Pulmonary Hypertension

Pulmonary Hypertension (PAH) is a disease in which blood pressure is constantly higher than normal in the blood vessels that connect the heart and lungs. These arteries tighten and constrict blood flow, causing the heart to work harder to pump enough blood to the lungs.

PAH is a progressive disease – it worsens over time and can be fatal. As less and less blood is able to flow through the arteries, symptoms will get worse. Although there is no cure for pulmonary hypertension, certain treatments can help ease the symptoms and prevent more lung damage.

HOW IS PULMONARY HYPERTENSION DIAGNOSED?

The following tests may be used to diagnose PAH:

• Echocardiogram: estimates the pressures in the lung and determines relevant cardiac aspects of pulmonary hypertension;

• Blood test;

• Chest x-ray;

• CT or MRI scans;

• Right heart catheterization: if an echocardiogram suggests a patient has pulmonary hypertension, the doctor will then perform a right heart catheterization to directly measure the pressure inside the lung and heart’s arteries using a thin tube inserted through the groin or neck;

• Lung function test;

• Perfusion lung scan: small amounts of radioactive substances are injected into the blood stream and pictures are taken by a special gamma camera so the doctor can study the blood flow through the lung and heart arteries.

After diagnosis, the doctor will classify each patient in one of the following classifications:

• Class I (no symptoms are showing)

• Class II (symptoms occur during physical activity)

• Class III (symptoms occur during all waking hours)

• Class IV (symptoms occur even during rest)

WHAT ARE MY TREATMENT OPTIONS?

Pulmonary hypertension cannot be cured. But, several medications and good lifestyle habits can help slow the progression of the disease and ease day-to-day symptoms.

Medications can be prescribed as pills, intravenous (IV) or subcutaneous infusions, or nebulized medications (inhaled through a breathing machine). Some of the available medications include:

• Blood vessel dilators: open constricted blood vessels and slow the growth of abnormal cells in the blood vessels of the lungs;

• Prostaglandins: medications that are either pumped into the body with a small device or inhaled into the lung via an nebulizer;

• Endothelin receptor antagonists: medications to stop the endothelin substance from constricting vessel walls;

• Phosphodiesterase inhibitors or Guanylate cyclase stimulators: medications that enhance nitric oxide’s ability to dilate blood vessels;

• Anticoagulants: to help prevent blood clots;

• Oxygen therapy: breathing supplemental oxygen can help certain patients.

Lung transplantation is an option for some cases in young patients.

Developing good lifestyle habits is crucial to help combat the progression of pulmonary hypertension.

• Exercise regularly and be active every day: exercising the lungs is essential to maintaining healthy tissue and encouraging healthy blood flow. Pure oxygen may be needed if exercise induces symptoms or is too exhausting for patients with Class III or Class IV PAH;

• Do not smoke: smoking cessation is unavoidable for patients with PAH. Second hand smoke should also be avoided;

• Do not become pregnant or use birth control pills: women should not become pregnant or take oral contraceptive pills that can cause blood clots;

• Eat a low-salt diet: salt can cause tissue to swell and blood pressure to increase.