Clin Transplant Res.  2024 Jun;38(2):154-162. 10.4285/ctr.24.0009.

Clinical features and outcomes of posterior reversible encephalopathy syndrome after heart transplantation: a case series

• Lim JH
• Kim SH
• Mo C
• Kim HW
• Lee SY

• KMID: 2557610
• DOI: http://doi.org/10.4285/ctr.24.0009

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a rare neurological disease that may be associated with hypertension, autoregulatory failure, and the use of calcineurin inhibitors following heart transplantation (HT). In this article, we present a case series of PRES, discussing its potential causes and management strategies. Among the 126 HT recipients at our hospital, four were diagnosed with PRES. Three of these patients developed PRES within 7 days after HT. Prior to the onset of PRES, all patients experienced sustained hypertension, and strict blood pressure (BP) control was maintained. Three of the four patients recovered without PRES recurrence, while one patient died of sepsis after an episode of altered consciousness. Hypertension was observed in all patients prior to the onset of PRES, and the majority experienced symptom improvement with BP control. While most cases of PRES were reversible with conservative treatment, including the administration of antiepileptics, one irreversible case resulted in in-hospital mortality. Thus, PRES can have serious outcomes and is not invariably benign.

Keyword

Posterior reversible encephalopathy syndrome; Calcineurin inhibitors; Heart transplantation; Case report

Figure

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  Fig. 1 Radiological examination results of four patients with PRES following heart transplantation. Case 1: CT reveals subtle areas of decreased density in both parietal lobes. MRI demonstrates diffuse high signal intensity along the sulci of the frontal, parietal, and occipital lobes on DWI (arrows). Additionally, multiple small microbleeds (yellow arrows) are scattered throughout both cerebral hemispheres. Case 2: CT scan does not reveal specific findings. However, MRI with DWI shows areas of restricted fluid (arrows) in the right parieto-occipital lobe, and multiple microbleeds (yellow arrows) are observed in the cerebrum and cerebellum on SWI. Case 3: CT indicates a subtle area of decreased density with a mass effect in both parieto-occipital lobes. MRI indicates a localized area of restricted diffusion (arrow) in the right parieto-occipital lobe on both FLAIR and T2-weighted sequences. Furthermore, multiple microbleeds (yellow arrows) are present in the cerebral and cerebellar hemispheres as well as the brainstem. Case 4: CT shows a gradually resolving small SDH (arrow) along the right parietotemporal cerebral convexity. MRI displays a small chronic SDH (arrow) in the right parietal cerebral convexity. Several microbleeds (yellow arrow) are observed in both cerebral hemispheres. CT, computed tomography; DWI, diffusion-weighted imaging; ADC, apparent diffusion coefficient; SWI, susceptibility-weighted imaging; FLAIR, fluid-attenuated inversion recovery; PRES, posterior reversible encephalopathy syndrome; MRI, magnetic resonance imaging; SDH, subdural hematoma.

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  Fig. 2 (A) Blood pressure (BP) trends before and after posterior reversible encephalopathy syndrome (PRES) symptom onset. Measurements were taken at 3-hour intervals. Persistently elevated BP was observed before the onset of symptoms. (B) BP trends before and after transplantation for the four patients with PRES.

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  Fig. 3 The two primary hypotheses of posterior reversible encephalopathy syndrome (PRES) are illustrated in the box. The left side presents the hyperperfusion-hypoperfusion hypothesis. According to this concept, elevated blood pressure that surpasses the autoregulatory capacity of blood vessels causes them to dilate. This dilation leads to sustained hyperemia and, consequently, perivascular edema. In turn, the hypoperfusion theory posits that an overactive autoregulatory response to high blood pressure results in excessive vasoconstriction. This can cause ischemia, swelling, and hemorrhage due to the overly constricted blood vessels. The right side of the box illustrates the hypothesis that PRES is induced by toxicity from calcineurin inhibitors (CNIs). This hypothesis proposes that CNIs stimulate the vascular endothelium to release cytokines, which in turn activate cytotoxic T lymphocytes. The activated T cells inflict damage on the endothelium, causing hemorrhage and edema. Furthermore, this hypothesis includes the increased secretion of endothelin, which induces vasoconstriction and subsequent ischemia. It also involves the activation of astrocyte vascular endothelial growth factor (VEGF) secretion, which damages endothelial cell junctions, resulting in hemorrhage and edema.

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