Pulmonary Hypertension

A diagnosis of pulmonary hypertension (PH) can be overwhelming and bewildering, leading to questions and concerns about therapies, outcomes, causes and lifestyle changes. PH refers to any issue that causes a problem in the blood vessels of the lungs. PH is a broad term, encompassing 5 different groups of the condition, based on the causes.

Dr. Denise Sese, an MUSC Health physician who specializes in pulmonary hypertension, explains what PH is, the different types of it, and the importance of early diagnosis.

Q. What is pulmonary hypertension?

A. Researchers have found that PH occurs when there is injury to the vessels of the lung. Because of some insult, medication, related disease, genetics, or for some unknown reason the blood vessels of the lungs abnormally constrict, or narrow, leading to elevated blood pressure in the lungs. This can then lead to failure of the right side of the heart.

Q. What are the different types of pulmonary hypertension?

A. Pulmonary hypertension is classified into different groups: pulmonary arterial hypertension (PAH), pulmonary hypertension caused by left heart disease, pulmonary hypertension caused by lung disease, PH due to blood clots, and secondary PH, usually associated with another disease or condition.

Q. How do they differ?

A. In PAH, the artery walls in the lungs become diseased, causing stiffness and thickening and narrowing of the arteries. PAH can be caused by genetics, or it can be idiopathic, meaning its cause is unknown. PAH can also be caused by drug use, such as amphetamines, diet pills, methamphetamines and cocaine, and diseases such as liver disease/cirrhosis, HIV, and connective disease such as lupus and scleroderma. The most common cause of PAH is an infection with a parasitic worm called schistosomiasis.

The most common form of PH is left heart disease, which occurs when the heart cannot adequately pump blood throughout the body. That weakened ability causes blood to back up in the lungs, raising pressure in the lungs.

The third group of PH is caused by lung disease, such as COPD, interstitial lung disease, cystic fibrosis, and sometimes sleep apnea. These conditions cause the arteries in the lungs to tighten, restricting air flow to areas that receive the most blood and oxygen.

The fourth group of PH is caused by blood clots in the pulmonary arteries, which restrict blood flow in the lungs and lead to high blood pressure. PH caused by blood clots is curable by removal of the clots.

The cause of secondary PH is not fully known, although it is linked to other conditions, including sickle cell anemia, metabolic disorder and removal of the spleen.

Q. What are the symptoms of PH?

A. The most common initial symptoms of PH are shortness of breath when moving, fatigue, a racing heart and/or passing out. If not treated, PH will become worse and may ultimately require oxygen or medications to treat heart failure.

Q. Is there a link between COVID-19 and PH?

A. Yes. PH is expected to increase as a result of COVID-19, primarily among patients whose lungs were scarred by the disease. COVID-19 also increases chances of blood clots, so some patients may develop PH as a consequence.

Q. Is PH treatable or curable?

A. No. PH is incurable and eventually will progress, but the good news is that there has been an exponential increase in the available treatments that can extend life expectancy and allow individuals to lead fulfilling lives if it is diagnosed early. That said, understanding the specific cause of one's PH is vital since different types of PH are treated differently.

Q. How is PAH treated?

A. It depends. For secondary PH, the treatment is to fix the underlying cause. For PAH, treatments include vasodilator medications that open up the blood vessels. These can be administered through an intravenous port, a subcutaneous pump, an inhaler or orally. These medications, along with other treatment protocols greatly improve symptoms and quality of life. It is important to distinguish the two because treatments for PAH can be harmful for PH.

Q. Can PH or PAH be prevented?

A. Unfortunately, PAH cannot be avoided, but if diagnosed early, can have as fruitful life unless it is caused by drugs or other foreign bodies. The chances of contracting secondary PH can be reduced by living a healthy lifestyle and avoiding diseases that can cause this secondary process, such as smoking, heart disease and vaccines.

Q. Does MUSC Health treat PH and PAH?

A. MUSC Health is home to South Carolina's only accredited pulmonary hypertension care center, which houses clinicians, counselors and cutting-edge researchers who are studying the mechanism of the disease and effects of new treatment. A highly experienced multidisciplinary care team from disciplines such as nephrology, gastroenterology, cardiology and rheumatology provide the latest advances in diagnostics and treatments, including clinical trials.

Learn more about MUSC Health’s lung treatment and specialists. To make an appointment with an MUSC Health lung specialist, call 843-792-9200.