What is Pulmonary Arterial Hypertension?

Pulmonary Arterial Hypertension (PAH) is a disease affecting the pulmonary arteries, the arteries that carry blood from the heart to the lungs. The right ventricle of the heart pumps blood into the lungs to gather oxygen for delivery to the rest of the body. In this condition, the blood pressure within those arteries is too high. With the difficulty pushing through the pulmonary arteries, the right ventricle of the heart strains under the pressure.

PAH is a life-threatening condition affecting 1 in 100,000 to 1 in 1,000,000. There are three forms of PAH. Familial PAH is linked to a genetic defect that is inherited. Idiopathic PAH is PAH with an unknown cause. Associated PAH is the most common and is connected to a specific underlying cause such as HIV, stimulant drug use, thyroid disorders, congenital lung disease or heart disease, collagen vascular disease and others.

The basic explanation for PAH is an issue with the pulmonary arteries themselves. Typically, due to any number of related factors, these arteries thicken, tighten, or are clogged with blood clots. Sometimes the arteries tighten due to muscle contractions. The arteries may be thickening with overgrowth of the cells and blood clots similarly narrow the passageway.

Because the heart must work extra hard to get blood through the pulmonary arteries for oxygenating the body organs and tissues, it begins to weaken over time. This begins to reduce oxygen levels. In some cases, heart failure manifests itself through swelling of the heart. This can cause fatigue, fainting, shortness of breath, chest pain, and dizziness.

PAH is more common among women than men. In newborns, it is called persistent pulmonary hypertension of the newborn (PPHN). It is more prolific among those with family histories of pulmonary hypertension or sudden death. And for pregnant women who have it, it can cause death during childbirth. PAH is a very serious condition, taking the lives of half of those diagnosed within five years of diagnosis. Those who are untreated tend to live only three years on average. Treatments can slow the progression of the disease and ensure a higher quality of life for patients with PAH.

Symptoms and Treatments for Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) generally results in the heart weakening from the strain of pumping blood through narrowed pulmonary arteries, resulting in reduced oxygen levels. Fatigue is one of the earlier signs, along with dizziness and chest pain. The symptoms can progress to leg and ankle swelling, shortness of breath during physical exertion, lightheadedness when exercising, weakness, and even fainting spells.

Because these symptoms are similar to other conditions, PAH is often misdiagnosed as diseases like left heart failure, pneumonia, chronic obstructive pulmonary disease (COPD), asthma, or coronary disease. It is important to get a valid diagnosis for this serious condition as it drastically shortens life span and requires treatment in order to prolong and improve the life of the patient.

The physician will diagnose the level of severity of PAH based on a class system. Class I means that the patient is in the earlier stages, with no resting symptoms and only milder symptoms upon physical exertion. Class II describes a patient who still has no resting symptoms, but activities are mildly limited by the symptoms that occur with exertion.

Class III involves marked limitations on activity levels of the patient with even low-stress activities bringing on symptoms. However, the patient is still comfortable when resting. In Class IV, the patient has symptoms even at rest and is unable to exert himself or herself for any physical activity.

Treatments of PAH may include medications like endothelin receptor antagonists to reduce the levels of endetholin in the circulatory system, which impacts blood flow and cell growth in the blood vessels. Another treatment method is oxygen therapy to help relieve shortness of breath. Calcium channel blockers help open the pulmonary arteries. Anticoagulants prevent blood clots. Diuretics reduce the volume of blood the heart must pump. Other drugs are also prescribed depending on the patient’s manifestation of the condition.

Surgical procedures, lifestyle changes, and other options may be recommended. The primary goals of treatment are to reduce strain on the heart, improve circulation, and maintain proper oxygen levels in the patient. These treatments slow the progression of the disease, allowing the patient to enjoy a longer and more fulfilling life.

Information on this page is for reference and educational purposes only. For more information about pulmonary arterial hypertension (PAH), talk to your doctor or primary care provider.

About Scott Ridl: Scott joined American Medical Sales and Rentals in 2008 as a Web Manager and Content Writer. He is a writer and designer. He is extensively trained on oxygen therapy products from leading manufacturers such as Inogen, Respironics, Chart, Invacare, ResMed and more. Scott works closely with respiratory therapists and oxygen specialists to educate the community about oxygen therapy products, COPD, asthma and lung diseases. He writes weekly columns and is passionate about educating the community on oxygen therapy and respiratory issues.

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  • [...] pressure to get dangerously high, causing stress on the heart and other complications including pulmonary arterial hypertension.  It often progresses gradually, causing small blood vessels in the lungs to become diseased until [...]


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