Prolongation of the QT interval in children with liver failure.

BACKGROUND: Alcoholic liver disease has been associated with QT prolongation and sudden cardiac death. HYPOTHESIS: We evaluated children with hepatic failure to determine whether they have abnormalities of ventricular repolarization. METHODS: Between October 1990 and January 1996, 38 pediatric patients (mean age 6.5 +/- 7.2 years) underwent evaluation for liver transplantation, including a 12-lead electrocardiogram and an echocardiogram. All patients had normal serum electrolytes, calcium, and magnesium at the time of cardiac evaluation and were not on any medications known to prolong repolarization. Follow-up electrocardiograms were performed on all survivors with QT prolongation following liver transplantation. RESULTS: Among those evaluated, seven (18%) were noted to have a prolonged QT interval corrected for rate (QTc > 450 ms; range 460-560 ms). All had a structurally normal heart, except one with an atrial and ventricular septal defect. When compared with patients with a normal QT interval, there was no significant difference in serum indices of liver function or indication for liver transplantation. None of the patients developed a ventricular arrhythmia. Two patients with a prolonged QTc died prior to transplant and another died immediately after surgery. All four survivors had normalization of the QTc following liver transplantation. CONCLUSION: QTc prolongation can be seen in a significant number of children with hepatic failure. While the mechanism is not known, it appears to be reversible following liver transplantation.

Recurrence of autoimmune hepatitis in children after liver transplantation.

BACKGROUND: Liver transplantation is recognized as the appropriate treatment for end-stage liver disease due to chronic active autoimmune hepatitis. While it was initially thought that the disease did not recur after transplant, it is now generally accepted that adult patients may develop recurrent disease, with studies reporting a recurrence rate of < or = 25%. We have noted a higher incidence of recurrent autoimmune hepatitis in our pediatric patients undergoing liver transplant, with a high incidence of associated morbidity. METHODS: We reviewed the records of six children followed up for autoimmune hepatitis who underwent orthotopic liver transplant for complications of end-stage liver disease. RESULTS: Of the six, five developed recurrent autoimmune hepatitis at a mean time of 11.4 months after transplant. The disease was aggressive, leading to cirrhosis and retransplant in three patients, within 1 year of recurrence. A second recurrence of disease occurred in all three retransplanted patients. One patient has received a third liver transplant, one has died, and one patient is asymptomatic on medical therapy. Autoimmune hepatitis recurred in all four patients receiving tacrolimus. CONCLUSION: We conclude that liver transplant for autoimmune hepatitis is likely to be palliative for most pediatric patients. Potent immunosuppressives such as tacrolimus do not protect against the development of recurrent autoimmune hepatitis.