As the pressure builds, your heart's lower right chamber (right ventricle) must work harder to pump blood through your lungs, and eventually fail.

► Symptoms

Common symptoms are shortness of breath, fatigue, malaise, decrease in exercise capacity, non-productive cough, chest pain, fainting or syncope, swelling around the ankles and feet, and rarely coughing up blood. Because symptoms may develop very gradually, patients may be initially misdiagnosed or delay seeing a physician for years.

► Causes

Some of the conditions that can lead to pulmonary hypertension are:

• Heart Disease
• COPD / Emphysema
• Sleep Apnea
• Pulmonary embolism (Blood clots)
• Scleroderma
• Liver Disease
• Diet pills
• Cocaine / Amphetamine use
• HIV / AIDS
• Sickle Cell Disease

► Diagnosis

The diagnosis of PAH requires an objective assessment of the pulmonary arterial pressures. In order to establish a definite diagnosis of PAH, a mean pulmonary artery pressure (mPAP) of more than 25 mm Hg must be measured. Normal pulmonary arterial pressure in a person living at sea level has a mean value of 12–16 mm Hg.

Mean pulmonary artery pressure (mPAP) should not be confused with systolic pulmonary artery pressure (sPAP), which is often reported on echocardiogram reports. A systolic pressure of 40 mm Hg typically implies a mean pressure of more than 25 mm Hg.

Although pulmonary arterial pressure can be estimated on the basis of echocardiography, pressure measurements with a Swan-Ganz catheter (via a heart catheterization) provides the most definite assessment.

Therefore diagnosis of PAH requires right-sided cardiac catheterization.

► Treatment

Multiple treatment options are available. These include pills, inhalers and infusions. Your doctor may use one or more of these in combination after carefully reviewing your case.

Treatment is individualized for each patient and it often takes some time to find the best treatment for you. The treatments are often complex and require extensive follow-up care.

Your doctor may also need to change your treatment if it's no longer effective. When pulmonary hypertension is caused by another condition, your doctor will treat the underlying cause whenever possible.