Pulmonary Arterial Hypertension (PAH) (WHO Group 1 PH)

Pulmonary Arterial Hypertension (PAH; WHO Group 1 PH)

Pulmonary arterial hypertension (PAH) is a rare, life-threatening disorder characterized by elevated pressure in the pulmonary arteries. This increased pressure forces the heart to work harder to pump blood through the lungs, leading to heart failure and other serious complications. Affected individuals often experience symptoms like shortness of breath, fatigue, swelling of the feet or ankles, and reduced exercise tolerance. PAH is a potentially fatal illness with no cure.

PAH occurs as a result of the narrowing and obstruction of the pulmonary arteries due to the thickening and fibrosis of the arterial walls. This obstruction makes it more difficult for blood to be pumped through the lungs, leading to elevated pressures in the pulmonary arteries. Over time, these pressures can cause damage to the heart, lungs, and other organs of the body due to lack of oxygen.

In addition to fibrosis of the arterial wall, PAH may be caused by other medical conditions such as connective tissue diseases, HIV, and cirrhosis. It is also associated with the abuse of certain drugs such as cocaine, amphetamines, diet drugs, and some stimulants.

The diagnosis of PAH is based on a combination of medical history, physical exam, chest X-ray, echocardiogram, and blood tests. Treatment varies depending on the severity of the disease, but may include medications such as phosphodiesterase inhibitors, endothelin receptor antagonists, prostacyclins, and diuretics. In some cases, surgical intervention may be necessary to correct abnormalities of the pulmonary arteries.

Symptoms of Pulmonary Arterial Hypertension (PAH)

• Dyspnea (shortness of breath)
• Fatigue and weakness
• Chest pain
• Lightheadedness
• Syncope (fainting)
• Swelling of the feet or ankles
• Reduced exercise tolerance

Treatment Options For Pulmonary Arterial Hypertension (PAH)

• Medications - such as phosphodiesterase inhibitors, endothelin receptor antagonists, prostacyclins, and diuretics
• Oxygen therapy
• Weight loss
• Pulmonary rehabilitation
• Surgical interventions - such as lung transplants, atrial septostomy, and pulmonary balloon angioplasty