PMID- 15791043 OWN - NLM STAT- MEDLINE DCOM- 20051018 LR - 20190819 IS - 1346-9843 (Print) IS - 1346-9843 (Linking) VI - 69 IP - 4 DP - 2005 Apr TI - Oral sildenafil improves primary pulmonary hypertension refractory to epoprostenol. PG - 461-5 AB - BACKGROUND: Epoprostenol (prostaglandin I(2)) has become recognized as a therapeutic breakthrough that can improve hemodynamics and survival in patients with primary pulmonary hypertension (PPH). However, a significant number of patients have PPH that is refractory to epoprostenol, and lung transplantation has been the only remaining treatment option. METHODS AND RESULTS: The study subjects included 20 consecutive patients with PPH (mean pulmonary arterial pressure: 65+/-15 mmHg) who had received epoprostenol for more than 12 months. The patients were divided into 2 groups; responders and non-responders. In the non-responders, New York Heart Association (NYHA) functional class did not improve and mean right atrial pressure (mRA) increased to 8 mmHg or more, and additional sildenafil, a phosphodiesterase-5 inhibitor, was started. Six patients were included in the non-responders, whose mRA was 9+/-5 mmHg before and significantly increased to 13+/-3 mmHg after epoprostenol administration (p < 0.05). One patient died and the other 5 patients received oral sildenafil. The mRA of 12+/-4 mmHg (value before sildenafil) improved to 8+/-5 mmHg after sildenafil administration. Three patients were classified in the NYHA functional class 4 and improved to class 3, and 2 patients were in class 3 and remained in the same class after the addition of sildenafil. CONCLUSIONS: In patients with severe PPH refractory to epoprostenol treatment, additional oral sildenafil can improve pulmonary hemodynamics and symptoms. The combination therapy of epoprostenol and sildenafil is a new medical treatment to attempt before progressing to lung transplantation for patients with PPH refractory to epoprostenol. FAU - Kataoka, Masaharu AU - Kataoka M AD - Cardiopulmonary Division, Department of Medicine, Keio University School of Medicine, Tokyo, Japan. FAU - Satoh, Toru AU - Satoh T FAU - Manabe, Tomohiro AU - Manabe T FAU - Anzai, Toshihisa AU - Anzai T FAU - Yoshikawa, Tsutomu AU - Yoshikawa T FAU - Mitamura, Hideo AU - Mitamura H FAU - Ogawa, Satoshi AU - Ogawa S LA - eng PT - Clinical Trial PT - Journal Article PL - Japan TA - Circ J JT - Circulation journal : official journal of the Japanese Circulation Society JID - 101137683 RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - BW9B0ZE037 (Sildenafil Citrate) RN - DCR9Z582X0 (Epoprostenol) SB - IM MH - Administration, Oral MH - Adult MH - *Drug Resistance MH - Drug Synergism MH - Drug Therapy, Combination MH - Epoprostenol/administration & dosage/*therapeutic use MH - Female MH - Humans MH - Hypertension, Pulmonary/*drug therapy MH - Male MH - Middle Aged MH - Piperazines/*administration & dosage MH - Purines MH - Salvage Therapy/methods MH - Sildenafil Citrate MH - Sulfones MH - Treatment Outcome EDAT- 2005/03/26 09:00 MHDA- 2005/10/19 09:00 CRDT- 2005/03/26 09:00 PHST- 2005/03/26 09:00 [pubmed] PHST- 2005/10/19 09:00 [medline] PHST- 2005/03/26 09:00 [entrez] AID - JST.JSTAGE/circj/69.461 [pii] AID - 10.1253/circj.69.461 [doi] PST - ppublish SO - Circ J. 2005 Apr;69(4):461-5. doi: 10.1253/circj.69.461.