Abstract
Traditional pulmonary angiography is still the diagnostic gold standard of chronic thromboembolic pulmonary hypertension (CTEPH). Diagnosis and potential operability should be discussed in a multidisciplinary team of chest physicians, cardiologists, radiologists, and surgeons. Patients with CTEPH may be cured by pulmonary endarterectomy (PEA). However, while up to 50% of patients are not operable, it is estimated that in 10–20% of cases pulmonary hypertension persists or recurs after surgery. For these patients, no medical treatments have been approved.
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References
Pengo V, Lensing AW, Prins MH, Marchiori A, Davidson BL, Tiozzo F, Albanese P, Biasiolo A, Pegoraro C, Iliceto S, Prandoni P (2004) Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 350(22):2257–2264
Bonderman D, Wilkens H, Wakounig S, Schäfers HJ, Jansa P, Lindner J, Simkova I, Martischnig AM, Dudczak J, Sadushi R, Skoro-Sajer N, Klepetko W, Lang IM (2009) Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir J 33(2):325–331 (Epub 2008 Sep 17)
Wolf M, Boyer-Neumann C, Parent F, Eschwege V, Jaillet H, Meyer D, Simonneau G (2000) Thrombotic risk factors in pulmonary hypertension. Eur Respir J 15(2):395–399
Egermayer P, Peacock AJ (2000) Is pulmonary embolism a common cause of chronic pulmonary hypertension? Limitations of the embolic hypothesis. Eur Respir J 15(3):440–448
Humbert M, Sitbon O, Simonneau G (2004) Treatment of pulmonary arterial hypertension. N Engl J Med 351(14):1425–1436
Keogh AM, Mayer E, Benza RL, Corris P, Dartevelle PG, Frost AE, Kim NH, Lang IM, Pepke-Zaba J, Sandoval J (2009) Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol 54(1 Suppl):67–77. http://www.ncbi.nlm.nih.gov/pubmed/19555860
Dartevelle P, Fadel E, Mussot S, Chapelier A, Hervé P, de Perrot M, Cerrina J, Ladurie FL, Lehouerou D, Humbert M, Sitbon O, Simonneau G (2004) Chronic thromboembolic pulmonary hypertension. Eur Respir J 23(4):637–648
Galiè N, Hoeper MM, Humbert M et al (2009) 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 30:2493–2537
Jensen KW, Kerr KM, Fedullo PF, Kim NH, Test VJ, Ben-Yehuda O, Auger WR (2009) Pulmonary hypertensive medical therapy in chronic thromboembolic pulmonary hypertension before pulmonary thromboendarterectomy. Circulation 120(13):1248–1254 (Epub 2009 Sep 14)
Condliffe R, Kiely DG, Gibbs JS, Corris PA, Peacock AJ, Jenkins DP, Hodgkins D, Goldsmith K, Hughes RJ, Sheares K, Tsui SS, Armstrong IJ, Torpy C, Crackett R, Carlin CM, Das C, Coghlan JG, Pepke-Zaba J (2008) Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med 177(10):1122–1127 (Epub 2008 Feb 21)
Kramm T, Eberle B, Guth S, Mayer E (2005) Inhaled iloprost to control residual pulmonary hypertension following pulmonary endarterectomy. Eur J Cardiothorac Surg 28(6):882–888 (Epub 2005 Oct 19)
Acknowledgments
This article is based on presentations given at the “5th International Ventavis® Symposium 2009, Athens”, sponsored by Bayer Schering Pharma AG.
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Humbert, M., Lang, I.M. & Mayer, E. Chronic thromboembolic pulmonary hypertension (CTEPH): specific disease characteristics and similarities to idiopathic pulmonary arterial hypertension. Clin Res Cardiol Suppl 5 (Suppl 2), 12–15 (2010). https://doi.org/10.1007/s11789-010-0019-6
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DOI: https://doi.org/10.1007/s11789-010-0019-6