title: Posterior reversible encephalopathy syndrome after pediatric heart transplantation: Increased risk for children with preexisting Glenn/Fontan physiology.
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.
authors.affiliation: School of Medicine, University of Washington, Seattle, WA, USA.
authors.forename: Braiden
authors.initials: B
authors.lastname: Eilers
authors.affiliation: Division of Pediatric Cardiology, Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
authors.forename: Erin
authors.initials: E
authors.lastname: Albers
authors.affiliation: Division of Pediatric Cardiology, Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
authors.forename: Yuk
authors.initials: Y
authors.lastname: Law
authors.affiliation: Division of Pediatric Cardiothoracic Surgery, Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
authors.forename: D Mike
authors.initials: DM
authors.lastname: McMullan
authors.affiliation: Division of Radiology, Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
authors.forename: Dennis
authors.initials: D
authors.lastname: Shaw
authors.affiliation: Division of Pediatric Cardiology, Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
authors.forename: Mariska
authors.initials: M
authors.lastname: Kemna
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D054038:Posterior Leukoencephalopathy Syndrome / Q000453:epidemiology / Q000209:etiology*; D011183:Postoperative Complications / Q000453:epidemiology / Q000209:etiology*; D015995:Prevalence; D012189:Retrospective Studies; D012307:Risk Factors; D055815:Young Adult
keywords: Fontan; heart transplantation; pediatric; posterior reversible encephalopathy syndrome