Transplantation as a primary treatment for hypoplastic left heart syndrome: intermediate-term results.

BACKGROUND: Hypoplastic left heart syndrome is a lethal malformation. For the last 10 years, orthotopic cardiac transplantation has been our preferred treatment for infants with hypoplastic left heart syndrome. METHODS: One hundred seventy-six infants with hypoplastic left heart syndrome were entered into a cardiac transplant protocol between November 1985 and November 1995. Interventional procedures to stent the ductus arteriosus or enlarge the interatrial communication were performed in 8 and 35 patients, respectively. Thirty-four patients (19%) died during the waiting period, and 142 infants underwent cardiac transplantation. Age at cardiac transplantation ranged from 1.5 hours to 6 months (median, 29 days). The majority of grafts were oversized, and the median graft ischemic time was 273 minutes (range, 60 to 576 minutes). The implantation procedure used a period of hypothermic circulatory arrest ranging from 23 to 110 minutes (median, 53 minutes). Repair of other significant defects included interrupted aortic arch and total or partial anomalous pulmonary venous connection. RESULTS: There were 13 early and 22 late deaths. Patient actuarial survival at 1 month and at 1, 5 and 7 years was 91%, 84%, 76%, and 70% respectively. Half of the late deaths were due to rejection. Severe graft vasculopathy was confirmed in 8 patients. Retransplantation was performed in 5 patients for graft vasculopathy 4 and rejection 1. Lymphoblastic leukemia developed in 1 patient 3 years after cardiac transplantation. CONCLUSIONS: Cardiac transplantation can be performed in infants with hypoplastic left heart syndrome with good operative and intermediate-term results. Improved survival can be achieved with increased donor availability, better management of rejection, and control of graft vasculopathy.

Neurologic sequelae of deep hypothermic circulatory arrest in cardiac transplant infants.

BACKGROUND: Considerable controversy exists experimentally and clinically regarding adverse neurologic effects that may follow deep hypothermic circulatory arrest. Moreover, the techniques of DHCA have never been standardized. METHODS: We prospectively studies the neurodevelopmental outcome in 38 infants undergoing cardiac transplantation using DHCA before the age of 4 months (mean age, 37.0 days). Neurodevelopmental outcome in the 22 boys and 16 girls was tested up to 2.5 years after transplantation using Bayley scale of infant development. Bayley scores were compared with the rate of core cooling and the length of DHCA in all patients. Deep hypothermic circulatory arrest was accomplished using an asanguineous prime resulting in hematocrits of 5% +/- 5% and ionized Ca2+, 0.4 +/- 0.1 mmol/L. No surface precooling was used, but the head was packed in ice. Mean cooling time was 14.0 +/- 3.5 minutes, resulting in rectal temperatures of 18 degrees +/- 2.5 degrees C. Duration of DHCA ranged from 42 to 70 minutes (mean duration, 56.0 +/- 6.6 minutes). RESULTS: Postoperatively, the mean Bayley psychomotor development index was 91 (range, 50 to 130) and mental development index was 88 (range, 50 to 130). No relationship was found between either the rate of cooling or the duration of DHCA and Bayley scores (r = 0.227 and r = 0.322, respectively). CONCLUSIONS: These data suggest that neither the rate of cooling nor DHCA times between 42 and 70 minutes using profoundly low hematocrits and low ionized calcium levels has any measurable effect on neurologic outcome up to 2.5 years postoperatively. It is possible that adverse neurologic outcomes from DHCA reflect particular methods of achieving DHCA.

Cardiac pacemakers in pediatric heart transplant recipients: incidence, indications, and associated factors. Pediatric Heart Transplant Group-Loma Linda.

This study was undertaken to assess the incidence, indications, and predisposing factors for pacemaker placement in a pediatric heart transplant population. From November 1985 to May 1994, 246 pediatric patients have undergone cardiac transplantation at Loma Linda University Medical Center. Seven (2.8%) have received pacemaker placement with an 8-50 month follow-up period. Median age at transplant was 462 days (0 days to 2.5 years). The median time to pacemaker placement was 190 days (18-1,672 days) after transplantation. Indications were sick sinus syndrome (SSS) in 5 and heart block in 2 patients (1 during acute rejection). Three patients with SSS underwent electrophysiology studies (EPS); 1 was normal and 2 showed sinus node dysfunction. The mode of pacing was VVIR in 6 patients and VVI in 1 patient. All 6 survivors are doing well and 5 patients' pacemakers still provide support. These 7 patients were compared with 185 pediatric patients (0 days to 12-years-old) transplanted during 1985 through 1993 who survived at least 6 months after transplantation. There was no correlation between the receipt of a pacemaker and graft cold ischemic time, rejection history, donor age, or recipient age at transplantation. The 5 patients with SSS had significantly lower average heart rates in the first month after transplantation (108 +/- 16 vs 130 +/- 12; P = 0.0002). The need for permanent pacemakers in this population is uncommon. Pacemakers, however, can be safely performed when necessary with excellent clinical results.

Rejection management and long-term surveillance of the pediatric heart transplant recipient: the Loma Linda experience.

At Loma Linda University Medical Center, 210 heart transplant procedures have been performed on 207 newborns, infants, and children since 1985. Actuarial survival rate at 5 years is 72% for the entire population and 82% for those receiving a transplant during the first month of life. These patients have been managed with a regimen that minimizes long-term steroid use and emphasizes the noninvasive diagnosis of rejection. This article describes in some detail the mechanics of this process. In addition, the rejection history of 154 children undergoing transplantation from 1989 through 1992 was reviewed. The average number of rejection episodes was 1.67 (standard deviation 1.65; median, 1; mode, 0). The vast majority of rejections occur in the first 3 months after transplantation. Long-term freedom from rejection was 19% for newborn recipients, 42% for infants, 25% for older children. Donor/recipient mismatch for gender, race, blood type, Rh factor, and HLA typing did not correlate with rejection history. Older age at transplantation and cytomegalovirus disease were correlated with more frequent rejection episodes. Five patients had posttransplantation coronary artery disease. This was strongly correlated with greater rejection frequency and death from rejection. In addition, there was a trend toward less posttransplantation coronary artery disease with antibody induction therapy, younger age at transplantation, and absence of cytomegalovirus disease.(ABSTRACT TRUNCATED AT 250 WORDS)