Posterior reversible encephalopathy syndrome (PRES) after pediatric heart transplantation: A multi-institutional cohort

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Journal of Heart and Lung Transplantation


Background: PRES is a relatively rare complication of heart transplantation (HTx), and its clinical course and risk factors remain poorly understood. We sought to examine the incidence, natural history, and risk factors for PRES after pediatric HTx. Methods: Fourteen-center retrospective study of 42 PRES-cases and 1,502 HTx-recipients without PRES, between 2000 and 2018. Demographic and clinical data were collected into a central database. Factors associated with PRES were identified by Cox Proportional Hazard multivariable analysis. Results: PRES occurred in 3.2% of HTx-recipients > 1 year of age; no infants developed PRES. Median onset of symptoms was 16 days (R 2-2468 days) after HTx, most commonly seizures (38/42) or altered mental status (24/42). Almost all recipients (40/42) recovered fully. MRI showed typical vasogenic edema in 33/39. Those with PRES had gradually worsening hypertension in the preceding 5 days, culminating in severely elevated blood pressure (>P99+5 mm Hg) for 34 recipients when presenting with PRES. Calcineurin inhibitor (CNI) levels were supratherapeutic in only 8/42, and CNI-management did not significantly impact outcome. Risk of PRES was 12.8x higher in those with pretransplant Glenn/Fontan (CI 1.69, 96.27; p = .013) compared to recipients with congenital heart disease without Glenn/Fontan, while sex, age, BMI or ischemic time did not contribute significantly. Conclusions: Pediatric PRES generally occurs early after HTx, with seizures as the most common presenting symptom. Most patients are severely hypertensive upon presentation, whereas only 19% have supratherapeutic CNI-levels. Pre-existing Glenn or Fontan physiology is a strong risk factor for PRES after HTx. Almost all recipients recovered fully within days of presentation.

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