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Contents of this page: | |
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Alternative Names Return to top
Pulmonary arterial hypertension; Sporadic primary pulmonary hypertension; Familial primary pulmonary hypertension; Idiopathic pulmonary arterial hypertension; Primary pulmonary hypertension; PPH; Secondary pulmonary hypertensionDefinition Return to top
Pulmonary hypertension is abnormally high blood pressure in the arteries of the lungs.
Causes Return to top
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:
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 Return to top
Exams and Tests Return to top
A physical examination may show:
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 Return to top
There is no known cure. The goal of treatment is to control symptoms.
Medicines used to treat pulmonary hypertension include:
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.
Outlook (Prognosis) Return to top
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.
When to Contact a Medical Professional Return to top
Call your health care provider if:
Most patients with primary pulmonary hypertension are treated at centers that specialize in the care of these patients.
References Return to top
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.
Update Date: 11/12/2008 Updated 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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Page last updated: 04 May 2009 |