Pulmonary Hypertension

The Singing River Health System Regional Pulmonary Hypertension Center, located in Ocean Springs, is the only pulmonary hypertension center on the Mississippi Gulf Coast.

About Pulmonary Hypertension

Pulmonary hypertension is an uncommon type of high blood pressure that affects only the arteries in the lungs and the right side of the heart. Patients will typically see many physicians before they are correctly diagnosed with pulmonary hypertension because it can appear to be any number of conditions. It is a serious illness that becomes progressively worse if left untreated.

Symptoms of Pulmonary Hypertension

The symptoms for all types of pulmonary hypertension may be similar, but the severity of the symptoms usually correlates with the progression of the disease.

• Breathlessness on exertion
• Shortness of breath that progresses without explanation and does not get better with common treatments
• Chest pain
• Dizziness upon standing, climbing stairs, straightening up from a bent position or even while just sitting
• Fainting
• Loss of energy
• Swollen ankles and legs
• Dry cough
• Reynaud’s phenomenon – chalky white and/or dusky blue fingers that may be painful and can sometimes be provoked by the cold

Diagnosing Pulmonary Hypertension

A patient will need a complete evaluation to establish a diagnosis of pulmonary hypertension. A series of tests will be performed and may include:

• Echocardiogram – ultrasound of the heart to estimate function
• Electrocardiogram (ECG) to record electrical activity of the heart
• Right heart catherization to obtain accurate pressures in the pulmonary arteries and determine how well the heart is pumping
• Six minute walk test to determine exercise capacity
• Ventilation-Perfusion scan (VQ scan) to determine if blood clots are present
• CT scan to check abnormalities in the lung tissue
• Pulmonary Function Test (PFT) to rule out other lung diseases that may contribute to symptoms
• Sleep study to assess for sleep apnea
• Lab test to rule out other associated diseases

Risk Factors and Causes

People of all ages, races and ethnic backgrounds can be diagnosed with pulmonary hypertension. Even though anyone can be diagnosed with pulmonary hypertension, certain risk factors and exposures can be associated with pulmonary hypertension and make some people more susceptible to the disease.

• Certain medical conditions such as collagen vascular diseases (including lupus, scleroderma and rheumatoid arthritis), chronic liver disease, HIV or congenital heart diseases
• Exposure to diet drugs such as fenphen or exposure to street drugs including cocaine and methamphetamine
• Obesity, obstructive sleep apnea and other respiratory diseases
• Chronic thromboembolic disease – clots that persist in the lung blood vessels
• Idiopathic and familial pulmonary hypertension is a rare condition and may be caused by a genetic defect

Treatment for Pulmonary Hypertension

After the complete evaluation, our physicians will determine which treatments and medications are best suited to the individual patient. Treatment options available include oral medications, inhaled medications and IV pump medications. Possible treatments may include:

• Anticoagulants to reduce blood clots
• Diuretics to rid the body of extra fluid
• Digoxin to improve pumping action of the heart
• Endothelin Receptor Antagonist (ERA) to help prevent blood vessels from narrowing
• Phosphodiesterase (PDE) inhibitors to help lungs vasodilate
• Prostacyclin analog therapies used to help relieve shortness of breath and open blood vessels

Resources

For more information on pulmonary hypertension, visit the sites below:

Pulmonary Hypertension Association
Pulmonary Arterial Hypertension Information
American Heart Association – Managing Your Medicines
National Heart, Lung, and Blood Institute – What Is Pulmonary Hypertension?Caring Voice Coalition
Primary Arterial Hypertension / Primary Pulmonary Hypertension