AI Article Synopsis

  • The study highlights the use of combination therapy for pulmonary hypertension (PH) in Japan, indicating that a significant percentage of patients are receiving advanced treatment options.
  • The research analyzed over 997 patients and found varying five-year transplant-free survival rates across different PH subtypes, with pulmonary arterial hypertension (PAH) at 74% and chronic thromboembolic PH (CTEPH) significantly higher at 92%.
  • Results showed that triple combination therapy for PAH, despite worse hemodynamic conditions, led to the best survival outcomes, while balloon angioplasty for CTEPH also improved survival rates.

Article 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.

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http://dx.doi.org/10.1016/j.ijcard.2019.11.139DOI Listing

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