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.

Liver transplantation during infancy: No increased rate of neurological complications

“…Relevant clinicoradiological differences are most apparent when comparing adult and pediatric patients . Moreover, it appears that within the pediatric collective PRES occurs markedly less often in younger than older children …”

Etiologies, Cerebral Vasomotion, and Endothelial Dysfunction in the Pathophysiology of Posterior Reversible Encephalopathy Syndrome in Pediatric Patients

“…7,10, 20,34,47,50,54,69,70,116,[165][166][167][186][187][188][189] The impressive, varied, and myriad conditions and diseases in patients developing PRES have both enhanced and complicated our theories on the pathogenesis of the condition. In general, the most common precipitants giving rise to PRES in the adult and pediatric populations include hypertension, renal disease, solid organ and hematologic malignancy, 9, 15,39,42,190 chemotherapy, [17][18][19][20][21][22][23][24][25][26] solid organ transplantation, 16,[35][36][37][191][192][193] and immunosuppressive therapy. 31,73,120,[194][195][196][197][198][199] Solid organ malignancies reported contemporaneously with the development of PRES include brainstem ependymoma, 200 hepatoblastoma, …”

“…The overall prevalence of PRES in hospitalized pediatric patients approximates 0.4%, 32 with renal disease, [327][328][329][330][331][332] hypertension, 32 malignancy, 34,71 and solid organ transplantation 16,[35][36][37][191][192][193] and HSCT 68,250,[256][257][258][259] representing the most frequent etiologies. Clinical findings of moderate to severe headache, decreased level of consciousness, visual disturbances, focal neurologic deficits, and epileptic activity, in conjunction with T2 and FLAIR hyperintensities of the supratentorial white and gray matter and, occasionally, contents of the posterior cranial fossa, effectively establish the diagnosis.…”

“…7 Afflicted patients often have renal dysfunction or arterial hypertension and an underlying solid organ or hematologic malignancy, or have recently received a solid organ or hematopoietic stem cell transplantation (HSCT), 7,8,10, 15,16 chemotherapy, [17][18][19][20][21][22][23][24][25][26] or immunosuppressive therapy. [27][28][29][30] In the pediatric population, renal disease, 31 hypertension, 32 hematologic disease, 22,33 malignancy, 34 chemotherapy, 32 and transplantation 16,32,[35][36][37] represent the most common precipitants of PRES. 12,13,20,34,35,[38][39][40][41][42][43][44][45][46][47][48][49][50][51][52] PRES may also arise postope...…”

Rapid cycling bipolar disorder post-heart transplant: A case report

“…Reports on psychiatric complications following transplant surgery are usually confined to younger people. In the case of heart transplants, posterior reversible encephalopathy syndrome (PRES) is well documented in both children [1] and adults [2], whereas reports about other psychiatric syndromes following heart transplant are rare. Depression [3][4][5], post-traumatic stress disorder, anxiety, adjustment disorder [6] and affective disorders [6,7] have all been documented within the first 3-6 years post-heart transplant with their prevalence being very high.…”