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What is World Pulmonary Hypertension Day?

World Pulmonary Hypertension Day is an annual global event to raise awareness of pulmonary hypertension (PH), a severe condition affecting the lungs and heart. On May 5, more than 80 organizations around the world participate in World PH Day activities to raise awareness of this frequently misdiagnosed disease and celebrate the lives of people living with PH.

What is pulmonary hypertension? 

Pulmonary hypertension is a condition in which there is high blood pressure in the lung arteries. How the disease starts is not always clear, but the arteries become narrow and there is less room for the blood to flow.

Over time, some of the arteries may stiffen and become completely blocked. The narrowing of the pulmonary arteries causes resistance to the blood flow, increasing the blood pressure. The right side of the heart has to work harder to pump blood through the arteries. This places an increasing strain on the heart, which causes it to enlarge and can lead to right heart failure.

Right heart failure is one of the most common causes of death in people who have pulmonary hypertension.

Symptoms

Difficulty in breathing or shortness of breath is the main early symptom of pulmonary hypertension. Other symptoms are fatigue, dizziness, swelling in the ankles or legs (edema), bluish lips and skin (cyanosis), chest pain, racing pulse and palpitations.

Classification 

  • Group 1 - Pulmonary arterial hypertension (PAH)
  • Group 2 - Pulmonary hypertension due to left-sided heart disease
  • Group 3 - Pulmonary hypertension due to lung diseases and/or hypoxia
  • Group 4 - Chronic thromboembolic pulmonary hypertension (CTEPH)
  • Group 5 – Pulmonary hypertension with unclear and/or multifactorial mechanisms

Specifics of pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a rare type of pulmonary hypertension characterized by high blood pressure (hypertension) in the arteries of the lungs (pulmonary artery). PAH can be heritable, induced by drugs and toxins or associated with underlying diseases but also without apparent reason (“idiopathic”). The pulmonary arteries are the blood vessels that carry blood from the right side of the heart through the lungs.

  • Symptoms of PAH include shortness of breath (dyspnea) especially during exercise, chest pain, and fainting episodes.
  • The exact cause of PAH is unknown and although treatable, there is no known cure for the disease.
  • PAH predominantly affects women between the ages of 30-60. Individuals with PAH may go for years without a diagnosis, either because their symptoms are mild, nonspecific, or only present during demanding exercise. However, it is important to treat PAH early because without treatment, high blood pressure in the lungs causes the right heart to work much harder, and over time, this heart muscle may fail.
  • The progressive nature of this disease means that an individual may experience only mild symptoms at first but will eventually require treatment and medical care to maintain a reasonable quality of life.
  • PAH has many potential causes. These include other diseases as well as treatments that can lead to the narrowing of pulmonary arteries. Examples include:
    - Heart abnormalities, such as congenital heart defects and Eisenmenger Syndrom
    - Viral infections, such as HIV
    - Connective tissue disorders, such as scleroderma and lupus 
    - Certain medicines, such as methamphetamines
    - Chronic liver disease, or cirrhosis
    - Glycogen storage disorders or von Gierke disease.

Approximately 15 - 20 percent of PAH patients have familial or heritable PAH, which is caused by genetic mutations that are passed down through the family.

Pulmonary hypertension and COVID-19 

There is no evidence to suggest that people with underlying health conditions are at higher risk of becoming infected with the COVID-19 virus. However, there is evidence that people with underlying health conditions, including all types of heart and lung diseases, have a higher risk of developing serious illness from the COVID-19 virus if they become infected.

Click here to learn more about safety precautions that PH patient should take!