Katsuya Kozu1, Koichiro Sugimura1, Masaaki Ito2, Ken-Ichi Hirata3, Koichi Node4, Takuya Miyamoto5, Shuichi Ueno6, Hiroshi Watanabe7, Hiroaki Shimokawa8. 1. Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan. 2. Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Tsu, Mie, Japan. 3. Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Kobe, Japan. 4. Department of Cardiovascular Medicine, Saga University, Saga, Japan. 5. Department of Cardiology, Pulmonology, and Nephrology, Yamagata University School of Medicine, Yamagata, Japan. 6. Division of Cardiovascular Medicine, Department of Medicine, Jichi Medical University, Tochigi, Japan. 7. Department of Clinical Pharmacology & Therapeutics, Hamamatsu University School of Medicine, Hamamatsu, Japan. 8. Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan. Electronic address: shimo@cardio.med.tohoku.ac.jp.
Abstract
BACKGROUND: In the current era of treatment of pulmonary hypertension (PH) in Japan, combination therapy has been frequently used thanks to the medical insurance system. Additionally, pulmonary balloon angioplasty (BPA) is widely performed for chronic thromboembolic PH (CTEPH). METHODS: To elucidate the long-term prognosis and the prognostic factors among all five subtypes of PH in this new era, we examined the current status of management of PH from November 2012 to April 2016 in the multicenter registry by the Japanese Pulmonary Circulation Society. RESULTS: Among 1253 consecutive patients registered from 20 PH centers in Japan, we analyzed 997 patients with mean pulmonary arterial pressure ≥ 25 mmHg by right heart catheterization. Transplant-free survival at 5 years in pulmonary arterial hypertension (PAH), PH due to left-heart disease, PH due to lung diseases, CTEPH, and miscellaneous PH were 74.0, 69.3, 63.7, 92.0, and 55.3%, respectively. Of note, 32% of PAH patients were treated with double combination therapy and 42% of those with triple combination therapy, and 66% of CTEPH patients with BPA. Although PAH patients with triple combination therapy had worse hemodynamic parameters than those with other medications, triple combination therapy showed the best prognosis. BPA in CTEPH improved survival even when adjusted for the key background factors. CONCLUSIONS: In the current era of PH treatment in Japan, the five-year transplant-free survival rate in this study was 74% for PAH and 92% for CTEPH, in which active combination medical therapy for PAH and higher performance rate of BPA for CTEPH may be involved.
BACKGROUND: In the current era of treatment of pulmonary hypertension (PH) in Japan, combination therapy has been frequently used thanks to the medical insurance system. Additionally, pulmonary balloon angioplasty (BPA) is widely performed for chronic thromboembolic PH (CTEPH). METHODS: To elucidate the long-term prognosis and the prognostic factors among all five subtypes of PH in this new era, we examined the current status of management of PH from November 2012 to April 2016 in the multicenter registry by the Japanese Pulmonary Circulation Society. RESULTS: Among 1253 consecutive patients registered from 20 PH centers in Japan, we analyzed 997 patients with mean pulmonary arterial pressure ≥ 25 mmHg by right heart catheterization. Transplant-free survival at 5 years in pulmonary arterial hypertension (PAH), PH due to left-heart disease, PH due to lung diseases, CTEPH, and miscellaneous PH were 74.0, 69.3, 63.7, 92.0, and 55.3%, respectively. Of note, 32% of PAHpatients were treated with double combination therapy and 42% of those with triple combination therapy, and 66% of CTEPHpatients with BPA. Although PAHpatients with triple combination therapy had worse hemodynamic parameters than those with other medications, triple combination therapy showed the best prognosis. BPA in CTEPH improved survival even when adjusted for the key background factors. CONCLUSIONS: In the current era of PH treatment in Japan, the five-year transplant-free survival rate in this study was 74% for PAH and 92% for CTEPH, in which active combination medical therapy for PAH and higher performance rate of BPA for CTEPH may be involved.