Pulmonary hypertension is abnormally high blood pressure in the arteries of the lungs.

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

Pulmonary hypertension occurs when the small arteries of the lung become narrow, which makes it hard for blood to flow. Blood pressure increases. The right side of the heart must work harder to pump blood, and may eventually become enlarged. Eventually, heart failure may develop.

Pumonary hypertension may be caused by a genetic defect, certain diet medications, or medical conditions such as lung or heart valve disease or history of a blood clot in the lung. In some cases, the cause is unknown.

If it occurs without a known cause, it is called primary pulmonary hypertension.

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

Pulmonary hypertension is rare. It affects more women than men.

• Shortness of breath during activity
• Chest pain
• Weakness
• Fatigue
• Fainting
• Light-headedness during exercise
• Dizziness
• Leg swelling

A physical examination may show:

• Enlargement of the veins in the neck
• Normal breathing sounds
• Heart murmur
• Feeling of a pulse over the breastbone
• Liver and spleen swelling
• Leg swelling

In early stages of the disease, the exam may be normal or almost normal. The condition may take several months to diagnose. Asthma causes similiar symptoms and must be ruled out.

Tests may include:

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

There is no known cure. The goal of treatment is to control symptoms.

Medicines used to treat pulmonary hypertension include:

• Ambrisentan (Letairis)
• Bosentan
• Calcium channel blockers
• Diuretics
• Prostacyclin

Your doctor will decide which medicine is best for you.

Persons with advanced cases of pulmonary hypertension may need oxygen. If treatment with medications fails, suitable candidates may be helped by lung or heart-lung transplant.

The outlook has traditionally been poor, but new therapies may produce better results. Some people with this condition may develop progressive heart failure leading to death.

Call your health care provider if:

• You begin to develop shortness of breath when you are active
• Shortness of breath worsens
• Chest pain develops
• Other symptoms develop

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

McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004 Jul;126(1 Suppl):14S-34S.

Sahara M, Takahashi T, Imai Y, et al. New insights in the treatment strategy for pulmonary arterial hypertension. Cardiovasc Drugs Ther. 2006 Oct;20(5):377-86.

Hayes D Jr. Idiopathic pulmonary arterial hypertension misdiagnosed as asthma. J Asthma. 2007 Jan-Feb;44(1):19-22.

Austin ED, Loyd JE. Genetics and mediators in pulmonary arterial hypertension. Clin Chest Med. 2007 Mar;28(1):43-57.