Acute care medicine: perioperative management of adult congenital heart disease.

Since the advent of neonatal cardiac surgery in the 1970s, an increasing number of patients suffering from congenital heart disease (CHD) have survived into adulthood. In 2010, it is estimated that 1.2 million or 1 in 150 of young adults have some form of CHD in the United States. Current birth, incidence, and survival rate predict an increase in the CHD population between 10,000 and 300,000 patients per year. Data from large adult CHD (ACHD) centers (UCLA, Toronto, Mayo Clinic) show that as many as 50% of these patients with complex physiology are 40 years of age or older and that two-thirds of them can be categorized as medium or high risk to demonstrate signs and symptoms of low cardiac output. As this population ages, it is very likely for hospital-based physicians to encounter such patients in their procedural and/or surgical practice. Risk stratification and interdisciplinary approach in the care of these patients will assure a safe outcome. The assessment of the patient must consider the variable expression of CHD. Because most lesions are "fixed but not cured," the periprocedural practitioner must consider the altered physiology of the heart in context of the physiological challenges of percutaneous and open surgical interventions. The 2008 American Heart Association/American College of Cardiology guidelines for the Management of Adults with CHD establish for the first time a streamlined approach to the care of these patients. This review will apply these guidelines to the patients with ACHD undergoing noncardiac surgery and intervention.