Abstract
The fixed incidence of congenital heart defects and improved survival have resulted in increasing numbers of adults with congenital heart disease (CHD) who have undergone complex repairs and/or palliations. Eventually, there will be more adults with CHD than children. They will require cardiac surgical interventions associated with progression of their CHD or for age-related disease, such as coronary revascularization. During bypass, anatomical shunts may exist within or without the heart. Left-to-right shunts can result in dramatically lower systemic blood flow than pump flow due to ‘steal’, while pulmonary edema ensues due to excessive pulmonary flow. Right-to-left shunts carry risks of massive air embolism and double or triple venous cannulation may be necessary. Cannulation of composite reconstructed aortas may be difficult, risking dissection or aortic obstruction, and double arterial cannulation may be indicated. Aberrant coronary arterial and venous anatomy may preclude adequate myocardial preservation with common techniques and can be complicated by aortic insufficiency. Valves and conduits may exhibit failure. Conventional monitoring, such as central venous oximetry, may be misleading. Monitoring, such as serial lactate measurement, near-infrared spectroscopy and transcranial Doppler blood velocity, offer advantages for such patients. The perfusionist needs to be aware of such conditions as much congenital aberrancy may present unexpectedly during cardiac surgery.
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