Posterior reversible encephalopathy syndrome after pediatric heart transplantation: Increased risk for children with preexisting Glenn/Fontan physiology

Pediatr Transplant. 2016 Jun;20(4):552-8. doi: 10.1111/petr.12702. Epub 2016 May 3.

Abstract

Identification of risk factors for PRES after organ transplant can improve early detection and avoid permanent neurological injury. High calcineurin-inhibitor levels and hypertension are recognized risk factors for PRES in adult transplant recipients. Limited data exist regarding PRES after pediatric HTx, with studies limited to case reports. We performed a retrospective review of 128 pediatric HTx recipients to identify risk factors for PRES. Seven of 128 (5.5%) recipients developed PRES at a median of 10 days (5-57) after HTx. The median age of recipients with PRES was 10.0 yr (5.7-19.0), compared to 1.4 yr (0.0-19.8) for recipients without PRES (p = 0.010). Fewer than half of recipients with PRES had elevated post-transplant calcineurin-inhibitor levels (n = 3) and/or preceding severe hypertension (n = 3). Four of seven who developed PRES (57%) had pretransplant Glenn or Fontan physiology (G/F). G/F was a significant risk factor for PRES (RR 4.99, 95% CI: 1.19-21.0, p = 0.036). Two recipients (29%), both with severe PRES, had residual neurological symptoms. In summary, PRES occurred in 5.5% of pediatric HTx recipients and presented early after HTx. All recipients with PRES were > 5 yr. Patients with pretransplant G/F were at increased risk, a risks factor not previously described.

Keywords: Fontan; heart transplantation; pediatric; posterior reversible encephalopathy syndrome.

Publication types

  • Observational Study

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Female
  • Fontan Procedure
  • Heart Transplantation*
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Posterior Leukoencephalopathy Syndrome / epidemiology
  • Posterior Leukoencephalopathy Syndrome / etiology*
  • Postoperative Complications / epidemiology
  • Postoperative Complications / etiology*
  • Prevalence
  • Retrospective Studies
  • Risk Factors
  • Young Adult