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Pulmonary hypertension

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Pulmonary hypertension is abnormally high blood pressure in the arteries of the lungs. It makes the right side of the heart work harder than normal.

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Causes
The right side of the heart pumps blood through the lungs, where it picks up oxygen. Blood returns to the left side of the heart, where it is pumped to the rest of the body.

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, this causes the right side of the heart to become larger. This condition is called right-sided heart failure, or cor pulmonale.

Pulmonary hypertension may be caused by: In many patients, the cause of pulmonary hypertension is unknown. In this case, the condition is called idiopathic pulmonary arterial hypertension (IPAH). Idiopathic means the cause of a disease is not known. IPAH is rare. It affects more women than men.

If pulmonary hypertension is caused by a known medicine or medical condition, it is called secondary pulmonary hypertension.

Symptoms
Shortness of breath or light-headedness during activity is often the first symptom. Fast heart rate (palpitations) may be present. Over time, symptoms occur with lighter activity or even while at rest.

Other symptoms include: People with pulmonary hypertension often have symptoms that come and go. They report good days and bad days.

Exams and Tests
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 and other diseases may cause similar symptoms and must be ruled out.

Tests may include:
Treatment
There is no cure for pulmonary hypertension. The goal of treatment is to control symptoms and prevent more lung damage. It is important to treat medical disorders that cause pulmonary hypertension, such as obstructive sleep apnea, lung conditions, and heart valve problems.

Many treatment options for pulmonary arterial hypertension are available. If you are prescribed medicines, they may be taken by mouth (oral), received through the vein (intravenous, or IV), or breathed in (inhaled). 

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 are responding to the medicine. Do not stop taking your medicines without talking to your doctor.

Other treatments may include: Other important tips to follow:
Outlook (Prognosis)
How well you do depends on what caused the condition.

As the illness gets worse, you will need to make changes in your home to help you get around the house.

When to Contact a Medical Professional
Call your health care provider if:
Alternative Names
Pulmonary arterial hypertension; Sporadic primary pulmonary hypertension; Familial primary pulmonary hypertension; Idiopathic pulmonary arterial hypertension; Primary pulmonary hypertension; PPH; Secondary pulmonary hypertension

References
Channick RN, Rubin LJ. Pulmonary hypertension. In: Mason RJ, Broaddus VC, Martin TR, et al., eds. Murray and Nadel's Textbook of Respiratory Medicine . 5th ed. Philadelphia, PA: Elsevier Saunders; 2010:chap 52.

McLaughlin VV, Archer SL, Badesch DB, et al: American College of Cardiology Foundation Task Force on Expert Consensus Documents; American Heart Association; American College of Chest Physicians; American Thoracic Society, Inc; Pulmonary Hypertension Association. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians: American Thoracic Society, Inc; and the Pulmonary Hypertension Association. J Am Coll Cardiol . 2009;53:1573-1619.

Rich S. Pulmonary hypertension. In: Bonow ROL, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine . 9th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 78.

Update Date: 4/26/2014
Denis Hadjiliadis, MD, Associate Professor of Medicine, Pulmonary, Allergy and Critical Care, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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