Management of advanced adult congenital heart disease

Abstract

As surgical techniques continue to develop, for example as seen with the Norwood procedure for hypoplastic left heart syndrome, the number of patients with the most complex forms of CHD will continue to increase along with their associated long term complications. Our ability to manage these patients appropriately, therefore, must also develop alongside. The purpose of this review is to examine the management of these patients once they have developed advanced disease, including drug therapy, devices, transplant and end of life care. In particular, the unique problems posed by patients with a systemic right ventricle (RV) and univentricular circulations are discussed. DRUG THERAPY IN HEART FAILUREIn patients with complex congenital heart disease, either palliated or unoperated, the development of ventricular dysfunction and clinical heart failure are common and as discussed above, is a common cause of morbidity and mortality in this population.