Connect with others who understand.

sign up log in
Resources
About MyHemophiliaTeam

Connect with others who understand.

sign up log in
Resources
About MyHemophiliaTeam

Hemophilia A and Cardiovascular Disease: Connection and Risk

Updated on October 18, 2022
Medically reviewed by
Todd Gersten, M.D.
Article written by
Amanda Jacot, PharmD

Over the past few decades, improved treatments have extended the life span of those living with hemophilia A. The average life expectancy for someone with hemophilia has increased from 66 years old in 1986 to 77 years old in 2018. As people with hemophilia A live longer, the risk of age-related health conditions — including cardiovascular disease (CVD) — has also risen.

An aging population with hemophilia has led to an increase in the number of people with hemophilia and CVD overall. However, experts disagree on whether hemophilia affects CVD risk, and how. Read on to learn about what influences your risk for heart disease with hemophilia and what you can do to prevent it.

What Is Cardiovascular Disease?

Cardiovascular disease is sometimes called heart disease and per the World Health Organization, it is the leading cause of death worldwide. CVD includes problems with the heart and blood vessels and can refer to several different conditions, including:

  • Heart attack — The supply of blood to your heart is suddenly blocked.
  • Stroke — The supply of blood to the brain is blocked, usually by a blood clot.
  • Ischemic heart disease — The arteries in the heart are narrowed, so the heart muscle gets less blood and oxygen.
  • Heart failure — The heart doesn’t pump blood as well as it should.
  • Arrhythmia — The heart has trouble keeping a normal rhythm.
  • Heart valve problems — The valves in the heart don’t open or close properly.

Members of MyHemophiliaTeam have shared their diagnoses with different forms of CVD. “I found out I have a superficial venous thrombosis in my vein in my right arm,” shared one. “My husband has been in and out of the hospital with a bad heart,” wrote another whose partner has a bleeding disorder. “He has an enlarged heart, scar tissue around his heart, and pericarditis.” “Just found out I have a pulmonary embolism [blood clot in the lung],” posted another member.

Treatment for CVD

Treatment for CVD often involves blood thinners — medications such as heparin and warfarin (sold as Coumadin) — to decrease the risk of thrombosis (blood clot forming in blood vessels). Blood thinners increase the risk of bleeding, which complicates treatment of CVD in people with hemophilia A.

Some MyHemophiliaTeam members report being placed on blood thinners due to clotting problems or CVD risk. One member shared, “I am on blood thinners for the rest of my life because of the clotting that has caused heart attacks and extremely high blood pressure.” A parent described their child’s struggles with both a bleeding disorder and CVD: “My daughter has Von Willebrand disease [a bleeding disorder when your blood doesn’t clot properly], and on top of that, due to an artificial heart valve, is now on Coumadin, which they are having a problem regulating.”

Some forms of heart disease require surgery. People with severe hemophilia A can safely undergo surgery with careful, expert management of a medication or a treatment plan designed to prevent a disease from occurring and monitor for complications.

Cardiovascular Risk Factors

The risk of CVD is influenced by your lifestyle, other health conditions, family history, and age. You may be more likely to develop CVD if you have:

  • Hypertension (high blood pressure)
  • High cholesterol levels
  • Atherosclerosis (buildup of a fatty substance called plaque in the arteries)
  • Diabetes
  • Obesity
  • Family history of CVD
  • Older age
  • HIV infection
  • Diet high in salt and fats
  • Tobacco use
  • Alcohol use

Does Hemophilia A Protect Against CVD?

Serious CVD-related problems often occur because a blood vessel is blocked by a blood clot. A study from the British Journal of Haemophilia found that people with bleeding disorders are thought to be protected from these issues because they have lower levels of clotting factors (molecules that help form clots).

One five-year study recently published in Blood Advances found that people with hemophilia had a lower-than-predicted number of CVD events such as heart disease or stroke when compared to the general population. However, another study published in the journal Haemophilia found that hospitalized men with hemophilia developed heart attacks and chest pain at about the same rate as men without the condition.

Certain people with hemophilia may be more at risk for CVD than others. For example, research shows that people with hemophilia B, a type of hemophilia caused by a lack of clotting factor 9, are less likely to have plaque buildup in the arteries.

The severity of hemophilia can also affect a person’s risk of CVD. People with moderate or severe hemophilia may have a lower risk of CVD than people with mild hemophilia because they have lower clotting factor levels in their blood. One study found that people with severe hemophilia had fewer heart attacks when compared to the general population (0.5 percent versus 4.8 percent). However, people with mild or moderate hemophilia had similar rates of heart attacks as those without hemophilia.

CVD Risk Factors in People Living With Hemophilia A

People living with hemophilia generally experience the same risk factors for CVD as the general population. Studies have found the incidence of diabetes and tobacco use is the same for those living with hemophilia and the general population.

However, hemophilia can increase certain risk factors such as hypertension. The reason for this difference is unknown.

Hypertension

Hypertension is more common in people with hemophilia compared to the general population. Normal blood pressure is considered to be 120/80 millimeters of mercury (mm Hg), and high blood pressure is usually considered to be over 140/90 mm Hg.

A MyHemophiliaTeam member shared, “I have Von Willebrand disease [bleeding disorder when your blood doesn’t clot properly] and factor 11 deficiency, and I have very high blood pressure. Anyone else? I am 39 years old.”

A Dutch study found that 57 percent of adults with hemophilia had hypertension. Another study in the United States found that 49 percent of people living with hemophilia also had hypertension compared to 32 percent in the general population.

Researchers are not sure why hemophilia increases the risk of hypertension, but some scientists suspect it has to do with microbleeds in the kidney. When the kidneys are damaged, it can cause high blood pressure.

People living with hemophilia are more likely to develop hypertension if they have hemophilia B, moderate or severe hemophilia, diabetes, or kidney injury.

Atherosclerosis

Atherosclerosis is a buildup of fats and cholesterol causing plaque formation on artery walls. These buildups of fat and cholesterol cause the artery to become hard and narrow, blocking blood flow and raising the risk of clots.

Although people with hemophilia are less likely to die from CVD than the general population, they still have the same degree of atherosclerosis. In an article published in the Journal of Thrombosis and Haemostasis, researchers found that neither hemophilia A nor hemophilia B protects against the development of plaques.

Hemophilia may help protect against CVD through reduced thrombin formation. Thrombin is an enzyme involved in clotting. People with hemophilia have lower thrombin levels, which may help strengthen the blood vessels and help plaques from breaking open.

Reduced Mobility and Chronic Pain

People living with chronic (long-term) pain are at a higher risk for CVD. Between 40 percent and 70 percent of people with hemophilia experience chronic pain. Hemophilia causes pain due to bleeding in the soft tissues and joints. Over time, ongoing bleeding can lead to changes in the joints, causing disability and pain.

Additionally, people with hemophilia and von Willebrand disease may develop inhibitors — immune system antibodies that block clotting factors — that prevent bleeding treatments from working. People with inhibitors may be more likely to experience joint pain and reduced mobility.

HIV

The Centers for Disease Control and Prevention (CDC) reports that before all blood products were screened for HIV in 1992, people with hemophilia were at a high risk of contracting HIV through a blood transfusion. Due to the development of effective HIV treatment, many people who were infected during this time are still alive today. However, many of the medications used to treat an HIV infection increase the risk of heart disease.

Protecting Yourself From Cardiovascular Disease

Treating CVD can be challenging, so it is important to prevent and treat risk factors. Take these steps to help lower your risk of developing CVD:

  • Manage your blood pressure as recommended by your doctor.
  • Control your cholesterol level.
  • Maintain a healthy weight.
  • Limit saturated fat, sodium, and sugar in your diet.
  • Exercise at least 150 minutes per week.
  • Drink alcohol in moderation.
  • Stop smoking.

Talk to your hematologist to learn more about how you can be affected by cardiovascular disease and discuss strategies to prevent it. You may need a multidisciplinary hematology team of doctors that includes a cardiologist who treats people with CVD and hemophilia A.

Talk With Others Who Understand

MyHemophiliaTeam is the social network for people with hemophilia and their loved ones. On MyHemophiliaTeam, more than 6,000 members come together to ask questions, give advice, and share their stories with others who understand life with hemophilia.

Are you living with hemophilia and CVD? What steps do you take to manage CVD? Share your experience in the comments below, or start a conversation by posting on your Activities page.

References
  1. Mortality, Life Expectancy, and Causes of Death of Persons With Hemophilia in the Netherlands 2001-2018 — Journal of Thrombosis and Haemostasis
  2. A Cross-Sectional Analysis of Cardiovascular Disease in the Hemophilia Population — Blood Advances
  3. Consensus Review of the Treatment of Cardiovascular Disease in People With Hemophilia A and B — Cardiology in Review
  4. Cardiovascular Risk in Patients With Hemophilia — Blood
  5. Cardiovascular Diseases (CVDs) — World Health Organization
  6. What Is Cardiovascular Disease? — American Heart Association
  7. Know Your Risk for Heart Disease — Centers for Disease Control and Prevention
  8. Haemophilia Protects Against Ischaemic Heart Disease: A Study of Risk Factors — British Journal of Haemotology
  9. Reduced Cardiovascular Morbidity in Patients With Hemophilia: Results of a 5-Year Multinational Prospective Study — Blood Advances
  10. Atherosclerotic Heart Disease: Prevalence and Risk Factors in Hospitalized Men With Hemophilia A — Haemophilia
  11. Consensus Review of the Treatment of Cardiovascular Disease in People With Hemophilia A and B — Cardiology in Review
  12. High Blood Pressure Symptoms and Causes — Centers for Disease Control and Prevention
  13. Prevalence and Risk Factors for Hypertension in Hemophilia — Hypertension
  14. Renal Disease Among Males With Haemophilia — Haemophilia
  15. Atherosclerosis: Arterial Disease — Cleveland Clinic
  16. Reduced Cardiovascular Morbidity in Patients With Hemophilia: Results of a 5-Year Multinational Prospective Study — Blood Advances
  17. Factor VIII Deficiency Does Not Protect Against Atherosclerosis — Journal of Thrombosis and Haemostasis
  18. The Association Between Chronic Pain and Cardiac Disease: A Cross-Sectional Population Study — The Clinical Journal of Pain
  19. MASAC Document 260 — Management of Chronic Pain in Persons With Bleeding Disorders: Guidance for Practical Application of the Centers for Disease Control’s Opioid Prescribing Guidelines — National Hemophilia Foundation
  20. Inhibitors and Hemophilia — Centers for Disease Control and Prevention
  21. Management of Haemophilia A With Inhibitors: A Regional Cross-Talk — Haemophilia
  22. U.S. Public Health Service Guidelines for Testing and Counseling Blood and Plasma Donors for Human Immunodeficiency Virus Type 1 Antigen — Centers for Disease Control and Prevention
  23. HIV and Opportunistic Infections, Coinfections, and Conditions — HIV Info.nih.gov
  24. Non-Fatal Cardiovascular Disease, Malignancies, and Other Co-Morbidity in Adult Haemophilia Patients — Thrombosis Research
  25. Unfavourable Cardiovascular Disease Risk Profiles in a Cohort of Dutch and British Haemophilia Patients — Journal of Thrombosis and Haemostasis
  26. How To Prevent Heart Disease — MedlinePlus
  27. How Much Physical Activity Do Adults Need? — Centers for Disease Control and Prevention
  28. Perioperative Management of Hemophilia A Patients Undergoing Cardiac Surgery: A Literature Review of Published Cases — Journal of Cardiothoracic and Vascular Anesthesia

All updates must be accompanied by text or a picture.
Todd Gersten, M.D. is a hematologist-oncologist at the Florida Cancer Specialists & Research Institute in Wellington, Florida. Review provided by VeriMed Healthcare Network. Learn more about him here.
Amanda Jacot, PharmD earned a Bachelor of Science in biology from the University of Texas at Austin in 2009 and a Doctor of Pharmacy from the University of Texas College of Pharmacy in 2014. Learn more about her here.

Recent articles

Hemophilia A is a rare bleeding disorder where the blood doesn’t clot easily. It occurs when a...

Genetic Testing for Hemophilia A Explained

Hemophilia A is a rare bleeding disorder where the blood doesn’t clot easily. It occurs when a...
Hemophilia A is a rare bleeding disorder in which the blood doesn’t clot properly. People with...

What Do Inhibitors Do in Hemophilia A?

Hemophilia A is a rare bleeding disorder in which the blood doesn’t clot properly. People with...
Hemophilia A and hemophilia B are two types of hemophilia that produce similar symptoms and are...

Hemophilia A vs. B

Hemophilia A and hemophilia B are two types of hemophilia that produce similar symptoms and are...
If you’re living with hemophilia, you’re already aware of the impact the condition can have on...

Hemophilia Awareness: How To Get Involved

If you’re living with hemophilia, you’re already aware of the impact the condition can have on...
Below the surface, living with hemophilia can mean severe pain, mobility difficulty, and...

Hemophilia: What People Don't See (Infographic)

Below the surface, living with hemophilia can mean severe pain, mobility difficulty, and...
Hemophilia is a chronic, genetic bleeding disorder in which the blood has low levels of certain...

Periods and Hemophilia: Your Guide

Hemophilia is a chronic, genetic bleeding disorder in which the blood has low levels of certain...
MyHemophiliaTeam My hemophilia Team

Thank you for subscribing!

Become a member to get even more:

sign up for free

close