Circulation. 2006;113:2011-2020
doi: 10.1161/CIRCULATIONAHA.105.602565
doi: 10.1161/CIRCULATIONAHA.105.602565
(Circulation. 2006;113:2011-2020.)
© 2006 American Heart Association, Inc.
Contemporary Reviews in Cardiovascular Medicine |
Chronic Thromboembolic Pulmonary Hypertension
From the Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany (M.M.H.); Department of Cardiothoracic and Vascular Surgery, Johannes Gutenberg University, Mainz, Germany (E.M.); Centre of Maladies Vasculaires Pulmonaires, Hôpital Antoine Béclère, Clamart, France (G.S.); and Pulmonary Vascular Program, University of California, San Diego (L.J.R.).
Correspondence to Marius M. Hoeper, MD, Department of Respiratory Medicine, Hannover Medical School, 30623 Hannover, Germany. E-mail hoeper.marius@mh-hannover.de
Key Words: embolism • pulmonary hypertension
An extract of the first 250 words of the full text is provided, because this article has no abstract. |
 |
Introduction |
|---|
Chronic thromboembolic pulmonary hypertension (CTEPH) has emerged as one of the leading causes of severe pulmonary hypertension. The disease is notoriously underdiagnosed, and the true prevalence is still unclear. CTEPH is characterized by intraluminal thrombus organization and fibrous stenosis or complete obliteration of pulmonary arteries.1 The consequence is an increased pulmonary vascular resistance resulting in pulmonary hypertension and progressive right heart failure. Vascular disobliteration by pulmonary endarterectomy (PEA) is the preferred treatment for patients with CTEPH,2 but not all patients are eligible for surgery. Recent research has provided evidence suggesting that the mechanistic view of CTEPH as a disease caused solely by obliteration of central pulmonary arteries due to organized thrombi may have been too simplistic. Pulmonary embolism, either as a single episode or as recurrent episodes, is thought to be the initiating event followed by progressive pulmonary vascular remodeling. This concept explains the clinical observation that CTEPH patients may have severe pulmonary hypertension out of proportion to the pulmonary vascular obliteration seen on a pulmonary angiogram. Thus, treatment of CTEPH often requires a multidisciplinary approach and may involve surgery, medical treatment, or both. However, many aspects of the pathogenesis of CTEPH are poorly understood, the diagnostic approach to these patients has not been standardized, and neither randomized controlled trials nor guidelines have been published in this field.
In July 2005, a group of international experts met in Zurich, Switzerland, for a comprehensive discussion of CTEPH. The members of this group are listed in the Appendix (in the online-only Data
This article has been cited by other articles:
 |
|
 |
|
  The Task Force for the Diagnosis and Treatment of, N. Galie, M. M. Hoeper, M. Humbert, A. Torbicki, J-L. Vachiery, J. A. Barbera, M. Beghetti, P. Corris, S. Gaine, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension Eur. Respir. J., December 1, 2009; 34(6): 1219 - 1263. [Full Text] [PDF]
|
|
|
|
 |
|
 N. Saouti, N. Westerhof, F. Helderman, J. T. Marcus, N. Stergiopulos, B. E. Westerhof, A. Boonstra, P. E. Postmus, and A. Vonk-Noordegraaf RC time constant of single lung equals that of both lungs together: a study in chronic thromboembolic pulmonary hypertension Am J Physiol Heart Circ Physiol, December 1, 2009; 297(6): H2154 - H2160. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Authors/Task Force Members, N. Galie, M. M. Hoeper, M. Humbert, A. Torbicki, J.-L. Vachiery, J. A. Barbera, M. Beghetti, P. Corris, S. Gaine, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT) Eur. Heart J., October 2, 2009; 30(20): 2493 - 2537. [Full Text] [PDF]
|
|
|
|
 |
|
 O. Sanchez, B. Planquette, and G. Meyer Update on acute pulmonary embolism Eur. Respir. Rev., September 1, 2009; 18(113): 137 - 147. [Full Text] [PDF]
|
|
|
|
 |
|
 A. M. Keogh, E. Mayer, R. L. Benza, P. Corris, P. G. Dartevelle, A. E. Frost, N. H. Kim, I. M. Lang, J. Pepke-Zaba, and J. Sandoval Interventional and surgical modalities of treatment in pulmonary hypertension. J. Am. Coll. Cardiol., June 30, 2009; 54(1 Suppl): S67 - S77. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. M. Hoeper, J. A. Barbera, R. N. Channick, P. M. Hassoun, I. M. Lang, A. Manes, F. J. Martinez, R. Naeije, H. Olschewski, J. Pepke-Zaba, et al. Diagnosis, assessment, and treatment of non-pulmonary arterial hypertension pulmonary hypertension. J. Am. Coll. Cardiol., June 30, 2009; 54(1 Suppl): S85 - S96. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Berman, E. Pavlushkov, E. Abraham, J. Dunning, S. Tsui, R. Hall, A. Klein, and D. P. Jenkins Pulmonary endarterectomy - an example of treatment of right ventricular after load failure MMCTS, June 12, 2009; 2009(0612): 3491. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N. Saouti, W. J. Morshuis, R. H. Heijmen, and R. J. Snijder Long-term outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: a single institution experience Eur. J. Cardiothorac. Surg., June 1, 2009; 35(6): 947 - 952. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 O. Mercier, E. Sage, M. de Perrot, L. Tu, E. Marcos, B. Decante, B. Baudet, P. Herve, P. Dartevelle, S. Eddahibi, et al. Regression of flow-induced pulmonary arterial vasculopathy after flow correction in piglets. J. Thorac. Cardiovasc. Surg., June 1, 2009; 137(6): 1538 - 1546. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
V. V. McLaughlin, S. L. Archer, D. B. Badesch, R. J. Barst, H. W. Farber, J. R. Lindner, M. A. Mathier, M. D. McGoon, M. H. Park, R. S. Rosenson, et al. ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension: A Report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association Developed in Collaboration With the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association J. Am. Coll. Cardiol., April 28, 2009; 53(17): 1573 - 1619. [Full Text] [PDF]
|
|
|
|
 |
|
 Writing Committee Members, V. V. McLaughlin, S. L. Archer, D. B. Badesch, R. J. Barst, H. W. Farber, J. R. Lindner, M. A. Mathier, M. D. McGoon, M. H. Park, et al. ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension: A Report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: Developed in Collaboration With the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association Circulation, April 28, 2009; 119(16): 2250 - 2294. [Full Text] [PDF]
|
|
|
|
 |
|
 M. Humbert Update in Pulmonary Hypertension 2008 Am. J. Respir. Crit. Care Med., April 15, 2009; 179(8): 650 - 656. [Full Text] [PDF]
|
|
|
|
 |
|
 J. Shehatha, P. Saxena, B. Clarke, J. Dunning, and I. E. Konstantinov Surgical Management of Extensive Pulmonary Artery Sarcoma Ann. Thorac. Surg., April 1, 2009; 87(4): 1269 - 1271. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
I. M. Lang Managing chronic thromboembolic pulmonary hypertension: pharmacological treatment options Eur. Respir. Rev., March 1, 2009; 18(111): 24 - 28. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. Bonderman, H. Wilkens, S. Wakounig, H-J. Schafers, P. Jansa, J. Lindner, I. Simkova, A. M. Martischnig, J. Dudczak, R. Sadushi, et al. Risk factors for chronic thromboembolic pulmonary hypertension Eur. Respir. J., February 1, 2009; 33(2): 325 - 331. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 F. Haddad, P. Couture, C. Tousignant, and A. Y. Denault The Right Ventricle in Cardiac Surgery, a Perioperative Perspective: II. Pathophysiology, Clinical Importance, and Management Anesth. Analg., February 1, 2009; 108(2): 422 - 433. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N. Skoro-Sajer, N. Hack, R. Sadushi-Kolici, D. Bonderman, J. Jakowitsch, W. Klepetko, M. A. R. Hoda, M. P. Kneussl, P. Fedullo, and I. M. Lang Pulmonary Vascular Reactivity and Prognosis in Patients With Chronic Thromboembolic Pulmonary Hypertension: A Pilot Study Circulation, January 20, 2009; 119(2): 298 - 305. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
X. Jais, A. M. D'Armini, P. Jansa, A. Torbicki, M. Delcroix, H. A. Ghofrani, M. M. Hoeper, I. M. Lang, E. Mayer, J. Pepke-Zaba, et al. Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial. J. Am. Coll. Cardiol., December 16, 2008; 52(25): 2127 - 2134. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Rich and M. Rabinovitch Diagnosis and Treatment of Secondary (Non-Category 1) Pulmonary Hypertension Circulation, November 18, 2008; 118(21): 2190 - 2199. [Full Text] [PDF]
|
|
|
|
|
|
H. A. Ghofrani, M. W. Wilkins, and S. Rich Uncertainties in the Diagnosis and Treatment of Pulmonary Arterial Hypertension Circulation, September 9, 2008; 118(11): 1195 - 1201. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Authors/Task Force Members, A. Torbicki, A. Perrier, S. Konstantinides, G. Agnelli, N. Galie, P. Pruszczyk, F. Bengel, A. J.B. Brady, D. Ferreira, et al. Guidelines on the diagnosis and management of acute pulmonary embolism: The Task Force for the Diagnosis and Management of Acute Pulmonary Embolism of the European Society of Cardiology (ESC) Eur. Heart J., September 2, 2008; 29(18): 2276 - 2315. [Full Text] [PDF]
|
|
|
|
|
|
D. H. Freed, B. M. Thomson, S. S.L. Tsui, J. J. Dunning, K. K. Sheares, J. Pepke-Zaba, and D. P. Jenkins Functional and haemodynamic outcome 1 year after pulmonary thromboendarterectomy Eur. J. Cardiothorac. Surg., September 1, 2008; 34(3): 525 - 530. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. Kearon, S. R. Kahn, G. Agnelli, S. Goldhaber, G. E. Raskob, and A. J. Comerota Antithrombotic Therapy for Venous Thromboembolic Disease: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition) Chest, June 1, 2008; 133(6_suppl): 454S - 545S. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. Condliffe, D. G. Kiely, J. S. R. Gibbs, P. A. Corris, A. J. Peacock, D. P. Jenkins, D. Hodgkins, K. Goldsmith, R. J. Hughes, K. Sheares, et al. Improved Outcomes in Medically and Surgically Treated Chronic Thromboembolic Pulmonary Hypertension Am. J. Respir. Crit. Care Med., May 15, 2008; 177(10): 1122 - 1127. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. E. Orfanos and S. Zakynthinos Heart biomarkers as prognostic tools for chronic thromboembolic pulmonary hypertension: a step forward by the fatty acid-binding protein Eur. Respir. J., May 1, 2008; 31(5): 915 - 917. [Full Text] [PDF]
|
|
|
|
|
|
M. Lankeit, C. Dellas, A. Panzenbock, N. Skoro-Sajer, D. Bonderman, M. Olschewski, K. Schafer, M. Puls, S. Konstantinides, and I. M. Lang Heart-type fatty acid-binding protein for risk assessment of chronic thromboembolic pulmonary hypertension Eur. Respir. J., May 1, 2008; 31(5): 1024 - 1029. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Scheffel, P. Stolzmann, A. Plass, A. Weber, R. Pretre, B. Marincek, and H. Alkadhi Primary intimal pulmonary artery sarcoma: A diagnostic challenge. J. Thorac. Cardiovasc. Surg., April 1, 2008; 135(4): 949 - 950. [Full Text] [PDF]
|
|
|
|
|
|
J. Suntharalingam, K. Goldsmith, V. van Marion, L. Long, C. M. Treacy, F. Dudbridge, M. R. Toshner, J. Pepke-Zaba, J. C. J. Eikenboom, and N. W. Morrell Fibrinogen A{alpha} Thr312Ala polymorphism is associated with chronic thromboembolic pulmonary hypertension Eur. Respir. J., April 1, 2008; 31(4): 736 - 741. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. Bonderman, J. Jakowitsch, B. Redwan, H. Bergmeister, M.-K. Renner, H. Panzenbock, C. Adlbrecht, A. Georgopoulos, W. Klepetko, M. Kneussl, et al. Role for Staphylococci in Misguided Thrombus Resolution of Chronic Thromboembolic Pulmonary Hypertension Arterioscler Thromb Vasc Biol, April 1, 2008; 28(4): 678 - 684. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. Haddad, R. Doyle, D. J. Murphy, and S. A. Hunt Right Ventricular Function in Cardiovascular Disease, Part II: Pathophysiology, Clinical Importance, and Management of Right Ventricular Failure Circulation, April 1, 2008; 117(13): 1717 - 1731. [Full Text] [PDF]
|
|
|
|
 |
|
 National Pulmonary Hypertension Centres of the UK Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland Heart, March 1, 2008; 94(Suppl_1): i1 - i41. [Full Text] [PDF]
|
|
|
|
 |
|
 National Pulmonary Hypertension Centres of the UK Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland Thorax, March 1, 2008; 63(Suppl_2): ii1 - ii41. [Full Text] [PDF]
|
|
|
|
|
|
B. Thomson, S. S.L. Tsui, J. Dunning, A. Goodwin, A. Vuylsteke, R. Latimer, J. Pepke-Zaba, and D. P. Jenkins Pulmonary endarterectomy is possible and effective without the use of complete circulatory arrest--the UK experience in over 150 patients Eur. J. Cardiothorac. Surg., February 1, 2008; 33(2): 157 - 163. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N. Skoro-Sajer, F. Mittermayer, A. Panzenboeck, D. Bonderman, R. Sadushi, R. Hitsch, J. Jakowitsch, W. Klepetko, M. P. Kneussl, M. Wolzt, et al. Asymmetric Dimethylarginine Is Increased in Chronic Thromboembolic Pulmonary Hypertension Am. J. Respir. Crit. Care Med., December 1, 2007; 176(11): 1154 - 1160. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. Reichenberger, R. Voswinckel, B. Enke, M. Rutsch, E. El Fechtali, T. Schmehl, H. Olschewski, R. Schermuly, N. Weissmann, H. A. Ghofrani, et al. Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension Eur. Respir. J., November 1, 2007; 30(5): 922 - 927. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. McNeil and J. Dunning Chronic thromboembolic pulmonary hypertension (CTEPH) Heart, September 1, 2007; 93(9): 1152 - 1158. [Full Text] [PDF]
|
|
|
|
|
|
M. de Perrot, E. Fadel, K. McRae, K. Tan, P. Slinger, N. Paul, S. Mak, and J. T. Granton Evaluation of Persistent Pulmonary Hypertension After Acute Pulmonary Embolism Chest, September 1, 2007; 132(3): 780 - 785. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. Suntharalingam, R. D Machado, L. D Sharples, M. R Toshner, K. K Sheares, R. J Hughes, D. P Jenkins, R. C Trembath, N. W Morrell, and J. Pepke-Zaba Demographic features, BMPR2 status and outcomes in distal chronic thromboembolic pulmonary hypertension Thorax, July 1, 2007; 62(7): 617 - 622. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N. Tunariu, S. J.R. Gibbs, Z. Win, W. Gin-Sing, A. Graham, P. Gishen, and A. AL-Nahhas Ventilation-Perfusion Scintigraphy Is More Sensitive than Multidetector CTPA in Detecting Chronic Thromboembolic Pulmonary Disease as a Treatable Cause of Pulmonary Hypertension J. Nucl. Med., May 1, 2007; 48(5): 680 - 684. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. Bonderman, N. Skoro-Sajer, J. Jakowitsch, C. Adlbrecht, D. Dunkler, S. Taghavi, W. Klepetko, M. Kneussl, and I. M. Lang Predictors of Outcome in Chronic Thromboembolic Pulmonary Hypertension Circulation, April 24, 2007; 115(16): 2153 - 2158. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 V. F. Tapson and M. Humbert Incidence and Prevalence of Chronic Thromboembolic Pulmonary Hypertension: From Acute to Chronic Pulmonary Embolism Proceedings of the ATS, September 1, 2006; 3(7): 564 - 567. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
I. Lang and K. Kerr Risk Factors for Chronic Thromboembolic Pulmonary Hypertension Proceedings of the ATS, September 1, 2006; 3(7): 568 - 570. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N. Galie and N. H. S. Kim Pulmonary Microvascular Disease in Chronic Thromboembolic Pulmonary Hypertension Proceedings of the ATS, September 1, 2006; 3(7): 571 - 576. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N. H. S. Kim Assessment of Operability in Chronic Thromboembolic Pulmonary Hypertension Proceedings of the ATS, September 1, 2006; 3(7): 584 - 588. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Mayer and W. Klepetko Techniques and Outcomes of Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension Proceedings of the ATS, September 1, 2006; 3(7): 589 - 593. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. Bresser, J. Pepke-Zaba, X. Jais, M. Humbert, and M. M. Hoeper Medical Therapies for Chronic Thromboembolic Pulmonary Hypertension: An Evolving Treatment Paradigm Proceedings of the ATS, September 1, 2006; 3(7): 594 - 600. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. J. Rubin, M. M. Hoeper, W. Klepetko, N. Galie, I. M. Lang, and G. Simonneau Current and Future Management of Chronic Thromboembolic Pulmonary Hypertension: From Diagnosis to Treatment Responses Proceedings of the ATS, September 1, 2006; 3(7): 601 - 607. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Peacock, G. Simonneau, and L. Rubin Controversies, Uncertainties and Future Research on the Treatment of Chronic Thromboembolic Pulmonary Hypertension Proceedings of the ATS, September 1, 2006; 3(7): 608 - 614. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. de Perrot, J. Granton, and E. Fadel Pulmonary hypertension after pulmonary emboli: an underrecognized condition Can. Med. Assoc. J., June 6, 2006; 174(12): 1706 - 1706. [Full Text] [PDF]
|
|