Pulmonary hypertension

Respiratory system

Primary pulmonary hypertension

Definition

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.

Alternative Names

Pulmonary arterial hypertension; Sporadic primary pulmonary hypertension; Familial primary pulmonary hypertension; Idiopathic pulmonary arterial hypertension; Primary pulmonary hypertension; PPH; Secondary pulmonary hypertension

Causes, incidence, and risk factors

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:

• Any condition that causes chronic low oxygen levels in the blood
• Autoimmune diseases that damage the lungs, such as scleroderma and rheumatoid arthritis
• Certain birth defects of the heart
• Certain diet medications
• Congestive heart failure
• History of a blood clot in the lung
• HIV infection
• Lung or heart valve disease
• Obstructive sleep apnea

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.

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:

• Ankle and leg swelling
• Bluish color of the lips or skin (cyanosis)
• Chest pain or pressure, usually in the front of the chest
• Dizziness or fainting spells
• Fatigue
• Weakness

People with pulmonary hypertension report good days and bad days.

Signs and tests

A physical examination may show:

• Abnormal heart sounds (especially a splitting of the second heart sound)
• Enlargement of the veins in the neck
• Feeling of a pulse over the breastbone
• Heart murmur
• Leg swelling
• Liver and spleen swelling
• Normal breathing sounds

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:

• Cardiac catheterization
• Chest x-ray
• CT scan of the chest
• Echocardiogram
• ECG
• Nuclear lung scan
• Pulmonary arteriogram
• Pulmonary function tests
• Sleep study

Treatment

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:

• Ambrisentan (Letairis)
• Bosentan (Tracleer)
• Calcium channel blockers
• Diuretics
• Prostacyclin or similar medications
• Sildenafil

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:

• Avoid pregnancy
• Avoid strenuous activities and heavy lifting
• Avoid traveling to high altitudes
• Keep up to date with yearly flu and pneumococcal pneumonia vaccines
• Stop smoking

Expectations (prognosis)

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.

Calling your health care provider

Call your health care provider if:

• You begin to develop shortness of breath when you are active
• Shortness of breath worsens
• You develop chest pain
• You develop other symptoms

Most patients with pulmonary arterial hypertension are treated at centers that specialize in the care of these patients.

References

Badesch DB, Abman SH, Simonneau G, et al. Medical Therapy for Pulmonary Arterial Hypertension Updated ACCP Evidence-Based Clinical Practice Guidelines. Chest. 2007: 131(6).

McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, 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.

Read More

• Blood pressure
• Heart failure

Review Date: 4/27/2010
Reviewed By: Allen J. Blaivas, DO, Clinical Assistant Professor of Medicine UMDNJ-NJMS, Attending Physician in the Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Veterans Affairs, VA New Jersey Health Care System, East Orange, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.