Definition:
Pulmonary hypertension is abnormally high blood pressure in the arteries of the lungs.
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:
Pulmonary hypertension is usually caused by a narrowing of the small arteries of the lung, 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 become enlarged over time. Eventually, heart failure may develop.
Pumonary hypertension may be caused by:
- A genetic defect
- Certain diet medications
- History of a blood clot in the lung
- Lung or heart valve disease
- Obstructive sleep apnea
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 medical condition, it is called secondary pulmonary hypertension.
Primary pulmonary hypertension is rare. It affects more women than men.
Symptoms:
Signs and tests:
A physical examination may show:
- 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 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:
Treatment:
There is no known cure. The goal of treatment is to control symptoms.
Medicines used to treat pulmonary hypertension include:
- Ambrisentan (Letairis)
- Bosentan (Tracleer)
- Calcium channel blockers
- Diuretics
- Prostacyclin
- Sildenafil
Your doctor will decide which medicine is best for you.
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 hypertension may need oxygen. If treatment with medications fails, suitable candidates may be helped by a lung or heart-lung transplant.
Expectations (prognosis):
The outlook has been poor, but new therapies may produce better results. Some people with this condition may develop heart failure that worsens and may lead to death.
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 primary pulmonary hypertension are treated at centers that specialize in the care of these patients.
References:
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.
Hayes D Jr. Idiopathic pulmonary arterial hypertension misdiagnosed as asthma. J Asthma. 2007 Jan-Feb;44(1):19-22.
Barst RJ. Pulmonary hypertension. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 67.
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Review Date:11/12/2008
Reviewed By:David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Benjamin Medoff, MD, Assistant Professor of Medicne, Harvard Medical School, Pulmonary and Critical Care Unit, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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