Pulmonary Arterial Hypertension [PAH] - Symptoms & Signs - Causes - Diagnosis - Treatment

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Other Names - Primary Pulmonary Arterial Hypertension (PAH), or idiopathic Pulmonary Arterial Hypertension (PAH)

Pronunciation - (PULL-mun-ary HI-per-TEN-shun)

Pulmonary arterial hypertension (PAH) is defined as mean pulmonary arterial pressure >25mm Hg at rest and >30mm Hg on exertion. Symptoms and Signs of PAH generally do not appear until the right side of the heart begins to fail. The causes are many and its diagnosis is done by Echocardiogram (ECHO) and other tests if necessary. For mild to moderate cases treatment is medication but more severe cases may need lung transplantation.

The pulmonary arteries carry blood from the heart to the lungs. PAH increases the resistance of blood flow through the lungs. Due to the increased strain the right side of the heart pumps blood less effectively, this can eventually lead to heart failure, known as right ventricular failure.

Pulmonary Arterial Hypertension

Causes

PAH can be primary where no known cause is known or secondary where a cause is present. This also is known as idiopathic PAH. Secondary PAH is more common and the cause include -

Bullet Mitral valve disease like stenosis or regurgitation (Mitral Valve lies between atrium and ventricle of heart)

Bullet Lung conditions like chronic obstructive pulmonary disease (COPD) Sleep apnea

Bullet Sickle cell anemia

Bullet Sarcoidosis

Risks of Developing PAH

Bullet Family History of PAH

Bullet Use of anti-obesity drugs, dexfenfluramine

Bullet Prolonged use of cocaine/amphetamines

Bullet Connective tissue disorders (like SLE)

Bullet HIV infection

Bullet Liver disorders

Bullet Use of cocaine

Signs & Symptoms

Bullet Breathlessness

Bullet Dizziness

Bullet Fainting

Bullet Non-productive cough, sometimes hemoptysis (coughing out blood)

Bullet Swelling around the ankle and knees (peripheral edema)

Bullet Chest pain

Bullet Palpitations (awareness of the heartbeat)

Diagnosis

Echocardiogram (ECHO -ultrasound of the heart)- it measures size and shape of the heart, assess the functioning of the heart chambers and valves

Bullet Right heart catheterization- a catheter is inserted through the femoral vein/subclavian vein advancing it to the right ventricle. This catheter is connected to a device to monitor the arterial pressure.

Bullet Electrocardiogram (ECG)

Bullet Chest X-ray (may show enlargement of the right ventricle)

Bullet MRI

Bullet PET (positron emission tomography)- shows the level of the chemical activity of the heart

Treatment

Bullet Medication -

Bullet Anticoagulant medicines- warfarin

Bullet Calcium channel blockers- relaxes the blood vessels and increases blood and oxygen supply to the heart

Bullet Epoprostenol- widens the arteries of the lungs and thus prevents formation of clots. It is given intravenously

Bullet Iloprost- patients are nebulized with this medicine. It reaches the lungs directly and prevents formation of clots.

Bullet Treprostinil- relaxes vessels and increases blood supply to the lungs

Bullet Bosentan- widens arteries of the lung and reduces blood pressure

Bullet Nitric oxide inhalation- widens arteries of the lung and reduces blood pressure

Bullet Diuretics

Bullet Sildenafil

Bullet Oxygen Treatment

Bullet Lung Transplantation

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