Surgical and interventional management of adult congenital heart disease

Survival of pediatric patients after surgery for congenital heart disease has consistently improved over the past 2 decades. As a consequence, more young adults are presenting with both historical and contemporary repairs, and with both anticipated and completely unanticipated complications. Surgery for adult congenital heart disease continues to evolve in Canada, and even the recent rapid progress in percutaneous and hybrid approaches to congenital heart disease has had no impact on the volume or the complexity of surgical cases. Technological advances now permit the treatment of both relatively simple and very complex anatomical and pathophysiological problems using percutaneous techniques. Managing adult congenital heart disease patients now means choosing from the surgical and interventional options available and determining which cases will truly benefit from novel therapies and which will require only what has always been done. We will continue to need wellintegrated congenital heart disease programs that permit collaboration between adult and pediatric medical and surgical subspecialists. Over the past 2 decades the survival of pediatric patients after surgery for congenital heart disease (CHD) has consistently improved, with in-hospital mortality rates now routinely below 3%. With this advance more children are now surviving and presenting as young adults with both historical and more contemporary repairs and therefore with both anticipated and completely unanticipated complications. This means we must look at the different levels of complexity of adult CHD and the array of surgical and interventional options, and determine which cases will truly benefit from novel therapies and which will require only what has always been done. Surgical management Modern surgical management of congenital heart disease began on 26 August 1938 with the first documented successful ligation of a patent ductus arteriosus (PDA) in a 7-year-old patient by Dr Robert Gross in Boston. The operation was scheduled while surgeon-in-chief Dr William Ladd was on vacation for fear that Ladd would not allow the groundbreaking surgery to go ahead. Later this led to an irreparable rift between the two surgeons, despite confirming that native defects of the heart and great vessels were amenable to surgical repair. As Dr Gross stated in his case report, at that time 50% of the children affected by PDA could be expected to die in infancy and the remainder would suffer the consequences of heart failure and, potentially, Eisenmenger syndrome, which would lead to an early death in the third or fourth decade of life. PDA ligation is now performed at the bedside in the neonatal intensive care unit in 30 minutes or less. Since this beginning, cardiac surgery for congenital defects has made great strides, but at every step there has been tension between the possible benefits of innovation and the risk of causing more harm than existing therapies or the natural history of the disease. This tension was very apparent in the 1980s when therapy for transpoDr Campbell is a congenital heart disease surgeon in the Pacific Adult Congenital Heart (PACH) clinic, Division of Cardiology, St. Paul’s Hospital. He is also a director in the Cardiac Surgery Residency Program and assistant professor at the University of British Columbia. Dr Carere is an interventional cardiologist in the PACH clinic. He is also a clinical professor in the Division of Cardiology at the University of British Columbia. This article has been peer reviewed.