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Posterior Reversible Encephalopathy Syndrome After Pediatric Heart Transplantation: Increased Risk for Children with Preexisting Glenn/Fontan Physiology.

M. Kemna,1 B. Eilers,2 E. Albers,1 Y. Law.1

1Pediatric Cardiology, Seattle Children's Hospital, Seattle, WA
2School of Medicine, University of Washington, Seattle, WA.

Meeting: 2016 American Transplant Congress

Abstract number: B149

Keywords: Heart transplant patients, Neurotoxicity, Pediatric, Post-transplant hypertension

Session Information

Session Name: Poster Session B: Hearts and VADs in Depth - The Force Awakens

Session Type: Poster Session

Date: Sunday, June 12, 2016

Session Time: 6:00pm-7:00pm

 Presentation Time: 6:00pm-7:00pm

Location: Halls C&D

Study aim:

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 that recipients with Glenn or Fontan physiology (G/F) at time of transplant are at higher risk for PRES, and we explored this group separately.

Methods:

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 with pretransplant G/F.

Results:

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). Four of seven who developed PRES (57%) had pre-transplant 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). Five (71.0%) recipients returned to their neurological baseline. Two recipients (29%), both with severe PRES, have residual ongoing neurological symptoms.

Conclusion:

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.

CITATION INFORMATION: Kemna M, Eilers B, Albers E, Law Y. Posterior Reversible Encephalopathy Syndrome After Pediatric Heart Transplantation: Increased Risk for Children with Preexisting Glenn/Fontan Physiology. Am J Transplant. 2016;16 (suppl 3).

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To cite this abstract in AMA style:

Kemna M, Eilers B, Albers E, Law Y. Posterior Reversible Encephalopathy Syndrome After Pediatric Heart Transplantation: Increased Risk for Children with Preexisting Glenn/Fontan Physiology. [abstract]. Am J Transplant. 2016; 16 (suppl 3). https://atcmeetingabstracts.com/abstract/posterior-reversible-encephalopathy-syndrome-after-pediatric-heart-transplantation-increased-risk-for-children-with-preexisting-glennfontan-physiology/. Accessed May 9, 2025.

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