Congenital heart disease (CHD) occurs in close to 1 percent of births with over 90 percent living to adulthood. There are now more adults living with CHD than children due to successful surgical and medical treatment over the last six decades. Unfortunately, many adults with CHD have fallen out of congenital heart care either because they were told they were surgically “fixed” or because they were never referred to adult congenital heart disease (AHCD) providers upon reaching adulthood.
It is now known from decades of follow-up that there can be life-long consequences of even the simplest congenital heart surgeries. In addition, we now know that many individuals with complex CHD repairs that have a single ventricle or a systemic right ventricle will experience early heart failure, arrhythmias, and far-reaching manifestations of surgical procedures, such as the Fontan operation, that subject the body to systemic venous hypertension leading to liver cirrhosis, pulmonary hypertension, esophageal varices, protein-losing enteropathy, plastic bronchitis, etc. Many of these individuals will go on to need a heart transplant or multi-organ transplantation in their second to fourth decade of life. Continue reading