Surgical and post-operative treatment of chronic thromboembolic pulmonary hypertension

Patients with chronic thromboembolic pulmonary hypertension (CTEPH) have fibrous obstructions in their proximal pulmonary arteries, as well as a variable degree of secondary vascular remodelling of distal, surgically inaccessible, small pulmonary vessels. Pulmonary endarterectomy (PEA) is currently the only potentially curative treatment option for CTEPH, although it is not known whether it has a beneficial effect on established secondary remodelling. Medical pre-treatment with therapies specific for pulmonary hypertension is often used as a bridge to PEA. The aim of PEA is the mechanical removal of the proximal fibrous pulmonary artery obstruction as completely as possible while avoiding vascular injury within the lung. The challenge is to find and follow the correct pulmonary artery endarterectomy plane in the time allowed during brief periods of circulatory arrest. Learning the operative technique is a complex process and, thus, early mortality after PEA decreases with increasing levels of surgical training and experience. Most patients experience lasting symptomatic and haemodynamic improvements after PEA, with only 10-20% having persistent or recurrent pulmonary hypertension after surgery.