Survival trends in patients with chronic thromboembolic pulmonary hypertension: an observational study

Background Treatment options for patients with chronic thromboembolic hypertension (CTEPH) have increased over the past decade. However, it is unknown whether the outcomes of patients with CTEPH have changed as well. Methods This retrospective study analysed the data of 834 patients with CTEPH, categorised into early (April 1980–December 1999), middle (January 2000–September 2010) and current (October 2010–December 2023) eras. The primary end-point was all-cause mortality. Multivariable Cox proportional hazards models were used to assess changes in all-cause mortality after adjusting for confounding variables. Results 95, 210 and 529 patients were from the early, middle and current eras, respectively. The proportion of patients who did not receive CTEPH therapy decreased from 65% in the early era to 36% and 3% in the middle and current eras, respectively. Meanwhile, the adoption of multimodal treatment increased from 0% to 58% over time. The Kaplan–Meier analysis revealed significant improvements in overall survival (log-rank p<0.001). The 5-year survival rates improved from 68% in the early era to 85% and 93% in the middle and current eras, respectively. The adjusted hazard ratios for mortality were 0.291 (95% CI 0.154–0.550; p<0.001) for the early versus middle era, 0.447 (95% CI 0.249–0.804; p=0.007) for the middle versus current era and 0.130 (95% CI 0.067–0.254; p<0.001) for the early versus current era. Conclusion The long-term prognosis of patients with CTEPH has significantly improved in recent decades.