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CTEPH


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


Haemodynamics


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


Recurrent


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


30


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


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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.


Conclusions


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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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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