Bravo-Valenzuela Nathalie Jeanne Magioli, Navarro Flavia, Silva Socrates Pereira
Department of Pediatrics, Federal University of Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
PEDICOR-Pediatric and Perinatal Cardiology Center, Sao José dos Campos, SP, Brazil.
Ann Pediatr Cardiol. 2021 Jan-Mar;14(1):75-78. doi: 10.4103/apc.APC_63_19. Epub 2020 Sep 23.
Pulmonary arterial hypertension (PAH) is a chronic, progressive, multifactorial disease. Currently, combination therapy is an attractive option for PAH management because three pathways (endothelin, nitric oxide, and prostacyclin) are involved in this disease. Selexipag is a novel oral prostacyclin pathway drug and is a highly selective IP prostacyclin receptor agonist with vasodilatory and antiproliferative effects. We report the case of a teenage patient with idiopathic PAH who presented in World Health Organization functional Class IV and showed no clinical improvement with dual therapy. We added oral selexipag to the treatment regimen and observed substantial improvement in her quality of life at the short-time follow-up. Despite the lack of childhood data regarding the use of selexipag in pediatric patients with PAH, the use of this drug in the current teenage patient improved her quality of life and exercise capacity.
肺动脉高压（PAH）是一种慢性、进行性、多因素疾病。目前，联合治疗是PAH管理的一个有吸引力的选择，因为三种途径（内皮素、一氧化氮和前列环素）参与了这种疾病。司来帕格是一种新型口服前列环素途径药物，是一种具有血管舒张和抗增殖作用的高选择性IP前列环素受体激动剂。我们报告了一例青少年特发性PAH患者的病例，该患者表现为世界卫生组织功能分级IV级，接受双重治疗后无临床改善。我们在治疗方案中添加了口服司来帕格，在短期随访中观察到她的生活质量有了显著改善。尽管缺乏关于司来帕格在儿童PAH患者中使用的儿童数据，但在目前这位青少年患者中使用这种药物改善了她的生活质量和运动能力。
