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Objectives Portal hypertension (PHT) is the main complication of cystic fibrosis liver disease (CFLD) but severe liver dysfunction occurred only in 0.5% of the patients. The early diagnosis of CFLD is elusive and a gold standard for diagnosis of liver disease is lacking. The aim of this study was to evaluate liver stiffness measurement (LSM) and CT with IV injected contrast medium in CF adult patients. Median LSM in 241 CF patients was 5.9 kPa. Methods 57 CF patients (28F, 33 yrs −18; 51) had LSM and CT done for lung transplant assessement or after lung transplantation. Results LSM was obtained in 53 patients (92%). PHT was evident on CT in 25 patients (portosystemic collaterals 12, splenomegaly 19, proeminent portal vein 25). PHT was present in 60% of the patients when LSM was >5.9 and in 12% when LSM was Conclusion CFLD with PHT could be present in patient with low LSM. Decompensation of PHT is unfrequent even in adults with end stage pulmonary disease or after lung transplantation. Portal venopathy without cirrhosis could explain PHT. The mechanism remains obscure but could explain the rare occurence of hepatocellular deficiency in CFLD.

The prevalence of CF related liver disease (CFLD) ranges from 26% to 45%. The pathogenesis is allegedly related to small bile duct obstruction leading to focal biliary cirrhosis and cirrhosis. Portal hypertension (PHT) without hepatocellular insufficiency is the main presentation of CFLD. The aim of this study was to establish the pathologic pattern in CFLD patients with severe PHT. Patients and Methods In 2 CF centers, all consecutive patients who underwent liver transplantation were retrospectively analysed. Results Eight patients (7 males; 14–32 y) were analysed. All presented with severe PHT: esophageal varices more than grade 2 (bleeding in 2 patients), splenomegaly 18–30 cm, collaterals or ascites. None had signs of hepatocellular insufficiency. Liver tests were less than 1.5 times elevated. Macroscopically, all explants (mean weight 1.6 kg) were dysmorphic with left atrophy and irregular macronodular aspect. There was no thrombosis of the large veins and large bile ducts were normal. Microscopically, all explants showed septal fibrosis without cirrhosis (F2 or F3 Metavir Score, n = 4 each). Diffuse obliterative portal venopathy with sinusoidal dilatation and regenerative nodular hyperplasia was observed in all, associated with frequent ductopenia in small portal tracts, without cholestasis or inflammation. Biliary plugs with ductular reaction were scarce, medium or large biliary ducts were normal. Conclusion According to histological examination of the native livers, CFLD with PHT is not related to biliary cirrhosis but responds to vascular damages as observed in idiopathic non-cirrhotic PHT. The mechanism leading to vascular lesion in CF is unknown.

Objectives PHT is the main manifestation of liver disease in adults CF patients. The risk of hepatic decompensation after isolated LT is unknown. The model for end-stage liver disease (MELD) score is not relevant for the indication to combined lungs-liver transplantation. We presented the postoperative outcome of isolated lung transplantation in CF patients with asymptomatic PHT. Methods From 2008 to 2014, 14 isolated LT (over 299) were proceeded over CF patients with PHT. Results All Patients had asymptomatic liver disease before LT but all had PHT on the CT scan: splenomegaly (14–19 cm) in 10 patients, portosystemic collaterals in 8, all had portal vein dilatation >14 (diameter 17±2.3 mm). The liver stiffness measurement (LMS) was elevated 18±8 kPa when in our adult CF center median LSM was 5.9 kPa in 241 CF patients. Thirteen patients had normal liver and renal function. Gastroesophageal varices were absents in 10 screened patients. After LT, 4 patients required ECMO, 6 developed easy to treat ascites associated with transient renal insufficiency (4) or intestinal complications (2). One developed transient ascitis 7 months after LT associated with sepsis and renal dysfunction. Hepatocellular function remain normal in all patients. Every patient is still alive (mean time 42±22 months). Conclusion Carefully selected CF patients with end-stage lung disease and portal hypertension can tolerate isolated lung transplantation. The non-invasive evaluation of the severity of PHT has to be evaluated. Prospective multicentric study is need to clearly define the indications of combined lung–liver transplantation.