Pulmonary Hypertension

Symptoms of pulmonary hypertension are sometimes hard to recognize. People may have symptoms for years before being diagnosed. This is because many symptoms of pulmonary hypertension are also symptoms of other medical conditions.
Some symptoms of pulmonary hypertension include:

• Chest pain
• Shortness of breath
• Swelling of the abdomen, legs, or feet
• Wheezing, which is a whistling sound when you breathe out
• Coughing that is dry or produces blood
• Fatigue and weakness
• Dizziness that may lead to fainting
• Nausea and vomiting
• Hoarseness

Symptoms can get worse over time. For example, in the early stages of pulmonary hypertension, you may only have shortness of breath with exercise. As the disease progresses, shortness of breath will occur more often.

To understand pulmonary hypertension, it is helpful to understand the job of the heart and lungs, and the flow of blood. As the left side pumping chamber of the heart beats, it sends blood throughout the body. The tissues of the body need oxygen from the blood to function properly. After the tissues use oxygen from the blood, oxygen-poor blood travels through veins to the right-sided chamber of the heart, then flows through the pulmonary arteries to the lungs so the lungs can add oxygen to the blood. The oxygen-rich blood then goes from the lungs back to the left side of the heart which then pumps the blood to the body again.

The force (pressure) of the blood against the walls of the pulmonary arteries is called the pulmonary pressure. When this pressure gets too high, the pulmonary arteries become narrow or blocked. This makes it more difficult for the blood to flow and makes the right side of the heart work harder to maintain blood flow into the lungs. Over time, this can cause damage to both the heart and lungs.

The underlying cause of pulmonary hypertension is not always clear. Certain medical conditions can damage, change, or block the blood vessels of the pulmonary arteries, which can lead to pulmonary hypertension. Some examples of medical conditions include:

• Left heart diseases, such as left heart failure and diseases of the heart valves which at late stages can lead to fluid and pressure backing up from the left pumping chamber of the heart to the lung arteries.
• Lung diseases such as chronic obstructive pulmonary disease (COPD), interstitial lung disease, emphysema sleep apnea or history of blood clots in the lung arteries. If severe enough, these disorders can all elevate the pressure inside the lung arteries over time.
• Other heart and blood vessel diseases such as congenital (inherited) heart defects which can causing mixing of blood between the two sides of the heart leading to increased blood volume sent into the lung arteries. Over time, this extra volume leads to changes in the pressure and the pulmonary arteries.
• Other medical conditions such as liver disease, sickle cell disease, or connective tissue disorders like scleroderma can lead to pulmonary hypertension by different mechanisms.

Several factors can increase your risk of developing pulmonary hypertension.

• Age: Pulmonary hypertension can occur at any age, but your risk increases as you get older. The condition is usually diagnosed between ages 30 and 60.
• Sex: Pulmonary hypertension is more common in women than in men. Pulmonary hypertension with certain types of heart failure is also more common in women.
• Environment: You may be at an increased risk of pulmonary hypertension if you have or are exposed to Asbestos or certain infections caused by parasites.
• Family history and genetics: A family history of heart diseases can increase the risk of heart problems that can then lead to pulmonary hypertension. A family history of blood clots also increases your risk. Genetic disorders, such as Down syndrome, congenital heart disease, and Gaucher disease, can increase your risk of pulmonary hypertension.
• Lifestyle habits: Unhealthy lifestyle habits such as smoking and illegal drug use can raise your risk of developing pulmonary hypertension.
• Medicine: Some prescribed medicines used to treat cancer, and depression may increase your risk of pulmonary hypertension.

To diagnose pulmonary hypertension, your doctor may ask you questions about your medical history and do a physical exam. Based on your symptoms and risk factors, your doctor may refer you to a lung specialist (pulmonologist) or a heart and blood vessel specialist (cardiologist). Your doctor will diagnose you with pulmonary hypertension if tests show higher-than-normal pressure in the arteries of the lungs (pulmonary arteries).

Medical History and Physical Exam – Your doctor may ask you about any symptoms you have been experiencing and any risk factors such as other medical conditions you have. Your doctor will also perform a physical exam to look for signs that may help diagnose your condition. As part of this exam, your doctor may do the following:

• Measure your blood pressure.
• Look at the veins in your neck to see if they are larger than normal.
• Listen to your heart to see if there are changes in how it sounds, and to find out if your heartbeat is faster than normal or irregular or if you have a new heart murmur.
• Listen to your lungs for sounds that could be caused by heart failure or significant underlying lung disease.
• Feel your liver to see if it is larger than normal.
• Look for swelling in your abdomen and legs that may be caused by fluid buildup.
• Check whether the oxygen levels in your blood are low. This may be done by pulse oximetry, in which a probe is placed on your finger to check your oxygen levels.

Diagnostic Tests – There are many tests that doctors can use to tell if you have pulmonary hypertension. The most common tests to measure the pressure in your pulmonary arteries are cardiac catheterization and echocardiography. Normal pressure in the pulmonary arteries is between 11 and 20 millimeters of mercury (mm Hg). If the pressure is too high, you may have pulmonary hypertension. A pressure of 25 mm Hg or greater measured by cardiac catheterization or 35 to 40 mm Hg or greater on echocardiography suggests pulmonary hypertension.

Other tests may include:

• Blood tests look for blood clots, stress on the heart, or anemia.
• Electrocardiogram (ECG or EKG) looks for changes in the electrical activity of your heart. This can help detect if certain parts of the heart are damaged or working too hard due to pulmonary hypertension.
• Heart and lung imaging tests, such as chest X-ray, cardiac CT, or MRI, take detailed pictures of the structure and functioning of the heart, lungs, and surrounding blood vessels.

Test for Other Medical Conditions – Your doctor may order additional tests to see whether another medical condition or medicine may be causing your pulmonary hypertension. Doctors can use this information to develop your treatment plan.

After you are diagnosed with pulmonary hypertension, it is important to follow your treatment plan, get regular care, and learn how to monitor your condition. Taking these steps can slow down the progression of the disease and may improve your condition. Your specific treatment plan will depend on the cause of your pulmonary hypertension, as well as how advanced it is. Pulmonary hypertension can get worse over time and lead to serious problems, including:

• Anemia, which can cause your body to not get enough oxygen-rich blood
• Arrhythmias, which are problems with the speed of your heartbeat
• Blood clots in the pulmonary arteries
• Bleeding in the lungs
• Heart failure
• Liver damage
• Pericardial effusion, which is a collection of fluid around the heart
• Pregnancy complications that can be life-threatening for the mother and baby

Receive Routine Follow-up Care – Your follow-up care may include recommendations such as these:

• Participate in support groups, counseling, and education efforts that can help you manage the activities of daily living, experience a successful pregnancy, and generally improve the quality of your life.
• Get the recommended vaccines, which often include a vaccine for pneumococcus and influenza or flu, shot every year at the start of flu season.
• Monitor your condition.

Talk to your doctor about new or concerning symptoms. People who have pulmonary hypertension may need regular tests. Your doctor may recommend the following to monitor your condition and treatment response:

• Six-minute Walk test to monitor your ability to exercise
• Blood tests to check hemoglobin, iron, and electrolyte levels; kidney, liver, and thyroid function; your blood’s ability to clot; and signs of stress on the heart
• Cardiac catheterization
• Cardiac MRI to monitor your heart’s size and how well it is working
• Chest X-ray
• Echocardiography to monitor your heart’s size and how well it is working, and measure the pressure in your right heart chambers
• Electrocardiogram to check for irregular heartbeats
• Lung function tests to check for any change in your lung function

Prevent Complications Over Your Lifetime – To help prevent some of the complications of pulmonary hypertension, your doctor may recommend the following:

•  Treat other medical conditions, such as COPD, heart conditions, and sleep apnea. Make sure you take prescribed medications that treat these disorders.
• Make heart-healthy lifestyle changes such as heart-healthy eating if your pulmonary hypertension is due to heart failure from heart disease or high blood pressure.
• Engage in regular physical activity. Before starting any exercise program, ask your doctor about what level of physical activity is right for you.
• Avoid high altitudes when possible and discuss with your doctor any plans for air travel or visits to places at high altitudes.
• Talk to your doctor if you are planning to get pregnant, as there is an increased risk of pregnancy complications.

Prevention is not always possible since the cause of pulmonary hypertension is not always clear. Your doctor may suggest a preventative screening if you have a known risk factor or medical condition that causes pulmonary hypertension. Your doctor may also recommend prevention strategies to help you lower your risk of developing pulmonary hypertension. Talk to your doctor about your risk factors and concerns to learn more.