Treatment of hyperhomocysteinemia in pediatric heart transplant recipients.

BACKGROUND: Graft coronary artery vasculopathy is the main cause of late morbidity and mortality in pediatric cardiac allograft recipients. Growing evidence suggests that elevated plasma homocysteine levels are associated with cardiac allograft vasculopathy following heart transplantation. The purpose of this study was to evaluate the effect of vitamin supplementation as a potential strategy for reducing homocysteine levels in pediatric heart transplant recipients and examine creatinine levels as potential determinants of plasma homocysteine concentration after transplantation. METHODS: We studied 27 pediatric heart transplant patients with homocysteine levels higher than normal. All children received vitamin supplementation (vitamin B(12), vitamin E, vitamin A and folic acid). During treatment, levels of homocysteine, vitamins and creatinine were evaluated after 3, 6, 9 and 12 months. RESULTS: We observed a significant homocysteine concentration decrease after treatment at every determination, whereas no significant change occurred for creatinine. Vitamin B(12) serum level increased markedly, whereas folic acid, vitamin E and vitamin A serum levels showed only minor increases. CONCLUSIONS: We observed a significant increase of mean levels of vitamin B(12) and a moderate increase in the other 3 vitamins. We also observed a significant reduction in homocysteine levels, which returned to normal levels for age. In our patients, there was a correlation, before and after treatment, between homocysteine and creatinine levels, but there was no a direct correlation between creatinine serum levels and homocysteine reduction. We conclude that vitamin supplementation reduces and may normalize homocysteine serum level after pediatric heart transplantation.

Total anomalous pulmonary venous connection: long-term appraisal with evolving technical solutions.

OBJECTIVES: To evaluate late outcome of non-isomeric total anomalous pulmonary venous connection (TAPVC) repair, controlling for anatomic subtypes and surgical technique. METHODS: Between 1983 and 2001, 89 patients (median age 54 days) underwent repair for supracardiac (38), cardiac (26), infracardiac (16) or mixed (nine) TAPVC. Ten patients (11.2%) presented associated anomalies other than PDA. Twenty-eight patients (31.5%) were emergencies, due to obstructed drainage. Supracardiac and infracardiac TAPVC repair included the double-patch technique with left atrial enlargement in 29 patients and side-to-side anastomosis between the pulmonary venous (PV) confluence and the left atrium in 29 patients. Coronary sinus unroofing was preferred for cardiac TAPVC repair. Total follow-up was 727.16 patient-years (mean 8.55 years, 98.8% complete). RESULTS: Early mortality was 7.86% (7/89). Ten patients (11.2%) underwent reintervention, including reoperation (eight), balloon dilation (one) and intraoperative stents placement (one), for anastomotic (four) or diffuse PV stenosis (six), with four late deaths. Kaplan-Meier survival is 87.3+/-0.036 SE% at 18.07 years with no difference according to anatomic type or surgical technique. Freedom from PV reintervention for operative survivors is 86.7+/-0.052 SE% at 18.07 years. Cox proportional hazard indicates associated anomalies (P=0.008) and reoperation for intrinsic PV stenosis (P=0.034) as independent predictors of mortality. According to logistic analysis, preoperative obstruction predicts higher risk of reintervention for intrinsic PV stenosis (P=0.022), while the double-patch technique increased the risk of late arrhythmias (P=0.005). CONCLUSIONS: Side-to-side anastomosis provides excellent results for TAPVC repair while left atrial enlargement procedures appear to be associated with higher risk of late arrhythmias. Although early and aggressive reintervention for recurrent PV obstruction is mandatory, intrinsic PV stenosis remains a predictor of adverse outcome.