Yuichi Tamura1, Hiraku Kumamaru2, Toru Satoh3, Hiroaki Miyata2,4, Aiko Ogawa5, Nobuhiro Tanabe6, Masaru Hatano7, Atsushi Yao8, Kohtaro Abe9, Ichizo Tsujino10, Keiichi Fukuda11, Hiroshi Kimura12, Masataka Kuwana13, Hiromi Matsubara5, Koichiro Tatsumi14. 1. Pulmonary Hypertension Center, International University of Health and Welfare Mita Hospital. 2. Department of Healthcare Quality Assessment, Graduate School of Medicine, The University of Tokyo. 3. Department of Cardiology, Kyorin University School of Medicine. 4. Department of Health Policy and Management, Keio University School of Medicine. 5. Department of Clinical Science, National Hospital Organization Okayama Medical Center. 6. Department of Advanced Medicine in Pulmonary Hypertension, Graduate School of Medicine, Chiba University. 7. Department of Cardiology, The University of Tokyo Hospital. 8. Division for Health Service Promotion, The University of Tokyo. 9. Department of Cardiovascular Medicine, Kyushu University Hospital. 10. First Department of Medicine, Hokkaido University School of Medicine. 11. Department of Cardiology, Keio University School of Medicine. 12. Second Department of Internal Medicine, Nara Medical University. 13. Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine. 14. Department of Respirology, Graduate School of Medicine, Chiba University.
Abstract
BACKGROUND: The trend of the initial treatment strategy for pulmonary arterial hypertension (PAH) has changed from monotherapies to upfront combination therapies. This study analyzed treatments and outcomes in Japanese patients with PAH, using data from the Japan PH Registry (JAPHR), which is the first organized multicenter registry for PAH in Japan.Methods and Results: We studied 189 consecutive patients (108 treatment-naïve and 81 background therapy patients) with PAH in 8 pulmonary hypertension (PH) centers enrolled from April 2008 to March 2013. We performed retrospective survival analyses and analyzed the association between upfront combination and hemodynamic improvement, adjusting for baseline NYHA classification status. Among the 189 patients, 1-, 2-, and 3-year survival rates were 97.0% (95% CI: 92.1-98.4), 92.6% (95% CI: 87.0-95.9), and 88.2% (95% CI: 81.3-92.7), respectively. In the treatment-naïve cohort, 33% of the patients received upfront combination therapy. In this cohort, 1-, 2-, and 3-year survival rates were 97.6% (95% CI: 90.6-99.4), 97.6% (95% CI: 90.6-99.4), and 95.7% (95% CI: 86.9-98.6), respectively. Patients on upfront combination therapy were 5.27-fold more likely to show hemodynamic improvement at the first follow-up compared with monotherapy (95% CI: 2.68-10.36). CONCLUSIONS: According to JAPHR data, initial upfront combination therapy is associated with improvement in hemodynamic status.
BACKGROUND: The trend of the initial treatment strategy for pulmonary arterial hypertension (PAH) has changed from monotherapies to upfront combination therapies. This study analyzed treatments and outcomes in Japanese patients with PAH, using data from the Japan PH Registry (JAPHR), which is the first organized multicenter registry for PAH in Japan.Methods and Results: We studied 189 consecutive patients (108 treatment-naïve and 81 background therapy patients) with PAH in 8 pulmonary hypertension (PH) centers enrolled from April 2008 to March 2013. We performed retrospective survival analyses and analyzed the association between upfront combination and hemodynamic improvement, adjusting for baseline NYHA classification status. Among the 189 patients, 1-, 2-, and 3-year survival rates were 97.0% (95% CI: 92.1-98.4), 92.6% (95% CI: 87.0-95.9), and 88.2% (95% CI: 81.3-92.7), respectively. In the treatment-naïve cohort, 33% of the patients received upfront combination therapy. In this cohort, 1-, 2-, and 3-year survival rates were 97.6% (95% CI: 90.6-99.4), 97.6% (95% CI: 90.6-99.4), and 95.7% (95% CI: 86.9-98.6), respectively. Patients on upfront combination therapy were 5.27-fold more likely to show hemodynamic improvement at the first follow-up compared with monotherapy (95% CI: 2.68-10.36). CONCLUSIONS: According to JAPHR data, initial upfront combination therapy is associated with improvement in hemodynamic status.