Most people are not aware that our body has two different blood pressures. This is because our heart is divided into two chambers, called ventricles, that pump blood in two different circulations. The right ventricle pumps blood to your lungs through the pulmonary circulation and the left ventricle pumps blood to the rest of your body through the systemic circulation. High blood pressure refers to the blood pressure in the systemic circulation and is usually measured with a blood pressure cuff on your arm. 

Two circulations for blood flow 

The two circulations for blood flow are separated by the ventricle that is pumping blood through our body’s systems. Blood coming from the right ventricle pumps blood into the pulmonary artery and to the lungs, where it is oxygenated and drains through the pulmonary veins to the left ventricle. The left ventricle then pumps the blood to every organ in the body via the systemic circulation where the oxygen is absorbed before the blood drains into veins that carry it back to the right ventricle and the process starts over again. 

That small trip from the right ventricle to the lungs requires less pressure than it takes to pump it throughout the entire body. As a result, blood pressure in the lungs is very low, about 25/10 mmHg compared to a normal blood pressure of about 120/80 in the rest of our body. 

Because the lung blood pressure is so low, the right ventricle is not nearly as strong as the left ventricle and does not tolerate large increases in blood pressure in the lungs. 

What is pulmonary hypertension? 

Pulmonary hypertension is caused when blood pressure in the lungs rises too high. This increase in pressure causes the right ventricle to fail. 

Symptoms of pulmonary hypertension 

The most common symptom of pulmonary hypertension is shortness of breath, especially when trying to do more stressful activities such as climbing stairs, running, or carrying heavy objects. Other symptoms include: 

  • swelling in the legs 
  • chest pain or pressure 
  • feeling faint when trying to do too much 

These symptoms also occur with a condition called congestive heart failure or sometimes just heart failure. 

What is congestive heart failure? 

Congestive heart failure occurs when the left ventricle becomes unable to pump all the blood coming to it from the lungs. When this happens, blood backs up into the lungs causing congestion. 

The differences between congestive heart failure and pulmonary hypertension 

Congestive heart failure is caused by a failure of the left ventricle and can lead to the development of pulmonary hypertension as blood flow backs up into the lungs. If the pulmonary blood pressure becomes too high it can cause failure of the right ventricle. Congestive heart failure can be distinguished from pulmonary hypertension and right ventricular failure by looking at changes in the chest X-ray, physical exam or an ultrasound study of the heart, called an echocardiogram, which allows healthcare providers to determine if it is the right or left ventricle that is failing. 

Pulmonary hypertension is often seen in patients who have congestive heart failure or chronic lung diseases such as COPD or pulmonary fibrosis. In these situations, treating the underlying heart or lung disease is the best approach. 

Pulmonary arterial hypertension (PAH) 

Rarely, pulmonary hypertension is seen in the absence of any underlying heart or lung disease and is caused instead by a narrowing of the small arteries in the lungs. This disease is called pulmonary arterial hypertension (PAH) and usually leads to severe elevation in lung blood pressure that can be life-threatening. 

Early signs of PAH include: 

  • shortness of breath with stressful activity 
  • fatigue 
  • leg edema 
  • chest pressure and lightheadedness that occur with exertion 

As the disease progresses, patients can develop swelling in the abdomen, palpitations and fainting. 

Treatment for pulmonary arterial hypertension 

Fortunately, there are a large number of drugs that have been developed for the treatment of PAH over the last 30 years. Although none are curative, they have been shown to improve patients’ symptoms and prolong life. 

In extreme cases, patients whose disease progresses despite all medical therapies may require lung transplantation. Some cases of PAH are caused by genetic mutations that can be readily identified to see if patient’s family members may be at risk. Patients with a family history of PAH or connective tissue diseases such as scleroderma, CREST or mixed connective tissue disease are at increased risk of PAH and should be screened annually for the disease with a physical exam, echocardiogram and six-minute walk test. 

Patients who remain short of breath after being treated for blood clots in the lung (known as pulmonary embolism), should also be checked for pulmonary hypertension. 

Treatment options for pulmonary hypertension or congestive heart failure 

There are many options for treating both pulmonary hypertension and congestive heart failure, including medications, diuretics, and potentially lung or heart transplants. Specific treatment plans are tailored to each individual based on the severity of their diseases. Pulmonologists and cardiologists, along with other members of a patient’s care team, work together to personalize the treatment plans. 

Lifestyle changes for managing pulmonary hypertension and PAH 

Lifestyle changes that can help manage pulmonary hypertension and PAH include: 

  • maintaining a healthy weight 
  • avoiding excess salt and fluids 
  • maintaining a low-level exercise program 

Many rehabilitation centers offer supervised exercise training referred to as pulmonary rehabilitation that has been shown to help patients feel better and do more. 

Low oxygen levels cause the blood pressure in the lungs to rise and should be avoided in patients with pulmonary hypertension. This may occur in patients who live at high altitude or who have obstructive sleep apnea. 

Pulmonary hypertension is increasingly recognized as an important lung disease and should be considered in any patient who has shortness of breath that is not explained by other diseases. At the Center for Advanced Lung Care, we’re leading the way with advanced treatment options for pulmonary hypertension and many other advanced lung and heart-lung diseases. To learn more about us, visit our website or call 401-606-8250.

James R. Klinger, MD

James R. Klinger, MD

Dr. James R. Klinger is director of the Rhode Island Hospital Pulmonary Hypertension Center.