Despite advances in knowledge about HPS, there are few publications about patients with portal hypertension, who do not have cirrhosis, but who do have this syndrome. There are a number of hepatic entities for which there have been reported cases of HPS other than cirrhosis. They include idiopathic portal hypertension, portal thrombosis, congenital hepatic fibrosis, Budd - Chiari syndrome, hepatic metastases, neonatal hepatitis and nodular regenerative hyperplasia.
Son varias la entidades hepáticas asociadas a SHP diferentes a cirrosis; aunque no son claros los mecanismos mediante los cuales se desarrolla SHP en patologías tales como hipertensión portal idiopática, trombosis portal, fibrosis hepática congénita, síndrome de Budd-Chiari, metástasis hepáticas, hepatitis neonatal y la hiperplasia nodular regenerativa en las que se han reportado casos de SHP 13.