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Pulmonary hypertension - Treatment

Alternative Names

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

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.

Respiratory system

Primary pulmonary hypertension

Reviewed last on: 11/12/2008
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.

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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