Congenital Heart Disease in Adults PDF

Preface

The outcome of surgical correction of congenital heart disease (CHD) has reached major advances over the past 30 years. New and more complex surgical and interventional procedures, applicable in infants, are emerging. As a result, some cardiologists have started to believe that CHDs in adulthood are of little importance, because they have been either surgically managed in childhood or they are of little hemodynamic significance. They also believed that severe, inoperable CHD cannot reach adulthood. Because of this misconception, but also because of its inherent complexity, until recently CHD was outside the main focus of interest in adult cardiologists.

However, the numbers of adults with CHD are today growing rapidly. Patients undergoing surgery as children in the 1950s–1970s have now become middle-aged individuals. The 1980s saw the advent of new surgical procedures and catheter-based interventions, the long-term outcomes of which we are now learning of. In addition, patients undergoing palliative surgical procedures in childhood also survive and, if provided reasonable conservative therapy, some patients with inoperable CHD may also live to reach adulthood. Some types of CHD, considered of little importance in childhood, may require surgery in adulthood. CHDs which are first operated on in adulthood may have a worse prognosis and more residual findings than if corrected in childhood.

Residual findings and arrhythmias may not manifest themselves clinically until some time after surgery. Even hemodynamically significant residual defects may be tolerated well for some time; however, they result in structural changes in the myocardium. These do not manifest themselves by clinical problems until several decades after surgery. The problem is, by that time, the myocardial changes may have already become irreversible. This explains why all these patients must be on constant follow-up and in time indicated for intervention or reoperation.

CHDs in adulthood show a low prevalence and huge heterogeneity. No controlled randomized studies in adults with CHD which furnish data to use in our decision-making have been conducted to date. Current guidelines are based on empirical observations and on data provided by studies involving small numbers of patients, and often date several decades back when the range of diagnostic and therapeutic options was much smaller compared with the arsenal available today. Criteria for indication and management applicable to children cannot be automatically applied to CHD in adulthood. In adulthood, CHD are joined by other conditions and complications such as coronary artery disease, hypertension, arrhythmia, acquired heart disease, diabetes mellitus, chronic obstructive lung disease, renal and hepatic dysfunction, impaired coagulation, and so on, appreciably increasing the risk associated with surgery. Making decisions about future strategy in adults is often very difficult. A decision has to be made between surgery carrying a risk and a life limited by CHD, even though these patients may have become accustomed to, and have come to terms with, their condition. Even today, the outcome of operation and reoperation for CHD in adulthood depends primarily on the body of experience that the department of cardiac surgery has with surgery for CHD and subsequent postoperative care.

The issue of CHD is becoming an integral part of adult cardiology and is receiving increased attention worldwide.