Canadian Cardiovascular Society Position Statement on Familial Hypercholesterolemia Flashcards

1
Q

What are the Simon Broome Registry criteria for FH?

A
  1. A plasma measurement of either: TC >6.7 (<16yo) or LDL >4.0 (<16yo)
  2. Tendon xanthomas in the patient or any of the patient’s first or second degree relatives
  3. DNA-based evidence in the patient of mutation in LDLR or other FH-related gene
  4. Family history of MI at <50yo in any first or second degree relative OR <60yo in any first degree relative
  5. Family history of of plasma TC >7.5 in any first or second degree relative
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2
Q

What are the Dutch Lipid Clinic Network criteria for FH?

A
  1. First degree relative with premature CVD or LDL-C >95th percentile or personal history of premature peripheral or cerebrovascular disease or LDL-C 4.01-4.89mmol/L
  2. First degree relative with tendinous xanthoma or corneal arcus, or first degree relative <18yo with LDL-C >95th percentile or personal history of coronary artery disease
  3. LDL-C between 4.91-644 mmol/L
  4. Presence of corneal arcus in patient <45yo
  5. LDL-C between 6.46-8.51mmol/L
  6. Presence of tendon xanthoma
  7. LDL-C > 8.53 mmol/L or functional mutation in LDLR gene

Definite FH >8points
Probably FH 6-7 points
Possible FH 3-5 points

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3
Q

What is the recommendation regarding screening in children and adolescents?

A

Screening plasma lipid profile in children with positive family history, poor lifestyle or cardiovascular disease, obesity, smoking, hypertension, or type 2 DM

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4
Q

What is the primary prevention targets for adult FH patients?

A
  1. Consider high risk

2. >50% reduction in LDL-C from baseline

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5
Q

What is the secondary prevention target for adult FH patients?

A

Target LDL-C <2.0mmol/L

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6
Q

What are the recommendations regarding management of FH in children?

A
  1. Lifestyle changes x 12m first (diet, exercise, tobacco-free)
  2. Statins as first line therapy, with ezetimibe and bile acid binding resins as second-line therapy
  3. Niacin is no longer recommended
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7
Q

When should children be referred to a specialized centre and considered for extracorporeal plasma exchange or LDL apheresis and emerging therapies?

A

Homozygous FH patients >7yo and >15kg

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8
Q

What are the recommendations regarding pregnancy and FH?

A

Stop lipid-lowering therapy excld. bile-acid binding resins at least 4 weeks prior to contraception and until cessation of breastfeeding.
Statins are contraindicated in pregnancy

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