Guidelines Summary

The following guidelines are available:

Chronic thromboembolic pulmonary hypertension, from the Proceedings of the 6th World Symposium on Pulmonary Hypertension ^[2]
2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension, from the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology and the European Respiratory Society and endorsed by the Association for European Paediatric and Congenital Cardiology and the International Society for Heart and Lung Transplantation ^[31]

Medication

Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019 Jan. 53 (1):[QxMD MEDLINE Link].
Kim NH, Delcroix M, Jais X, Madani MM, Matsubara H, Mayer E, et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019 Jan. 53 (1):[QxMD MEDLINE Link].
Fedullo P, Kerr KM, Kim NH, Auger WR. Chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2011 Jun 15. 183 (12):1605-13. [QxMD MEDLINE Link].
Wagenvoort CA. Pathology of pulmonary thromboembolism. Chest. 1995 Jan. 107 (1 Suppl):10S-17S. [QxMD MEDLINE Link].
Hoeper MM, Madani MM, Nakanishi N, Meyer B, Cebotari S, Rubin LJ. Chronic thromboembolic pulmonary hypertension. Lancet Respir Med. 2014 Jul. 2 (7):573-82. [QxMD MEDLINE Link].
Piazza G, Goldhaber SZ. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2011 Jan 27. 364 (4):351-60. [QxMD MEDLINE Link].
Lang IM, Dorfmüller P, Vonk Noordegraaf A. The Pathobiology of Chronic Thromboembolic Pulmonary Hypertension. Ann Am Thorac Soc. 2016 Jul. 13 Suppl 3:S215-21. [QxMD MEDLINE Link].
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Dorfmüller P, Günther S, Ghigna MR, Thomas de Montpréville V, Boulate D, Paul JF, et al. Microvascular disease in chronic thromboembolic pulmonary hypertension: a role for pulmonary veins and systemic vasculature. Eur Respir J. 2014 Nov. 44 (5):1275-88. [QxMD MEDLINE Link].
Yan L, Li X, Liu Z, Zhao Z, Luo Q, Zhao Q, et al. Research progress on the pathogenesis of CTEPH. Heart Fail Rev. 2019 Nov. 24 (6):1031-1040. [QxMD MEDLINE Link].
Mahmud E, Madani MM, Kim NH, Poch D, Ang L, Behnamfar O, et al. Chronic Thromboembolic Pulmonary Hypertension: Evolving Therapeutic Approaches for Operable and Inoperable Disease. J Am Coll Cardiol. 2018 May 29. 71 (21):2468-2486. [QxMD MEDLINE Link].
Karimi M, Cohan N. Cancer-associated thrombosis. Open Cardiovasc Med J. 2010 Feb 23. 4:78-82. [QxMD MEDLINE Link].
Bonderman D, Wilkens H, Wakounig S, Schäfers HJ, Jansa P, Lindner J, et al. Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir J. 2009 Feb. 33 (2):325-31. [QxMD MEDLINE Link].
Yaoita N, Satoh K, Satoh T, Sugimura K, Tatebe S, Yamamoto S, et al. Thrombin-Activatable Fibrinolysis Inhibitor in Chronic Thromboembolic Pulmonary Hypertension. Arterioscler Thromb Vasc Biol. 2016 Jun. 36 (6):1293-301. [QxMD MEDLINE Link].
Pepke-Zaba J, Delcroix M, Lang I, et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation. 2011 Nov 1. 124 (18):1973-81. [QxMD MEDLINE Link].
Quarck R, Wynants M, Verbeken E, Meyns B, Delcroix M. Contribution of inflammation and impaired angiogenesis to the pathobiology of chronic thromboembolic pulmonary hypertension. Eur Respir J. 2015 Aug. 46 (2):431-43. [QxMD MEDLINE Link].
Wolf M, Boyer-Neumann C, Parent F, Eschwege V, Jaillet H, Meyer D, et al. Thrombotic risk factors in pulmonary hypertension. Eur Respir J. 2000 Feb. 15 (2):395-9. [QxMD MEDLINE Link].
Kim NH. Group 4 Pulmonary Hypertension: Chronic Thromboembolic Pulmonary Hypertension: Epidemiology, Pathophysiology, and Treatment. Cardiol Clin. 2016 Aug. 34 (3):435-41. [QxMD MEDLINE Link].
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Lang IM. Chronic thromboembolic pulmonary hypertension--not so rare after all. N Engl J Med. 2004 May 27. 350 (22):2236-8. [QxMD MEDLINE Link].
Becattini C, Agnelli G, Pesavento R, Silingardi M, Poggio R, Taliani MR, et al. Incidence of chronic thromboembolic pulmonary hypertension after a first episode of pulmonary embolism. Chest. 2006 Jul. 130 (1):172-5. [QxMD MEDLINE Link].
Poli D, Grifoni E, Antonucci E, Arcangeli C, Prisco D, Abbate R, et al. Incidence of recurrent venous thromboembolism and of chronic thromboembolic pulmonary hypertension in patients after a first episode of pulmonary embolism. J Thromb Thrombolysis. 2010 Oct. 30 (3):294-9. [QxMD MEDLINE Link].
Kitamukai O, Sakuma M, Takahashi T, Kagaya Y, Watanabe J, Shirato K. Incidence and characteristics of pulmonary thromboembolism in Japan 2000. Intern Med. 2003 Nov. 42 (11):1090-4. [QxMD MEDLINE Link].
Held M, Grün M, Holl R, Walter F, Schäfers HJ, Graeter T, et al. [Chronic thromboembolic pulmonary hypertension: Time delay from onset of symtoms to diagnosis and clinical condition at diagnosis]. Dtsch Med Wochenschr. 2014 Aug. 139 (33):1647-52. [QxMD MEDLINE Link].
Gall H, Hoeper MM, Richter MJ, Cacheris W, Hinzmann B, Mayer E. An epidemiological analysis of the burden of chronic thromboembolic pulmonary hypertension in the USA, Europe and Japan. Eur Respir Rev. 2017 Mar 31. 26 (143):[QxMD MEDLINE Link].
D'Armini AM, Zanotti G, Ghio S, Magrini G, Pozzi M, Scelsi L, et al. Reverse right ventricular remodeling after pulmonary endarterectomy. J Thorac Cardiovasc Surg. 2007 Jan. 133 (1):162-8. [QxMD MEDLINE Link].
Delcroix M, Lang I, Pepke-Zaba J, et al. Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry. Circulation. 2016 Mar 1. 133 (9):859-71. [QxMD MEDLINE Link].
Mayer E, Jenkins D, Lindner J, D'Armini A, Kloek J, Meyns B, et al. Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg. 2011 Mar. 141 (3):702-10. [QxMD MEDLINE Link].
Moser KM, Auger WR, Fedullo PF. Chronic major-vessel thromboembolic pulmonary hypertension. Circulation. 1990 Jun. 81 (6):1735-43. [QxMD MEDLINE Link].
Marshall PS, Kerr KM, Auger WR. Chronic thromboembolic pulmonary hypertension. Clin Chest Med. 2013 Dec. 34 (4):779-97. [QxMD MEDLINE Link].
[Guideline] Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint 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: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016 Jan 1. 37 (1):67-119. [QxMD MEDLINE Link].
Wilkens H, Konstantinides S, Lang IM, et al. Chronic thromboembolic pulmonary hypertension (CTEPH): Updated Recommendations from the Cologne Consensus Conference 2018. Int J Cardiol. 2018 Dec 1. 272S:69-78. [QxMD MEDLINE Link].
Farber HW, Foreman AJ, Miller DP, McGoon MD. REVEAL Registry: correlation of right heart catheterization and echocardiography in patients with pulmonary arterial hypertension. Congest Heart Fail. 2011 Mar-Apr. 17 (2):56-64. [QxMD MEDLINE Link].
Frost A, Badesch D, Gibbs JSR, Gopalan D, Khanna D, Manes A, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019 Jan. 53 (1):[QxMD MEDLINE Link].
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Tunariu N, Gibbs SJ, Win Z, Gin-Sing W, Graham A, Gishen P, 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 May. 48 (5):680-4. [QxMD MEDLINE Link].
Lisbona R, Kreisman H, Novales-Diaz J, Derbekyan V. Perfusion lung scanning: differentiation of primary from thromboembolic pulmonary hypertension. AJR Am J Roentgenol. 1985 Jan. 144 (1):27-30. [QxMD MEDLINE Link].
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Bergin CJ, Park KJ. Lymph node enlargement in pulmonary arterial hypertension due to chronic thromboembolism. J Med Imaging Radiat Oncol. 2008 Feb. 52 (1):18-23. [QxMD MEDLINE Link].
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Media Gallery

Ventilation perfusion scan showing bilateral large wedge-shaped mismatched perfusion defects and areas of gray indicating decreased perfusion. Right-sided heart catheterization for this patient showed combined precapillary and postcapillary pulmonary hypertension and the following: right atrial pressure 10 mm Hg, right ventricular pressure 82/8 mm Hg, pulmonary artery wedge pressure 22 mm Hg, and pulmonary artery pressure 83/27 mm Hg with a mean of 49 mm Hg. He was referred to an expert center for pulmonary endarterectomy evaluation, where he underwent pulmonary angiography. Findings from the right side showed an occluded upper lobe anterior segment, a proximal web in the upper lobe, and disease in all lower segments. Findings from the left side showed an occluded superior segment of the lower lobe with disease in basal segments, proximal web in lingula, and intact upper lobe vessels. He underwent pulmonary endarterectomy with intraoperative University of California San Diego classification of thrombus as right 1 and left 2. Postoperatively, he has had dramatic improvement in his symptoms and is off all pulmonary arterial hypertension therapy.
Extensive pulmonary emboli are seen in both lungs, with a dominant, eccentric thrombus related to the left main pulmonary artery. More distal emboli were noted in the subsegmental pulmonary arteries (not shown). This is a 42-year-old woman with a history of multiple pulmonary emboli and protein S deficiency who initially presented with severe dyspnea (New York Heart Association functional class IV) and was initiated on multiple pulmonary arterial hypertension medications, including intravenous prostacyclin. She underwent pulmonary endarterectomy, and the images of the clots that were removed intraoperatively are shown in another image.
The heart is enlarged, with right atrial and ventricular enlargement in addition to reflux of contrast into the intrahepatic inferior vena cava. The main pulmonary artery itself measures approximately 3 cm. There was mosaic attenuation of both lungs, most pronounced related to the right-middle and upper lobes (not shown).
Mosaic pattern seen on expiratory images of a chest CT scan in a patient with chronic thromboembolic pulmonary hypertension. The same patient’s ventilation/perfusion scan is also shown. The areas of hypovascularity in blood vessels with clots cause areas of relative hypoperfusion, which appear darker than normal lung and give rise to the differential opacities seen on this image.
Intraoperative clot during pulmonary endarterectomy. This is a 42-year-old woman with a history of multiple pulmonary emboli and protein S deficiency who initially presented with severe dyspnea (New York Heart Association functional class IV) and was initiated on multiple pulmonary arterial hypertension medications, including intravenous prostacyclin. Preoperative right-sided heart catheterization showed right atrial pressure of 16 mm Hg, pulmonary artery pressure of 124/29 mm Hg with a mean of 67 mm Hg, cardiac output of 4.53, and pulmonary vascular resistance of 12 Wood units. Immediate postoperative right-sided heart catheterization showed right atrial pressure of 9 mm Hg, pulmonary artery pressure of 40/15 mm Hg with a mean of 25 mm Hg, cardiac output of 5.15, and pulmonary vascular resistance of 2.9 Wood units. She came off all her pulmonary arterial hypertension therapy immediately postoperatively and continues to do well.

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