Pulmonary hypertension is pretty rare, and in the US, only about 1-2 people in every one million¹ have it. Although the disease is life-threatening and worsens over time, treatments can relieve the symptoms and ensure patients have a better life. While that requires some planning, many people with this disease find ways to live their lives and continue doing everything they did before the diagnosis.
We make it easy for you to participate in a clinical trial for High blood pressure, and get access to the latest treatments not yet widely available - and be a part of finding a cure.
Pulmonary hypertension (PH) refers to abnormally high blood pressure in the arteries that carry blood from the heart to the lungs.
The human body has two sets of blood vessels facilitating blood flow from the heart, known as pulmonary circulation and systemic circulation:
Pulmonary circulation: This set of blood vessels allows the deoxygenated blood to flow from the right side of the heart to the lungs, where it's oxygenated, and then back to the left side of the heart.
Systemic circulation: This set facilitates the flow of oxygenated blood from the left side of the heart to the entire body.
Pulmonary circulation normally has lower pressure than systemic circulation. However, if the blood pressure in the pulmonary section increases by an unusually high amount, it results in what is known as pulmonary hypertension.
So, what is the difference between pulmonary hypertension and systemic hypertension?
Systemic hypertension results from increased pressure in the arteries throughout the body.
Pulmonary hypertension is more specific to the unusual increase in pressure in the arteries that carry blood from the heart to the lungs.
Pulmonary arterial hypertension (PAH) is a sub-group of pulmonary hypertension and refers to the tightening and stiffening of the walls of the arteries of the lungs. PAH is chronic and currently incurable.
In people with pulmonary arterial diseases, the right side of the heart works harder to pump blood through the narrowed arteries in the lungs. As a result, the right side of the heart enlarges, and its flexibility decreases, affecting its ability to pump blood to the lungs and into the entire body.
Higher pressure in the pulmonary arteries that cause PH does not necessarily mean you have pulmonary arterial hypertension. It may be caused by other diseases such as lung disease, obstructive sleep apnea (OSA), diastolic heart failure, or other reasons altogether.
Symptoms of pulmonary hypertension usually begin to show when the condition progresses. So, you may not realize you have pulmonary hypertension in its early stages.
Early symptoms of pulmonary hypertension
Bluish lips and skin
Shortness of breath when you perform daily activities, e.g., climbing stairs
Swelling in the ankles, abdomen, or legs
These symptoms range in severity, and you may not necessarily suffer from all of them.
Symptoms of advanced pulmonary hypertension
Passing out or dizziness
Irregular heartbeat (palpitations or extreme throbbing sensation)
Difficulty breathing while at rest
Tachycardia or racing pulse, where the heartbeat exceeds 100 per minute
Continuous shortness of breath during exercise or activity
As the condition worsens, you may find it challenging to carry out everyday activities.
The underlying conditions that could lead to pulmonary hypertension include:
Hyperthyroidism – This condition involves the production of excess hormones by the thyroid gland, causing irregular or rapid heartbeat
Inflamed pulmonary blood vessels
Chronic lung diseases, including chronic bronchitis and cystic fibrosis
Recurring blood clot formation in the pulmonary arteries or blood clots that travel from the legs to the pulmonary arteries
Rheumatologic conditions including lupus, scleroderma (systemic sclerosis), or rheumatoid arthritis
Use of illicit drugs such as cocaine or methamphetamine
Certain heart diseases, including mitral valve disease, aortic valve disease, and congestive heart failure (left side)
Advanced liver disease
Low-oxygen conditions such as high altitude living, obstructive sleep apnea, and obesity
Certain medications, e.g., Fen-Phen medication used for weight loss
Conditions that interfere with blood flow from the heart to other body parts lead to blood stacking, which causes pulmonary hypertension by increasing pulmonary venous pressure.
Changes in blood vessels
Some infections and diseases, including cancer, may cause narrowing of the inner lining of the blood vessels when tissues grow around and within the vessels. Also, scarring and masses from other conditions, like pulmonary fibrosis and sarcoidosis, increase blood pressure by narrowing and compressing blood vessels.
Hypoxic pulmonary vasoconstriction
This refers to lung vessels narrowing as they try to redirect blood flow from the parts of the lungs that are functioning poorly.
For example, pneumonia causes inflammation in part of the lung, affecting its ability to oxygenate and deoxygenate blood. Hypoxic pulmonary vasoconstriction diverts the blood to better functioning areas of the lung that aren’t affected by pneumonia, in an effort to maintain optimum oxygenation. However, this process involves the constriction of the pulmonary artery vessels and raises pulmonary blood pressure.
Blockage of blood vessels
If you have a parasitic infection such as schistosomiasis, it can cause blockages in the blood vessels that lead to pulmonary hypertension.
Constriction of blood vessels
If you take certain substances, including methamphetamines, cocaine, and anti-obesity drugs, e.g., dexfenfluramine (Redux), they may cause constriction of the blood vessels, which elevates blood pressure in the pulmonary artery.
Some diseases cause pulmonary hypertension for unknown reasons. It is thought, for example, that certain chemical or toxin effects may cause constriction or growth of tissue around or within the vessel.
The World Health Organization (WHO) puts pulmonary hypertension into five groups according to the cause or associated underlying conditions.
Pulmonary arterial hypertension (PAH)
Heart problems present during childbirth (congenital heart disease)
Conditions such as HIV infection, connective tissue disorders (lupus, scleroderma, etc.), and chronic liver disease (cirrhosis)
Alterations in a gene passed down through generations in a family (heritable PAH)
Unknown cause (idiopathic PAH)
Prescription diet medications or illegal drugs, like meth
Results from heart disease affecting the right side of the heart
Diseases in the left-sided heart valve, e.g., aortic valve or mitral valve disease
Failure of the left ventricle
Caused by lung disease
Scarred tissues between the lung's air sacs (pulmonary fibrosis)
Obstructive sleep apnea (OSA)
Chronic obstructive pulmonary disease (COPD)
Living in high altitude areas while being at a higher risk of pulmonary hypertension
Results from chronic blood clots
Clotting disorders such as chronic blood clotting in the lungs (pulmonary emboli)
Triggered by other health conditions
Metabolic disorders, such as glycogen storage disease
Blood disorders, e.g., essential thrombocythemia and polycythemia vera
Tumors exerting pressure on the pulmonary arteries
Inflammatory disorders, e.g., vasculitis and sarcoidosis
Pulmonary hypertension may lead to complications such as:
Pulmonary hypertension can lead to irregular heartbeats, resulting in heart palpitations (pounding heartbeat). Sometimes, it may cause dizziness or fainting.
Bleeding in the lungs
Another complication of pulmonary hypertension is bleeding in the lungs. The situation may worsen to the point of coughing up blood (called hemoptysis).
Enlargement of the right ventricles and heart failure
Enlargement of the right ventricles makes the heart pump blood harder than normal as it tries to force the flow through the narrow or blocked arteries. As a result, the heart walls thicken while the right ventricle expands to accommodate more blood. However, the thickening and enlargement creates more strain on the heart, which may ultimately cause heart failure.
People with pulmonary hypertension are at a higher risk of developing blood clots in the small arteries of their lungs.
Pulmonary hypertension puts the mother at risk of severe cardiac outcomes, especially during labor and delivery.
Pulmonary hypertension may result from many medical conditions. Therefore, ruling out the diseases and making the correct diagnosis requires an analysis of your full medical history, physical examination, and symptoms. Below are the physical exams and tests performed to diagnose pulmonary hypertension.
The doctor will complete these processes during a physical exam:
Establish whether you have abnormal heart sounds, e.g., a loud pulmonic valve sound, a galloping due to ventricular failure, or systolic murmuring of tricuspid regurgitation
Check for any enlargement or swelling of the jugular vein in the neck
Look out for fluid retention in the abdomen, legs, and ankles
Check for a bluish tint to the nail beds
Examine any signs of other underlying conditions that could be causing pulmonary hypertension
There are several blood tests used in diagnosing pulmonary hypertension. For example:
Thyroid-stimulating hormone (TSH) test to screen for thyroid problems
Screening for Human Immunodeficiency Virus (HIV)
Complete blood count (CBC) test to check for infection, increased hemoglobin, and/or anemia
B-type natriuretic peptide (BNP) test that is a marker for heart failure
Arterial blood gases (ABG) test to establish the level of oxygen in arterial blood
Complete metabolic panel (CMP) test to check liver and kidney function
Autoantibody blood test, e.g., ANA, ESR, and others, to screen for collagen vascular conditions
This test helps understand the likelihood of someone developing pulmonary hypertension. Echocardiogram tests done with a Doppler study help your healthcare provider better understand the pressure in the pulmonary artery. This is a painless ultrasound test that uses high-frequency sound waves to help evaluate blood flow.
Right heart catheterization
The right heart catheterization is a gold-standard test in diagnosing pulmonary hypertension. It involves inserting a catheter via the groin into the femoral vein until it reaches the right side of the heart. The catheter connects to a machine that tracks and measures the blood pressure in the pulmonary arteries and the right side of the heart.
Chest x-rays check for the enlargement of pulmonary arteries and right ventricle.
A six-minute walk test to determine your tolerance level to exercise and blood oxygen saturation level during the activity
Pulmonary function test to establish whether there are other lung conditions such as idiopathic pulmonary fibrosis and chronic obstructive pulmonary disease
Overnight oximetry or polysomnogram test screens for sleep apnea as it leads to reduced oxygen levels at night
Ventilation-perfusion scan (V/Q scan) test establishes whether there is any blood clotting along the pathway to the lungs.
Pulmonary angiogram tests screen for blood clots blocking the pulmonary arteries
It is important to obtain the correct pulmonary hypertension diagnosis before commencing any treatment. PH treatment varies by patient, depending on the underlying causes. Adhering to the treatment plan provided by your doctor is the best way to manage pulmonary hypertension.
The general treatments include:
Several types of medication can treat pulmonary hypertension, and each has a specific goal. The medication prescribed will depend on the underlying causes of the disease, how severe it is, the livelihood it will worsen, and a person’s drug tolerance.
Diuretics remove excess fluid from the bloodstream and tissues, helping to reduce swelling and enabling easier breathing. Examples include Torsemide (Demadex®), Spironolactone (Aldactone®)], and Furosemide (Lasix®).
Also known as blood-thinning drugs, such as Warfarin sodium (Coumadin®), anticoagulants reduce blood clot formation, improving blood flow in the blood vessels. However, people taking blood thinners need to monitor for bleeding complications and undergo regular lab tests to check the level of these drugs in their bloodstream.
Prevention of narrowing blood vessels
Taking Macitentan (Opsumit), Ambrisentan (Letairis®), or Bosentan (Tracleer®) helps counteract the vasoconstriction effects of the protein endothelin. These medications allow the arteries to relax, which lowers pulmonary blood pressure. If you take Bosentan, you will need monthly lab work to monitor your liver function.
Reduce blood pressure
Certain medications can reduce pulmonary blood pressure and may improve the pumping ability of the right side of the heart. These drugs include Diltiazem (Cardizem®) and Nifedipine (Procardia®).
Relaxation of pulmonary cells
Taking drugs such as Tadalafil (Adcirca®) and Sildenafil (Revatio®) widens the pulmonary arteries by relaxing the pulmonary smooth muscle cells.
Oxygen therapy can help people with low oxygen concentration in their blood.
Blood clot prevention
Medicines such as treprostinil sodium (Orenitram ®, Remodulin®, Tyvaso®), Iloprost (Ventavis®), and Epoprostenol (Veletri, Flolan®) widen pulmonary arteries and prevent blood clots from forming.
These can replace the potassium the body loses through increased urination from using diuretics. An example of a potassium drug is K-dur®.
Improve heart pumping
Inotropic agents like Digoxin enhance the heart’s pumping ability.
A quick guide to taking pulmonary hypertension medications
Taking pulmonary hypertension medication requires you to pay close attention to avoid any negative side effects. Ensure you do the following:
Keep a list of your medications and understand how each of them works
Avoid quitting a dose or changing your drugs unless you talk with your doctor first
Avoid taking herbal products alongside medications used for pulmonary hypertension as you may experience some side effects.
Avoid taking over-the-counter drugs with labels warning those with high blood pressure not to take them. You should ask your doctor before taking any OTC drug. Decongestants such as Sudafed®, Dimetapp®, and Benadryl® or any medications containing pseudoephedrine or ephedrine and non-steroidal anti-inflammatory agents (NSAIDs) such as Indocin®, Motrin®, and Advil® can cause issues in people with heart failure.
Ensure you take the medications at the same time every day. If you forget a dose, don’t take two doses to compensate for the one you missed, as it’s safer to miss a dose than to potentially overdose.
Another way to manage pulmonary hypertension is by making changes to your diet.
Consider making the following dietary changes:
Switch to foods with high levels of magnesium, e.g., broccoli, peanuts, and tofu, and potassium, e.g., dried fruits, oranges, and bananas
Limit your daily calorie intake
Reduce consumption of foods containing cholesterol, refined sugar, and saturated fats
Eat fiber-rich foods such as fruits, vegetables, whole grains, and bran
Check your fluid intake and reduce it if you notice swelling or realize you are becoming short of breath
You can also ask the doctor whether you should limit your vitamin K intake. These vitamins are present in some oils and green leafy vegetables and may interfere with how well blood thinners work
Changing your lifestyle can help in managing pulmonary hypertension. Below are some of the lifestyle habits and changes you should consider:
Avoid shoving, pushing, or lifting items above 20 pounds since they increase pressure in your lungs and arteries.
Walking helps improve circulation and maintain strong muscles, but you should avoid strenuous aerobic exercise since your lungs may not sustain the oxygen demands of your body during the activities. Also, stop any activity that causes chest pain, dizziness, or shortness of breath and reach out to your doctor if such symptoms occur.
Record your weight daily and talk to your doctor if you notice a sudden weight gain of two pounds in a day or five pounds over a week
If you are overweight, ensure you stick to your doctor's recommendation on weight management.
Go for annual health check-ups with your primary care doctor
Seek emotional support. Having pulmonary hypertension may cause stress, worry, anxiety, or depression. Share your feelings with your doctor so they can direct you to the help you need, including taking medicine for depression, going for counseling, or joining a patient support group.
Below are the surgical interventions that can help manage pulmonary hypertension:
This surgical therapy involves removing any blood clots to improve blood flow and lung function.
A lung transplant is an option if advanced pulmonary hypertension doesn’t respond to treatment, as the right side of the heart generally goes back to functioning normally after the procedure. However, the shortage of lung donors is a major barrier to this therapy in the US.
A lung/heart transplant involves replacing both the heart and the lung. This treatment is rare but necessary for PH patients with lung and left heart failure.
You should contact your doctor if you experience the following symptoms:
Shortness of breath that is worsening
Reduced tolerance for an activity or extreme fatigue with regular activities
Chest pain episodes
Confusion or restlessness
Worsening swelling in the legs, abdomen, and/or legs
Difficulty breathing at rest or constant lightheadedness or dizziness
A respiratory infection like a cough that is worsening
Nausea or poor appetite
Increased heart rate reaching 120 beats per minute
Weight gain at a rate of two pounds in a day or five pounds in a week
Patients with pulmonary hypertension generally see cardiologists and pulmonologists (pulmonary physicians). However, other physicians such as rheumatologists, sleep medicine doctors, and infectious disease specialists may be involved, depending on the cause.
Some questions to ask your doctor include:
What could be the cause of my pulmonary hypertension?
Which tests will I need, and does insurance cover them?
Should I limit fluid or salt intake?
How often should I visit the doctor for my problem?
Will treatment of PH interfere with treating other health problems that I have?
Will I need surgical therapy?
Preventing pulmonary hypertension focuses on reducing the risk factors that lead to the underlying cause. However, some risk factors, such as lupus, are uncontrollable; so, you cannot prevent them. Luckily, early diagnosis and appropriate treatment reduce the chances of advancement of this condition.
If you're diagnosed with pulmonary hypertension, you should have regular follow-ups with your doctor for signs, symptoms, blood pressure, and oxygen level checks. In a nutshell, pulmonary hypertension should be diagnosed and treated early to ensure it does not advance into a life-threatening condition.
We make it easy for you to participate in a clinical trial for High blood pressure, and get access to the latest treatments not yet widely available - and be a part of finding a cure.