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Delcroix, M; Lang, I; Pepke-Zaba, J; Jansa, P; D'Armini, AM; Snijder, R; Bresser, P; Torbicki, A; Mellemkjaer, S; Lewczuk, J; Simkova, I; Barberà, JA; de Perrot, M; Hoeper, MM; Gaine, S; Speich, R; Gomez-Sanchez, MA; Kovacs, G; Jaïs, X; Ambroz, D; Treacy, C; Morsolini, M; Jenkins, D; Lindner, J; Dartevelle, P; Mayer, E; Simonneau, G.
Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry.
CIRCULATION. 2016; 133(9): 859-871. Doi: 10.1161/CIRCULATIONAHA.115.016522 [OPEN ACCESS]
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Kovacs Gabor
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Abstract:
Chronic thromboembolic pulmonary hypertension, a rare complication of acute pulmonary embolism, is characterized by fibrothrombotic 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 patients who have chronic thromboembolic pulmonary hypertension. A total of 679 patients newly diagnosed with chronic thromboembolic pulmonary hypertension were prospectively included over a 24-month period. 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) 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, pulmonary arterial hypertension-targeted therapy did not affect survival estimates significantly. Mortality was associated with New York Heart Association functional class IV (hazard ratio [HR], 4.16; 95% CI, 1.49-11.62; P=0.0065 and HR, 4.76; 95% CI, 1.76-12.88; P=0.0021), increased right atrial pressure (HR, 1.34; 95% CI, 0.95-1.90; P=0.0992 and HR, 1.50; 95% CI, 1.20-1.88; P=0.0004), and a history of cancer (HR, 3.02; 95% CI, 1.36-6.69; P=0.0065 and HR, 2.15; 95% CI, 1.18-3.94; P=0.0129) in operated and not-operated patients, respectively. Additional correlates of mortality were bridging therapy with pulmonary arterial hypertension-targeted drugs, postoperative pulmonary hypertension, surgical complications, and additional cardiac procedures in operated patients, and comorbidities such as coronary disease, left heart failure, and chronic obstructive pulmonary disease in not-operated patients. The long-term prognosis of operated patients currently is excellent and better than the outcome of not-operated patients. © 2016 American Heart Association, Inc.

Find related publications in this database (Keywords)
endarterectomy
hypertension
pulmonary
pulmonary embolism
survival
thromboembolism
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