Heart Complications and Sickle Cell Disease

Sickle cell disease (SCD) is the most common inherited blood disorder in the United States. It is also a global health issue. The abnormally-shaped red blood cells can block blood flow and cause damage to organs, including the heart. The chronic anemia that results from SCD contributes to these issues.1,2

Heart problems are some of the most common causes of death in people with SCD. Knowing more about these issues can help you be aware of any changes in your health.1

What heart problems can happen with sickle cell disease?

The most common heart problems with SCD are:1

  • Cardiomyopathy
  • Diastolic dysfunction
  • Pulmonary hypertension
  • Sudden cardiac death

Cardiomyopathy

Cardiomyopathy is not one disease, but many. It represents a variety of conditions of the heart muscle. In cardiomyopathy, the heart muscle can become thick or stiff. It can also fill with abnormal material that is usually not in the heart. This makes it harder for your heart to pump blood to the rest of your body. Over time, this can lead to heart failure.2,3

Diastolic dysfunction

Diastolic dysfunction is also called diastolic left heart disease. When your heart beats, it usually relaxes after each beat. When your heart cannot relax fast enough, it is called diastolic dysfunction. This is dangerous, even with no other heart problems. This happens if the heart muscles are stiff.4,5

When the heart muscles are not flexible, the ventricles (chambers in the heart) cannot completely fill. This can cause blood to back up in other organs.5

Pulmonary hypertension

Pulmonary hypertension is a type of high blood pressure that affects the blood vessels that go from the heart to the lungs. This causes poor blood flow and low levels of oxygen in the blood.6

There are different kinds of pulmonary hypertension. Several of them are linked to complications of sickle cell disease and increase the risk of death in adults with SCD.4

Sudden cardiac death

Sudden cardiac death (SCD) is also known as sudden cardiac arrest. It is the largest cause of natural death in the United States. This is not the same as a heart attack.7

Sudden cardiac arrest is when the electrical system of the heart malfunctions and becomes irregular. The heart beats very fast, and blood is not pumped to the body. Without emergency treatment, death occurs.7

In contrast, a heart attack happens when there is a blockage in one or more of the coronary arteries. If the blockage prevents enough oxygenated blood from getting to the heart, it causes damage.7

Why do heart problems happen with sickle cell disease?

Heart problems are common in people with SCD because of:1

  • The disease itself
  • The effects of the inflexible, sickled red blood cells

Over time, sickled blood cells block blood vessels. This can damage the heart and lungs.

Chronic (long-term) anemia in SCD lowers the ability of blood to carry oxygen. This makes the heart work harder to make up for the lower oxygen levels in blood. This increases stress and pressure on the heart, which increases the risk of heart failure.1

When sickle cells burst, they also release molecules that can trigger immune cells and promote inflammation. This inflammation can also cause heart problems.1

How heart complications are treated

In most cases, acute heart failure is treated with diuretics and supportive care. Diuretics, also called water pills, are drugs that reduce the amount of fluid in your blood vessels. This helps lower your blood pressure. Supportive care focuses on quality of life while managing the symptoms and problems linked to heart failure.

Treatment for people with sickle cell disease and heart failure is based on each person’s unique needs. In most cases, your hematologist and cardiologist will work together to create a treatment plan that is right for you.

Treatment usually includes:1

  • Increasing therapies for sickle cell disease
  • Avoiding both dehydration and too much fluid
  • Blood transfusions
  • Hydroxyurea

Other treatments may include:1

  • Aspirin
  • Nitroglycerin
  • Beta-blockers
  • Angiotensin converting enzyme (ACE) inhibitors

If there is too much iron in your blood, you will likely need iron chelation therapy. Depending on your specific heart issues and overall health, your doctor may also recommend surgery to treat your heart problem.1

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Written by: Jaime Rochelle Herndon | Last reviewed: February 2021