P271
SUCCESSFUL LIVING-RELATED RENAL TRANSPLANTATION IN A CHILD
WITH A FONTAN CIRCULATION
Woodside, J1, Savage, J2, Marks, S3.
1
Department of Paediatric Nephrology, Royal Belfast Hospital for Sick Children,
2
Department of Child Health, Queen’s University Belfast, Belfast, 3Department of
Paediatric Nephrology, Great Ormond Street Hospital for Children and University
College London Institute of Child Health, London
INTRODUCTION: There is no reported case in the literature of renal transplantation in a
child with a Fontan circulation (which is used as long-term palliation on patients with a single
ventricle, involving anastomosis between the inferior vena cava and the right pulmonary
artery).
AIM: To describe the first successful case of living-related renal transplantation in a child
with a Fontan circulation.
METHODS: Retrospective case note review of child with complex congenital heart disease
who underwent living-related renal transplantation.
RESULTS: A 13-year old girl presented with severe chronic kidney disease (plasma
creatinine of 250μmol/l) with lower limb rash and abdominal pain on the background of
known complex congenital heart disease (with AVSD, atrial and ventricular components
complete and ostium secundum ASD, right aortic arch, bilateral SVC, patent arterial duct and
right ventricular hypoplasia, pulmonary atresia, total anomalous pulmonary venous drainage
(intra and supracardiac)). She has a clinical heterotaxy Ivemark syndrome with right
isomerism with asplenia and had undergone creation of Fontan circulation at 7 years of age
after bilateral bidirectional superior cavopulmonary Glenn procedure at 16 months of age.
She had some renal impairment after her Fontan procedure but this had normalised within a
few days.
At re-presentation, she had significant proteinuria and secondary
hyperparathyroidism. Initial renal ultrasound showed bright kidneys with poor corticomedullary differentiation. MAG-3 scan showed normal perfusion, uptake and drainage with
no evidence of renal infarction athought there was a high background count. She underwent a
renal biopsy as her plasma creatinine increased four months later. There was evidence of
glomerulosclerosis in two-thirds of her glomeruli and the remaining glomeruli were very
large with focal and segmental sclerosis. A pre-emptive renal transplant was planned but her
renal function continued to deteriorate and due to her Fontan circulation, she was felt not to
be a suitable candidate for haemodialysis and was commenced on peritoneal dialysis 18
months following her initial presentation. It was felt that renal transplantation was possible in
view of her cardiac catherisation showing no systemic desaturation with oxygen saturation of
96% in the ascending aorta and mean systemic venous pressure of 15mmHg with
unobstructed flow through the IVC conduit and following balloon inflation in the IVC for 5
minutes duration there was no change in heart rate or oxygen saturations. She successfully
underwent a living-related renal transplant from her mother (mismatch 1,1,0) and recovered
well. Her complications included a large tear in the renal vein requiring blood transfusions,
mild chronic changes on pre-implantation biopsy, a lymphatic collection and new-onset of
diabetes mellitus after transplantation (NODAT requiring insulin for six weeks). At nine
months of follow-up, she has good renal allograft function (with estimated glomerular
filtration rate of 62mls/min/1.73m2) with no episodes of acute rejection.
CONCLUSION: Renal transplantation is possible in children with complex congenital heart
disease and Fontan circulation.