Although the brain is the organ most sensitive to total bodily ischemia, and probably also most sensitive to the deleterious effects of cardiopulmonary bypass, it is also the most difficult organ in which to quantitate function accurately. Clinical studies allow sensitive neuropsychometric testing, as discussed elsewhere in this issue (see Wernovsky et al, p. 308). This is not possible in studies using animals. Even testing of gross neurological integrity in animals is complicated by the associated dysfunction of other organs which accompanies the insult of cardiopulmonary bypass with or without circulatory arrest on the whole body. For example, how should one evaluate an animal undergoing neurological testing which is unable to walk or eat, perhaps because of severe cardiorespiratory or gastrointestinal dysfunction following cardiopulmonary bypass and circulatory arrest. What is the contribution of neurological dysfunction to this state? Most importantly, how is the animal scored which does not survive, even though this may be a result of multiorgan dysfunction?

The dramatic reduction in surgical mortality associated with repair of congenital heart anomalies in recent decades has been accompanied by a growing recognition of adverse neurologic sequels in some of the survivors. Abnormalities of the central nervous system may be a function of coexisting cerebral abnormalities or acquired events unrelated to surgical management (such as paradoxical embolus, cerebral infection, or effects of chronic cyanosis), but insults to the central nervous system appear to occur most frequently during or immediately after surgery. In particular, techniques of support used during neonatal and infant cardiac surgery—cardiopulmonary bypass, profound hypothermia and circulatory arrest—have been implicated as important causes of cerebral injury. This paper will review the effects of bypass and deep hypothermic circulatory arrest on neurodevelopmental outcome.

Obstruction to the left ventricular outflow tract is not uncommon in patients with congenitally corrected transposition. A case of congenitally corrected transposition is described with significant subpulmonary obstruction which was due to a subpulmonary tag. The patient required an aortopulmonary shunt. Her clinical course was subsequently significantly influenced by arrhythmias associated with an accessory pathway. She died suddenly from an arrhythmia. Post-mortem examination confirmed a large subpulmonary tag which originated from both sides of the ventricular septum—a previously unreported finding. Careful evaluation of the nature of left ventricular outflow obstruction is important in this condition as it may alter the surgical management.

With substantial effort and research devoted to improving surgical techniques and myocardial protection, superb results have been achieved for repair of complex congenital heart defects in children. As a result, investigative efforts now have begun to examine the quality of life for patients surviving these operations. Because these neonates and infants are exposed to severe physiologic extremes of temperature (15–18 °C) and severe alterations from normal perfusion (total circulatory arrest), the nature of long-term neuropsychological outcome has been a prominent concern. Recent preliminary reports suggest that transient and permanent neuropsychologic injury occur in as many as 25% of all infants undergoing hypothermic cardiopulmonary bypass with circulatory arrest.'Since improved surgical techniques have significantly reduced rates of operative mortality and cardiac morbidity, one of the greatest risks remaining for the patient with congenital heart disease may be long term neuropsychologic and developmental abnormalities.

We report a case of totally anomalous pulmonary venous connection in which the two pulmonary veins from the left lung joined to form a common vein which then passed across the midline into a hypoplastic right lung and, after receiving small veins from the right lung, passed inferiorly, exiting the lung below the hilum as a “scimitar” vein and terminating in the inferior caval vein. A separate pulmonary vein from the right lung passed inferiorly independently and joined the “scimitar” vein before it entered the inferior caval vein. There was an associated hypoplastic left heart syndrome.

Mycotic aneurysms of the pulmonary trunk are a rare but recognized complication of infective endocarditis. They are usually associated with infected congenital heart lesions, and especially with persistent patency of the arterial duct. This report deals with a patient with a persistent duct and aortic coarctation who developed a mycotic aneurysm of the pulmonary trunk secondary to infective endocarditis. The lesion was evaluated before and after surgery by magnetic resonance imaging.