The rare, progressive disease known as pulmonary arterial hypertension (PAH) is characterized by elevated blood pressure in the pulmonary arteries, which supply the lungs. Increased blood flow resistance results from the narrowing, occlusion, or destruction of the tiny arteries in the lungs. Because of this, the right side of the..
The rare, progressive disease known as pulmonary arterial hypertension (PAH) is characterized by elevated blood pressure in the pulmonary arteries, which supply the lungs. Increased blood flow resistance results from the narrowing, occlusion, or destruction of the tiny arteries in the lungs. Because of this, the right side of the heart has to work harder to pump blood through the lungs, which, if untreated, can eventually result in right-sided heart failure.Although the precise etiology of PAH is frequently unknown, it can be idiopathic or linked to other disorders such connective tissue illnesses, congenital cardiac abnormalities, or exposure to specific chemicals or medicines. Additionally, PAH may run in families, with some people being predisposed to the illness due to gene abnormalities . Shortness of breath, exhaustion, chest pain, lightheadedness, fainting, and swelling in the ankles and legs are some of the nonspecific symptoms of PAH. As the illness worsens over time, these symptoms frequently get worse, significantly lowering exercise tolerance and quality of life.A comprehensive medical history, physical examination, pulmonary function tests, echocardiography, right heart catheterization, and blood tests to evaluate hormone levels and indicators of heart and lung function are sometimes required for the diagnosis of PAH. The degree of lung and heart involvement may also be determined by imaging tests such chest X-rays, CT scans,and MRI.The goals of PAH treatment are to lessen symptoms,increase activity tolerance, and delay the onset of the illness.Prostacyclin analogs, endothelin receptor antagonists, phosphodiesterase-5 inhibitors,and calcium channel blockers are among the drugs that are frequently used in conjunction for this. Supplemental oxygen treatment could be required in some circumstances to treat hypoxemia and enhance respiratory health.Apart from pharmaceutical treatment, lifestyle adjustments including quitting smoking, keeping a healthy weight, and taking part in pulmonary rehabilitation programs can also aid in symptom management and enhance general health. As a last resort, lung transplantation may be considered for patients with severe PAH who do not respond to medical treatment.All things considered, PAH is a dangerous illness with a high chance of death that needs to be continuously managed by a multidisciplinary team of medical specialists, including cardiologists, pulmonologists, and expert nurses. Improving prognoses and quality of life for those with this difficult condition requires early identification and treatment.
