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sub-segmental vascular bed in order to perform a complete endarterectomy and obtain optimal decrease in PVR. Once the right side endarterectomy is complete, circulation is transiently restored, and subsequently the procedure is repeated with an incision in the left main pulmonary artery. While the patient is being rewarmed, the surgeon can complete any other surgical procedure that needs performed such as coronary artery bypass, or closure of a PFO. In addition to complications that are common to other types of cardiothoracic surgery such as arrhythmias, bleeding, infection, pericardial effusions and delirium, patients post PEA may also experience severe reperfusion oedema. Although usually self limited, and treated supportively, it can result in severe V/Q mismatch, profound hypoxaemia and prolonged mechanical ventilation.21

Outcomes after surgery Peri-operative mortality seems to have decreased substantially over the years from 16% pre-1990, to less than 5% in recent series.6,17,22


usually improve dramatically and immediately after a successful PEA surgery. Pulmonary artery pressures and pulmonary vascular resistance can decrease by up to 70% and PVRs in the range of 200 to 350 dyne seconds/cm5 are frequently achieved.23,24

Long-term outcomes after successful surgery are also impressive. In those that survive the post-operative period, mortality is low, with one study showing a 4% PH-related mortality in 137 patients during a four-year follow-up.25


thromboembolism post operatively is extremely rare, especially in those who are well maintained on long-term anticoagulation, and who have an inferior vena cava (IVC) filter placed pre- operatively.

The haemodynamic improvements seen immediately post-operatively persist long term. Impressive functional improvements also occur. In one study, the vast majority of patients were NYHA class III or IV pre-operatively, and improved to NYHA class I or II post- operatively.26


The reasons for the residual, post-operative PH may include an incomplete endarterectomy and/or pre-existing small vessel arteriopathy from long-term exposure to elevated

A certain percentage of patients, 5–35% remain with persistent PH despite PEA.27–29

pulmonary flow and pressures. These patients may be candidates for medical therapy, which is discussed elsewhere.


Although CTEPH is a common cause of PH, it remains consistently underdiagnosed. The diagnosis of this condition requires a systematic approach including echocardiography, V/Q lung scanning, CTA and usually right heart catheterisation as well as pulmonary angiography. MRI angiography may play a role in some centres. Referral of all diagnosed patients to an expert centre to confirm the diagnosis, and assess for surgical resectability is mandatory. PEA can be curative, and portends good short- and long-term prognoses in suitable patients. In patients deemed surgically inoperable, and in those with persistent PH post PEA surgery, medical therapy could be considered. l

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7. Raisinghani A, Ben-Yehuda O. Echocardiography in chronic thromboembolic pulmonary hypertension. Semin Thorac Cardiovasc Surg 2006;18:230–5.

8. Tunariu N 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.

9. Kim NH et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol 2013;62:D92.

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12. Bergin CJ et al. Chronic thromboembolism: diagnosis with helical CT and MR imaging with angiographic and surgical correlation. Radiology 1997;204:695–702.

13. Kreitner KF et al. Chronic thromboembolic pulmonary hypertension – assessment by magnetic resonance imaging. Eur Radiol 2003;13(10):2365–71.

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

17. Jamieson SW et al. Experience and results with 150 pulmonary thromboendarterectomy operations over a 29-month period. J Thorac Cardiovasc Surg 1993;106:116–26; discussion 126–7.

18. Zund G et al. Improved exposure of the pulmonary arteries for thromboendarterectomy. Ann Thorac Surg 1998;66:1821–3.

19. Thistlethwaite PA et al. Technique and outcomes of pulmonary endarterectomy surgery. Ann Thorac Cardiovasc Surg 2008;14:274–82.

20. Madani MM, Jamieson SW. Technical advances of pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. Semin Thorac Cardiovasc Surg 2006;18:243–9.

21. Levinson RM et al. Reperfusion pulmonary edema after pulmonary artery thromboendarterectomy. Am Rev Respir Dis 1986;134:1241–5.

22. Jamieson SW et al. Pulmonary endarterectomy: experience and lessons learned in 1,500 cases. Ann Thorac Surg 2003;76:1457–62; discussion 1462–4.

23. Skoro-Sajer N et al. Surgical specimens, haemodynamics and long-term outcomes after pulmonary endarterectomy. Thorax 2014;69:116–22.

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

25. Corsico AG et al. Long-term outcome after pulmonary endarterectomy. Am J Respir Crit Care Med 2008;178:419–24

26. Archibald CJ et al. Long-term outcome after pulmonary thromboendarterectomy. Am J Respir Crit Care Med 1999;160:523–8.

27. Bonderman D et al. Predictors of outcome in chronic thromboembolic pulmonary hypertension. Circulation 2007;115:2153–8.

28. Thistlethwaite PA et al. Outcomes of pulmonary endarterectomy for treatment of extreme thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg 2006;131:307–13.

29. Freed DH et al. Survival after pulmonary thromboendarterectomy: effect of residual pulmonary hypertension. J Thorac Cardiovasc Surg 2011;141:383–7.

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