This retrospective study tries to determine the safety and efficacy of medically assisted reproduction in women with known Budd-Chiari syndrome, portal vein thrombosis or porto-sinusoidal vascular disease. Women with vascular liver diseases who underwent medically assisted reproduction between 2000 and 2018 will be included. This includes controlled pharmacologic stimulation of ovarian follicles, paired or not with assisted reproduction technologies. Vascular liver disease should be diagnosed prior to medically assisted reproduction.
Liver and spleen stiffness by 2D-shear-wave elastography to detect porto-sinusoidal vascular liver disorder in patients with signs of portal hypertension
In 2019, VALDIG proposed the term of porto-sinusoidal vascular liver disease (PSVD) to describe a group of rare vascular liver entities characterized by histological lesions involving portal venules and/or sinusoids and absence of cirrhosis, with or without signs of portal hypertension (1). Recently, the Baveno VII consensus workshop preferred the term “disorder” instead of “disease” so that PSVD now stands for “porto-sinusoidal vascular liver disorder”. PSVD is defined as follows:
- Adequate liver biopsy (≥20 mm long, and not too fragmented, or considered adequate for interpretation by an expert pathologist) or liver explant without cirrhosis AND one sign specific for portal hypertension or one histological lesion specific for PSVD;
- Or adequate liver biopsy (≥20 mm long, and not too fragmented, or considered adequate for interpretation by an expert pathologist) or liver explant without cirrhosis AND one sign not specific for portal hypertension AND one histological lesion not specific for PSVD.
Histology is therefore mandatory for the diagnosis of PSVD, with a high quality liver biopsy, and an interpretation by an expert pathologist. Non-invasive tests appear useful tools to screen for PSVD. Elkrief et al. showed that liver stiffness measurement using transient elastography is able to raise suspicion of PSVD in patients with signs of portal hypertension with high accuracy (2). Ferreira-Silva et al. found that spleen stiffness measurement using transient elastography has a good performance in predicting high-risk varices in patients with non-cirrhotic portal hypertension (3), but this result was not observed by other groups (4).