Chronic thromboembolic pulmonary hypertension: A 10-year analysis from a Portuguese referral center

INTRODUCTION AND OBJECTIVES:Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive, but potentially curable, form of pulmonary hypertension. Pulmonary endarterectomy (PEA) is a complex surgery that frequently achieves hemodynamic normalization and symptom resolution, although not all patients are suitable for the procedure. We aimed to assess long-term outcomes of CTEPH, namely all-cause mortality and hospital admission for decompensated heart failure, according to treatment modalities in patients who underwent PEA or non-surgical therapy. METHODS:A 10-year retrospective study of patients with CTEPH at a referral center was conducted. Forty-five patients were included and median follow-up time was 57 (IQR 24-93) months. Survival analysis was performed and a multivariate Cox regression model was used to identify independent predictors of outcomes. RESULTS:Patients were mostly female (59%) and mean age was 63±16 years. Two-thirds were severely symptomatic at diagnosis, with 62.2% of patients presenting in WHO functional class (WHO FC) III or IV. One-, two- and three-year survival was 93.3%, 82.4% and 75.9%, respectively. Serum BNP (HR 1.003; 95% CI: 1.001-1.005; p=0.003) and creatinine (HR 12.092; 95% CI: 1.121-130.390; p=0.040) were predictors of death. Mortality was numerically lower in those who underwent PEA (p=0.135). PEA was associated with decreased risk of the combined endpoint of all-cause mortality and hospital admission for decompensated heart failure (HR 0.198; 95% CI: 0.040-0.982; p=0.047), as were lower serum BNP (HR 1.003; 95% CI: 1.001-1.005; p=0.008) and mPAP (HR 1.073; 95% CI: 1.022-1.128; p=0.005) at diagnosis. Most patients who underwent PEA presented improved WHO FC (92.9%) and post-surgical residual pulmonary hypertension was identified in only 21.4%. CONCLUSION:PEA provided a better overall prognosis than non-surgical therapy, improving symptoms and frequently achieving hemodynamic normalization, with a numerical trend for lower mortality. Higher serum BNP, creatinine and mPAP at diagnosis were independently associated with worse outcomes.