No abstract

No abstract

The association of biliary atresia (BA) with congenital heart diseases has been extensively described, and there are a number of reports on the outcomes of patients in this group who undergo liver transplantation (LT). The intraoperative management and the timing of LT for patients with end-stage liver disease are matters of debate, especially when complex heart diseases are involved. This report describes the outcome after LT for a pediatric recipient with BA and hypoplastic left heart syndrome. The patient underwent Norwood-Sano and Glenn procedures for heart palliation before LT. He was cyanotic, was severely malnourished, and had complications secondary to chronic liver failure. At the time of transplantation, the child was 16 months old and weighed 5175 g. Despite the critical clinical scenario and the long hospitalization period, there were no cardiac, vascular, or biliary complications after LT. At the age of 48 months, the patient was awaiting the final cardiac repair. In conclusion, the presence of complex cardiac malformations may not be a contraindication to LT. An experienced surgical team and a multidisciplinary approach are key to a successful outcome.

IntroductionDonor shortage and organ allocation is the main problem in pediatric heart transplant. Mechanical circulatory support is known to increase waiting list survival, but it is not routinely used in pediatric programs in Latin America.MethodsAll patients listed for heart transplant and supported by a mechanical circulatory support between January 2012 and March 2016 were included in this retrospective single-center study. The endpoints were mechanical circulatory support time, complications, heart transplant survival and discharge from the hospital.ResultsTwenty-nine patients from our waiting list were assessed. Twelve (45%) patients were initially supported by extracorporeal membrane oxygenation (ECMO) and a centrifugal pump was implanted in 17 (55%) patients. Five patients initially supported by ECMO were bridged to another device. One was bridged to a centrifugal pump and four were bridged to Berlin Heart Excor®. Among the 29 supported patients, 18 (62%) managed to have a heart transplant. Thirty-day survival period after heart transplant was 56% (10 patients). Median support duration was 12 days (interquartile range [IQR] 4 - 26 days) per run and the waiting time for heart transplant was 9.5 days (IQR 2.5-25 days). Acute kidney injury was identified as a mortality predictor (OR=22.6 [CI=1.04-494.6]; P=0.04).ConclusionMechanical circulatory support was able to bridge most INTERMACS 1 and 2 pediatric patients to transplant with an acceptable complication rate. Acute renal failure increased mortality after mechanical circulatory support in our experience.