Porto-sinusoidal vascular disease: redefining indications for liver transplantation

Introduction. Portal hypertension (PH), traditionally regarded as a consequence of liver cirrhosis (LC), in recent decades become the focus of new conceptual frameworks describing its development in non-cirrhotic liver diseases. These observations have led to the identification of a new nosological entity termed a porto-sinusoidal vascular disease of the liver (PSVD), and first proposed by the VALDIG expert group in 2019.

Case report. This report describes a clinical case of a 34-year-old female patient with PSVD complicated by severe PH, who underwent living donor liver transplantation (LDLT). The presented case highlights the diagnostic challenges and clinical heterogeneity of PSVD, emphasizing the importance of timely recognition of this condition and the need for an individualized approach to determining indications for liver transplantation.

Discussion. Despite the emergence of the new PSVD concept, many key aspects of management remain controversial. In particular, there is ongoing debate regarding the selection criteria for liver transplant candidates and the effectiveness of existing therapeutic algorithms. The applicability of standard scoring systems such as Child–Turcotte–Pugh and MELD 3.0 in this pathology also remains uncertain.

Conclusion. The diagnosis of PSVD presents significant challenges, largely due to limited awareness among clinicians, as well as the lack of standardized diagnostic criteria and clinical guidelines. Furthermore, indications for liver transplantation in PSVD have not yet been fully standardized. This report aims to discuss these issues and raise awareness about PSVD, which we believe may contribute to improving the quality and effectiveness of patient care.

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