Long-term outcomes in congenital heart disease

The long-term survival of children with congenital heart disease (CHD) continues to improve due to advancements in cardiac surgery and perioperative care and enhancements in cardiovascular diagnostic and interventional capabilities. As the mortality rate associated with congenital heart defects has continued to decline, the number of adults with CHD has increased exponentially. In fact, it is estimated that there are now more than one million adults living with CHD. While ongoing improvement in short-term results remains important, the clinical focus of medical caregivers has expanded to evaluating late outcomes. Understanding the long-term outcomes of our CHD survivors allows for intervention opportunities to prevent these late complications and treat them if they are present. The late outcomes encompass not only cardiac specific physiologic and anatomic abnormalities, but also important non-cardiac sequelae such as neurodevelopmental and psychosocial morbidity on the individual and their families. This chapter will describe the long-term outcomes of specific CHD lesions. The specific CHD lesions are grouped into broad categories including left-to-right shunts, right and leftsided obstructive lesions, mixing lesions, and single ventricle anatomies. The evaluation of neurodevelopmental outcomes is discussed as we attempt to better understand brain development in CHD patients and minimize brain injury that may result from the underlying CHD lesion or the therapies required to repair or palliate the defect. Each section begins with an overview of the anatomy and physiology of the lesion followed by a brief description of the surgical intervention. Complications and long-term outcomes are then described for each lesion.