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An ever-increasing number of congenital heart disease patients are surviving into and beyond adolescence. Despite the encouraging cardiac results, with the vast majority asymptomatic and requiring no medication and having few, if any, limitations, there are many challenges for this patient population. They are likely to find obstacles to attaining education, social development, health and life insurance, and employment. Most of these obstacles are attributable to the stresses upon children with congenital heart disease and their families, and the prejudices of a society that knows little about congenital heart disease. Practitioners caring for these young people must be familiar with these issues and knowledgeable about the facts of individual cases, so that they may anticipate needs and offer support to their patients in their progress toward adulthood.

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The major ethical issues involved in decision-making in the care of extremely low birth weight newborns are analyzed here. We propose a schema for assessment and management of these infants that is consistent with ethical principles broadly accepted by the pediatric community, and which takes into account mortality risk at any given institution rather than arbitrary weight limits, with a major decision-making role for the infant's parents. When possible, the decision of whether or not to resuscitate should be made before delivery; when not possible, delivery room resuscitation is recommended, and the decision to continue or withdraw care should be made subsequently based on likelihood of survival and the wishes of the parents.

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METHODS: Applying generally accepted criteria for selection of adult heart donors, we conducted a retrospective study of brain-dead infants and children for assessment of suitability as donors for heart transplantation. Cardiac histopathologic studies were evaluated in all subjects undergoing autopsy. RESULTS: In 5 years there were 58 such patients, the majority of whom had head injury, near-drowning, near-miss sudden infant death syndrome, infection, or asphyxia. Of these, only five met the proposed clinical criteria. Most prospective donors were eliminated on the basis of prolonged cardiac arrest (n = 33), pressor dependency (n = 25), and/or infection (n = 10). Forty-two subjects underwent autopsy, of whom 36 would not have been excluded as donors except on the basis of ischemic cardiac insult. Of these, 18 subjects were found to have essentially normal myocardium, nine had abnormal but potentially reversible microscopic changes, and nine had myocardial infarction. The pathologic findings were not predicted by the selection criteria, but severe chest trauma was not associated with infarction, eight of the nine patients with infarction had had cardiac arrest, and most of those with infarction had drowned or had had sudden infant death syndrome. CONCLUSIONS: The supply of donor organs for pediatric heart transplantation is very limited if selection criteria used for adult donors are applied. These criteria, however, do not correlate well with myocardial pathologic findings in infants and children. More accurate predictors of donor suitability are needed.

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Since April 1976, nine infants less than 1 month of age underwent a Mustard intraatrial baffle procedure. All had transposition of the great arteries and were persistently symptomatic or hypoxic after the Rashkind balloon atrioseptostomy. Two had an associated large ventricular septal defect patched at the time of physiologic correction. The average age of the infants was 11.6 days (range 36 hours to 28 days). Weight averaged 3.4 kg (range 2.5 to 4.2). In all the Mustard procedure was performed using deep hypothermic circulatory arrest, averaging 71 minutes (range 48 to 88) at a mean core temperature of 13 degrees C. An average of 2.8 days of postoperative ventilatory assistance was required. One death occurred in a 28 day old male infant with an associated ventricular septal defect who, the morning after operation, had a sudden unresponsive cardiac standstill. The postoperative hospitalization period averaged 15 days (range 10 to 23). Follow-up evaluation has extended from 4 to 63 months (average 31). Six patients were restudied 2 to 21 months postoperatively. One had obstruction of the superior limb of the baffle, which had separated from the right atrial wall permitting a modest right to left shunt. She has had uneventful repair employing a unique modification of Senning's operation. Another child has asymptomatic partial obstruction of the superior limb of the baffle demonstrated angiographically. None has pulmonary venous obstruction and all have prevailing normal sinus rhythm. Significant right ventricular dysfunction has been demonstrated in one. Early correction in these persistently symptomatic or hypoxic neonates with transposition of the great arteries with or without a ventricular septal defect has obviated the need for palliative operations and produced gratifying early and late results.

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A unique concept of right ventricular outflow tract reconstruction is presented. Applications of this concept, employing a glutaraldehyde-preserved heterograft mitral valve leaflet mounted directly to the right ventricle, was studied in six immature goat models. Interval evaluation of these models demonstrated no significant outflow tract obstruction or aneurysm. Only one animal had significant pulmonary outflow regurgitation angiographically at the time of sacrifice. All heterograft valve leaflets showed a degree of calcification and/or retraction directly related to the time interval between implant and sacrifice. Encouraging early results were obtained with this method of pulmonary outflow tract reconstruction in a 2,400 gram neonate with type I truncus arteriosus.

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Anatomic correction of interrupted aortic arch complex by direct aortic anastomosis was accomplished in five neonates ranging in age from 2 to 19 days. Three had type B and two had type A interruption of the aortic arch. All operations were performed through a transverse bilateral thoracotomy by use of hypothermic circulatory arrest. The descending thoracic aorta is widely mobilized to the level of the diaphragm. The ductus arteriosus is completely excised and the descending aortic segment is anastomosed end to side to the ascending aorta. The large ventricular septal defect is patched with Dacron velour, and atrial communications are closed with running suture. Three of the babies survived surgery and are making satisfactory clinical progress. All three have been restudied on a routine basis. They were found to have excellent anatomic repairs with no residual intracardiac shunts. There was a peak systolic gradient of 8 mm Hg at the aortic anastomosis in the second survivor; however, she does not have ascending aortic hypertension. The first and last survivors have no aortic gradients. It is recommended that a newborn with interrupted aortic arch complex be subjected to corrective surgery as the procedure of choice. Excellent results may be expected when surgery is done prior to irreversible metabolic decompensation of the patient.

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