Portal hypertension is associated with poor prognosis in cirrhotic patients

A large analysis of healthcare records revealed that, despite optimal current care, the prospects are gloomy for adults and children with portal hypertension – independent of aetiology. This underlines the need for novel therapeutic options to improve the long-term survival of these patients.

In 2017, the Global Burden of Disease study reported over 1.32 million cirrhosis-related deaths globally, which is approximately 2.4% of all deaths worldwide [1]. At present, there is limited data estimating the prognosis of portal hypertension in children. A retrospective cohort study was designed to assess the prevalence of prespecified aetiologies of cirrhosis-related portal hypertension and to compare the prognosis among children and adults [2]. Data was derived from the electronic healthcare record TriNetX´s Analytics. Data from January 2007 to December 2020 was included with ICD-10 codes of prespecified aetiological diagnoses associated with cirrhotic portal hypertension. Assessed were event rates for complications and recorded mortality occurring within a 24-month follow-up period.

The 3 most commonly occurring prespecified aetiologies in the adult cohort (n=60,579) were alcoholic liver disease, followed by chronic viral hepatitis and non-alcoholic steatohepatitis. In contrast, most cases in the paediatric cohort (n=1,679) occurred due to biliary atresia (23%), followed by primary sclerosing cholangitis (14%). In the paediatric cohort, almost half of the study population (49.1%) had a claim of at least 1 adverse event in 2 years of follow-up, most frequently variceal haemorrhage in 33.8% and liver transplant in 26.3%. A further 15.9% underwent variceal band ligation to prevent upper GI bleeding. The 24-month survival probability was below 70% in patients developing variceal haemorrhage without intervention.

The composite event rate of liver outcomes and death in the adult cohort approached 75% and 20% in patients with or without variceal haemorrhage, respectively, with more than half of this incidence happening within the first 6 months.

The researchers summarised that, despite optimal current care, disease progression including variceal haemorrhage, need for liver transplant, and ultimately death is common in both adults and children with portal hypertension. This highlights the urgent need for new medical therapies to improve the prognosis of these patients.

1. Sepanlou SG, et al. Lancet Gastroenterol Hepatol. 2020;5:245–66.
2. Kulkarni S, et al.Portal hypertension secondary to cirrhosis is associated with poor prognosis, irrespective of age and aetiological differences – a large epidemiological study. LB05, UEG Week 2022, 8-11 October, Vienna, Austria.

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Posted on 2 December, 2022