Portal vein thrombosis in non-cirrhotic individuals, although uncommon, is an increasingly explored condition that affects mainly young people, consequently representing a significant disease burden. Reports primarily including western European populations have recently shed light regarding the pathophysiology, risk factors, natural history, treatment, and prognosis of this entity. Underlying predisposing conditions are documented in ~70% of cases, encompassing local risk factors, inherited and acquired thrombophilia, cancer, and systemic inflammatory conditions. Non-cirrhotic portal vein thrombosis can cause significant portal hypertension in the acute setting, but, more frequently, significant portal hypertension-related complications arise when the condition becomes chronic and portosystemic collaterals develop, increasing the risk for variceal bleeding and ascites. The diagnostic approach to screen for underlying thrombophilia remains a challenge, and recommendations in this regard, although scarce and backed by scarce evidence, have changed notably in the last years, leaning toward a universal screen in patients who develop this condition without a clear provoking factor. Recently, studies have shown that long-term anticoagulation may be appropriate even in the absence of clear provoking factors or underlying thrombophilia. Future studies should address which patients may benefit from this approach, which patients may not need it, and what the most appropriate strategies are to approach patients who do not recover portal vein patency with anticoagulation to further prevent portal hypertension-related complications.

Portal venous system thrombosis (PVT) outside the setting of cirrhosis is uncommon with limited information available about the etiological and clinical characteristics across varied racial and ethnic groups. This retrospective cohort study examines the long-term outcomes of non-cirrhotic, Hispanic adults diagnosed with PVT at a single center in Mexico City between January 2000 and August 2023. Patients with conditions predisposing to PVT were excluded. We included 100 Hispanic adults diagnosed with non-cirrhotic PVT. Thrombophilia was identified in 49%, with antiphospholipid syndrome (APS) being the most prevalent thrombophilia (23%), followed by JAK2 mutation (18%). Chronic PVT, observed in 70% of cases, predominantly affected the portal vein (50%), followed by porto-mesenteric (41%) and porto-splenic (9%) territories. At diagnosis, 55% had esophageal varices. Anticoagulant therapy was administered to over half of the patients for more than 12 months. Over a median follow-up of 55 months, the 5-year risk of re-thrombosis was 24%, and the 5-year risk of variceal bleeding (VB) was 45%. The 4-year overall survival (OS) was 97%. Comparative analysis between thrombophilia-associated and idiopathic PVT did not reveal significant differences in VB, re-thrombosis, and OS. This study underscores the unique clinical profile of Hispanic patients with non-cirrhotic PVT, highlighting a high prevalence of APS and substantial risks of VB. These findings contribute to a better understanding of PVT in Hispanics and emphasize the importance of tailored management strategies. Our results' generalizability may be limited by the thrombophilia testing approach, the excluded populations, and its retrospective, single center nature.