Coronary Heart Disease
Positive Monogenic Risk
Monogenic risk for Coronary Heart Disease (CHD) results from a pathogenic variant(s) in any of the following genes: LDLR, APOB, PCSK9, and LDLRAP1. A positive monogenic risk increases CHD risk by 2-3-fold. For participants with monogenic risk, recommendations include:
- Referral to a genetic counselor.
- Check a lipid profile and Lp(a) level.
- Consider lipid-lowering therapy (e.g., statin):
- Reduce LDL-cholesterol using maximally tolerated statin therapy +/- ezetimibe; PCSK9 inhibitors may be considered if target LDL-C is not achieved despite maximal statin dose.
- Follow the 2018 AHA/ACC Guideline on the Management of Blood Cholesterol in patients with Familial Hypercholesterolemia.
- Consider referral to a lipid specialist.
- Emphasize a heart-healthy lifestyle.
High PRS
A high PRS (top 5th percentile) is associated with a 1.7-2.3 times increased risk for developing CHD relative to a person not in the high-risk category. The data is based on populations of European, Hispanic/Latino, Asian, and African descent. Information is insufficient or not available for populations of other descent. For participants with a high PRS, recommendations include:
- Check a lipid profile and Lp(a) level.
- In those aged ≥40 years, consider further screening such as a coronary calcium scan and treatment with a statin to reduce CHD risk. Shared decision-making is recommended.
- In those aged <40 years, consider carotid ultrasound to detect plaque/measure intima-media thickness to assess for early disease if such an imaging modality is available.
- Treat risk factors if present: high blood pressure, diabetes, and high cholesterol.
- Lifestyle changes: smoking cessation if a smoker, increase physical activity, weight loss if elevated body mass index, and heart-healthy diet.
Positive Family History
Positive family history of CHD is defined as onset of CHD in a male first-degree relative before age 55 years, or in a female first-degree relative before age 65 year. A positive family history of CHD increases CHD risk by 1.5–2 folds. For participants with a positive family history, recommendations include:
- Check a lipid profile and Lp(a) level.
- In those aged ≥40, consider further screening such as a coronary calcium scan and treatment with a statin to reduce CHD risk. Shared decision-making is recommended.
- In those aged <40, consider carotid ultrasound to detect plaque/measure intima-media thickness to assess for early disease if such an imaging modality is available.
- Treat risk factors if present: high blood pressure, diabetes, and high cholesterol.
- Lifestyle changes: smoking cessation if a smoker, increase physical activity, weight loss if elevated body mass index, and heart-healthy diet.
10-year CHD Integrated Score
For participants with monogenic risk/high PRS/family history, the GIRA report displays two estimates for the 10-year CHD risk: one calculated using the PCE and the other calculated by combining PRS and PCE. The 10-year CHD risk is calculated for those aged 40-79 years and is applicable only to individuals without known CHD.
More PCE details
For participants with a 10-y CHD risk ≥7.5%, consider shared-decision making regarding initiation of statin therapy to reduce the risk of CHD.