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Hokkaido University Collection of Scholarly and Academic Papers >
Graduate School of Medicine / Faculty of Medicine >
Peer-reviewed Journal Articles, etc >
Efficacy and safety of oral pulmonary vasodilators in pulmonary veno-occlusive disease
| Title: | Efficacy and safety of oral pulmonary vasodilators in pulmonary veno-occlusive disease |
| Authors: | Nakamura, Junichi Browse this author | | Tsujino, Ichizo Browse this author →KAKEN DB | | Shima, Hideki Browse this author | | Nakaya, Toshitaka Browse this author | | Sugimoto, Ayako Browse this author | | Sato, Takahiro Browse this author | | Watanabe, Taku Browse this author | | Ohira, Hiroshi Browse this author | | Suzuki, Masaru Browse this author | | Tsuneta, Satonori Browse this author | | Hisada, Ryo Browse this author | | Kato, Masaru Browse this author | | Konno, Satoshi Browse this author →KAKEN DB |
| Keywords: | capillaries | | pulmonary hypertension | | treatment | | vasodilation | | veins |
| Issue Date: | Oct-2022 |
| Publisher: | John Wiley & Sons |
| Journal Title: | Pulmonary circulation |
| Volume: | 12 |
| Issue: | 4 |
| Start Page: | e12168 |
| Publisher DOI: | 10.1002/pul2.12168 |
| Abstract: | Pulmonary veno-occlusive disease (PVOD) or pulmonary capillary hemangiomatosis (PCH) is a rare subtype of pulmonary hypertension with dismal prognosis. Limited data are available on the efficacy and safety of orally administered pulmonary vasodilators for PVOD/PCH. Whether and how systemic sclerosis (SSc) affects the clinical outcomes of PVOD/PCH is also unknown. This study aimed to determine the clinical and hemodynamic efficacy and safety of oral pulmonary vasodilators for PVOD/PCH and clarify the possible effects of SSc on the clinical presentation of PVOD/PCH. We retrospectively analyzed the clinical data of 15 patients with PVOD/PCH treated with oral pulmonary vasodilators in our department since 2001. Six of them had SSc. Oral pulmonary vasodilators were administered either as single agents (n = 10) or in combination (n = 5). Treatment improved the functional class of five patients, and pulmonary arterial pressure and pulmonary vascular resistance decreased by 10 +/- 12 mmHg and 36 +/- 19%, respectively (p < 0.05 for both, n = 13), whereas pulmonary edema developed in three patients. The mean survival was 3.9 years, and the 1- and 3-year survival rates were 93% and 65%, respectively. The clinical presentation, including survival, was similar between patients with and without SSc. In our PVOD/PCH cohort, oral pulmonary vasodilators caused pulmonary edema in 20% of patients, but more than 80% of patients experienced significant pulmonary vasodilatory effects, and the overall prognosis was better than that previously reported. SSc does not adversely affect the clinical outcomes of PVOD/PCH. |
| Type: | article |
| URI: | http://hdl.handle.net/2115/88012 |
| Appears in Collections: | 医学院・医学研究院 (Graduate School of Medicine / Faculty of Medicine) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)
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