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Pulmonary Hypertension: Lungs Under Pressure

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What is Pulmonary Hypertension?


Pulmonary Hypertension (PH) is a disease in which the blood vessels connecting the heart and the lungs develop high blood pressure. This can lead to fluid congestion in the lungs and dysfunction of the right side of the heart, known as Right Heart Failure.


Symptoms of Pulmonary Hypertension


Symptoms of PH vary from person to person, but commonly include the following:

  • Shortness of Breath (worse with exercise or lying flat)

  • Fatigue

  • Dizziness/lightheadedness

  • Poor activity tolerance

  • Swelling in the feet, legs, and abdomen

  • Chest pressure

  • Bluish tint around the lips (cyanosis)

  • Palpitations (sensation of racing, skipping, or pounding heart)


Different Types of Pulmonary Hypertension


Pulmonary Hypertension is classified into different Groups by the World Health Organization (WHO) based on the underlying cause. These groups help providers determine the best approaches to treatment. 


Group 1 - Pulmonary Arterial Hypertension (PAH)

  • WHO Group 1 describes Pulmonary Hypertension due to narrowing or stiffening of the pulmonary arteries (PAH).  

  • The pulmonary arteries are the blood vessels that carry oxygen-poor blood from the right side of the heart to the lungs, where it gets reloaded with oxygen. From there, the oxygen-rich blood is sent back to the left side of the heart, which pumps it out to the rest of the body. 

  • When pulmonary arteries narrow, they cannot carry enough blood to the lungs to meet the body’s demands. Ultimately, blood backs up in the heart and blood vessels, increasing the pressure and causing tissue damage. Also, less oxygen-rich blood is available to supply oxygen to the heart and body.

  • PAH is a condition that sometimes runs in families. It may also be caused by some drugs, such as stimulants, or by other health conditions,  such as HIV or lupus, that change the structure of the arteries.

  • Some cases of PAH are idiopathic, which means their exact cause is not known.


Group 2 - Pulmonary Hypertension due to Left-Sided Heart Disease

  • WHO Group 2 includes patients with Pulmonary Hypertension due to left-sided heart dysfunction.  

  • Left-sided heart failure is also known as Congestive Heart Failure (CHF). It can be due to coronary artery disease, heart valve disease, high blood pressure, or diseases that cause damage to the heart muscle, such as myocardial infarction (heart attack). CHF is often due to a combination of these factors.


Group 3 - Pulmonary Hypertension due to Lung Disease

  • WHO Group 3 consists of cases of pulmonary Hypertension due to lung disease.  

  • These conditions cause the blood’s oxygen levels to drop to dangerously low levels. This state is known as hypoxia and can result in tissue death due to lack of oxygen.

  • Examples of such lung diseases include:

    • COPD

    • Emphysema

    • Pulmonary Sarcoidosis

    • Sleep Apnea

    • Pulmonary Fibrosis 


Group 4 - Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

  • WHO Group 4 involves Pulmonary Hypertension due to blood clots in the lungs (pulmonary thrombus/pulmonary embolism).  

  • Typically, PH occurs with clots that do not fully dissolve with standard blood clot treatment, or when embolism occurs repeatedly due to blood-clotting disorders.  

  • The accumulation of clots in the lungs blocks blood flow and damages both the pulmonary vessels and the tissues that rely on oxygen carried from the lungs. Sometimes this can cause ischemia and infarction of the lungs, meaning portions of lung tissue die from lack of oxygen.

  • Patients who have a history of blood clots in the legs (DVT) or who have certain cancers or autoimmune disorders are at higher risk of developing pulmonary embolism and CTEPH.


Group 5 - Pulmonary Hypertension due to unclear or multifactorial causes

  • WHO Group 5 describes PH cases that don’t fit well into other categories and may be due to multiple or unclear causes. Examples of these causes include:

    • Hematological disorders 

      • Certain types of anemia 

      • History of splenectomy (spleen removal) 

      • Myeloproliferative disorders, such as Leukemia or Polycythemia Vera

      • Sickle Cell Disease

    • Systemic or Metabolic Disorders

      • Thyroid Disease

      • Gaucher Disease

      • Chronic Kidney Disease

      • Glycogen Storage Disease

    • Complex Congenital Heart Disease

    • Other Rare Diseases


Patients sometimes fall into more than one WHO group. For instance, a patient with Pulmonary Hypertension due to a combination of heart and lung disease would be classified under both WHO Groups 2 and 3.


Diagnosis


Physical Exam: 

The first step in diagnosing Pulmonary Hypertension is the physical exam. During this face-to-face encounter, a licensed provider will check vital signs and interview the patient about symptoms and medical history. The provider will then use their eyes and hands to examine the patient’s body for abnormalities. They will also listen to the lungs and heart with a stethoscope.


Based on symptom severity, the doctor will assign the patient a NYHA Functional Class, ranging from I to IV, with I representing mild symptoms and IV representing the most severe symptoms.


The doctor may then order further testing based on findings from the physical exam. Pulmonary Hypertension is not generally treated based solely on physical exam findings.


Right Heart Catheterization

 The “gold standard” method for diagnosis of Pulmonary Hypertension is Right Heart Catheterization (cath). This minimally invasive procedure is usually performed on an outpatient basis and is always done by a licensed physician.


During a Right Heart Cath, the doctor will have the patient lie on their back and will give light sedation. A tube, called a Pulmonary Artery Catheter, is passed via a vein in the neck into the right side of the heart. The doctor collects pressure measurements in the pulmonary vessels and right heart before removing the tube.  


A Right Heart Cath is often performed at the same time as a Coronary Angiogram (Left Heart Catheterization). Data from a Right Heart Cath helps the provider better understand disease severity and guide treatment selection.


Echocardiogram

 A second key method for evaluating pulmonary Hypertension is an Echocardiogram (Echo), which is an ultrasound of the heart. An Echo uses sound waves to help doctors see the structures and pumping function of the heart muscle, valves, and some connected blood vessels.


Other Tests

Several other tests may be necessary to further clarify a patient’s PH diagnosis. These may be repeated over time to determine how well treatment is working. These tests include:

  • Pulmonary Function Tests (PFTs)

  • Labwork involving blood and urine collection

  • Six Minute Walk Test

  • Nuclear Cardiac Stress Test

  • EKG/ECG

  • Chest X-Ray

  • Cardiac MRI or CT scan

  • Lung Ventilation/Perfusion Scan (V/Q Scan)

  • Genetic Testing



Treatment


Pulmonary Hypertension does not have a cure, but multiple treatments are available to help decrease symptoms of the disease.  


The type of treatment depends on disease severity, underlying causes, and the presence of other existing health conditions. Specific therapies for one WHO group may not be appropriate for another group.


The majority of treatments for Pulmonary Hypertension aim to manage the underlying diseases that cause or worsen PH.


First-line treatments:

  • Antihypertensives - medicines that lower blood pressure

  • Antiarrhythmicsmedicationsnes that control heart rate and rhythm

  • Anticoagulantsmedicationsnes that thin the blood and prevent clotting

  • Diureticsmedicationsnes that help the body get rid of excess fluid by urination

  • Steroids, inhaled or oral - for patients with autoimmune or inflammatory disease

  • Cardiac glycosidesmedicationsnes that help the heart contract with more force

  • Nitratesmedicationsnes that dilate the blood vessels

  • Inhaled supplemental Oxygen (O2)


Specialty Drugs


Many patients with Pulmonary Hypertension will eventually require the addition of stronger, more tailored drugs to manage the disease. These include:


  • Phosphodiesterase-5 (PDE-5) Inhibitors

    - work by helping to dilate the pulmonary arteries and increase blood flow to the lungs

    • Examples: 

      • Sildenafil (Revatio, Viagra) 

      • Tadalafil (Adcirca, Cialis)

  • Endothelin Receptor Antagonists (ERAs)

    - work by blocking endothelin, a naturally occurring substance in the body that leads to blood vessel narrowing

    • Examples: 

      • Ambrisentan (Letairis) 

      • Bosentan (Tracleer) 

      • Macitentan (Opsumit )

  • Prostaglandin-Related

    - work by increasing prostaglandin, a hormone that decreases blood clotting and dilates the blood vessels, leading to increased blood flow to the lungs. These drugs may be delivered by mouth, by inhaler, or by IV.

    • Examples: 

      • Epoprostenol (Flolan, Veletri) 

      • Treprostinil (Orenitram, Remodulin, Tyvaso)

      • Iloprost (Ventavis)

      • Selexipag (Uptravi)

  • Soluble Guanylate Cyclase Inhibitors (SGCs)

    - work by causing blood vessels to dilate and reducing lung pressure

    • Example:

      • Riociguat (Adempas)



These specialty drugs carry higher risks and are more expensive to produce. Thus, they usually require special authorization from insurance as well as close, regular checking of labwork and other pertinent test results.  


Some of them are only available through programs called REMS, which the FDA requires to ensure drug adherence and safety monitoring. Drugs requiring REMS have dedicated programs through the drug manufacturers. If a provider prescribes any of these medicines, they will assist you with REMS program enrollment and follow-up.


End-Stage Pulmonary Hypertension


For patients whose disease becomes severe enough to be classified as end-stage, they may be eligible for a heart and/or lung transplant or other Advanced Heart Failure therapies. If this is a possibility, the physician managing the Pulmonary Hypertension will refer patients to a specialist in Advanced Heart Failure.


Resources


Many Resources are available to help patients with Pulmonary Hypertension and their caregivers.


Pulmonary Rehabilitation 

These are structured programs in which nurses help qualified patients develop an exercise regimen to improve or maintain lung function. These programs take place in group settings, so patients benefit from the support of both peers and professionals.


Financial Assistance 

For many patients, assistance programs or financial grants exist to help offset the monetary cost of PH medications. If available, your healthcare provider can assist you in finding and applying for these opportunities.


Support Groups  

Networks of support exist on local, national, and international levels to help patients with PH find resources, understand their condition, and connect with others affected by PH. More information about various support groups can be found through the Pulmonary Hypertension Association (https://phassociation.org/living-with-ph/support/explore-groups) and the PAH Initiative (https://www.pahinitiative.com/pah-information-support/groups-specialists).





References


American Lung Association. (2025, August 20). Pulmonary Rehabilitation. Retrieved November 4, 2025, from https://www.lung.org/lung-health-diseases/lung-procedures-and-tests/pulmonary-rehab



Cleveland Clinic. (n.d.). Pulmonary Arteries. Retrieved October 28, 2025, from https://my.clevelandclinic.org/health/body/21486-pulmonary-arteries.




LeVarge, B., & Ford, H. J. (2021). Epidemiology, Pathogenesis, and Clinical Approach in Group 5 Pulmonary Hypertension. Frontiers in Medicine, 7, 616720. https://doi.org/10.3389/fmed.2020.616720.


Pulmonary Associates of Brandon. (2018, June 1). 5 Pulmonary Hypertension Groups You Should Know About. Retrieved October 28, 2025, from https://floridachest.com/pulmonary-blog/5-pulmonary-hypertension-groups-you-should-know-about.


Pulmonary Hypertension R.N. (n.d.). Types of Pulmonary Hypertension. Retrieved October 28, 2025, from https://pulmonaryhypertensionrn.com/types-of-pulmonary-hypertension/.


Sabbula B.R., Sankari A., Akella J. (2024, March 4). Chronic Thromboembolic Pulmonary Hypertension. In StatPearls. StatPearls Publishing. Retrieved October 28, 2025, from https://www.ncbi.nlm.nih.gov/books/NBK549836/ . 


U.S. Food & Drug Administration. (2025, May 20). Risk Evaluation and Mitigation Strategies | REMS. Retrieved November 4, 2025, from https://www.fda.gov/drugs/drug-safety-and-availability/risk-evaluation-and-mitigation-strategies-rems


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