Pulmonary Arterial Hypertension (PAH)
Pulmonary Arterial Hypertension (PAH) is increased pressure in the pulmonary arteries. These arteries carry blood from your heart to your lungs to pick up oxygen. The cells that line your pulmonary arteries become inflamed and lead to tightening, stiffness, and blood clots in the arteries. There are five different types of PAH, and many different causes that can contribute to the disease. Other conditions/diseases that can contribute are HIV, Congenital Heart Disease, and Sickle Cell disease. The disease can also be inherited or have an unknown cause. Symptoms include breathlessness, chest pain, dizziness, fainting, edema, fatigue, and decreased appetite.
If you or your doctor suspect you have PAH a diagnosis will be determined using a combination of medical and family histories, physical exam, and results from various tests. Chest X-rays, Echocardiogram, Electrocardiogram (ECG), Pulmonary Function Tests are often used to diagnose. Once the initial tests support the diagnoses a Right- Heart Catheterization is completed by measuring the pressure inside the pulmonary arteries. A catheter is placed through a large vein in the neck or groin and it travels to the heart to measure the blood pressure in the right side of the heart and lungs.
Treatment of PAH
PAH has no cure, but there are medications available to help alleviate symptoms and slow the progression of the disease. Therapy depends on the type of PAH and severity of disease and should be discussed with a healthcare professional. Different medication classes available include conventional therapies like Calcium Channel Blockers (CCB), Digoxin, Diuretics, and Warfarin. Newer therapies include Endothelin Receptor Antagonists (ERAs), Phosphodiesterase Inhibitors (PDE 5 Inhibitors), Prostacyclin Analogue, Soluble Guanylate Cyclase Stimulators, and Prostacyclins. Therapies come in all forms (inhaled, injected, infused, and oral) to help meet the needs of individual patients and their disease. By following a treatment plan developed by the patient and provider patients with PAH can lead normal healthy lives.
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