This chapter summarizes current knowledge about chronic thromboembolic pulmonary hypertension (CTEPH), and discusses the perioperative and anesthetic management of patients scheduled for pulmonary endarterectomy (PEA). It discusses the pathophysiology, prognosis, diagnosis, and treatment for CTEPH. Approximately half of the patients exhibit known thrombotic tendencies like protein-C deficiency, factor V Leiden deficiency, anti-phospholipid syndrome or other autoimmune disorders. Computerized tomography scanning with pulmonary angiography is a useful tool in distinguishing between proximal and distal disease. The peri-operative management of patients undergoing pulmonary endarterectomy is complex and involves a multidisciplinary team of physicians, surgeons, anesthetists and intensivists. Thorough preoperative investigation and ascertaining the appropriateness of surgery is essential. Generally quoted and accepted peri-operative mortality risk is 10%, down to 4% in centers with large experience. In most surviving patients, a marked reduction in the pulmonary arterial pressure is observed and right heart function will return to normal within a few months.

This chapter deals with anesthesia for surgery to major intrathoracic structures excluding the heart and great vessels in neonates, infants and children. It focuses on thorough pre-operative assessment, intra-operative management including techniques for one-lung ventilation (OLV) and post-operative care including analgesia as well as a brief discussion of several conditions which present specifically in the pediatric patient population. Each phase of the anesthetic management of the pediatric thoracic patient should be carefully planned in advance and anesthetic nursing staff should be fully briefed. Two-lung ventilation is usually a perfectly acceptable and a simpler technique for many surgical procedures. To help reduce the chance of damaging the lung, apnea can be maintained while the thoracic cavity is entered. Providing satisfactory peri-operative management of the pediatric thoracic patient requires the highest standards of pre-operative assessment, technical expertise and communication between team members, but is both challenging and rewarding.