#210 - Lp(a) and its impact on heart disease | Benoît Arsenault, Ph.D.

Jun 13, 2022 Episode Page ↗
Overview

Benoît Arsenault, a research scientist, discusses Lp(a), the most significant genetically inherited trait for cardiovascular disease risk. The episode covers Lp(a)'s biology, measurement, and its role in ASCVD and aortic stenosis, alongside current and future therapies like statins, PCSK9 inhibitors, and antisense oligonucleotides.

At a Glance
10 Insights
2h 7m Duration
17 Topics
7 Concepts

Deep Dive Analysis

Benoit Arsenault's Introduction to Lp(a) Research

Epidemiology and Historical Challenges of Lp(a) Measurement

Genetic Insights into Lp(a) and Cardiovascular Risk

Lp(a) Biology, Production, and Measurement Methods

Statin Effects on Lp(a) and its Unique Atherogenicity

Apolipoprotein(a) Structure and Plasminogen Homology

Lp(a)'s Role in Aortic Valve Stenosis

Lp(a) Isoform Size vs. Particle Number for Risk

Inheritance and Clinical Testing of Lp(a) Levels

Physician Awareness and Holistic Management of High Lp(a)

Variability in Disease Expression with High Lp(a)

Lp(a) Association with Other Cardiovascular Diseases

Niacin's Ineffectiveness in Lowering Lp(a) and Improving Outcomes

PCSK9 Protein Biology and its Impact on LDL Receptors

PCSK9 Inhibitors: Effects on Lp(a) and Residual Risk

Future Therapies: Antisense Oligonucleotides and siRNA for Lp(a)

Horizon Trial and the Future of Lp(a) Treatment

Lp(a) (Lipoprotein(a))

A supercharged low-density lipoprotein (LDL) particle that is particularly nefarious due to an additional apolipoprotein(a) (Apo(a)) covalently bound to the ApoB-100 of an LDL particle. It is a significant genetically inherited risk factor for atherosclerotic cardiovascular disease (ASCVD) and aortic valve stenosis.

Apolipoprotein(a) (Apo(a))

A glycoprotein that binds to an LDL particle, forming Lp(a). Its structure includes Kringle repeats, particularly Kringle 4 type 2 (which dictates isoform size variability) and Kringle 4 type 9 (responsible for covalent binding to ApoB). Apo(a) shares sequence homology with plasminogen, influencing thrombosis.

Mendelian Randomization

A research method that uses genetic variants as instrumental variables to infer causal relationships between a modifiable risk factor (like Lp(a) levels) and a disease outcome. It leverages the random assortment of genes at conception to mimic a randomized controlled trial, helping to overcome confounding in observational studies.

Oxidized Phospholipids (OxPLs)

These are much higher on Lp(a) particles compared to LDL particles. OxPLs on Lp(a) send signals that drive pro-inflammatory, pro-thrombotic, and pro-calcifying processes in cells like endothelial cells, smooth muscle cells, macrophages, and valvular interstitial cells, making Lp(a) more atherogenic.

Aortic Valve Stenosis

A disease characterized by the calcification and narrowing of the aortic valve, significantly associated with high Lp(a) levels. Lp(a) is believed to initiate and accelerate this process by promoting osteoblastic changes in valvular interstitial cells and contributing to inflammation and calcification.

PCSK9 (Proprotein Convertase Subtilisin/Kexin Type 9)

A protein that regulates the LDL receptor. PCSK9 can bind to the LDL receptor inside cells, leading to its degradation in the lysosome, or extracellularly, preventing the LDL receptor from binding LDL particles and hindering its recycling, thereby reducing LDL clearance from the blood.

Antisense Oligonucleotides (ASOs) / siRNA

A new class of therapeutic agents designed to lower Lp(a) levels. ASOs are single-stranded synthetic nucleic acids that bind to specific mRNA sequences, preventing the production of the target protein (Apo(a)). siRNA (small interfering RNA) are double-stranded RNA molecules that silence gene expression by degrading specific mRNA. Both aim to reduce Apo(a) production in the liver.

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What is Lp(a) and why is it important for cardiovascular health?

Lp(a) is a lipoprotein particle similar to LDL but with an additional apolipoprotein(a) attached, making it highly atherogenic. It is the single most important genetically inherited trait for atherosclerotic cardiovascular disease (ASCVD) risk, affecting about 15-20% of the population.

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Why was Lp(a) research previously overlooked, and what revived interest in it?

Early studies on Lp(a) often yielded negative results due to unreliable assays that couldn't accurately measure its complex structure. Interest was revived around 2009-2011 by genetic association studies (GWAS) and Mendelian randomization, which convincingly linked genetic variants associated with high Lp(a) levels to cardiovascular events, bypassing assay limitations.

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How is Lp(a) measured clinically, and what is the preferred unit of measurement?

Lp(a) is measured using immunoturbidometric assays. The preferred measurement is in nanomoles per liter (nmol/L) as it provides a better sense of the number of Lp(a) particles, which is the most important factor for risk assessment, rather than milligrams per deciliter (mg/dL) which can be influenced by isoform size.

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How does Lp(a) contribute to atherosclerosis and cardiovascular disease?

Lp(a) is more atherogenic than standard LDL particles on a per-particle basis, primarily due to carrying a higher number of oxidized phospholipids. These oxidized phospholipids drive pro-inflammatory, pro-thrombotic, and pro-calcifying signals, contributing to plaque formation, progression, and aortic valve stenosis.

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How does Lp(a) affect the efficacy of statins?

Statins primarily lower LDL by upregulating LDL receptors on liver cells, which clear ApoB-containing lipoproteins. However, statins do not effectively lower Lp(a) and may even cause a small increase, because Lp(a) is not catabolized by the LDL receptor in the same way as LDL.

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Is Lp(a) isoform size important for assessing cardiovascular risk?

No, genetic studies have shown unequivocally that Lp(a) isoform size itself is not directly associated with the risk of heart attacks and strokes. Instead, the isoform size matters because it is associated with different levels (number) of Lp(a) particles, and it is the number of Lp(a) particles that truly dictates risk.

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How is Lp(a) inherited, and should children of parents with high Lp(a) be tested?

Lp(a) follows an autosomal dominant pattern of inheritance, meaning you typically need only one genetic variant from a parent to have high Lp(a). However, due to the complexity of many variants, you cannot reliably predict offspring's Lp(a) levels from parents' levels; direct measurement is required. The Lp(a) gene is fully expressed by age two, and levels are relatively stable through adulthood, so testing can be done early.

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Why is physician awareness of Lp(a) low, and what can be done about it?

Physician awareness is low partly because guidelines for Lp(a) measurement are relatively new and take time to be widely adopted. Additionally, some physicians are reluctant to measure it due to the historical lack of specific treatments, fearing it might cause anxiety without offering solutions. Increased education and the emergence of new therapies are crucial to improve awareness.

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What is the role of Niacin in lowering Lp(a) and is it recommended?

Niacin can reduce Lp(a) levels by about 20-30% by inhibiting its production. However, large cardiovascular outcome trials (AIM-HI, HPS2-THRIVE) have shown no cardiovascular benefits from niacin treatment, despite its effects on lipids, and it carries significant side effects like flushing. Therefore, niacin is generally not recommended for Lp(a) lowering.

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How do PCSK9 inhibitors affect Lp(a) levels?

PCSK9 inhibitors reduce Lp(a) levels by approximately 25-30% on average, primarily by reducing the production rate of apolipoprotein(a) in the liver. The variability in reduction can be significant, and while beneficial for LDL, this reduction in Lp(a) may not be sufficient to fully ameliorate Lp(a)-specific residual risk.

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What are the most promising future treatments for high Lp(a)?

Antisense oligonucleotides (ASOs) and small interfering RNA (siRNA) are highly promising. These therapies target the LPA gene to reduce the production of apolipoprotein(a) in the liver, leading to substantial (e.g., 80% or more) reductions in Lp(a) levels. Clinical trials, such as the Horizon trial for an ASO, are currently underway to assess their impact on cardiovascular outcomes.

1. Demand LP(a) Test

Demand your physician checks your LP(a) level, as there’s a significant chance it’s elevated (10-20% of the population) and many doctors don’t routinely test it. A milligram per deciliter mass measurement is likely sufficient if nanomoles/L is not available, as both methods will identify high-risk levels.

2. Aggressively Manage All CV Risk Factors

If you have high LP(a), aggressively manage all other cardiovascular risk factors (smoking, diet, physical activity, body weight, LDL cholesterol, diabetes, blood pressure) to significantly reduce your overall risk of events by up to two-thirds.

3. Aggressive ApoB Lowering & PCSK9

For patients with elevated LP(a), aggressively lower ApoB to a physiological level (30-40 mg/dL) and liberally use PCSK9 inhibitors, which can reduce LP(a) by approximately 30% on average, as part of a comprehensive lipid management strategy.

4. Screen Aortic Valve for Stenosis

If you have elevated LP(a), get a baseline echocardiogram or cardiac MRI to check for early signs of aortic stenosis, as early identification and intervention lead to better outcomes.

5. Measure LP(a) in Children

Measure LP(a) levels in children by age two to five, especially if there’s a family history of early heart attack or stroke, as the gene is fully expressed by age two and levels are stable from age five into adulthood.

6. Avoid Niacin for LP(a) Lowering

Do not use niacin to lower LP(a) levels, despite its ability to reduce LP(a) by 20-30%, because large cardiovascular outcome trials have shown no cardiovascular benefits and significant side effects.

7. Do Not Avoid Statins for High LP(a)

Do not avoid prescribing or taking statins if you have high LP(a), as statin treatment is beneficial in patients with high LP(a) levels, potentially even more so than in those with low LP(a), despite a small potential increase in LP(a) levels.

8. Prefer Nanomole/Liter LP(a) Measurement

When measuring LP(a), ideally seek a lab that provides results in nanomoles per liter, as this gives a more accurate sense of the number of LP(a) particles, which is the most important risk factor.

9. LP(a) Measurement Once in Lifetime

Measure LP(a) once in a lifetime, as its levels are remarkably stable over time and do not require repeated testing for risk assessment.

10. Advocate for Physician Education on LP(a)

Advocate for increased physician education and awareness regarding LP(a) measurement and management, as it is a highly prevalent and important driver of cardiovascular disease often overlooked.

Lp(a) was actually the strongest of them that was predicting residual cardiovascular risk.

Benoit Arsenault

Lp(a) is the single highest genetically inherited trait that confers high risk of ASCVD.

Peter Attia

On a per-particle basis, Lp(a) is much more atherogenic than an equivalent LDL particles.

Benoit Arsenault

It's really the Lp(a) number that matters.

Benoit Arsenault

You cannot predict the phenotype of the offspring from the phenotype of the parents.

Peter Attia

Even though there's no specific therapy for high Lp(a), it doesn't mean you can't do anything.

Benoit Arsenault

If you can hit a 15% relative risk reduction, which ultimately turned into a bigger risk reduction in 2.2 years on a group of patients who show up on the maximum dose of a statin, whose LDL is already at the 10th percentile, you've changed the field of cardiovascular medicine.

Peter Attia

General Management of High Lp(a) Risk Factors

Benoit Arsenault
  1. Measure Lp(a) levels at least once in a lifetime.
  2. Target and manage other cardiovascular risk factors including smoking, diet, physical activity, body weight, LDL cholesterol, diabetes, and blood pressure.
  3. Consider statin therapy to lower LDL cholesterol aggressively, as statins have shown benefits in patients with high Lp(a) by reducing overall cardiovascular risk.

Peter Attia's Clinical Approach to Managing Patients with Elevated Lp(a)

Peter Attia
  1. Aggressively eradicate ApoB by targeting an ApoB level of approximately 30-40 mg/dL (a physiologic level).
  2. Liberally use PCSK9 inhibitors, as they can reduce Lp(a) by about 30% on average, in addition to their potent ApoB-lowering effect.
  3. Insist on at least one baseline echocardiogram to check for even the earliest signs of aortic stenosis, as early intervention improves outcomes.
20%
Approximate percentage of world population with high-risk Lp(a) Varies by ethnicity; Peter Attia previously cited 8-12%.
1963
Year Lp(a) was discovered By Swedish scientist Cary Berg.
50 milligrams per deciliter
Lp(a) level considered high risk Equivalent to 125 nanomoles per liter.
10%
Approximate increment in Lp(a) levels with statin treatment In most studies, can be up to 20% in some cases like aortic valve stenosis patients.
50% to 100%
Increased risk for aortic valve stenosis with high Lp(a) For Lp(a) levels around 50 mg/dL or 125 nmol/L.
Age 2
Age by which Lp(a) gene is fully expressed Levels obtained at age 5 are likely to remain stable through adulthood.
20-30%
Mean reduction in Lp(a) levels with niacin therapy Niacin reduces Lp(a) production.
50-100 milligrams per deciliter
Lp(a) reduction needed for cardiovascular benefits (Mendelian randomization estimate) Over a lifetime, to achieve benefits comparable to statin treatment.
6%
Reduction in cardiovascular events with anacetrapib (CETP inhibitor) Observed in the REVEAL trial, attributed to ApoB lowering rather than HDL increase.
2%
Percentage of population with common PCSK9 variant (loss of function) Associated with lower PCSK9 levels, lower LDL, and protection against CVD.
20%
LDL reduction associated with common PCSK9 variant Lifelong reduction.
15%
Relative risk reduction in cardiovascular events with PCSK9 inhibitors Observed in the Fourier trial over 2.2 years in patients already on high-intensity statins.
25-30%
Average reduction in Lp(a) levels with PCSK9 inhibitors Variability is significant; reduction in patients with high Lp(a) in specific trials was ~15%.
80%
Mean reduction in Lp(a) levels with Ionis antisense oligonucleotide Observed in phase two dose-ascending studies, with 90% of patients achieving Lp(a) below 50 mg/dL.
Under 8,000
Number of patients in the Horizon cardiovascular outcomes trial for Ionis ASO Patients with stable cardiovascular diseases.
2025
Estimated year for Horizon trial results Due to longer treatment period and COVID-related recruitment challenges.