Chronic thromboembolic pulmonary hypertension (CTEPH), a rare complication of acute pulmonary embolism, is characterized by fibro-thrombotic obstructions of large pulmonary arteries combined with small vessel arteriopathy. It can be cured by pulmonary endarterectomy, and can be clinically improved by medical therapy in inoperable patients. A European registry was set up in 27 centers to evaluate long-term outcome and outcome correlates in 2 distinct populations of operated and not-operated CTEPH patients.
METHODS AND RESULTS: -A total of 679 newly diagnosed CTEPH patients were prospectively included over a 24-month period. Estimated survival at 1, 2, and 3 years was 93% (95% CI, 90-95), 91% (95% CI, 87-93), and 89% (95% CI, 86-92) in operated patients (n=404), and only 88% (95% CI, 83-91), 79% (95% CI, 74-83), and 70% (95% CI, 64-76) in not-operated patients (n=275). In both operated and not-operated patients, PAH-targeted therapy did not affect survival estimates significantly. Mortality was associated with NYHA functional class IV (HR 4.16, CI 1.49-11.62, p=0.0065 and HR 4.76, CI 1.76-12.88, p=0.0021), increased right atrial pressure (HR 1.34, CI 0.95 -1.90, p=0.0992 and HR 1.50, CI 1.20-1.88, p=0.0004), and a history of cancer (HR 3.02, CI 1.36-6.69, p=0.0065 and HR 2.15, CI 1.18-3.94, p=0.0129) in operated and not-operated patients, respectively. Additional correlates of mortality were bridging therapy with PAH-targeted drugs, postoperative PH, surgical complications, and additional cardiac procedures in operated patients, and comorbidities such as coronary disease, left heart failure, and COPD in not-operated patients.
CONCLUSIONS: -The long-term prognosis of operated patients is nowadays excellent and better than the outcome of not-operated patients.
