Faircloth Jenna M, Bhatt Neelam D, Chartan Corey A, Coleman Ryan D, Villafranco Natalie, Ruiz Fadel E, Morales-Demori Raysa, Whalen Elise, Ely Erin, Fombin Rozmeen, Varghese Nidhy P
Department of Pharmacy, Texas Children's Hospital, Houston, TX, United States.
Department of Pediatrics, Division of Critical Care and Pulmonology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, United States.
Front Pediatr. 2023 Mar 8;11:1050508. doi: 10.3389/fped.2023.1050508. eCollection 2023.
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 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.
司来帕格是一种选择性前列环素受体激动剂，已被批准用于治疗WHO第1组成年患者的肺动脉高压。与胃肠外前列环素制剂相比，司来帕格因其口服制剂、给药频率和作用机制，显著改善了患者和护理人员的生活质量。尽管儿科人群的经验仅限于年龄较大的青少年患者的病例报告，且司来帕格未被批准用于儿科肺动脉高压人群，但许多儿科中心正在将这种治疗方法扩大应用于该人群。我们报告了我们机构使用司来帕格治疗10岁以下、体重10至30公斤儿童肺动脉高压的经验。7例患者开始接受司来帕格治疗，包括从静脉注射曲前列尼尔起始和转换为口服司来帕格。所有患者在基线时均接受稳定的背景治疗，使用磷酸二酯酶-5抑制剂和内皮素受体拮抗剂治疗。所有患者在住院期间均达到了计划的司来帕格目标剂量，无需更改滴定方案，也没有血流动力学恶化。根据我们的经验，口服司来帕格在患有肺动脉高压的小儿患者中是安全且耐受性良好的。基于我们的良好经验，我们制定了一个机构特定的司来帕格治疗流程算法，以便在儿科人群中持续成功使用。
