Table 2 Variant syndromes and definitions for selection to the adult elective liver transplant waiting list
i. Diuretic resistant ascitesAscites unresponsive to or intolerant of maximum diuretic dosage and non responsive to TIPS or where TIPS deemed impossible or contraindicated and in whom the UKELD score at registration is less than or equal to 49.
ii. Hepatopulmonary syndromeArterial Po2 less than 7.8 kPa. Alveolar–arterial oxygen gradient less than 20 mm Hg. Calculated shunt fraction greater than 8% (brain uptake following technetium macro-aggregate albumin), pulmonary vascular dilatation documented by positive contrast enhanced trans-thoracic echo in the absence of overt chronic lung disease.
iii. Chronic hepatic encephalopathyConfirmed by EEG or trail making tests with at least two admissions in 1 year due to exacerbations of encephalopathy that has not been manageable by standard therapy. Structural or neurological disease must be excluded by appropriate imaging and if necessary psychometric testing.
iv. Persistent and intractable pruritusPruritus consequent on cholestatic liver disease which is intractable after therapeutic trials which might include cholestyramine, ursodeoxycholic acid, rifampicin, ondansetron, naltrexone and after exclusion of psychiatric co-morbidity that might contribute to the itch.
v. Familial amyloidosisConfirmed transthyretin mutation in the absence of significant debilitating cardiac involvement or autonomic neuropathy.
vi. Primary hyperlipidaemiasHomozygous familial hypercholesterolaemia with absent LDL receptor expression and LDL receptor gene mutation.
vii. Polycystic liver diseaseIntractable symptoms due to the mass of liver or pain unresponsive to cystectomy or severe complications secondary to portal hypertension.
  • EEG, electroencephalogram; LDL, low density lipoprotein; TIPS, transjugular intra-hepatic stent.