Congenital Heart Disease in Adults

Advances in perioperative care for children with congenital heart disease (CHD) over the past several decades have resulted in an ever-increasing number of these children reaching adulthood with their cardiac lesions palliated or repaired. The first paper on adult CHD (ACHD) was published in 1973. The field has grown such that several texts are now devoted to it, and a dedicated specialty society, the International Society for Adult Congenital Heart Disease ( http://www.isachd.org ), was formed in the 1990s. Each year an estimated 32,000 new cases of CHD occur in the United States and 1.5 million worldwide. More than 85% of infants born with CHD are expected to grow to adulthood. It is estimated that there are more than 1 million adults with CHD in the United States and 1.2 million in Europe, and this population is growing at approximately 5% per year; 55% of these adults remain at moderate-to-high risk, and more than 115,000 in the US have complex disease. These patients can be seen by anesthesiologists for primary cardiac repair, repair following a prior palliation, revision of repair due to failure or lack of growth of prosthetic material, or conversion of a suboptimal repair to a more modern operation ( Box 16.1 ). In addition, these adults with CHD will be seen for the other common ailments of aging and trauma that require surgical intervention. Although it has been suggested that teenagers and adults can have repair of congenital cardiac defects with morbidity and mortality approaching that of surgery done during childhood, these data are limited and may reflect only a relatively young and acyanotic sampling. Other data suggest that, in general, adults over 50 years of age represent an excessive proportion of the early postoperative mortality encountered, and the number of previous operations and cyanosis are both risk factors.

Box 16.1

Indications for Cardiac Surgery in Adults With Congenital Heart Disease

• Primary repair

• Total correction following palliation

• Revision of total correction

• Conversion of suboptimal obsolescent operation into more modern repair

• Heart transplantation

These patients bring with them anatomic and physiologic complexities of which physicians accustomed to caring for adults may be unaware, as well as medical problems associated with aging or pregnancy that might not be familiar to physicians used to caring for children. This has led to the establishment of the growing subspecialty of ACHD. The American College of Cardiology reviewed the available evidence and published superb guidelines for the care of these patients in 2008. A major recommendation was that adult patients with moderate or complex CHD be cared for in specialized adult congenital heart centers. An informed anesthesiologist is a critical member of the team required to care optimally for these patients. A specific recommendation was that noncardiac surgery on adult patients with moderate-to-complex CHD be done at an adult congenital heart center (regional centers) with the consultation of an anesthesiologist experienced with ACHD. In fact, one of the founding fathers of the subspecialty wrote, “A cardiac anesthesiologist with experience in CHD is pivotal…The cardiac anesthesiologist and the attending cardiologist are more important than the noncardiac surgeon.” Despite this recommendation, the majority of adult patients with CHD having ambulatory surgery appear not to be having their surgery at ACHD centers.