Preoperative Risk Assessment and Perioperative Management of Adults with Congenital Heart Disease Undergoing Non-Cardiac Surgery

Adults with congenital heart disease (ACHD) constitute a growing population with complex cardiac physiopathology and frequent extra-cardiac involvement. The recent dramatic improvement of their life expectancy has resulted in an increasing proportion of ACHD patients requiring non-cardiac surgery. While a large body of evidence demonstrated the importance of an accurate risk assessment in patients with acquired heart disease before non-cardiac surgery in order to reduce perioperative morbidity and mortality and detailed algorithms have been released by international societies, no specific guidelines are available for the perioperative management in this population. Nonetheless, understanding the complex anatomy and unusual physiology of both repaired and unrepaired congenital heart disease is paramount to meet the unique needs of these patients and to ensure an adequate perioperative management and prevention of complications. Furthermore, anaesthesiologists and surgeons unfamiliar with congenital heart disease may be not aware of the variety of different disease-related issues, which may arise in ACHD patients during the hemodynamic changes of the perioperative phase, with possible severe adverse effects on cardiac performance. We herein review the limited evidence from the literature and summarize our personal experience in a tertiary ACHD centre in order to propose a first structured approach for preoperative risk assessment and perioperative management to reduce the mortality and morbidity risk in adults with congenital heart disease undergoing non-cardiac surgery.