Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH), a potentially curable form of pulmonary hypertension with pulmonary thromboendarterectomy surgery, has been the focus of significant medical advances. In addition to new imaging modalities that are now used to help recognize and diagnose CTEPH, additional treatment options have emerged for inoperable cases. These include a newly approved medical therapy for inoperable disease or persistent/recurrent CTEPH as well as percutaneous balloon angioplasty of the pulmonary arteries. In this article, we summarize these recent advances in the field and review the related literature.
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Pengo V, Lensing AW, Prins MH, et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med. 2004;350:2257–64. Seminal prospective study that examined the incidence of chronic thromboembolic pulmonary hypertension in patients with acute pulmonary embolus and without prior history of venous thromboembolism.
Guerin L, Couturaud F, Parent F, et al. Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. Prevalence of CTEPH after pulmonary embolism. Thromb Haemost. 2014;112:598–605. Prospective study examining the prevalence of chronic thromboembolic pulmonary hypertension during a median of 26 months after an acute pulmonary embolus.
McLaughlin VV, Langer A, Tan M, et al. Contemporary trends in the diagnosis and management of pulmonary arterial hypertension: an initiative to close the care gap. Chest. 2013;143:324–32. Large study that compared reported clinical practice in pulmonary arterial hypertension patients to published guidelines by the American College of Chest Physicians using an electronic data management system.
Pepke-Zaba J, Delcroix M, Lang I, et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation. 2011;124:1973–81. Study examining clinical characteristics and management data of chronic thromboembolic pulmonary hypertension patients using a large international registry.
Tunariu N, Gibbs SJ, Win Z, et al. 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. 2007;48:680–4. Classic study showing increased sensitivity of ventilation perfusion scitnigrahy compared to CT-pulmonary angiography in the screening of patients with suspected chronic thromboembolic pulmonary hypertension.
Clements PJ, Tan M, McLaughlin VV, et al. The pulmonary arterial hypertension quality enhancement research initiative: comparison of patients with idiopathic PAH to patients with systemic sclerosis-associated PAH. Ann Rheum Dis. 2012;71:249–52.
Freeman LM. Don’t bury the V/Q scan: it’s as good as multidetector CT angiograms with a lot less radiation exposure. J Nucl Med. 2008;49:5–8.
Bergin CJ, Sirlin CB, Hauschildt JP, et al. Chronic thromboembolism: diagnosis with helical CT and MR imaging with angiographic and surgical correlation. Radiology. 1997;204:695–702.
Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62:D92–9. Recent review summarizing the conclusions of the chronic thromboembolic pulmonary hypertension task force and meeting discussions from the 5th World Symposium on Pulmonary Hypertension.
He J, Fang W, Lv B, et al. Diagnosis of chronic thromboembolic pulmonary hypertension: comparison of ventilation/perfusion scanning and multidetector computed tomography pulmonary angiography with pulmonary angiography. Nucl Med Commun. 2012;33:459–63.
Rajaram S, Swift AJ, Telfer A, et al. 3D contrast-enhanced lung perfusion MRI is an effective screening tool for chronic thromboembolic pulmonary hypertension: results from the ASPIRE Registry. Thorax. 2013;68:677–8.
Ley S, Ley-Zaporozhan J, Pitton MB, et al. Diagnostic performance of state-of-the-art imaging techniques for morphological assessment of vascular abnormalities in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Eur Radiol. 2012;22:607–16.
Liu M, Ma Z, Guo X, Zhang H, Yang Y, Wang C. Computed tomographic pulmonary angiography in the assessment of severity of chronic thromboembolic pulmonary hypertension and right ventricular dysfunction. Eur J Radiol. 2011;80:e462–9.
Sugiura T, Tanabe N, Matsuura Y, et al. Role of 320-slice CT imaging in the diagnostic workup of patients with chronic thromboembolic pulmonary hypertension. Chest. 2013;143:1070–7.
Reichelt A, Hoeper MM, Galanski M, Keberle M. Chronic thromboembolic pulmonary hypertension: evaluation with 64-detector row CT versus digital substraction angiography. Eur J Radiol. 2009;71:49–54.
Hoey ET, Mirsadraee S, Pepke-Zaba J, Jenkins DP, Gopalan D, Screaton NJ. Dual-energy CT angiography for assessment of regional pulmonary perfusion in patients with chronic thromboembolic pulmonary hypertension: initial experience. AJR Am J Roentgenol. 2011;196:524–32.
Rossi A, Attina D, Borgonovi A, et al. Evaluation of mosaic pattern areas in HRCT with Min-IP reconstructions in patients with pulmonary hypertension: could this evaluation replace lung perfusion scintigraphy? Eur J Radiol. 2012;81:e1–6.
Hurdman J, Condliffe R, Elliot CA, et al. ASPIRE registry: Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre. Eur Respir J. 2012;39:945–55.
Rajaram S, Swift AJ, Capener D, et al. Diagnostic accuracy of contrast-enhanced MR angiography and unenhanced proton MR imaging compared with CT pulmonary angiography in chronic thromboembolic pulmonary hypertension. Eur Radiol. 2012;22:310–7.
Sugiyama M, Fukuda T, Sanda Y, et al. Organized thrombus in pulmonary arteries in patients with chronic thromboembolic pulmonary hypertension; imaging with cone beam computed tomography. Jpn J Radiol. 2014;32:375–82.
Auger WR, Kim NH, Trow TK. Chronic thromboembolic pulmonary hypertension. Clin Chest Med. 2010;31:741–58.
Condliffe R, Kiely DG, Gibbs JS, et al. Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2008;177:1122–7.
Olschewski H, Simonneau G, Galiè N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002;347:322–9.
Suntharalingam J, Treacy CM, Doughty NJ, et al. Long-term use of sildenafil in inoperable chronic thromboembolic pulmonary hypertension. Chest. 2008;134:229–36.
Jaïs X, D’Armini AM, Jansa P, 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. 2008;52:2127–34. Seminal randomized control trial showing no significant improvement in outcomes in patients with chronic thromboembolic pulmonary hypertension when the endothelin receptor antagonist bosentan was compared to placebo.
Ghofrani HA, D’Armini AM, Grimminger F, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013;369:319–29. Seminal randomized control trial showing a significant improvement in outcomes in patients with chronic thromboembolic pulmonary hypertension when the soluble guanylate cyclace stimulator riociguat was compared to placebo.
Simonneau G, D’Armini AM, Ghofrani HA, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J. 2015;45:1293–302.
Jensen KW, Kerr KM, Fedullo PF, et al. Pulmonary hypertensive medical therapy in chronic thromboembolic pulmonary hypertension before pulmonary thromboendarterectomy. Circulation. 2009;120:1248–54.
Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62:D34–41.
Mo M, Kapelanski DP, Mitruka SN. Reoperative pulmonary thomboendarterectomy. Ann Thorac Surg. 1999;68:1770–7.
Voorburg JA, Cats VM, Buis B, Bruschke AV. Balloon angioplasty in the treatment of pulmonary hypertension caused by pulmonary embolism. Chest. 1988;94:1249–53.
Feinstein JA, Goldhaber SZ, Lock JE, Ferndandes SM, Landzberg MJ. Balloon pulmonary angioplasty for treatment of chronic thromboembolic pulmonary hypertension. Circ J. 2001;103:10–3. The first larger case series describing the use of balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension in lieu of surgery.
Pitton MB, Herber S, Mayer E, Thelen M. Pulmonary balloon angioplasty of chronic thromboembolic pulmonary hypertension (CTEPH) in surgically inaccessible cases. RoFo. 2003;175:631–4.
Sugimura K, Fukumoto Y, Satoh K, et al. Percutaneous transluminal pulmonary angioplasty markedly improves pulmonary hemodynamics and long-term prognosis in patients with chronic thromboembolic pulmonary hypertension. Circ J. 2012;76:485–8. Recent case series of balloon pulmonary angioplasty with the help of optical coherence tomography in patients with more distal chronic thromboembolic pulmonary hypertension.
Kataoka M, Inami T, Hayashida K, et al. Percutaneous transluminal pulmonary angioplasty for the treatment of chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv. 2012;5:756–62. Recent case series of balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension that also showed a correlation between worse clinical presentation and/or hemodynamics and post-procedure reperfusion pulmonary edema.
Mizoguchi H, Ogawa A, Munemasa M, Mikouchi H, Ito H, Matsubara H. Refined balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv. 2012;5:748–55. Largest case series to date, reporting on the use of balloon pulmonary angioplasty in the treatment of chronic thromboembolic pulmonary hypertension using itnravascular utlrasonographic guidance and with a follow up of more than 2 years.
Yoshimi S, Tanabe N, Masuda M, et al. Survival and quality of life for patients with peripheral type chronic thromboembolic pulmonary hypertension. Circ J. 2008;72:958–65.
Andreassen AK, Ragnarsson A, Gude E, Geiran O, Andersen R. Balloon pulmonary angioplasty in patients with inoperable chronic thromboembolic pulmonary hypertension. Heart. 2013;99:1415–20. Smaller case series on the use of balloon pulmonary angioplasty in chronic thromboembolic pulmonray hypertension with a relatively high mortality rate.
Fukui S, Ogo T, Morita Y, et al. Right ventricular reverse remodelling after balloon pulmonary angioplasty. Eur Respir J. 2014;43:1394–402.
Taniguchi Y, Miyagawa K, Nakayama K, et al. Balloon pulmonary angioplasty: an additional treatment option to improve the prognosis of patients with chronic thromboembolic pulmonary hypertension. EuroIntervention. 2014;10:518–25. Retrospective study comparing outcomes in chronic thromboembolic pulmonary hypertension patients treated with either pulmonary thromboendarterectomy or balloon pulmonary angioplasty.
Archibald CJ, Auger WR, Fedullo PF, et al. Long-term outcome after pulmonary thromboendarterectomy. Am J Respir Crit Care Med. 1999;160:523–8.
Acknowledgments
We would like to thank Dr. Deepa Gopalan for providing the images for this manuscript.
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Dr. Papamatheakis has received speaking honoraria by Actelion Pharmaceuticals. Dr Kim has received consultancy and speaking honoraria from Actelion Pharmaceuticals and Bayer HealthCare.
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This article does not contain any studies with human or animal subjects performed by any of the authors.
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This article is part of the Topical Collection on Pulmonary Hypertension
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Papamatheakis, D.G., Kim, N.H. Advances in the Management of Chronic Thromboembolic Pulmonary Hypertension. Curr Hypertens Rep 17, 75 (2015). https://doi.org/10.1007/s11906-015-0582-9
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DOI: https://doi.org/10.1007/s11906-015-0582-9