PMID- 37544296 OWN - NLM STAT- MEDLINE DCOM- 20230906 LR - 20240412 IS - 1423-0356 (Electronic) IS - 0025-7931 (Linking) VI - 102 IP - 8 DP - 2023 TI - Long-Term Safety, Outcome, and Clinical Effects of Subcutaneous and Intravenous Treprostinil Treatment in Patients with Severe Chronic Pulmonary Arterial Hypertension. PG - 579-590 LID - 10.1159/000531169 [doi] AB - BACKGROUND: Current guidelines recommend treatment with parenteral prostacyclin analogs in patients with severe pulmonary arterial hypertension (PAH), who have insufficient response to treatment. Real-life data are sought to help physicians in treatment decisions and clinical care of patients. OBJECTIVE: This study analyzed safety, clinical effects, and long-term outcomes of subcutaneous (sc) and/or intravenous (iv) treprostinil via different pump systems in consecutive patients with PAH. METHODS: Thirty-seven patients with severe progressive PAH despite dual combination therapy (20 female, mean age: 52.3 +/- 15 years, mean pulmonary vascular resistance: 12.1 +/- 5.1 WU) were initiated with add-on treprostinil sc and were routinely clinically assessed. Changes in clinical parameters, adverse events, and outcome were analyzed retrospectively. RESULTS: In 24 of 37 patients, treprostinil administration was continued iv via implantation of LENUS Pro(R) pump after 3 +/- 1.3 months, 6 patients continued with sc therapy, and 7 discontinued treatment. After 3, 6, 9, and 12 months of treprostinil treatment, patients showed a significant improvement in mean 6-min walk distance and tricuspid annular plane systolic excursion compared to baseline. In 8 of the 24 patients, iv pumps required surgical revision. During a mean follow-up of 2.82 +/- 1.95 years, 12 patients died, four received lung transplantation. Transplant-free survival after 1, 2, and 3 years was 85.7%, 69.2%, and 65.3%, respectively. CONCLUSION: sc treprostinil as add-on to double combination treatment significantly improved exercise capacity and right heart function. In most patients, treprostinil could be continued via more tolerable iv administration approach (LENUS Pro(R) pump), showing reasonable overall survival with respect to the severity of PAH. CI - (c) 2023 S. Karger AG, Basel. FAU - Harutyunova, Satenik AU - Harutyunova S AD - Center for Pulmonary Hypertension, Thoraxklinik at Heidelberg University Hospital, Heidelberg, Germany, satenik.harutyunova@med.uni-heidelberg.de. AD - Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Heidelberg, Germany, satenik.harutyunova@med.uni-heidelberg.de. FAU - Benjamin, Nicola AU - Benjamin N AD - Center for Pulmonary Hypertension, Thoraxklinik at Heidelberg University Hospital, Heidelberg, Germany. AD - Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Heidelberg, Germany. FAU - Eichstaedt, Christina AU - Eichstaedt C AD - Center for Pulmonary Hypertension, Thoraxklinik at Heidelberg University Hospital, Heidelberg, Germany. AD - Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Heidelberg, Germany. AD - Institute of Human Genetics, Laboratory for Molecular Genetic Diagnostics, Heidelberg University, Heidelberg, Germany. FAU - Marra, Alberto M AU - Marra AM AD - Center for Pulmonary Hypertension, Thoraxklinik at Heidelberg University Hospital, Heidelberg, Germany. AD - Internal Medicine Section, Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy. FAU - Xanthouli, Panagiota AU - Xanthouli P AD - Center for Pulmonary Hypertension, Thoraxklinik at Heidelberg University Hospital, Heidelberg, Germany. AD - Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Heidelberg, Germany. FAU - Nagel, Christian AU - Nagel C AD - Center for Pulmonary Hypertension, Thoraxklinik at Heidelberg University Hospital, Heidelberg, Germany. AD - Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Heidelberg, Germany. AD - Department of Pneumology, Clinic Baden-Baden, Baden-Baden, Germany. AD - Practice of Pneumology, Max Grundig Clinic Buhlerhohe, Buhl, Germany. FAU - Grunig, Ekkehard AU - Grunig E AD - Center for Pulmonary Hypertension, Thoraxklinik at Heidelberg University Hospital, Heidelberg, Germany. AD - Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Heidelberg, Germany. FAU - Egenlauf, Benjamin AU - Egenlauf B AD - Center for Pulmonary Hypertension, Thoraxklinik at Heidelberg University Hospital, Heidelberg, Germany. AD - Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Heidelberg, Germany. LA - eng PT - Journal Article DEP - 20230804 PL - Switzerland TA - Respiration JT - Respiration; international review of thoracic diseases JID - 0137356 RN - RUM6K67ESG (treprostinil) RN - 0 (Antihypertensive Agents) RN - DCR9Z582X0 (Epoprostenol) SB - IM MH - Humans MH - Female MH - Adult MH - Middle Aged MH - Aged MH - *Pulmonary Arterial Hypertension/drug therapy MH - Antihypertensive Agents/adverse effects MH - Retrospective Studies MH - Treatment Outcome MH - Epoprostenol MH - *Hypertension, Pulmonary MH - Familial Primary Pulmonary Hypertension OTO - NOTNLM OT - Prostacyclin OT - Pulmonary arterial hypertension OT - Treatment OT - Treprostinil EDAT- 2023/08/07 00:42 MHDA- 2023/09/06 06:42 CRDT- 2023/08/06 18:23 PHST- 2023/01/23 00:00 [received] PHST- 2023/05/16 00:00 [accepted] PHST- 2023/09/06 06:42 [medline] PHST- 2023/08/07 00:42 [pubmed] PHST- 2023/08/06 18:23 [entrez] AID - 000531169 [pii] AID - 10.1159/000531169 [doi] PST - ppublish SO - Respiration. 2023;102(8):579-590. doi: 10.1159/000531169. Epub 2023 Aug 4.