title: Four cases with group 3 out-of-proportion pulmonary hypertension with a favorable response to vasodilators.
abstract: Some patients with group 3 pulmonary hypertension (PH) (PH due to lung disease and/or hypoxia) exhibit disproportionately advanced or "out-of-proportion" PH. In the present case series, we document four consecutive patients with progressive out-of-proportion group 3 PH. All patients exhibited progressive dyspnea or peripheral edema and were treated by pulmonary artery hypertension (PAH)-specific vasodilator(s). At the follow-up assessment 3-4 months later, symptoms/signs and pulmonary hemodynamic measurements improved in all four patients (45 ± 8% decrease in pulmonary vascular resistance). Pulmonary oxygenation deteriorated in one patient but improved or did not significantly change in the remaining three cases. Importantly, the background lung parenchymal disease (early-onset chronic obstructive pulmonary disease, rheumatoid arthritis-associated interstitial pneumonia, and combined pulmonary fibrosis and emphysema) was stable upon progression of the right heart failure symptoms/signs, and also during the 3-4-month follow-up period in all cases. We herein describe the clinical features of the four cases and discuss the potential benefits and risks of PAH-specific treatment in this emerging population.
authors.affiliation: First Department of Medicine, Hokkaido University Hospital, North 14, West 5, Kita-ku, Sapporo, Hokkaido 060-8648, Japan.
authors.forename: Ayako
authors.initials: A
authors.lastname: Igarashi
authors.affiliation: First Department of Medicine, Hokkaido University Hospital, North 14, West 5, Kita-ku, Sapporo, Hokkaido 060-8648, Japan.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Sato
authors.affiliation: First Department of Medicine, Hokkaido University Hospital, North 14, West 5, Kita-ku, Sapporo, Hokkaido 060-8648, Japan.
authors.forename: Ichizo
authors.initials: I
authors.lastname: Tsujino
authors.affiliation: First Department of Medicine, Hokkaido University Hospital, North 14, West 5, Kita-ku, Sapporo, Hokkaido 060-8648, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ohira
authors.affiliation: First Department of Medicine, Hokkaido University Hospital, North 14, West 5, Kita-ku, Sapporo, Hokkaido 060-8648, Japan.
authors.forename: Asuka
authors.initials: A
authors.lastname: Yamada
authors.affiliation: First Department of Medicine, Hokkaido University Hospital, North 14, West 5, Kita-ku, Sapporo, Hokkaido 060-8648, Japan.
authors.forename: Taku
authors.initials: T
authors.lastname: Watanabe
authors.affiliation: First Department of Medicine, Hokkaido University Hospital, North 14, West 5, Kita-ku, Sapporo, Hokkaido 060-8648, Japan.
authors.forename: Masaru
authors.initials: M
authors.lastname: Suzuki
authors.affiliation: Division of Respiratory Medicine, Department of Internal Medicine, Hokkaido University Graduate School of Medicine, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Konno
authors.affiliation: Division of Respiratory Medicine, Department of Internal Medicine, Hokkaido University Graduate School of Medicine, Japan.
authors.forename: Masaharu
authors.initials: M
authors.lastname: Nishimura
keywords: Chronic obstructive pulmonary disease; Combined pulmonary fibrosis and emphysema; Interstitial pneumonia; Pulmonary hypertension; Sildenafil; Vasodilator