Science and Research

Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry

BACKGROUND: 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. METHODS AND RESULTS: 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. CONCLUSIONS: The long-term prognosis of operated patients currently is excellent and better than the outcome of not-operated patients.

  • Delcroix, M.
  • Lang, I.
  • Pepke-Zaba, J.
  • Jansa, P.
  • D'Armini, A. M.
  • Snijder, R.
  • Bresser, P.
  • Torbicki, A.
  • Mellemkjaer, S.
  • Lewczuk, J.
  • Simkova, I.
  • Barbera, J. A.
  • de Perrot, M.
  • Hoeper, M. M.
  • Gaine, S.
  • Speich, R.
  • Gomez-Sanchez, M. A.
  • Kovacs, G.
  • Jais, X.
  • Ambroz, D.
  • Treacy, C.
  • Morsolini, M.
  • Jenkins, D.
  • Lindner, J.
  • Dartevelle, P.
  • Mayer, E.
  • Simonneau, G.

Keywords

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Chronic Disease
  • Female
  • Humans
  • Hypertension, Pulmonary/diagnosis/*epidemiology/*therapy
  • *Internationality
  • Male
  • Middle Aged
  • Prospective Studies
  • Pulmonary Embolism/diagnosis/*epidemiology/*therapy
  • *Registries
  • Time Factors
  • Treatment Outcome
  • Young Adult
  • endarterectomy
  • hypertension, pulmonary
  • pulmonary embolism
  • survival
  • thromboembolism
Publication details
DOI: 10.1161/CIRCULATIONAHA.115.016522
Journal: Circulation
Pages: 859-71 
Number: 9
Work Type: Original
Location: BREATH, UGMLC
Disease Area: PH
Partner / Member: JLU, MHH
Access-Number: 26826181
See publication on PubMed

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