Aiko Ogawa1, Seiichiro Sakao2, Nobuhiro Tanabe3, Hiromi Matsubara4, Koichiro Tatsumi5. 1. Department of Clinical Science, National Hospital Organization Okayama Medical Center, 1711-1 Tamasu, Kita-ku, Okayama 701-1192, Japan. Electronic address: aiko-oky@umin.ac.jp. 2. Department of Respirology, Graduate School of Medicine, Chiba University, Chiba, Japan. Electronic address: sakaos@faculty.chiba-u.jp. 3. Department of Respirology, Graduate School of Medicine, Chiba University, Chiba, Japan; Department of Advanced Medicine in Pulmonary Hypertension, Graduate School of Medicine, Chiba University, Chiba, Japan. Electronic address: ntanabe@faculty.chiba-u.jp. 4. Department of Clinical Science, National Hospital Organization Okayama Medical Center, 1711-1 Tamasu, Kita-ku, Okayama 701-1192, Japan. 5. Department of Respirology, Graduate School of Medicine, Chiba University, Chiba, Japan. Electronic address: tatsumi@faculty.chiba-u.jp.
Abstract
BACKGROUND: There are several medications available to treat pulmonary arterial hypertension (PAH): PAH-targeted drugs. However, in patients with pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis (PVOD/PCH), rare diseases that cause pulmonary hypertension, the effectiveness and safety of vasodilators, including PAH-targeted drugs, are unclear. METHODS: We searched English-language publications listed in three electronic databases (PubMed, Cochrane Library, and the Japan Medical Abstracts Society). Reports with efficacy outcomes (survival, improvement in 6-minute walk distance, and pulmonary vascular resistance) and data on development of pulmonary edema after administration of vasodilators to patients with PVOD/PCH were selected (1966 to August 2015). RESULTS: We identified 20 reports that met our criteria. No randomized controlled or prospective controlled studies were reported. The survival time ranged from 71 minutes to 4 years or more after initiation of vasodilators. Most of the reported cases showed an improvement in the 6-minute walk distance and pulmonary vascular resistance. Pulmonary edema was reported in 15 articles, some cases of which were lethal. CONCLUSIONS: The present study demonstrates the potential efficacy and difficulties in the use of vasodilators in patients with PVOD/PCH; however, drawing a firm conclusion was difficult because of the lack of randomized controlled trials. Further research is needed to ascertain if vasodilator use is beneficial and safe in patients with PVOD/PCH.
BACKGROUND: There are several medications available to treat pulmonary arterial hypertension (PAH): PAH-targeted drugs. However, in patients with pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis (PVOD/PCH), rare diseases that cause pulmonary hypertension, the effectiveness and safety of vasodilators, including PAH-targeted drugs, are unclear. METHODS: We searched English-language publications listed in three electronic databases (PubMed, Cochrane Library, and the Japan Medical Abstracts Society). Reports with efficacy outcomes (survival, improvement in 6-minute walk distance, and pulmonary vascular resistance) and data on development of pulmonary edema after administration of vasodilators to patients with PVOD/PCH were selected (1966 to August 2015). RESULTS: We identified 20 reports that met our criteria. No randomized controlled or prospective controlled studies were reported. The survival time ranged from 71 minutes to 4 years or more after initiation of vasodilators. Most of the reported cases showed an improvement in the 6-minute walk distance and pulmonary vascular resistance. Pulmonary edema was reported in 15 articles, some cases of which were lethal. CONCLUSIONS: The present study demonstrates the potential efficacy and difficulties in the use of vasodilators in patients with PVOD/PCH; however, drawing a firm conclusion was difficult because of the lack of randomized controlled trials. Further research is needed to ascertain if vasodilator use is beneficial and safe in patients with PVOD/PCH.
Authors: Baukje M Zaaijer; Nienke Duppen; Brigitte W M Willemse; Martijn V Verhagen; Marcus T R Roofthooft; Wim Timens; Rolf M F Berger; Johannes M Douwes Journal: Respir Med Case Rep Date: 2021-12-01