Management of Congenital Heart Disease in the Adult
Duke Cameron, MD
Baltimore, Maryland
Surgery for congenital heart disease in the adult may take several forms: total correction when diagnosis is made late in life, total correction after palliative surgery earlier in life, repair of late complications after total correction (such as restenosis of pathways and valve stenosis/regurgitation), and rarely cardiopulmonary transplantation for organ failure.
The spectrum of disease is broad and the natural history for many congenital defects is less-well documented than with acquired heart disease, particularly among patients who have had early palliation or corrective surgery. To further complicate matters, patients are frequently cared for by four distinct physician groups (pediatric cardiologist, adult cardiologist, pediatric cardiac surgeons, and adult cardiac surgeons) and the coordination of evaluation and care is frequently suboptimal. Despite the proliferation of "adult congenital" clinics throughout North America, only a few centers have large clinical experience with management of these challenging patients. Fortunately, several texts are available on the subject; the one by Reddington et al is unusually lucid and concise.
It is estimated that approximately 5,000 adult congenital heart operations are performed annually in the United States, considerably less than more than the half million open heart procedures performed for acquired adult cardiac disease. The most common procedure is closure of an secundum type atrial septal defect. Operations on the left ventricular outflow tract (including aortic valve replacement), coarctation, repair of tetralogy of Fallot or its late postoperative sequelae, and conduit changes are the other operations commonly performed.
Special considerations to be addressed perioperatively in the evaluation and management of adult congenital patients are: anatomic features and vascular resistance of the pulmonary arterial bed, the possibility of concomitant coronary artery disease (or anomalies), the technical hazards of reoperation, the effect of aortopulmonary collaterals on myocardial protection and systemic perfusion during cardiopulmonary bypass, and the hemostatic and renal implications of chronic cyanosis.
A broad review of all lesions likely to be encountered in this group of patients will be impossible in the short time allotted, but specific attention will be given to these questions:
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