Abstract

Introduction: Severe Pulmonary hypertension (PH) has a high morbidity and increased mortality. Worst case scenario is an acute PH crisis, being triggered by infections or anesthesia. Taking this into consideration, prior to a surgery or anesthetic procedure, children with PH in treatment with subcutaneous (SC) treprostinil were transitioned to intravenous (IV).

Methods: Retrospective review of all pediatric patients at Vall d'Hebron hospital (Barcelona, Spain) with PH who underwent transition from SC treprostinil to IV (2022-present).

Results: 6 patients mean age was 9 (IQR from 4-15) years with severe PH diagnosis receiving triple therapy requiring risky procedures (cardiac catheterization =5, atrial septostomy =3, liver transplant =2, hepaticojejunostomy, cholangiography, percutaneous drainage of perihepatic collection, adenoidectomy, and esophagogastroduodenoscopy). Two patients underwent several surgical procedures requiring to repeat the transition protocol. In order to achieve an accurate management of the treprostinil during surgeries, a transition protocol was established from SC to IV. The etiology of PH was idiopathic (3), Porto pulmonary (3). The transition procedure was made in 3 intervals. First step was reducing SC treprostinil ? and 15 minutes later IV treprostinil was started ?.  The procedure of lowering the SC dose and increasing the IV dose is repeated every 60 minutes. No complications registered during the transition.

Conclusions: This is our center's first experience performing the transition of SC to IV treprostinil. The procedure can be safely made by harsh supervision in a specialized center without having any complications.