Identification of risk factors for posterior reversible encephalopathy syndrome (PRES) after organ transplant can improve early detection and avoid permanent neurologic injury. High calcineurin-inhibitor levels and hypertension are recognized risk factors for PRES in adult transplant recipients. Limited data exist regarding PRES after pediatric heart transplant (HTx), with studies limited to case reports. Our aim was to determine the prevalence and clinical features of PRES in pediatric HTx recipients. Based on our own clinical experience, we hypothesized a priori that recipients with Glenn or Fontan physiology (G/F) at time of transplant are at higher risk for PRES. We performed a retrospective review of 128 pediatric HTx recipients < 21 yo, who received their transplant at our institution between 1994-2014. Demographic and clinical risk factors were analyzed and Fisher’s exact test was used to estimate relative risk (RR) of PRES in recipients. 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 years (5.7-19.0), compared to 1.4 years (0.0-19.8) for recipients without PRES (p=0.010). Recipients with PRES did not differ from those without PRES when comparing gender, BMI, total allograft ischemic time, aortic cross-clamp time, or cardiopulmonary bypass time. Fewer than half of recipients with PRES had elevated post-transplant calcineurin-inhibitor levels (n=3) and/or preceding severe hypertension (n=3), both considered traditional risk factors for PRES. Four of seven who developed PRES (57%) had pre-transplant G/F. G/F was a significant risk factor for PRES (RR 4.99, 95% CI: 1.19-21.0, p=0.036). Five (71.0%) recipients returned to their neurological baseline. Two recipients (29%), both with severe PRES, have residual ongoing neurological symptoms. In summary, PRES occurred in 5.5% of pediatric HTx recipients, a higher prevalence than reported in adults. It presented early after HTx, and in older children: all recipients with PRES were > 5 yo. Patients with pre-transplant G/F were at increased risk, a risk factor not previously described.

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 HT x, with studies limited to case reports. We performed a retrospective review of 128 pediatric HT x recipients to identify risk factors for PRES . Seven of 128 (5.5%) recipients developed PRES at a median of 10 days (5–57) after HT x. 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 HT x recipients and presented early after HT x. All recipients with PRES were > 5 yr. Patients with pretransplant G/F were at increased risk, a risks factor not previously described.