Top

Published in:

Open Access 01-12-2014 | Review Article

Chronic thromboembolic pulmonary hypertension

Authors: B. E. Schölzel, R. J. Snijder, J. J. Mager, H W. van Es, H. W. M. Plokker, H. J. Reesink, W. J. Morshuis, M. C. Post

Published in: Netherlands Heart Journal | Issue 12/2014

Abstract

Chronic pulmonary thromboembolic disease is an important cause of severe pulmonary hypertension, and as such is associated with significant morbidity and mortality. The prognosis of this condition reflects the degree of associated right ventricular dysfunction, with predictable mortality related to the severity of the underlying pulmonary hypertension. Left untreated, the prognosis is poor. Pulmonary endarterectomy is the treatment of choice to relieve pulmonary artery obstruction in patients with chronic thromboembolic pulmonary hypertension and has been remarkably successful. Advances in surgical techniques along with the introduction of pulmonary hypertension-specific medication provide therapeutic options for the majority of patients afflicted with the disease. However, a substantial number of patients are not candidates for pulmonary endarterectomy due to either distal pulmonary vascular obstruction or significant comorbidities. Therefore, careful selection of surgical candidates in expert centres is paramount. The current review focuses on the diagnostic approach to chronic thromboembolic pulmonary hypertension and the available surgical and medical therapeutic options.

Available only for authorised users

Fedullo PF, Auger WR, Kerr KM, et al. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2001;345:1465–72.PubMedCrossRef

Fedullo P, Kerr KM, Kim NH, et al. Chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2011;183:1605–13.PubMedCrossRef

Klok FA, van Kralingen KW, van Dijk AP, et al. Prospective cardiopulmonary screening program to detect chronic thromboembolic pulmonary hypertension in patients after acute pulmonary embolism. Haematologica. 2010;95:970–5.PubMedCentralPubMedCrossRef

Pengo V, Lensing AW, Prins MH, et al. Thromboembolic pulmonary hypertension study group. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med. 2004;350:2257–64.PubMedCrossRef

Den Exter PL, van der Hulle T, Lankeit M, et al. Long-term clinical course of acute pulmonary embolism. Blood Rev. 2013;27:185–92.CrossRef

Bonderman D, Jakowitsch J, Adlbrecht C, et al. Medical conditions increasing the risk of chronic thromboembolic pulmonary hypertension. Thromb Haemost. 2005;93:512–6.PubMed

Condliffe R, Kiely DG, Gibbs JS, et al. Prognostic and aetiological factors in chronic thromboembolic pulmonary hypertension. Eur Respir J. 2009;33:332–8.PubMedCrossRef

Moser KM, Auger WR, Fedullo PF. Chronic major-vessel thromboembolic pulmonary hypertension. Circulation. 1990;81:1735–43.PubMedCrossRef

Horlander KT, Mannino DM, Leeper KV. Pulmonary embolism mortality in the United States, 1979–1998: an analysis using multiple-cause mortality data. Arch Intern Med. 2003;163(14):1711–7.PubMedCrossRef

10.

Riedel M, Stanek V, Widimsky J, et al. Long-term follow-up of patients with pulmonary thromboembolism. Late prognosis and evolution of hemodynamic and respiratory data. Chest. 1982;81:151–8.PubMedCrossRef

11.

Archibald CJ, Auger WR, Fedullo PF, et al. Long-term outcome after pulmonary thromboendarterectomy. Am J Respir Crit Care Med. 1999;160:523–8.PubMedCrossRef

12.

Corsico AG, D’Armini AM, Cerveri I, et al. Long-term outcome after pulmonary endarterectomy. Am J Respir Crit Care Med. 2008;178:419–24.PubMedCrossRef

13.

Saouti N, Morshuis WJ, Heijmen RH, et al. Long-term outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: a single institution experience. Eur J Cardiothorac Surg. 2009;35:947–52.PubMedCrossRef

14.

Mayer E, Jenkins D, Lindner J, et al. Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg. 2011;141:702–10.PubMedCrossRef

15.

Auger WR, Fedullo PF. Chronic thromboembolic pulmonary hypertension. Semin Respir Crit Care Med. 2009;30:471–83.PubMedCrossRef

16.

Peacock A, Simonneau G, Rubin L. Controversies, uncertainties and future research on the treatment of chronic thromboembolic pulmonary hypertension. Proc Am Thorac Soc. 2006;3:608–14.PubMedCrossRef

17.

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.PubMedCrossRef

18.

Bonderman D, Wilkens H, Wakounig S, et al. Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir J. 2009;33:325–31.PubMedCrossRef

19.

de Perrot M, Fadel E, McRae K, et al. Evaluation of persistent pulmonary hypertension after acute pulmonary embolism. Chest. 2007;132:780–5.PubMedCrossRef

20.

Ribeiro A, Lindmarker P, Johnsson H, et al. Pulmonary embolism: one-year follow-up with echocardiography doppler and five-year survival analysis. Circulation. 1999;99:1325–30.PubMedCrossRef

21.

Moser KM, Bloor CM. Pulmonary vascular lesions occurring in patients with chronic major vessel thromboembolic pulmonary hypertension. Chest. 1993;103:685–92.PubMedCrossRef

22.

Lang IM, Pesavento R, Bonderman D, et al. Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding. Eur Respir J. 2013;41:462–8.PubMedCrossRef

23.

Goto K. Basic and therapeutic relevance of endothelin-mediated regulation. Biol Pharm Bull. 2001;24:1219–30.PubMedCrossRef

24.

Bauer M, Wilkens H, Langer F, et al. Selective upregulation of endothelin B receptor gene expression in severe pulmonary hypertension. Circulation. 2002;105:1034–6.PubMedCrossRef

25.

Reesink HJ, Meijer RC, Lutter R, et al. Hemodynamic and clinical correlates of endothelin-1 in chronic thromboembolic pulmonary hypertension. Circ J. 2006;70:1058–63.PubMedCrossRef

26.

Jaïs X, D’Armini AM, Jansa P, et al. Bosentan effects in iNopErable forms of chronIc thromboembolic pulmonary hypertension study group. 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.PubMedCrossRef

27.

van der Plas MN, Reesink HJ, Roos CM, et al. Pulmonary endarterectomy improves dyspnea by the relief of dead space ventilation. Ann Thorac Surg. 2010;89:347–52.PubMedCrossRef

28.

van Wolferen SA, Marcus JT, Westerhof N, et al. Right coronary artery flow impairment in patients with pulmonary hypertension. Eur Heart J. 2008;29:120–7.PubMedCrossRef

29.

Klok FA, Surie S, Kempf T, et al. A simple non-invasive diagnostic algorithm for ruling out chronic thromboembolic pulmonary hypertension in patients after acute pulmonary embolism. Thromb Res. 2011;128:21–6.PubMedCrossRef

30.

Piazza G, Goldhaber SZ. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2011;364:351–60.PubMedCrossRef

31.

Correale M, Montrone D, Ieva R, et al. Pulmonary arterial hypertension: an update. Neth Heart J. 2012;20:138.PubMedCentralPubMedCrossRef

32.

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.PubMedCrossRef

33.

Willemink MJ, van Es HW, Koobs L, et al. CT evaluation of chronic thromboembolic pulmonary hypertension. Clin Radiol. 2012;67:277–85.PubMedCrossRef

34.

Galiè N, Hoeper MM, Humbert M, et al. ESC committee for practice guidelines (CPG). 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. 2009;30:2493–537.PubMedCrossRef

35.

Reesink HJ, Marcus JT, Tulevski II, et al. Reverse right ventricular remodeling after pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension: utility of magnetic resonance imaging to demonstrate restoration of the right ventricle. J Thorac Cardiovasc Surg. 2007;133:58–64.PubMedCrossRef

36.

Kreitner KF, Ley S, Kauczor HU, et al. Chronic thromboembolic pulmonary hypertension: pre- and postoperative assessment with breath-hold MR imaging techniques. Radiology. 2004;232:535–43.PubMedCrossRef

37.

Auger WR, Fedullo PF, Moser KM, et al. Chronic major-vessel thromboembolic pulmonary artery obstruction: appearance at angiography. Radiology. 1992;182:393–8.PubMedCrossRef

38.

Jamieson SW, Kapelanski DP, Sakakibara N, et al. Pulmonary endarterectomy: experience and lessons learned in 1,500 cases. Ann Thorac Surg. 2003;76:1457–62.PubMedCrossRef

39.

Jenkins DP, Madani M, Mayer E, et al. Surgical treatment of chronic thromboembolic pulmonary hypertension. Eur Respir J. 2013;41:735–42.PubMedCrossRef

40.

Houk VN, Hufnagel CA, McClenathan JE, et al. Chronic thrombotic obstruction of major pulmonary arteries- report of a case successfully treated by thromboendarterectomy and a review of the literature. Am J Med. 1963;35:269–82.PubMedCrossRef

41.

Thistlethwaite PA, Kaneko K, Madani MM, et al. Technique and outcomes of pulmonary endarterectomy surgery. Ann Thorac Cardiovasc Surg. 2008;14:274–82.PubMed

42.

Keogh AM, Mayer E, Benza RL, et al. Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol. 2009;54(1 Suppl):S67–77.PubMedCrossRef

43.

Thistlethwaite PA, Mo M, Madani MM, et al. Operative classification of thromboembolic disease determines outcome after pulmonary endarterectomy. J Thorac Cardiovasc Surg. 2002;124:1203–11.PubMedCrossRef

44.

Levinson RM, Shure D, Moser KM. Reperfusion pulmonary edema after pulmonary artery thromboendarterectomy. Am Rev Respir Dis. 1986;134:1241–5.PubMed

45.

Kerr KM, Auger WR, Marsh JJ, et al. Efficacy of methylprednisolone in preventing lung injury following pulmonary thrombo-endarterectomy. Chest. 2012;141:27–35.PubMedCentralPubMedCrossRef

46.

Freed DH, Thomson BM, Berman M, et al. Survival after pulmonary thromboendarterectomy: effect of residual pulmonary hypertension. J Thorac Cardiovasc Surg. 2011;141:383–7.PubMedCrossRef

47.

Bonderman D, Skoro-Sajer N, Jakowitsch J, et al. Predictors of outcome in chronic thromboembolic pulmonary hypertension. Circulation. 2007;115:2153–8.PubMedCrossRef

48.

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.PubMedCrossRef

49.

Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D92–9.PubMedCrossRef

50.

McLaughlin VV, Badesch DB, Delcroix M, et al. End points and clinical trial design in pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54:S97–107.PubMedCrossRef

51.

Schölzel B, Snijder R, Morshuis W, et al. Clinical worsening after pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension. Neth Heart J. 2011;19:498–503.PubMedCentralPubMedCrossRef

52.

Schölzel BE, Post MC, Dymarkowski S, et al. Prediction of outcome after PEA in chronic thromboembolic pulmonary hypertension using indexed pulmonary artery diameter. Eur Respir J. 2014;43:909–12.PubMedCrossRef

53.

Reesink HJ, van der Plas MN, Verhey NE, et al. Six-minute walk distance as parameter of functional outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg. 2007;133:510–6.PubMedCrossRef

54.

Tscholl D, Langer F, Wendler O, et al. Pulmonary thromboendarterectomy–risk factors for early survival and hemodynamic improvement. Eur J Cardiothorac Surg. 2001;19:771–6.PubMedCrossRef

55.

Reichenberger F, Voswinckel R, Enke B, et al. Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension. Eur Respir J. 2007;30:922–7.PubMedCrossRef

56.

Hughes RJ, Jais X, Bonderman D, et al. The efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension: a 1-year follow-up study. Eur Respir J. 2006;28:138–43.PubMedCrossRef

57.

Suntharalingam J, Treacy CM, Doughty NJ, et al. Long-term use of sildenafil in inoperable chronic thromboembolic pulmonary hypertension. Chest. 2008;134:229–36.PubMedCrossRef

58.

Ghofrani HA, D’Armini AM, Grimminger F, et al. CHEST-1 study group. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013;369:319–29.PubMedCrossRef

59.

Bresser P, Fedullo PF, Auger WR, et al. Continuous intravenous epoprostenol for chronic thromboembolic pulmonary hypertension. Eur Respir J. 2004;23:595–600.PubMedCrossRef

60.

Pepke-Zaba J, Jansa P, Kim NH, et al. Chronic thromboembolic pulmonary hypertension: Role of medical therapy. Eur Respir J. 2013;41:985–90.PubMedCrossRef

61.

Reesink HJ, Surie S, Kloek JJ, et al. Bosentan as a bridge to pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg. 2010;139:85–91.PubMedCrossRef

62.

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.PubMedCrossRef

Title: Chronic thromboembolic pulmonary hypertension
Authors: B. E. Schölzel
R. J. Snijder
J. J. Mager
H W. van Es
H. W. M. Plokker
H. J. Reesink
W. J. Morshuis
M. C. Post
Publication date: 01-12-2014
Publisher: Bohn Stafleu van Loghum
Published in: Netherlands Heart Journal / Issue 12/2014
Print ISSN: 1568-5888
Electronic ISSN: 1876-6250
DOI: https://doi.org/10.1007/s12471-014-0592-2

ACC 2024 Congress Coverage

Heart Failure Video

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Chronic thromboembolic pulmonary hypertension

Abstract

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

Incentivised to exercise

New intervention for STEMI-related cardiogenic shock

» View more highlights on the ACC 2024 congress hub page

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Please log in to get access to this content

Other articles of this Issue 12/2014

A rare case of narrow QRS complex tachycardia

CMR-derived TAPSE measurement: a semi-quantitative method of right ventricular function assessment in patients with hypertrophic cardiomyopathy

Screening in asymptomatic coronary artery disease: helpful, redundant or harmful?

The history of the cardiac emergency room, and the patient’s history

Early rapidly developing constrictive pericarditis after aortic valve surgery

Cardiac magnetic resonance imaging: artefacts for clinicians

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

Incentivised to exercise

New intervention for STEMI-related cardiogenic shock

» View more highlights on the ACC 2024 congress hub page