Bleeding after cardiopulmonary bypass remains a significant problem for the cardiac surgeon. In addition to surgically-induced bleeding, the major cause of loss of blood is an acquired coagulopathy. Impaired function, produced either by the mechanical effect of cardiopulmonary bypass or by the influence of hemostatic activation of platelets during bypass, is the main cause of postoperative bleeding. The tendency towards bleeding in infants and neonates is additionally influenced by the type of operation, those with cyanotic heart disease demonstrating an increased tendency to bleeding. Most of the procedures are more complex than in adults, and many reoperations are necessary, particularly if primary correction is not possible. Furthermore, procedures for perfusion and cooling are mostly different from those used in adults. The operations are regularly performed using deep hypothermia or circulatory arrest, which may further impair the function of platelets. Yet the effect of low temperatures on hemostasis, with or without circulatory arrest, still remains unclear, since the immature system for coagulation in neonates may contribute to impaired hemostasis.

The current practice of cardiac surgery would be impossible without extracorporeal circulatory support. Although significant morbidity is still associated with these techniques of support, they are “tolerated” because the flexibility and practical advantages which they provide in clinical practice far outweigh the associated morbidity.

Twins of like sex and concordant for William's syndrome are presented. The first twin presented late with aortic arch atresia and has subsequently developed hypertension associated with renal arterial stenosis. The second twin died with myocardial ischemia late after surgery for supravalvar aortic and pulmonary stenosis.

A seven-month-old boy with severe respiratory distress because of absence of the leaflets of the pulmonary valve underwent banding of the pulmonary trunk. Postoperative recovery was uneventful with dramatic improvement of his general and hemodynamic status. Repair was performed successfully almost two years later when the child had grown satisfactorily and was in stable condition.

Balloon dilation of native aortic coarctation was performed in a three-month-old boy. Angiography immediately after dilation revealed a localized bulge on the anterior as pect of the descending aorta interpreted initially as an aneurysm. Angiography four months later, however, failed to detect this bulge. We speculate that the early aneurysmal appearance of the anterior wall of the aorta may have been caused by factors other than a tear in the aortic adventitia.

We describe a male infant with the combination ofan aortic interruption proximal to the brachiocephalic artery and a persistent fifth aortic arch providing a pulmonary-to-systemic shunt. As far as we know, interruption of the aorta at this site has not previously been reported. Documented cases of a persistent fifth aortic arch itself are few, although failure of recognition may account for its apparent rarity.

Cerebral hypothermia is the principal means of protecting the brain during cardiac surgery, permitting periods of reduced perfusion and/or circulatory arrest. For example, at 10–20°C, circulatory arrest for 45–75 minutes is tolerated in animals without subsequent behavioral or histopathologic evidence of neurologic injury. Nevertheless, clinically, die protective effect of hypothermia is far from complete. In children undergoing hypothermic circulatory arrest under equivalent conditions, coma, seizures, choreoathetosis, and developmental retardation are recognized as potential neurologic sequels, occurring widi a combined incidence of 10-30%. Neurologic complications (stroke, neuropsychologic change) also occur with distressing frequency in adults undergoing cardiac surgery, even in the absence of circulatory arrest. An improved understanding of the physiology of cerebral hypothermia during cardiopulmonary bypass and hypothermic arrest is needed to improve upon these results. This review will address current concepts and controversies regarding flow of blood to the brain and its metabolism during hypothermic cardiopulmonary bypass and their relationship to current techniques for cerebral protection.

We report a patient with tetralogy of Fallot and pulmonary atresia who displayed an atypical aortopulmonary collateral artery extending from the ascending aorta to the pulmonary trunk. The internal thoracic artery, instead of the right subclavian artery (which was aberrant), was used as the source ofblood during unifocalization of the right pulmonary arteries to avoid the potential formation of a vascular ring. Unifocalization in the left lung was carried out by enlargement with a patch of the previously stenotic Blalock-Taussig shunt. The palliation set the scene for final intracardiac repair achieved one month after unifocalization of the right sides. The postoperative course was good and uneventful.

Hypothermic circulatory arrest has been an important adjunct in pediatric cardiovascular surgery. Historically, use of hypothermia allowed the first intracardiac repair prior to the successful application of cardiopulmonary bypass and well before the advent of cardioplegic cardiac arrest. Today, the repair of many complex defectsis greatly facilitated by the use of hypothermic arrest. Despite the importance of the technique in the armamentarium of the congenital heart surgeon, the physiology of cerebral injury associated with hypothermic arrest has not been fully elucidated. To understand this physiology better, a number of investigators have focused on cerebral reperfusion after the event. In this review, we discuss findings from our own studies in the light of other available data on global and regional flow of blood to the brain following hypothermic arrest.

A syndrome with characteristic facial and skeletal features resulting from a specific deletion of material on the short arm of chromosome 17 was first described by Smith et al in 1982.1 The common findings are brachycephaly, hypoplasia of the middle face, and short broad hands associated with mental retardation. More than 50 cases have been reported, and about one third have congenital heart disease. We report here a patient with atrial and ventricular septal defects together with pulmonary valvar stenosis.

Over the past two decades, advances in equipment used for cardiopulmonary bypass and in operative techniques have resulted in a tremendous decrease in the mortality of patients undergoing surgical repair of congenital heart disease utilizing hypothermic circulatory arrest. Despite the widespread use of hypothermic arrest, opinion is not unanimous with regard to its safety. Previous studies which have examined neurological outcome following repair of congenital heart disease in infancy have generally agreed that when the period of arrest exceeds 60 minutes, there is increasing risk of cerebral injury.

Repair of severe hypoplasia of the aortic arch with coarctation must be based on the individual anatomy of the lesion and, where necessary, one should take into consideration associated cardiac abnormalities. We report a surgical technique which was employed when standard procedures for reconstruction of the arch had failed to relieve the obstruction adequately. A conduit from the carotid artery to the descending aorta was used successfully in two patients to abolish residual stenosis.

A right-sided modified Blalock-Taussig anastomosis was created using a 5 mm Gore-Tex tube in a 4½ month old infant with unilateral “absence” of the right pulmonary artery. Wide patency of the shunt was confirmed shortly after by angiography, but rarefaction of the peripheral right pulmonary arteries was seen at the age of 2½ years, when corrective surgery was performed. Postoperative hypoperfusion of the right lung, along with severe pulmonary vascular abnormalities seen in a lung biopsy, indicated failure of the two-stage correction in spite of appropriate surgery.

We describe an eleven-year-old boy with severe retardation in physical growth and severe rheumatic valvar disease. At surgery, a sample of the atrial appendage showed Aschoff nodules, although no antecedent of rheumatic fever was recorded. The patient was HLA-DR5 positive. The case emphasizes the strong association of malnutrition with severe rheumatic heart disease in northeast Brazil.

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?