Jackson Emma O, Brown Anna, McSweeney Julia, Parker Claire
Department of Cardiology, Seattle Children's Hospital, Seattle, WA, USA.
Division of Pulmonary Medicine, Department of Pediatrics, Vanderbilt Children's Hospital, Nashville, TN, USA.
Pulm Circ. 2021 Mar 2;11(1):2045894021994450. doi: 10.1177/2045894021994450. eCollection 2021 Jan-Mar.
Pulmonary arterial hypertension is a chronic, progressive, and life-threatening disease in children with diverse causes of pulmonary arterial hypertension. The most severe cases of pulmonary arterial hypertension require aggressive treatments with systemic administration of continuous prostacyclin therapy, including treprostinil and epoprostenol. The successful use of continuous subcutaneous treprostinil therapy eliminates the need for an indwelling central venous catheter and its associated risks. However, pain at the subcutaneous infusion site, an expected side effect of this therapy, is often a deterrent to its widespread use. Effective subcutaneous treprostinil site maintenance and pain management is essential to achieve success with this therapy, but strategies surrounding site maintenance and pain control vary significantly between pediatric pulmonary hypertension treatment centers. In an attempt to standardize practice, a survey on the use of subcutaneous treprostinil and site maintenance and pain management strategies, as well as its perceived effectiveness, was disseminated to 13 pediatric pulmonary hypertension centers of the Pediatric Pulmonary Hypertension Network. Responses to the survey were collected and analyzed and were developed into a set of formalized strategies to facilitate knowledge sharing and standardization of practice.
肺动脉高压是一种发生于儿童的慢性、进行性且危及生命的疾病，其肺动脉高压病因多样。最严重的肺动脉高压病例需要采用积极的治疗方法，即持续静脉输注前列环素类药物进行全身治疗，包括曲前列尼尔和依前列醇。成功使用皮下持续输注曲前列尼尔疗法可避免使用中心静脉留置导管及其相关风险。然而，皮下输注部位疼痛是该疗法的一个预期副作用，这常常阻碍其广泛应用。有效的皮下曲前列尼尔输注部位护理和疼痛管理对于该疗法的成功实施至关重要，但在儿科肺动脉高压治疗中心，围绕输注部位护理和疼痛控制的策略差异很大。为了规范实践，一项关于皮下曲前列尼尔的使用、输注部位护理和疼痛管理策略及其感知效果的调查被分发给了儿科肺动脉高压网络的13个儿科肺动脉高压中心。对调查的回复进行了收集和分析，并制定了一套正式策略，以促进知识共享和实践标准化。
