Department of Pediatric Cardiology, Ramon y Cajal Hospital, Madrid, Spain.
Department of Pediatric Cardiac Surgery, Ramon y Cajal Hospital, Madrid, Spain.
Pediatr Pulmonol. 2020 May;55(5):1254-1258. doi: 10.1002/ppul.24707. Epub 2020 Mar 9.
Prostanoid treatment in patients with severe pulmonary arterial hypertension (PAH) has been proven safe and effective. Subcutaneous administration of treprostinil has side effects, which limits their use and acceptance. An implantable pump for continuous intravenous treprostinil infusion has been recently approved. We describe our experience with the implantable pump in three pediatric patients.
The LENUS pro pump was implanted in three adolescents with severe PAH, who were treated with tadalafil, ambrisentan, and subcutaneous treprostinil. The indication of the Lenus pro pump implantation was the local side effects of subcutaneous treprostinil (pain, inflammation, and local infection) that were not well tolerated and that severely decreased their quality of life. The pump was surgically implanted under general anesthesia.One patient, in functional class IV, suffered postoperative hemodynamic instability and small pneumothorax, requiring an increase in treprostinil dose up to 85 ng/kg/min and a decrease 9 days after the pump implantation. The second patient who was discharged 4 days after surgery with treprostinil at 60 ng/kg/min reported improvement in his quality of life, but the dose requirement increased up to 92 ng/kg/min. After a 21-month follow-up, this patient received a lung transplant. The third patient presented a hematoma at the pump site with no other complications and had a follow-up of 9 months with an improvement in her quality of life.
Implantable pumps for continuous parenteral prostanoid infusion in pediatric patients are an alternative to external pumps, especially when familiar psychological or psychomotor issues hinder the use of external pumps.
在严重肺动脉高压（PAH）患者中，前列腺素类药物治疗已被证明是安全有效的。曲前列尼尔的皮下给药有副作用，这限制了它们的使用和接受。最近已批准一种用于持续静脉内曲前列尼尔输注的植入式输液泵。我们描述了在 3 名儿科患者中使用植入式输液泵的经验。
在 3 名患有严重 PAH 的青少年中植入了 LENUS pro 输液泵，他们接受了他达拉非、安贝生坦和曲前列尼尔皮下注射治疗。植入 LENUS pro 输液泵的指征是曲前列尼尔皮下注射的局部副作用（疼痛、炎症和局部感染）不能耐受，严重降低了他们的生活质量。输液泵在全身麻醉下进行手术植入。1 名功能分级为 IV 级的患者在术后出现血流动力学不稳定和小量气胸，需要将曲前列尼尔剂量增加至 85ng/kg/min，并在植入后 9 天减少剂量。第二位患者在手术后 4 天出院，以 60ng/kg/min 的曲前列尼尔剂量出院，报告生活质量改善，但剂量需求增加至 92ng/kg/min。经过 21 个月的随访，该患者接受了肺移植。第三位患者在输液泵部位出现血肿，但无其他并发症，随访 9 个月后生活质量得到改善。
在儿科患者中，用于持续肠外前列腺素输注的植入式输液泵是外部输液泵的替代方案，尤其是当熟悉的心理或运动问题妨碍外部输液泵的使用时。
