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Portal vein thrombosis, hepatic decompensation, and survival in patients with porto-sinusoidal vascular disease and portal hypertension

  • Original Article―Liver, Pancreas, and Biliary Tract
  • Published:
Journal of Gastroenterology Aims and scope Submit manuscript

Abstract

Background

Porto-sinusoidal vascular disease (PSVD) is a novel nomenclature to describe non-cirrhotic portal hypertension and characteristic histology without portal vein thrombosis (PVT). It is a more inclusive definition than the previously well-recognized entity idiopathic non-cirrhotic portal hypertension. There is a paucity of data on PSVD patients.

Methods

A total of 33 patients diagnosed with PSVD and portal hypertension (PH) between 2005 and 2021 were included. Data were retrieved from electronic medical record system and analyzed.

Results

Of the 33 patients, 6 (18%) occurred in post-transplant allograft liver. After a median follow-up of 96 months (interquartile range, IQR [52, 139]), 14 deaths occurred (42%), 4 directly related to decompensated liver disease. The Kaplan–Meier survival estimates at 1, 5, and 10 years were 94%, 87% and 58%. PVT occurred in 10 patients (30%). The Nelson–Aalen cumulative risk estimate for PVT at 1, 5 and 10 years were 16%, 25% and 48%. The median model for end-stage liver disease and Child–Pugh score at initial presentation were 8 (IQR [7–12]) and 5 [5–6], and increased to 13 [8, 18] and 7 [5, 8], respectively, at the end of follow-up. Of the 11 patients who presented with splenomegaly and no specific sign of PH, 7 (64%) developed varices and 3 (27%) ascites at a median follow-up of 100 months.

Conclusions

PSVD with PH is not a benign entity. Mortality, PVT and hepatic decompensation are common. Patients with PSVD must be closely monitored, including those who only have non-specific clinical signs (e.g., splenomegaly) of PH.

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Data availability

The data that support the findings of this study are available on request from the corresponding author, A.M. The data are not publicly available due to information that could compromise the privacy of research participants.

Abbreviations

PSVD:

Porto-sinusoidal vascular disease

PVT:

Portal vein thrombosis

PH:

Portal hypertension

INCPH:

Idiopathic non-cirrhotic portal hypertension

NCPH:

Non-cirrhotic portal hypertension

EGD:

Esophagogastroduodenoscopy

TIPS:

Transjugular intrahepatic portal systemic shunt

HVPG:

Hepatic venous pressure gradient

NRH:

Nodulr regenerative hyperplasia

OPV:

Obliterative portal venopathy

MELD-Na:

Model for end-stage liver disease-sodium score

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Acknowledgements

Dr. Michael Voigt contributed to the conceptualization of the study and initiated the cohort collection.

Funding

None.

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Authors and Affiliations

  1. Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of Iowa, 200 Hawkins Drive, Iowa City, IA, USA

    Xiaocen Zhang & Arvind R Murali

  2. Department of Internal Medicine, University of Iowa, 200 Hawkins Drive, Iowa City, IA, USA

    Katelin Marie Durham & Alexander Austin Garza

  3. Liver Center, Orlando Health Digestive Health Institute, 89 W. Copeland Dr., Orlando, FL, USA

    Arvind R Murali

Authors
  1. Xiaocen Zhang
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  2. Katelin Marie Durham
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  3. Alexander Austin Garza
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  4. Arvind R Murali
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Contributions

XZ, conceptualization of study, data analysis, manuscript, statistical analysis, graphics. KD and AG data collection, manuscript. A.M., conceptualization of study, data interpretation, critical review of manuscript.

Corresponding author

Correspondence to Arvind R Murali.

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The authors declare that they have no conflict of interests.

Ethical approval

This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committee of University of Iowa (IRB 202012058).

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No consent specific to the study was obtained from the patients as this was a retrospective chart review involving minimal risk.

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Zhang, X., Durham, K.M., Garza, A.A. et al. Portal vein thrombosis, hepatic decompensation, and survival in patients with porto-sinusoidal vascular disease and portal hypertension. J Gastroenterol 58, 268–276 (2023). https://doi.org/10.1007/s00535-023-01957-0

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  • DOI: https://doi.org/10.1007/s00535-023-01957-0

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