Lung High Blood Pressure (Primary Pulmonary Hypertension): Signs, Triggers, and Further Information
Pulmonary Arterial Hypertension (PAH) is a rare and progressive disorder that affects the blood vessels in the lungs, leading to high blood pressure in the lungs. Although the exact cause of PAH is unknown, it may develop due to various factors such as connective tissue disorders, cirrhosis, exposure to certain toxins and drugs, congenital heart abnormalities, infections, and other forms of pulmonary hypertension that include heart and lung diseases.
Despite there being no cure for PAH, treatments focus on improving the quality of life and slowing the progression of the disease. Common treatment options include oxygen therapy, diuretics, endothelin receptor antagonists, phosphodiesterase inhibitors (PDE5 inhibitors), prostacyclin analogue, selective IP receptor agonist, soluble guanylate cyclase (sGC) stimulators, anticoagulants, and in severe cases, lung or heart transplant.
Early diagnosis of PAH can significantly improve a person's survival. Symptoms such as chest pain, increased shortness of breath, swelling of the legs and feet, fatigue, lips and fingers turning blue, dizziness and fainting spells, heart palpitations, hoarseness, cough, and even misdiagnosis as other common lung diseases like COPD or asthma, may indicate PAH. A doctor may order genetic testing to check for genetic mutations associated with PAH.
Healthcare professionals diagnose about 500-1,000 new cases of PAH in the United States each year. Delays in PAH diagnosis may occur due to symptoms starting mild and nonspecific, younger people and people with a history of other lung disorders having delayed diagnosis, and a doctor potentially mistaking it for other conditions like congestive heart failure.
Treatment for PAH is personalized based on the patient's specific condition, underlying causes, comorbidities, severity, and response to therapy. Oxygen supplementation, lifestyle management, and individualized medication regimens play crucial roles in managing PAH. Lung transplant remains the last-resort option for refractory cases.
Key treatment classes and approaches include oxygen therapy to correct low blood oxygen levels, diuretics to manage fluid buildup and reduce swelling, prostacyclins to dilate pulmonary vessels and inhibit vessel wall thickening, endothelin receptor antagonists to block endothelin, a substance that narrows blood vessels, phosphodiesterase-5 inhibitors to enhance nitric oxide signaling and dilate vessels, activin-targeting therapy like sotatercept to address activin pathway imbalance, parenteral prostanoid therapy for severe cases, and surgery such as removal of blood clots or transplantation in advanced disease.
A person can manage PAH at home by staying active, eating a nutrient-dense diet, avoiding or quitting smoking, avoiding hot tubs, saunas, and traveling to high altitudes, discussing pregnancy with a doctor, seeking support groups and help for mental health conditions that may develop. Approximately 15-20% of people with PAH have a genetic mutation inherited from a parent.
By adhering to their treatment plan and managing the condition at home, a person may improve their outlook. The condition most commonly affects females between the ages of 30 and 60. This multifaceted approach reflects the complex nature of PAH and the need for individualized treatment plans.
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