Clinical worsening during long-term follow-up in inoperable chronic thromboembolic pulmonary hypertension

Background: Pulmonaryendarterectomy is the treatment of choice in chronic thromboembolic pulmonary hypertension (CTEPH). Modern pulmonary vasoactive medication (like endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, and prostacyclins) is used in patients with an inoperable disease and improved prognosis. We evaluate mortality and time to clinical worsening (TtCW) in inoperable CTEPH patients during long-term follow-up. Methods: All 32 patients with inoperable CTEPH were enrolled between June 2002 and January 2009. TtCW was defined as the combination of death, need for intravenous pulmonary arterial hypertension medication, or 15% decrease in 6-minute walk distance (6-MWD) without improvement in functional class. The Cox proportional hazard regression was used to identify predictors. Results: During a mean follow-up of 3.4 years (range = 0.2-10.2 years), 11 patients died (34%). The 1- and 3-year survival rates were 87 and 77%, respectively. Baseline functional class, 6-MWD, mean pulmonary artery pressure, and pulmonary vascular resistance were predictors for survival. Clinical worsening occurred in 16 patients (50%). The 1- and 3-year rates of freedom from clinical worsening were 74 and 60%, respectively. The only predictor for clinical worsening was the baseline 6-MWD. Conclusion: Despite the improvement in medical treatment of inoperable CTEPH, the mortality rate is still high, and clinical worsening occurred in a substantial number of patients during a follow-up of more than 3 years.