Pulmonary hypertension (or PH) is a medical condition that happens when you have high blood pressure in the vessels of your lungs. [1] High blood pressure in the lungs makes the heart work too hard, which causes symptoms like shortness of breath, lightheadedness, and chest pain. About 1% of people worldwide have pulmonary hypertension, and it can affect adults of all ages. [2,1] It is most common in adults with other medical conditions, like mitral valve or aortic valve disease, scleroderma, or sickle cell disease. [2]

Since pulmonary hypertension causes your heart to work harder to pump blood, it becomes dangerous, and even fatal, if not treated. Problems caused by pulmonary hypertension include:

  • Blood clots in the lungs’ arteries
  • Anemia: the body does not have enough red blood cells to carry oxygen to the rest of the body [3]
  • Abnormal heart rhythms
  • Pericardial effusion: too much fluid in the sac around the heart [4]

What causes pulmonary hypertension?

Multiple things can cause PH. This condition is divided into five groups based on these causes. [1,5]

Group 1

Group 1 is pulmonary arterial hypertension (PAH). This happens when the arteries in the lungs become small, thick, and stiff, making it harder for blood to get through. When this happens, blood pressure in these arteries gets too high. PAH has multiple causes, including some drugs and other health conditions.

Group 2

Group 2 pulmonary hypertension is caused by left-sided heart disease. The left side of the heart pumps blood to the rest of the body. Left-sided heart disease affects the arteries and veins that connect the heart and lungs. It causes a back-up of blood, which collects in the heart and raises blood pressure.

Group 3

Group 3 is pulmonary hypertension caused by lung disease or low oxygen in the body’s tissues (hypoxia). These problems can cause your lung arteries to become thick and narrow, making the pressure in the blood too high.

Group 4

Group 4 pulmonary hypertension is caused by blood clots in the lungs. Blood clots can cause scarring in the lung vessels, which slows down blood flow and raises blood pressure. [6]

Group 5

Group 5 is pulmonary hypertension from unknown causes or caused by other medical conditions. Some medical conditions that may cause pulmonary hypertension include sickle cell anemia, spleen removal (splenectomy), and certain metabolic diseases.

What are the symptoms of pulmonary hypertension?

People with PH may not have any symptoms at first. Usually, the first symptom noticed is feeling short of breath, even while resting. Symptoms develop and worsen over time. [1,7] Other symptoms include:

  • Shortness of breath
  • Grey or blue skin color
  • Chest pressure or pain
  • Pounding or racing heartbeat
  • Dizziness or lightheadedness
  • Ankle, leg, or tummy swelling
  • Fatigue
  • Feeling less hungry

How is pulmonary hypertension diagnosed?

First, your doctor will perform a medical exam to look for signs and symptoms of pulmonary hypertension. Then, your doctor will determine if you need certain tests to measure the blood pressure in your lung vessels. Your doctor may also recommend you see a heart or lung doctor (cardiologist or pulmonologist).

Tests that measure blood pressure in the lung vessels include an echocardiogram and right heart catheterization. [1]

  • An echocardiogram (echo) is a simple test that uses ultrasound to estimate blood pressure in the arteries of the lungs, measure the heart’s size, and see blood flowing through the heart.
  • During a right heart catheterization, a doctor inserts a flexible tube (catheter) into a vessel in the groin, arm, or wrist. Then, the doctor guides the tube to the pulmonary artery and measures the blood pressure.

If you have pulmonary hypertension, your doctor may order tests to find out why. These tests can include blood tests, CT scans, and chest X-rays.

How is pulmonary hypertension treated?

Your doctor will choose a treatment plan based on your type (group) of PH. Some types require treating the medical condition that’s causing pulmonary hypertension. Treatment may also include lifestyle changes and medications, including those that lower blood pressure, diuretics (“water pills”) to remove extra fluid, oxygen, and medicine to relax your arteries and lower blood pressure. In some cases, your doctor may recommend procedures or surgery. [1]

Looking Ahead: Life with Pulmonary Hypertension

Scientists have not yet discovered a cure for pulmonary hypertension. For some people, symptoms can get worse over time. However, there are treatment options that can help you feel better and manage PH. [7] Learn more and ask your doctor about your options and a treatment plan tailored to you. You can make decisions and work together to treat and manage your pulmonary hypertension.

References:

  1. Clinic C. Pulmonary Hypertension: Symptoms, Treatment. Cleveland Clinic. Published 2022. Accessed August 12, 2024. https://my.clevelandclinic.org/health/diseases/6530-pulmonary-hypertension-ph
  2. What Is Pulmonary Hypertension? NHLBI, NIH. Published May 2023. Accessed August 12, 2024. https://www.nhlbi.nih.gov/health/pulmonary-hypertension
  3. Clinic C. Anemia: Symptoms, Causes & Treatment. Cleveland Clinic. Published 2024. Accessed August 12, 2024. https://my.clevelandclinic.org/health/diseases/3929-anemia
  4. Clinic C. Pericardial Effusion: Symptoms & Causes. Cleveland Clinic. Published 2024. Accessed August 12, 2024. https://my.clevelandclinic.org/health/diseases/17351-pericardial-effusion
  5. Prins KW, Thenappan Thenappan. World Health Organization Group I Pulmonary Hypertension. Cardiology Clinics. 2016;34(3):363-374. doi:https://doi.org/10.1016/j.ccl.2016.04.001
  6. PHA A. Types of Pulmonary Hypertension: The WHO Groups. Pulmonary Hypertension Association. Published December 13, 2023. Accessed August 12, 2024. https://phassociation.org/types-pulmonary-hypertension-groups/
  7. Pulmonary hypertension – Symptoms and causes. Mayo Clinic. Published 2023. Accessed August 12, 2024. https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes/syc-20350697