Marion Delcroix1, Irene Lang2, Joanna Pepke-Zaba2, Pavel Jansa2, Andrea M D'Armini2, Repke Snijder2, Paul Bresser2, Adam Torbicki2, Sören Mellemkjaer2, Jerzy Lewczuk2, Iveta Simkova2, Joan A Barberà2, Marc de Perrot2, Marius M Hoeper2, Sean Gaine2, Rudolf Speich2, Miguel A Gomez-Sanchez2, Gabor Kovacs2, Xavier Jaïs2, David Ambroz2, Carmen Treacy2, Marco Morsolini2, David Jenkins2, Jaroslav Lindner2, Philippe Dartevelle2, Eckhard Mayer2, Gérald Simonneau2. 1. From KU Leuven - University of Leuven, University Hospitals of Leuven, Belgium (M.D.); Medical University of Vienna, Austria (I.L.); Papworth Hospital, Cambridge, United Kingdom (J.P.-Z., C.T., D.J.); Clinical Department of Cardiology and Angiology of the First Faculty of Medicine and General Teaching Hospital, Prague; Czech Republic (P.J., D.A., J.L.); San Matteo Hospital, University of Pavia, Italy (A.M.D., M.M.); St Antonius Ziekenhuis, Nieuwegein, The Netherlands (R.S.); OLVG, Amsterdam, The Netherlands (P.B.); Medical Center for Postgraduate Education, ECZ-Otwock, Poland (A.T.); Aarhus University Hospital, Skejby, Denmark (S.M.); Regional Specialist Hospital and Medical University, Wroclaw, Poland (J.L.); Slovak Medical University and National Institute of Cardiovascular Diseases, Bratislava, Slovakia (I.S.); Hospital Clínic- IDIBAPS-CIBER Enfermedades Respiratorias, Universtiy of Barcelona, Spain (J.A.B.); Toronto General Hospital, Canada (M.d.P.); Medizinische Hochschule Hannover, Germany, and German Center of Lung Research (DZL), Hannover, Germany (M.M.H.); Mater Misericordiae University Hospital, Dublin, Ireland (S.G.); Universitätspital Zürich, Switzerland (R.S.); Hospital Universitario 12 Octubre-CIBER Enfermedades Respiratorias, Madrid, Spain (M.A.G.-S.); Medical University of Graz, Austria and Ludwig Boltzmann Institute for Lung Vascular Research, Austria (G.K.); University Paris Sud (Paris XI), INSERM U 999, Hôpital Le Kremlin Bicêtre, France (X.J., P.D., G.S.); and Kerckhoff Heart and Lung Center, Bad Nauheim, Germany (E.M.). marion.delcroix@uzleuven.be. 2. From KU Leuven - University of Leuven, University Hospitals of Leuven, Belgium (M.D.); Medical University of Vienna, Austria (I.L.); Papworth Hospital, Cambridge, United Kingdom (J.P.-Z., C.T., D.J.); Clinical Department of Cardiology and Angiology of the First Faculty of Medicine and General Teaching Hospital, Prague; Czech Republic (P.J., D.A., J.L.); San Matteo Hospital, University of Pavia, Italy (A.M.D., M.M.); St Antonius Ziekenhuis, Nieuwegein, The Netherlands (R.S.); OLVG, Amsterdam, The Netherlands (P.B.); Medical Center for Postgraduate Education, ECZ-Otwock, Poland (A.T.); Aarhus University Hospital, Skejby, Denmark (S.M.); Regional Specialist Hospital and Medical University, Wroclaw, Poland (J.L.); Slovak Medical University and National Institute of Cardiovascular Diseases, Bratislava, Slovakia (I.S.); Hospital Clínic- IDIBAPS-CIBER Enfermedades Respiratorias, Universtiy of Barcelona, Spain (J.A.B.); Toronto General Hospital, Canada (M.d.P.); Medizinische Hochschule Hannover, Germany, and German Center of Lung Research (DZL), Hannover, Germany (M.M.H.); Mater Misericordiae University Hospital, Dublin, Ireland (S.G.); Universitätspital Zürich, Switzerland (R.S.); Hospital Universitario 12 Octubre-CIBER Enfermedades Respiratorias, Madrid, Spain (M.A.G.-S.); Medical University of Graz, Austria and Ludwig Boltzmann Institute for Lung Vascular Research, Austria (G.K.); University Paris Sud (Paris XI), INSERM U 999, Hôpital Le Kremlin Bicêtre, France (X.J., P.D., G.S.); and Kerckhoff Heart and Lung Center, Bad Nauheim, Germany (E.M.).
Abstract
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.
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.
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