Pulmonary Arterial hypertension (PAH) is a rare progressive disease also known as high blood pressure in the lungs. You may wonder what does the lung have to do with blood pressure? Well, the lung and the heart actually work hand in hand to provide oxygenated blood throughout our body. If there is damage to vessels in the lungs this prevents the oxygenated blood to get through from the lungs to the heart, leading the heart to pump harder thus increasing pressure. In other words, PAH is due to increased pressure in the vessels which is caused by obstruction in the small arteries in the lungs, thus blocking blood flow through the lungs. PAH can be difficult to diagnose, and someone may not know they have PAH until they experience severe symptoms. Although extremely rare, PAH is a very serious condition and if left untreated, it can worsen overtime eventually damaging the heart. The cause of PAH is unknown, but it is thought to be drug-related, or due to connective tissue or autoimmune disorders.
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Who is at risk of Pulmonary Arterial Hypertension (PAH)?
PAH is noticed more in young females of childbearing age than men as well as those with family history of PAH. Patients with lupus, scleroderma, cirrhosis, and HIV can also develop PAH. Drugs, such as methamphetamine and cocaine, may put you at greater risk as well.
What are symptoms of Pulmonary Arterial hypertension (PAH)?
Symptoms of PAH are not specific to the disease, however one with PAH may experience shortness of breath (especially with exercises), chest pain, syncope (fainting), dizziness, weakness, occasionally bloody cough, and swelling (feet, legs, belly, neck). In advanced PAH patients may present with bluish discoloration of skin due to low levels of oxygen circulating in blood.
How is Pulmonary Arterial hypertension (PAH) diagnosed?
PAH is only diagnosed when other causes of pulmonary hypertension have been ruled out. To diagnose several tests must be done by both a heart and lung specialist. Diagnostic tests include: breathing tests (pulmonary function tests), ECG, X-ray, blood tests, echocardiograph, and 6 min walk test.
How is Pulmonary Arterial hypertension (PAH) treated?
Unfortunately, there is no cure for PAH, however it is treatable. Treatment is patient specific depending on severity of the condition and medications can vary from oral pill, inhaled or IV formulations. Some patient’s may require pulmonary rehabilitation programs.
Treatment options include: Prostaglandins, Endothelin receptor antagonists and Phosphodiesterase type 5 inhibitors. These options help slow down progression of disease as well as help with decreasing pressure in the pulmonary arteries.
How should Pulmonary Arterial Hypertension be managed?
Always take your medication as prescribed. Avoid activities that may put strain on your lungs and heart. Consider making lifestyle and diet changes that are applicable to you, such as; avoid smoking and/or drug use, decrease salt and fluid intake, and monitor weight.
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