Welsh Familial Hypercholesterolaemia (FH) Scoring criteria

Welsh Familial Hypercholesterolaemia (FH) Score was developed based on a modification of the Dutch Lipid Clinic scoring criteria (1) to guide genetic testing of index patients presenting with hypercholesterolaemia.

• a numeric score is derived from measures of: family history, physical examination, cardiovascular clinical history, LDL-C and triglyceride concentrations

The key differences between these criteria and the Dutch criteria are:

• 1) the Welsh FH criteria, patients score 6 if they or their 1st or 2nd degree relatives have tendon xanthomata (TX)
  • this compares with the Dutch criteria, patients scored 6 if they themselves had tendon xanthomata (TX), but scored only 2 if only their 1st or 2nd degree relatives had TX
    
    
• 2) a minus score is given for raised triglycerides
  • the quantification of raised triglycerides is not a feature of the Dutch criteria, however, the Dutch criteria includes a caveat saying "HDL-cholesterol and triglycerides are normal"
    
    
• 3) the Welsh FH criteria includes a for the development of CHD at a younger age in the index patient (CHD <45 yrs 4, CHD <50yrs 3, CHD <60yrs 2)
  
  
• 4) the Welsh FH criteria has weighted family history of CHD to a greater degree than the Dutch criteria
  • in the Dutch criteria, one point was awarded for CHD in a 1st degree relative under the age of 60
  • in the Welsh FH score this criterion was modified so that a patient scores one point if their 1st or 2nd degree relative had CHD before the age of 60, and two points if their 1st or 2nd degree relative had CHD before the age of 45
    
    
• 5) an LDL-C correction factor table used to estimate the pretreated LDL-C levels of patients on cholesterol lowering medication has been updated to include all available statins with or without ezetimibe

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Reference:

• Dutch Lipid Clinic Network Criteria for making a diagnosis of Familial Hypercholesteroloemia (FH) in adults - http://www.racgp.org.au/your-practice/guidelines/redbook/appendices/appendix-1-dutch-lipid-clinic-network-criteria/
• Haralambos K et al. Clinical experience of scoring criteria for Familial Hypercholesterolaemia (FH) genetic testing in Wales.Atherosclerosis. 2015 May;240(1):190-6.