PULMONARY HYPERTENSION (High blood pressure)

 
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What is pulmonary hypertension?

  • Your heart is made up of two pump systems. The right side of your heart pumps blood to your lungs to pick up oxygen. The left side of your heart pumps blood to the rest of your body.

  • Blood pressure measured with a cuff on your arm is measuring the pressure it takes to pump blood to your body. The pressure it takes to pump blood to your lungs can also be measured. Pulmonary hypertension is when it takes high amounts of pressure to pump blood to the lungs.

  • Pulmonary hypertension is more common in people with sickle cell disease than in people without sickle cell disease.

 

The symptoms of pulmonary hypertension can happen suddenly, or can come on slowly, including:

 
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Shortness of breath

 
 
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Feeling very tired

 
 
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Chest pressure or chest pain

 
 
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Feeling your heart beat out of rhythm or very fast

 
 
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Feeling like you are going to faint

 
 
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Swelling of your hands, feet, or belly

 

Screening

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  • An ultrasound of the heart, or echocardiogram (also called an echo), can be done to screen for pulmonary hypertension.

  • It is also used to measure how fast blood is pumped through one of the heart valves on the right (or lung) side of the heart.

  • This measurement is called tricuspid regurgitant jet velocity, or TRJV. A TRJV higher than 2.5m/s is a warning sign for early death in people with sickle cell disease.

Treatment

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Treatment for pulmonary hypertension is different for people with sickle cell disease than for people without sickle cell disease.

 
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Pulmonary hypertension checklist

For you and your health care provider

 

Getting an echo:

🄯 If you are an adult or if you are preparing to transition to the adult clinic, and you have not already had an echo, talk with your provider about getting one.

🄯 If you have had an echo, talk with your provider about when you should get another one.

🄯 If your echo shows a TRJV higher than 2.5m/s, talk with your provider about the next steps to take.

🄯 If an echo is not available, talk with your provider about getting a blood test to measure the level of a hormone called BNP (brain natriuretic peptide) or NTproBNP (N-terminal-pro b-type natriuretic peptide). This hormone is made by your heart. The amount of BNP goes up when the heart has to work hard to pump blood to the rest of the body.

🄯 If you have pulmonary hypertension, talk with your health care provider about which treatment is right for you.

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SECTION OVERVIEW

  • An ultrasound of the heart, is used to screen for pulmonary hypertension or elevated TRJV in adults with sickle cell disease.

  • Treatment for pulmonary hypertension is different for people with sickle cell disease than for people without sickle cell disease.