What is primary arterial hypertension?

Pulmonary arterial hypertension (PAH), formerly known as primary pulmonary hypertension, is a rare type of high blood pressure. PAH affects the pulmonary arteries and capillaries. These blood vessels carry blood from the lower right chamber of your heart (right ventricle) into your lungs. As the pressure in the pulmonary blood vessels builds up, the heart must work harder to pump blood to the lungs. Over time, this weakens the heart muscle. Eventually, it can lead to heart failure and death. There’s no cure yet for PAH, but treatment options are available. Treatment may help relieve your symptoms, lower your chance of complications, and prolong your life.

Symptoms of pulmonary arterial hypertension

In the early stages of PAH, you might not have any noticeable symptoms. As the condition worsens, symptoms will become more noticeable. Common symptoms include:

• difficulty breathing
• fatigue
• dizziness
• fainting
• chest pressure
• chest pain
• rapid pulse
• heart palpitations
• bluish tint to your lips or skin
• swelling of your ankles or legs
• swelling with fluid inside your abdomen, particularly in the later stages of PAH

Causes of pulmonary arterial hypertension

PAH develops when the pulmonary arteries and capillaries that carry blood from your heart to your lungs become constricted or destroyed. Various conditions may trigger it, but the exact cause is unknown. Around 20 percent of PAH cases are inherited. It involves genetic mutations that can occur in the BMPR2 gene or other genes. The mutations can then be passed down through families, allowing the person with one of these mutations to have the potential to later develop PAH. Other potential conditions that can be associated with developing PAH include:

• chronic liver disease
• congenital heart disease
• certain connective tissue disorders
• certain infections, such as HIV or schistosomiasis
• sickle cell anemia

Diagnosis of pulmonary arterial hypertension

If your doctor suspects you might have PAH, they will likely order one or more tests to assess your pulmonary arteries and heart. Tests for diagnosing PAH may include:

• electrocardiogram to check for signs of strain or abnormal rhythms in your heart
• echocardiogram to examine the structure and function of your heart and measure pulmonary artery pressure
• chest X-ray to learn if your pulmonary arteries or the lower right chamber of your heart are enlarged
• CT scan to look for blood clots, narrowing, or damage in your pulmonary arteries
• right heart catheterization to measure the blood pressure in your pulmonary arteries and right ventricle of your heart
• pulmonary function test to assess the capacity and flow of air into and out of your lungs
• blood tests to check for substances associated with PAH or other health conditions

Stages of pulmonary arterial hypertension

PAH is divided into four stages based on the severity of symptoms. These stages are based on criteria established by the World Health Organization (WHO) Class 1. The condition doesn’t limit your physical activity. You don’t experience any noticeable symptoms during periods of ordinary physical activity or rest. Class 2. The condition slightly limits your physical activity. You experience noticeable symptoms during periods of ordinary physical activity, but not during periods of rest. Class 3. The condition significantly limits your physical activity. You experience symptoms during periods of slight physical exertion and ordinary physical activity, but not during periods of rest. Class 4. You’re unable to carry out any type of physical activity without symptoms. You experience noticeable symptoms, even during periods of rest. Signs of right-sided heart failure tend to occur in this stage.

What is the survival rate for pulmonary hypertension?

Background Primary pulmonary hypertension (PPH) is a severe and progressive disease. Without treatment, the median survival is 2.8 years, with survival rates of 68%, 48%, and 34% at 1, 3, and 5 years, respectively. Intravenous epoprostenol was the first Food and Drug Administration approved therapy for PPH.