Pulmonary arterial hypertension

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at April 18, 2016
StartDiseasesPulmonary arterial hypertension

Pulmonary arterial hypertension, sometimes referred to as PAH is a serious and life-threatening disease in which the patient experiences increased blood pressure in the arteries traveling from the heart to the lungs. Though very serious, with proper care and treatments patients can manage their symptoms and live well with PAH.


Definition & Facts

PAH is different from high blood pressure and is defined by increased pressure inside the patient’s lung vasculature, or pulmonary arteries and veins. This causes a decreased amount of oxygenated blood traveling to the rest of the body and forces the heart to work harder when pumping blood. This increased strain can eventually lead to heart failure and even death.

Symptoms & Complaints

There are many symptoms of pulmonary arterial hypertension, making it hard to distinguish from other conditions. Should one experience any of the symptoms listed below for an extended period of time, they should consult their doctor immediately. Oftentimes when patients are experiencing PAH, they will report feeling shortness of breath, increased heart rate, chest pain, sharp pains on the right side of their abdomen, and fainting.

They may also less commonly experience swelling in the legs and ankles, chronic lightheadedness, decreased appetite, and blue or purple coloration near the surface of the skin. Though these symptoms are often caused by many other diseases, it is important that the patient gets diagnosed and treated right away as PAH can be potentially life-threatening.


In a patient with pulmonary arterial hypertension, it becomes harder for the blood to flow through the veins, capillaries, or arteries in the lungs. This can be caused by stiffening or thickening of the vessels, or extra tissue forming on the sides of the vessels. These effects originate from many events or activities, and can be caused by genetic factors.

The patient is at risk if he or she has experienced congenital heart failure, blood clots, HIV, liver disease, lung disease, certain autoimmune diseases, sleep apnea, or if there is a family history of cardiovascular disease. Other activities can contribute to the development of PAH, such as smoking cigarettes or using illegal drugs like cocaine and methamphetamine.

Diagnosis & Tests

PAH can be difficult to diagnose because many of its symptoms are similar to those of other diseases. Though often diagnosed incorrectly or late into its development as it is hard to catch in a normal routine checkup, there are many ways for doctors to determine if a patient is suffering from PAH. Health care providers will test for certain risk factors such as being overweight or experiencing high blood pressure. They will also inquire about other risk factors like family history of heart disease, drug use, the use of certain appetite suppressant medications, or living at high altitudes.

Doctors may also use an echocardiogram by sending sound waves to the heart to create an image of its size, rhythm of beating, and to see the pressure in a patient’s pulmonary arteries. This may also involve a stress echocardiography, so that the doctor can see how the heart performs under stress. A chest X-ray might also be used to provide insight into the condition of a patient’s heart and arteries. If the doctor believes that the patient has PAH, they may place a catheter into a vein in the patient’s neck or groin and feed it into the right ventricle or pulmonary artery. This tests blood pressure.

Treatment & Therapy

Though it cannot be cured outright, proper treatment can ease the symptoms of pulmonary arterial hypertension and slow the progression of the condition. Many treatments may need to be tried over a long period of time, as the proper solution tends to vary across patients. It is critical that all patients with PAH adopt a healthy diet and cease smoking or drug use if applicable.

In addition, doctors may prescribe certain medications to reduce the intensity of symptoms. These medications include intravenous or inhaled blood vessel dilators to increase blood flow to the lungs, endothelin receptor antagonists to reduce shrinking of blood vessels, high-dose calcium channel blockers to relax the muscles in the walls of blood vessels, anticoagulants to thin blood and prevent clots, diuretics to reduce the amount of fluid the heart must pump, and oxygen therapy.

In more extreme or developed cases, surgery may also be used to reduce or eliminate the effects of PAH. Generally these surgeries include either a heart transplant or lung transplant, or an atrial septostomy in an attempt to relieve blood pressure.

Prevention & Prophylaxis

PAH can be preventable for many people with small lifestyle changes. Regular exercise and eating healthy foods like fruits and vegetables can reduce the risk of clots and constriction of blood vessels.

Finally, refraining from smoking and using illegal drugs is incredibly important in maintaining a healthy body and heart. These substances negatively affect a multitude of factors in maintaining proper functioning of the body’s systems, and cutting them out can greatly reduce the risk of PAH.