Pulmonary arterial hypertension (PAH) is an increase in the blood pressure in the pulmonary arteries. PAH is only one of five classifications of pulmonary hypertension (PH) and therefore testing must be done to correctly diagnose which type of PH you have.
Effects and Symptoms of Pulmonary Arterial Hypertension
The effects of pulmonary arterial hypertension are vasoconstriction or narrowing and tightening of the blood vessels that connect the heart to the lungs and those within the lungs. This action increases the difficulty for the heart to pump blood through the lungs.
As the condition progresses the vessels become thicker and stiffer which further impairs the blood flow and again increases the work load of the heart to pump the blood to the lungs. Due to the extra work the heart has to do the right ventricle of the heart begins to thicken and enlarge making it even harder to pump blood and leads to right sided heart failure.
Since the blood that is flowing through the lungs is decreasing because the heart cannot pump it there, the left side of the heart begins receiving less blood and the blood it is receiving carries less oxygen than normal. This causes insufficient oxygenation of the rest of the body.
The condition develops over time and symptoms appear gradually and many patients do not see a doctor until late in the process. Some symptoms are fatigue, shortness of breath, a non productive cough, fainting, swelling around the ankles or feet, angina and on rare occasions coughing up blood.
Diagnosing Pulmonary Arterial Hypertension
To diagnose PAH requires the presence of pulmonary hypertension and meeting the parameters of two other conditions. Pulmonary artery occlusion pressure (PAOP) which must be less than 15 mm/Hg and pulmonary vascular resistance (PVR) which must be greater than 3 Wood Units.
Echocardiography can be used to estimate the pulmonary arterial pressure but the most definite assessment of the pressure can be obtained using a Swan-Ganz catheter. PAOP and PVR are not able to be measured or estimated with echocardiography. Accurate diagnosis requires that right sided cardiac catheterization be performed.
Treatment for Pulmonary Arterial Hypertension
Conventional treatment for PAH includes changes in lifestyle and the use of diuretics, anticoagulants, digoxin and oxygen therapy. Some medications are prescribed based on the type of PAH you have such as calcium channel blockers which should only be used in patients who have vasoreactive PAH.
Prostaglandins are usually considered the most effective drug therapy for PAH, some forms of this medication need to be given intravenously however. It requires a semi-permanent central venous catheter for some of these medications. Your doctor can discuss the options with you and you can make an informed choice for your treatment.
The only cure for pulmonary arterial hypertension is a lung transplant. The average survival post transplant for PAH is just over five years.
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