Pulmonary hypertension is abnormally high blood pressure in the arteries of the lungs. It makes the right side of the heart need to work harder than normal.
The right side of the heart pumps blood through the lungs, where it can receive oxygen.
When the small arteries (blood vessels) of the lung become narrowed, they cannot carry as much blood. When this happens, pressure builds up. This is called pulmonary hypertension.
The heart needs to work harder to force the blood through the vessels against this pressure. Over time, the right side of the heart may become enlarged. At some point, not enough blood flows to the lungs to pick up oxygen and symptoms begin.
At this point, heart failure that involves the right side of the heart is present. This is called cor pulmonale.
Pumonary hypertension may be caused by:
In many cases the cause is unknown, in which case the condition is known as idiopathic pulmonary arterial hypertension (IPAH). IPAH is rare. It affects more women than men.
If it is caused by a known medicine or medical condition, it is called secondary pulmonary hypertension.
Shortness of breath or light-headedness during activity is often the first symptom.
Other symptoms include:
People with pulmonary hypertension report good days and bad days.
A physical examination may show:
In the early stages of the disease, the exam may be normal or almost normal. The condition may take several months to diagnose. Asthma causes similar symptoms and must be ruled out.
Tests may include:
There is no known cure. The goal of treatment is to control symptoms. It is important to treat medical disorders that cause pulmonary hypertension, such as obstructive sleep apnea, lung conditions, and heart valve disorders.
Many new treatment options for idiopathic pulmonary arterial hypertension (IPAH) and other forms of pulmonary aterial hypertension are becoming available. Medicines used to treat pulmonary hypertension include:
Your doctor will decide which medicine is best for you. You will be closely monitored during treatment to watch for side effects and to see how well you're responding to the medication. Never stop taking medicines without talking to your doctor.
Some patients are put on blood thinners to reduce the risk of blood clots in leg veins and lung arteries.
People with advanced cases of pulmonary arterial hypertension may need oxygen. If treatment with medications fails, suitable candidates may be helped by a lung or heart-lung transplant.
As the illness progresses, you will need to make changes in the home environment and get more help around the home.
Other important tips to follow:
The long-term outlook has been poor, but new therapies may produce better results. Some people with this condition may develop progressive heart failure that may lead to death.
Avoiding pregnancy is recommended.
Call your health care provider if:
Most patients with pulmonary arterial hypertension are treated at centers that specialize in the care of these patients.
Pulmonary arterial hypertension; Sporadic primary pulmonary hypertension; Familial primary pulmonary hypertension; Idiopathic pulmonary arterial hypertension; Primary pulmonary hypertension; PPH; Secondary pulmonary hypertension