Nobuhiro Tanabe1,2, Hiroyuki Taniguchi3, Ichizo Tsujino4, Fumio Sakamaki5, Noriaki Emoto6, Hiroshi Kimura7, Kei Takamura8, Masayuki Hanaoka9, Masaharu Nishimura4, Koichiro Tatsumi1. 1. Department of Respirology, Graduate School of Medicine, Chiba University, Chiba, Japan. 2. Department of Advanced Medicine in Pulmonary Hypertension, Graduate School of Medicine, Chiba University, Chiba, Japan. 3. Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan. 4. First Department of Medicine, Hokkaido University Hospital, Sapporo, Japan. 5. Department of Medicine, Tokyo Saiseikai Central Hospital, Tokyo, Japan. 6. Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Kobe, Japan. 7. Second Department of Internal Medicine, Nara Medical University, Kashihara, Japan. 8. First Department of Medicine, Obihiro-Kosei General Hospital, Obihiro, Japan. 9. First Department of Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
Abstract
BACKGROUND AND OBJECTIVE: Pulmonary hypertension (PH) is often associated with respiratory diseases, but only a small number of patients present with severe PH defined as mean pulmonary arterial pressure ≥ 35 mm Hg. We here conducted a multicenter, retrospective study of patients with severe PH associated with respiratory diseases (R-PH) to reveal their demographics, treatment, prognosis and determinants of prognosis. METHODS: From 101 patients with severe R-PH collected by postal survey at the first stage, 70 patients with four major diseases (chronic obstructive pulmonary disease (COPD), combined pulmonary fibrosis with emphysema (CPFE), interstitial pneumonia associated with connective tissue disease (CTD-IP), interstitial pneumonia (IP)) and normal pulmonary arterial wedge pressure were studied for clinical characteristics, treatment and prognosis. RESULTS: Three-year survival rates were 50% for COPD (n = 18), 35.7% for IP (n = 19) and 68.1% for CTD-IP (n = 20), and the 2-year survival rate for CPFE (n = 13) was only 22.6%. Eighty-one per cent of patients had been treated with pharmacotherapy specific for pulmonary arterial hypertension. Those patients who had received phosphodiesterase-5 inhibitors (PDE-5I) displayed significantly better survival from the date of diagnosis than those who had not (3-year survival: 61.8% vs 20.0% P < 0.0001), especially in the IP, CTD-IP and CPFE groups. Multivariate analysis also revealed that treatment with PDE-5I was a positive prognostic factor. CONCLUSIONS: We here demonstrated the dismal prognosis of patients with severe R-PH. The remarkably better survival in those patients who had received PDE-5I warrants and facilitates future prospective randomized studies in this particular population.
BACKGROUND AND OBJECTIVE:Pulmonary hypertension (PH) is often associated with respiratory diseases, but only a small number of patients present with severe PH defined as mean pulmonary arterial pressure ≥ 35 mm Hg. We here conducted a multicenter, retrospective study of patients with severe PH associated with respiratory diseases (R-PH) to reveal their demographics, treatment, prognosis and determinants of prognosis. METHODS: From 101 patients with severe R-PH collected by postal survey at the first stage, 70 patients with four major diseases (chronic obstructive pulmonary disease (COPD), combined pulmonary fibrosis with emphysema (CPFE), interstitial pneumonia associated with connective tissue disease (CTD-IP), interstitial pneumonia (IP)) and normal pulmonary arterial wedge pressure were studied for clinical characteristics, treatment and prognosis. RESULTS: Three-year survival rates were 50% for COPD (n = 18), 35.7% for IP (n = 19) and 68.1% for CTD-IP (n = 20), and the 2-year survival rate for CPFE (n = 13) was only 22.6%. Eighty-one per cent of patients had been treated with pharmacotherapy specific for pulmonary arterial hypertension. Those patients who had received phosphodiesterase-5 inhibitors (PDE-5I) displayed significantly better survival from the date of diagnosis than those who had not (3-year survival: 61.8% vs 20.0% P < 0.0001), especially in the IP, CTD-IP and CPFE groups. Multivariate analysis also revealed that treatment with PDE-5I was a positive prognostic factor. CONCLUSIONS: We here demonstrated the dismal prognosis of patients with severe R-PH. The remarkably better survival in those patients who had received PDE-5I warrants and facilitates future prospective randomized studies in this particular population.
Authors: Steven D Nathan; Joan A Barbera; Sean P Gaine; Sergio Harari; Fernando J Martinez; Horst Olschewski; Karen M Olsson; Andrew J Peacock; Joanna Pepke-Zaba; Steeve Provencher; Norbert Weissmann; Werner Seeger Journal: Eur Respir J Date: 2019-01-24 Impact factor: 16.671
Authors: Marius M Hoeper; Juergen Behr; Matthias Held; Ekkehard Grunig; C Dario Vizza; Anton Vonk-Noordegraaf; Tobias J Lange; Martin Claussen; Christian Grohé; Hans Klose; Karen M Olsson; Thomas Zelniker; Claus Neurohr; Oliver Distler; Hubert Wirtz; Christian Opitz; Doerte Huscher; David Pittrow; J Simon R Gibbs Journal: PLoS One Date: 2015-12-02 Impact factor: 3.240