A type of pulmonary hypertension (high blood pressure in the pulmonary arteries) that negatively affects the small blood vessels in the lungs, making them narrower, is called pulmonary arterial hypertension (PAH). Usually, people do not have any symptoms until the condition advances. In general, it is quite difficult to diagnose this condition, and doctors often perform multiple tests to confirm it. Physicians often recommend PAH medicines and lifestyle changes to improve your quality of life and lifespan.
Furthermore, this condition causes reduced blood flow to the lungs, which leads to increased blood pressure in the lung arteries. Normally, these arteries carry oxygen-rich blood from the heart to the lungs. When these arteries become narrowed, the heart should work harder to pump blood to the lungs. You should get treatment as soon as possible if you have this condition. Otherwise, it may lead to multiple health problems throughout the body.
This type of pulmonary hypertension is not as common as other forms of this condition (such as those caused by underlying heart or lung disease). Every year, about 500 to 1,000 people are diagnosed with PAH in the United States. In addition, in Western countries, approximately 25 out of 1 million people live with PAH.
Symptoms
While people do not have any symptoms in the early stages of the disease, when it advances, they may experience the following symptoms. Check below some examples:
- Blue lips or fingers
- Dizziness
- Chest pain or pressure
- Fatigue (extreme tiredness)
- Shortness of breath (this symptom may worsen over time)
- Edema (swelling in the feet and legs progressing to the abdomen and neck)
- Fast heart rate
If you do not get treatment for this condition, the previous symptoms may worsen over time. Immediately contact your doctor if any of the previous symptoms occur.
Causes
This type of pulmonary hypertension occurs due to damage to the lining of the blood vessels in the lungs. While it is not always possible to determine what exact causes damage to these small blood vessels (idiopathic pulmonary arterial hypertension), it is often associated with certain diseases, genetic mutations, and some medicines. Check below some health conditions that may contribute to PAH:
- HIV infection
- Lupus
- Liver disease
- Scleroderma
- Portal hypertension
- Congenital heart defects
- Glycogen storage disease
- Pulmonary capillary hemangiomatosis
- Schistosomiasis
- Pulmonary veno-occlusive disease
Check below some genetic mutations that may also lead to PAH:
- Mutations in the BMPR2 gene cause an accumulation of cells in the small arteries of the lungs. As a result, they become narrow and allow less blood to flow through them.
- Family history – Sometimes, genetic mutations that lead to PAH may run in families. In such cases, the condition is called heritable PAH, and about 80% of people with this form of PAH have mutations to their BMPR2 gene.
- Carriers – In some cases, people may carry the abnormal gene without developing the condition. Furthermore, they can pass this abnormal gene to their biological children in the future.
- Sporadic PAH – When people develop this type of pulmonary hypertension without a family history of the disease or genetic mutations, it is called sporadic PAH.
Additionally, the following drugs may also contribute to PAH. For example:
- Recreational drugs – For example, cocaine, methamphetamine, and others.
- Diet pills – While some of the diet pills are not available anymore, you may develop PAH even years after stopping the use of these medications. One example is “fen-phen”, which some research has shown can be dangerous.
Risk Factors
While sometimes it is not possible to determine the exact cause of pulmonary arterial hypertension, they have identified some factors that may increase your risk of developing it. Check below some examples:
- Persistent pulmonary hypertension in the neonate (PPHN)
- Sex – Females are more likely to develop PAH than males.
- Age – Usually, at increased risk of PAH are older adults (over 65 years old).
- Connective tissue disease
- Down syndrome
- Family history of PAH
- HIV infection
- Diet medicines (including Dexfenfluramine and Phentermine)
- Use of illegal drugs
You can perform some genetic tests if your parent or sibling has this condition. It helps understand the risks of developing it.
What Happens if Pulmonary Arterial Hypertension Goes Untreated?
People with this condition may also experience some complications, especially if they leave it untreated. Examples include:
- Blood clots
- Arrhythmia (irregular heart rhythm)
- Left main compression syndrome
- Pericardial effusion
- Bleeding in the lungs (also known as hemoptysis)
- Anemia (low red blood cell count)
- Pregnancy complications
- Liver damage
The previous list does not contain a full list of complications. However, you can consult your doctor about ways to reduce the risk of developing them.
How to Prevent Pulmonary Arterial Hypertension?
While some risk factors for PAH cannot be managed or controlled (such as genetic mutations), some of them can be avoided. For example, street drugs, excessive body weight, and other health problems. Moreover, doctors may recommend preventive screenings for PAH if you have risk factors for this condition.
Diagnosis
Usually, it is challenging to diagnose PAH because there are multiple health conditions that cause symptoms similar to this condition. However, the diagnosis often begins with a physical examination to check for abnormalities linked to the disease. When doctors suspect PAH, you may be referred to a pulmonologist or cardiologist for further testing. They often perform the following tests to confirm or rule out PAH. It is important to understand that PAH requires specific treatment that is not appropriate for other forms of pulmonary hypertension. Check below some tests:
- Blood tests – These tests are done to check organ function and hormone levels. They may also help identify underlying conditions. Doctors often perform a complete metabolic panel and complete blood count (CBC).
- Imaging tests – The following tests are used to get detailed images of the heart and lungs. They help doctors evaluate heart chambers and pulmonary arteries. Usually, doctors perform chest X-rays, chest CT (computerized tomography) scans, or heart MRI (magnetic resonance imaging) scans.
- PSG (polysomnogram) – This test is used to check whether you have sleep apnea. This sleep disorder can worsen PAH.
- Pulmonary function tests – These tests are used to check lung function.
- Pulmonary ventilation/perfusion (VQ) scan – This test is used to check for blood clots in the lungs. Commonly, it helps rule out chronic thromboembolic pulmonary hypertension.
- Right heart catheterization – It helps measure the pulmonary artery pressures. Furthermore, it is essential for diagnosing PAH.
- Six-minute walk test – During this test, doctors will examine how much exercise you can handle and how much oxygen circulates in the blood.
Treatment
In general, the treatment goal is to reduce the progression of the disease and improve your quality of life. However, there is no standard treatment for PAH, and doctors usually prescribe different treatments for people with this type of pulmonary hypertension. Check below some of them:
- BAS (balloon atrial septostomy) – This treatment is often used in babies with congenital heart defects, but it can also be used in adults with PAH. It helps reduce pressure on the right side of the heart, which allows more oxygen to circulate in the blood.
- Calcium channel blockers – This group of medicines is often used to reduce blood pressure in the pulmonary arteries as well as throughout the body.
- Diuretics (also known as water pills) – These medications are used to remove excess fluid from the body. It may also help relieve swelling.
- Oxygen therapy – Sometimes, people need supplemental oxygen during tests, sleep, or exercise.
- Pulmonary vasodilators – These medicines are used to relax pulmonary arteries, which often helps reduce strain on the heart muscle and ease the symptoms.
- A lung transplant – This treatment is usually recommended by doctors if you develop a severe form of PAH or the previous options do not work.
The medications used to treat PAH come in different forms, including oral, inhaled, portable infusion pump, and intravenous (IV). Check below some medicines approved by the FDA (Food and Drug Administration) for people with this type of pulmonary hypertension:
- Ambrisentan
- Bosentan
- Epoprostenol
- Iloprost
- Riociguat
- Selexipag
- Macitentan
- Tadalafil
- Sildenafil
- Treprostinil (this medicine is available in all forms listed above)
However, the previous medications may give you some adverse reactions, especially if they are used for long periods. These include lightheadedness, headaches, hypotension (low blood pressure), rash, upper respiratory congestion, flushing, gastrointestinal symptoms (including nausea, vomiting, diarrhea, and others), and pedal edema (swelling in the ankles and feet).
Frequently Asked Questions
How serious is pulmonary arterial hypertension?
This is a serious condition that sometimes can be life-threatening. The only way to prevent serious complications is early diagnosis and proper treatment.
How does pulmonary arterial hypertension affect my body?
This condition strains the right side of the heart, which normally pumps oxygen-poor blood to the lungs. In more severe cases, it may lead to right-sided heart failure. PAH may also slow down blood flow between the heart and lungs, which causes less blood to enter the lungs to get fresh oxygen. Sometimes, this condition can be fatal without treatment.
Can pulmonary arterial hypertension be reversed?
Nowadays, there are specific medications that may slow down the progression of this disease. However, these medicines cannot reverse the damage already done. In addition, researchers are working on new medicines that could help reverse this condition. Ask your healthcare provider if you have additional questions.
