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. When the pulmonary hypertension is brought on my another condition, then treatment is usually directed at that underlying disease.
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 how well you're responding to the medication.
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, changes in the home environment and more help around the home will be needed.
Expectations (prognosis)
The 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.
Complications
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.
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