Screening and diagnosis

Classification and risk stratification

Perioperative care

Quality improvement

Patient education

Nonpharmacologic interventions

Therapeutic procedures

Respiratory support

Follow-up and surveillance

Specific circumstances

Preventative measures

Medical management

Surgical interventions

Inpatient care

Diagnostic procedures

Diagnostic investigations

Insufficient evidence to make a recommendation.

Strongly favor this test or intervention.

The DLCN criteria for familial hypercholesterolemia (FH) calculator is designed to assess the likelihood that an individual has FH based on specific factors. To determine this, the calculator evaluates five key aspects and assigns a numerical score to each based on predefined criteria.

Family history considerations include whether a first-degree relative has premature coronary or vascular disease—males under 55 or females under 60—or elevated LDL-C levels above the 95th percentile, which contributes 1 point. If a relative has tendinous xanthomata or arcus cornealis, or if children under 18 have high LDL-C, the score is 2.

In regard to clinical history, if the patient has premature coronary artery disease, this is worth 2 points. Premature cerebral or peripheral vascular disease in the patient contributes 1 point.

Physical examination findings, such as tendinous xanthomata, are notably significant, adding 6 points to the score, while observing arcus cornealis before 45 years of age adds 4 points.

For LDL-C levels measured without treatment, a result of 8.5 mmol/L (325 mg/dL) or higher is assigned 8 points; levels between 6.5 and 8.4 mmol/L (251-325 mg/dL) score 5 points; a range of 5.0 to 6.4 mmol/L (191-250 mg/dL) adds 3 points; and 4.0 to 4.9 mmol/L (155-190 mg/dL) results in 1 point.

Discovering a functional mutation in the LDLR, apoB, or PCSK9 genes through DNA analysis also heavily influences the determination by adding 8 points.

After collecting all responses, the individual scores are summed to form a total score which guides the interpretation. If the score is greater than 8, the individual is considered to meet the criteria for definite FH. A score ranging from 6 to 8 suggests probable FH, while scores between 3 and 5 indicate possible FH. Scores falling below 3 imply that the criteria for FH are not met. These scores and interpretations guide clinicians in determining the likelihood of familial hypercholesterolemia based on the patient's information.

The calculator in question is the Dutch Lipid Clinic Network (DLCN) criteria for Familial Hypercholesterolemia (FH). This scoring system is used to diagnose patients with familial hypercholesterolemia, a genetic disorder characterized by high cholesterol levels, specifically very high levels of low-density lipoprotein (LDL), in the blood and early cardiovascular disease.

The DLCN criteria for FH is a comprehensive scoring system that takes into account the patient's personal and family history of premature coronary artery disease, LDL cholesterol levels, presence of physical signs related to high cholesterol (such as tendon xanthomas), and genetic testing results. 

The clinical utility of this calculator is to aid in the diagnosis of FH, which can be challenging due to the wide range of phenotypic expressions. Early diagnosis of FH is crucial as it allows for prompt initiation of lipid-lowering therapy to reduce the risk of atherosclerotic cardiovascular disease.

There are no specific exclusion criteria for the use of the DLCN criteria. However, it should be noted that this scoring system is less useful in populations with a high prevalence of polygenic hypercholesterolemia or in individuals with secondary causes of hypercholesterolemia, as it may overestimate the likelihood of

DLCN criteria for familial hypercholesterolemia

The Atherosclerotic Cardiovascular Disease Risk (ASCVD) risk score estimator is a tool designed to assess the 10-year risk of developing atherosclerotic cardiovascular disease for individuals between the ages of 40 and 79. It considers a variety of personal health inputs including age, gender, race, cholesterol levels, blood pressure, smoking status, diabetes status, and whether the individual is undergoing blood pressure treatment.

Inputs for the ASCVD calculator include:

- Age in years
- Gender: categorized as Male or Female
- Race: either African American or Other
- Total Cholesterol, measured in mg/dL or mmol/L
- HDL Cholesterol, measured in mg/dL or mmol/L
- Systolic Blood Pressure, measured in mmHg or kPa
- Smoking status: Yes or No
- Diabetes status: Yes or No
- Blood Pressure Treatment status: Yes or No

Here's how the ASCVD calculation unfolds:

1. **Unit Conversion**:
   - If the cholesterol levels are given in mmol/L, they're multiplied by 38.67 to convert to mg/dL.
   - If the systolic blood pressure is in kPa, it is converted to mmHg by multiplying by 7.50062.

2. **Selection of Coefficients**:
   - Coefficients specific to the individual's race and gender form the basis of the calculations. These include distinctive values related to age, cholesterol levels, HDL levels, and systolic blood pressure, as well as the statuses of smoking and diabetes.

3. **Logarithmic Transformation**:
   - Intermediate values are calculated using logarithmic functions for age, total cholesterol, HDL cholesterol, and systolic blood pressure.

4. **Risk Score Calculation**:
   - The calculator amalgamates various terms, including age-related factors, cholesterol and HDL contributions, systolic blood pressure, smoking, diabetes, and medication treatment, to derive a holistic score. This score is derived by combining the selected coefficients with the intermediate logarithmic values.

5. **Conversion to Risk Percentage**:
   - Using race- and gender-specific constants, the intermediate risk score is translated into a percentage risk value. This is achieved by adjusting the score and converting it to a percentage of 100 using exponential functions.

6. **Risk Interpretation**:
   - The calculated risk percentage is categorized into one of four distinct risk levels:
     - Low risk: Less than 5%
     - Borderline risk: Between 5% and 7.5%
     - Intermediate risk: Between 7.5% and 20%
     - High risk: 20% or more

The result of this calculation is a comprehensive output detailing the risk percentage and its corresponding risk category. This information is crucial for individuals and healthcare providers aiming to tailor preventive practices and interventions based on the assessed cardiovascular risk.

The ASCVD calculator estimates the 10-year risk of heart attack or stroke in adults aged 40-79 based on factors like age, cholesterol, blood pressure, diabetes, and smoking. It follows ACC/AHA guidelines to aid decisions on preventive measures, including statin use.

Atherosclerotic Cardiovascular Disease Risk (ASCVD) risk score

Dyslipidemia

Omega-3 fatty acid supplements

Patients with familial hypercholesterolemia

Patients with familial hypercholesterolemia (CVD risk stratification)

Patients with hypertriglyceridemia

Patients with hypertriglyceridemia, general principles

Fat intake

Patients with endocrine disorders

Patients with endocrine disorders (adult GHD)

Coronary computed tomography

Coronary CT

Patients on hemodialysis

Patients with atherosclerotic cardiovascular disease

Patients with ASCVD (ischemic stroke)

Patients with diabetes mellitus

Patients with diabetes mellitus, non-statin lipid-lowering therapy

Patients with diabetes mellitus, statin therapy

As per AAPA/ABC/ACC/…/PCNA 2019 guidelines, 

Apolipoprotein B

ApoB

Patients with chronic kidney disease

Patients with CKD, management

Female patients

Patients with inflammatory diseases and human immunodeficiency virus

Patients with inflammatory diseases and HIV

Pediatric patients

Pediatric patients, lifestyle modifications

Lipoprotein

Lipoprotein, a

Risk assessment

Pediatric patients, statin therapy

Patients with hypertriglyceridemia (niacin)

Bempedoic acid

Screening for hypothyroidism

Other supplements

Indications for screening

Indications for screening (pediatrics)

Patients with familial hypercholesterolemia, management

Treatment targets

Statins

Screening for anabolic steroid use

Patients with hypertriglyceridemia (management of pancreatitis)

Patients with familial hypercholesterolemia, diagnosis

Patients with hypertriglyceridemia, omega-3 fatty acids

Dietary modifications

PCSK9 inhibitors

Monitoring for adverse effects of statins

Bile acid sequestrants

Patients with severe hypercholesterolemia

Elderly patients

Patients with obesity

Patients with endocrine disorders (evaluation)

Indications for screening (adults, ApoB and LPa)

Lipid profile

Lipid profile, tests

Patients with ASCVD (PAD)

Ezetimibe

Patients with diabetes mellitus (hypertriglyceridemia)

Pediatric patients, genetic testing

Patients with diabetes mellitus, treatment targets

Pediatric patients, treatment targets

Patients with hypertriglyceridemia (fibrates)

Patients with hypertriglyceridemia (statins)

Patients with heart failure

Patients with HF

Patients with diabetes mellitus (lifestyle modifications)

Physical activity

Shared decision-making

Monitoring for adherence to treatment

Patients with endocrine disorders (Cushing's syndrome)

Patients with endocrine disorders (thyroid disease)

Patients with familial hypercholesterolemia (homozygous FH)

PCSK9-blocking siRNA

Patients with aortic valvular disease

Icosapent ethyl

Patients with diabetes mellitus (lipid profile monitoring)

Pediatric patients, screening

Arterial ultrasound

Indications for screening, adults, lipid profile

Pediatric patients (clinical assessment)

Patients with endocrine disorders (acromegaly)

Updated 2024 ADA guidelines for the management of dyslipidemia in diabetes


The American Association of Clinical Endocrinology (AACE) has published a focused update on pharmacotherapy for dyslipidemia in patients with or at high risk of atherosclerotic cardiovascular disease (ASCVD). A treatment target of LDL-C <70 mg/dL is suggested. Anti-PCSK9 monoclonal antibodies (evolocumab or alirocumab) are suggested for patients who remain above target despite maximally tolerated statins, and bempedoic acid is suggested for statin-intolerant patients. Inclisiran is not recommended due to insufficient evidence.


Dyslipidemia

Background

Overview

Guidelines

Key sources