal, and its use provides a meaningful improvement in quality of life compared with other prostacyclin formulations. However, when goals of care include aggressive disease management, a decision between improved quality of life and possible adverse outcomes must be considered, and its substitution should include cautious, close, long‐term follow‐up.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Transitionsmentioning

confidence: 99%

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Hemodynamic assessment of transitioning from parenteral prostacyclin to selexipag in pediatric pulmonary hypertension

et al. 2022

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“… 6 , 7 Most “rapid” transitions between prostacyclins and selexipag in pediatric patients take place over 4–8 days, in the hospital. 8 , 9 …”

Section: Introductionmentioning

confidence: 99%

Direct prostacyclin transition in pediatric patients with pulmonary hypertension

Merrill,

Davis,

Jackson

et al. 2024

Pulm. circ.

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Pediatric patients with pulmonary arterial hypertension (PAH) are commonly treated with the prostacyclin analog treprostinil in IV, SQ, inhaled or oral form, or the prostacyclin receptor agonist selexipag. Patients who transition between these medications often follow recommendations for gradual up‐ and down‐titrations that take place over several days in the hospital or several weeks as an outpatient. However, hospital resources are limited, and long transitions are inconvenient for patients and families. We report a case series of eight pediatric patients with PAH transitioned directly between prostacyclins with no overlapping doses. Direct medication transitions occurred in the cardiac intensive care unit (CICU), at home and in cardiology clinic. Equivalent doses for selexipag were estimated using information extrapolated from experience, published materials and selexipag study guidelines. All patients completed direct transition as planned and remained on transition dose for at least 1 week. In most cases selexipag was up‐titrated at home after establishing initial transition dose. In select patients, direct prostacyclin transition in pediatric patients with PAH is safe, effective, convenient for families and reduces the use of hospital resources.

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“…Given these advantages and pharmacokinetics, selexipag is an attractive therapy choice. Therefore, although experience in the pediatric population is limited primarily to case reports in adolescent patients and selexipag is not approved for use in the pediatric pulmonary hypertension population, many pediatric centers are expanding the use of this therapy to this population (4)(5)(6)(7)(8).…”

Section: Introductionmentioning

confidence: 99%

Case Report: Selexipag in pediatric pulmonary hypertension: Initiation, transition, and titration

et al. 2023

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Selexipag, a selective prostacyclin receptor agonist, is approved for treating pulmonary arterial hypertension in WHO Group 1 adult patients. Compared to parenteral prostacyclin formulations, selexipag offers a significant improvement in patient’s and caregiver’s quality of life because of its oral formulation, frequency of administration, and mechanism of action. Although experience in the pediatric population is limited to case reports in older adolescent patients and selexipag is not approved for use in the pediatric pulmonary hypertension population, many pediatric centers are expanding the use of this therapy to this population. We report our institution's experience in the use of selexipag to treat pulmonary hypertension in children under 10 years of age, between 10 and 30 kg. Seven patients were initiated on selexipag therapy including de novo initiation and transition from intravenous treprostinil to oral selexipag. All patients were on stable background therapy with phosphodiesterase-5 inhibitor and endothelin receptor antagonist therapies at baseline. All patients reached their planned goal selexipag dose during admission without the need for changes to the titration schedule and without hemodynamic deterioration. In our experience, oral selexipag is safe and well-tolerated in young pediatric patients with pulmonary hypertension. Based on our favorable experience, we developed an institution-specific selexipag process algorithm for continued successful use in the pediatric population.

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Safety and Tolerability of a Rapid Transition From Intravenous Treprostinil to Oral Selexipag in Three Adolescent Patients With Pulmonary Arterial Hypertension

Cited by 4 publications

References 19 publications

Hemodynamic assessment of transitioning from parenteral prostacyclin to selexipag in pediatric pulmonary hypertension

Hemodynamic assessment of transitioning from parenteral prostacyclin to selexipag in pediatric pulmonary hypertension

Direct prostacyclin transition in pediatric patients with pulmonary hypertension

Case Report: Selexipag in pediatric pulmonary hypertension: Initiation, transition, and titration

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