Long-term outcomes in patients with porto-sinusoidal vascular disorder: a single-centre 10-year follow-up study

Objective Porto-sinusoidal vascular disorder (PSVD) is a recently described nomenclature with limited data on long-term outcomes. We evaluated the outcomes in a cohort of PSVD patients and determined whether these differed from patients classified as per the previous idiopathic non-cirrhotic portal hypertension (INCPH) criteria. Design In this single-centre retrospective study, baseline characteristics and liver-related outcomes were evaluated in patients with PSVD. The prognostic performance of the nomogram from the Vascular Liver Disease Group (VALDIG) consortium was assessed. Outcomes were compared in patients categorised as PSVD (PSVD+/INCPH-) as per new nomenclature with those who, in addition, fulfilled previous INCPH (PSVD+/INCPH+) criteria. Results Eighty patients were included (mean age: 44 years and 38% females). History of PH-related complications was present in 36 (45%) patients at diagnosis. Median follow-up duration from diagnosis was 78 (IQR: 27–132) months. The cumulative 5-year and 10-year probability of liver-related events was as follows: PH-related bleed (15.4% and 21.8%), new onset/worsening ascites (21.6% and 35%), splanchnic vein thrombosis (15.9% and 21%) and transplant-free survival (70.5% and 60.72%), respectively. The VALDIG nomogram showed good discrimination (optimism-corrected C-statistic: 0.769) but over-estimated survival. Patients classified as PSVD+/INCPH+ (n=26) were younger (mean age: 39 vs 48 years, p=0.013), had a higher prevalence of varices (92% vs 59%, p=0.027), but had similar outcomes (10-year survival: 63.5% vs 59.3%; HR, 0.76 (95% CI 0.33 to 1.75), p=0.52) compared with the PSVD+/INCPH− group (n=54). Conclusion This study describes long-term outcomes in PSVD from a quaternary centre. VALDIG prognostic nomogram may need recalibration through further external validation. Outcomes were similar irrespective of the nomenclature used to define this cohort.