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Transplant surgeons are reluctant to use hearts that have undergone cardiopulmonary resuscitation for cardiac arrest because of the fear of poor early and late cardiac function. A policy of minimizing contraindications to use of donor hearts has led to the unique opportunity of assessing the effects of donor arrest and successful cardiopulmonary resuscitation on early and late cardiac function in pediatric heart transplantation. A number of 140 infants and children undergoing transplantation from birth to 17 years of age were studied retrospectively and divided into two groups on the basis of cardiopulmonary resuscitation status. Group 1 (72 patients) received donor hearts that were not subjected to cardiopulmonary resuscitation; group 2 (68 patients) received donor hearts that had cardiopulmonary resuscitation for a mean of 18.8 +/- 14.6 minutes, the longest period of time being 60 minutes. Mean ischemic times were almost identical in the two groups: 4.43 +/- 2.0 hours (cardiopulmonary resuscitation) versus 4.5 +/- 2.1 hours (no cardiopulmonary resuscitation). Early cardiac function was assessed on the basis of the number of days the recipient was supported by the ventilator, days receiving dopamine, days receiving isoproterenol, and the amount of inotropic agents required after the operation. The groups did not differ. Parameters of systolic function included fractional shortening, posterior wall thickening, and maximum velocity of change in left ventricular posterior wall dimension during systole. Diastolic function was measured on the basis of left ventricular end-diastolic volume, left ventricular mass, and maximum velocity of change in left ventricular posterior wall dimension during diastole. Both systolic and diastolic function were measured and analyzed from M-mode echocardiography at 1 week, 1 month, 6 months, 1 year, and 2 years after the operation. There were no statistically significant differences in graft function between the two groups in any of the echocardiographic parameters studied, even at 2 years. No group differed from ranges of normal. Our results suggest that hearts undergoing cardiopulmonary resuscitation for periods of up to 60 minutes can be used safely without evidence of deterioration of early or late cardiac function.

The cause of brain death and the physiologic sequelae of brain death may impair heart function. Pharmacologic attempts to maintain donor viability may further jeopardize myocardial performance and could only be justified if dysfunctional donor organs subsequently prove to recover normal function after transplantation. Survival data on heart transplantation with organs donated from infants with sudden infant death syndrome indicate that prolonged ischemia (cardiopulmonary resuscitation up to 60 minutes) and metabolic abnormalities a priori do not increase the risk of graft failure. To provide a donor organ to infants in immediate peril, we have used donor hearts with documented dysfunction (left ventricular shortening fraction [LVSF]28%, wall motion abnormalities, and mitral regurgitation). The results of heart transplantation with use of dysfunctional donor hearts (n = 22, LVSF = 24.5% +/- 3%) were compared with donors with normal left ventricular function (n = 133, LVSF28%). Early death (30 days) was similar for the dysfunctional donor group (14%) and normal function donor group (11%). Postoperative inotropic support was equally frequent in both groups. Graft function on echocardiography was normal at 30 days after transplantation for both types of donor organs. We conclude that donor hearts with decreased left ventricular function (LVSF 15% to 28% and/or asymmetric wall motion), despite massive inotropic support, can function normally in the recipient. Significant donor mitral regurgitation was seen in grafts that ultimately failed after transplantation. Research into the reversible mechanisms of myocardial dysfunction associated with brain death could enlarge the donor pool.

Early age at cardiac transplantation may favor successful engrafting with minimal chronic immunosuppression. Fifty-two newborns underwent orthotopic heart transplantation; 47 (90%) survived the operation, and 44 (85%) were late survivors. Actuarial survival was 92% at 1 month, 86% at 1 year, and 84% at 5 years. Forty-four infants who survived 12 weeks and the corresponding 100 rejection episodes were analyzed. Mean follow-up was 2.2 years. The mean number of rejections per year of follow-up was 1.2. No episodes of rejection were identified in six patients. Seven patients had a late rejection episode more than 1 year after transplantation, and only one had a late rejection episode after 2 years. Neonatal cardiac transplantation is effective and durable therapy for uncorrectable heart disease. Intermediate-term results are excellent. Severe rejection is uncommon, and few episodes occur after 1 year of follow-up.

We studied the feasibility of transesophageal echocardiography to guide endomyocardial biopsies in five heart transplant patients and two baboons with heart transplantations. The patients were 1 month to 1.5 years old with weight range of 2.9 to 9.5 kg. The two juvenile baboons weighed 6.6 and 7 kg. Transesophageal echocardiography was performed uneventfully in all cases with the use of sedation and anesthesia, which were necessary for catheterization. The combination of four-chamber and short-axis views easily identified the exact location of the bioptome within the heart, views that fluoroscopic imaging could not provide. Transesophageal echocardiography proved to be a safe and useful tool for guiding the endomyocardial biopsy procedure. With further refinement endomyocardial biopsy with only transesophageal echocardiography guidance could become the routine method for endomyocardial biopsies in infants, particularly when there are abnormalities of heart situs or position.

We examined the early and late results of operations in 29 consecutive neonates with pulmonary atresia and intact ventricular septum treated from 1980 to 1988. Transventricular pulmonary valvotomy and central aorta-pulmonary artery shunting were performed in 19 of 22 infants who had a patent infundibulum. Pulmonary valvotomy alone was performed in 3 of the 22 infants with a patent infundibulum, but 2 of these required subsequent systemic-pulmonary artery shunts. Primary shunting was used to palliate 7 infants who had absent infundibular portions of the right ventricle and a very diminutive right ventricular cavity. Tricuspid valve excision and atrial septectomy were also performed in 5 of these 7 infants to decompress large fistulous communications between the right ventricule and coronary artery. Two early deaths (2/29, 6.9%) occurred overall. Both were in infants who had a very small right ventricle. Definitive operation has been accomplished in 16 patients; 13 have had closure of residual interatrial communications and shunt ligation with no deaths, and 3 have undergone modified Fontan repair with 1 death. Actuarial survival rate for the entire group, including operative deaths, is 86% at 5 years. The technique of transventricular pulmonary valvotomy and systemic-pulmonary artery shunting offers a reliable means of palliating neonates with pulmonary atresia and intact ventricular septum and obtains good late right ventricular growth. Systemic-pulmonary shunting, tricuspid valvectomy, and atrial septectomy may offer a means of reducing or obliterating right ventricular-coronary artery fistulas.

To evaluate the role of orthotopic cardiac transplantation for infants with lethal cardiac disease, we reviewed the results from our first 25 patients. Data on the donors were also reviewed to define the characteristics of a successful cardiac donor. Patients had transplants between November 1985 and November 1988. Several primary cardiac diagnoses were indications for transplantation, including congestive cardiomyopathy, hypoplastic left-heart syndrome, and other types of native and postoperative complex congenital heart diseases. The ages ranged from birth to 7 months. Of 25 patients, 21 are still alive (84% survival rate) with follow-up from 4 to 40 months. No late deaths have occurred. Long-term immunosuppression was accomplished with cyclosporine and azathioprine. Rejection surveillance was performed noninvasively; only one child required an endomyocardial biopsy. Donors died from a variety of traumatic and metabolic causes, including sudden infant death syndrome. The majority (72%) of donors had a history of cardiac arrest requiring cardiopulmonary resuscitation. One third were receiving inotropic support at the time of cardiac evaluation. We conclude that orthotopic cardiac transplantation is an effective therapy for infants with lethal heart disease. A larger donor pool is required, and many dying infants, despite cardiac arrest and resuscitation, would be suitable donors.