Long-Term Outcome of Chronic Thromboembolic Pulmonary Hypertension

Study Questions:

What are the long-term outcomes for patients with chronic thromboembolic pulmonary hypertension (CTEPH)?


Across 27 centers in Europe, 679 patients with newly diagnosed CTEPH were prospectively followed for 24 months. Survival was estimated at 1, 2, and 3 years, stratified by pulmonary endarterectomy status, using Kaplan-Meier analysis. The impact of pulmonary artery hypertension (PAH)-targeted therapy on survival was assessed using multivariable Cox regression.


Estimated survival at 1, 2, and 3 years was 93% (95% confidence interval [CI], 90-95%), 91% (95% CI, 87-93%), and 89% (95% CI, 86-92%), respectively, among patients undergoing pulmonary endarterectomy (n = 404). Estimated survival at 1, 2, and 3 years was 88% (95% CI, 83-91%), 79% (95% CI, 74-83%), and 70% (95% CI, 64-76%), respectively, among patients not undergoing surgery (n = 275). Use of PAH-targeted therapy did not affect survival for either cohort. Increased mortality was associated with New York Heart Association functional class IV (HR, 4.16; 95% CI, 1.49-11.62 and HR, 4.76; 95% CI, 1.76-12.88), increased right atrial pressure (HR, 1.34; 95% CI, 0.95-1.90 and HR, 1.50; 95% CI, 1.20-1.88) and a history of cancer (HR, 3.02; 95% CI, 1.36-6.69 and HR, 2.15; 95% CI, 1.18-3.94) in operated and nonoperated patients, respectively.


The authors concluded that long-term prognosis is better for CTEPH patients who underwent pulmonary endarterectomy than patients managed solely with medical therapy.


This registry provides reassuring data on the 1-, 2-, and 3-year mortality estimates for CTEPH patients. It also highlights the importance of surgical endarterectomy for eligible patients. While the observational design is prone to potential confounding, this multicenter registry highlights the importance of mechanical obstruction in CTEPH patients that differs from other forms of PAH. Referral to high-volume multi-disciplinary programs with surgical expertise may offer optimal outcomes for CTEPH patients.

Clinical Topics: Anticoagulation Management, Arrhythmias and Clinical EP, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Vascular Medicine, Anticoagulation Management and Venothromboembolism, Pulmonary Hypertension, Interventions and Vascular Medicine, Hypertension

Keywords: Anticoagulants, Atrial Pressure, Endarterectomy, Hypertension, Hypertension, Pulmonary, Neoplasms, Pulmonary Artery, Pulmonary Embolism, Survival, Thromboembolism, Venous Thromboembolism

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