Pulmonary Arterial Hypertension: Understanding a Complex and Progressive Condition - Healthcare-netizens/arpita-kamat GitHub Wiki
Pulmonary Arterial Hypertension: Understanding a Complex and Progressive Condition Pulmonary arterial hypertension (PAH) is a rare and progressive disorder characterized by the narrowing of the small blood vessels in the lungs, specifically the pulmonary arteries. This narrowing increases the resistance to blood flow through the lungs, leading to elevated pressure within the pulmonary arteries – hence the name pulmonary hypertension. As the pressure builds, the right ventricle of the heart, responsible for pumping blood through the lungs, has to work harder to overcome this resistance. Over time, this increased workload can cause the right ventricle to weaken and eventually fail, leading to serious complications and a shortened lifespan if left untreated.
PAH is classified under the broader umbrella of pulmonary hypertension (PH), which encompasses several distinct groups with different underlying causes. PAH, classified as Group 1 PH, has various associated conditions, including idiopathic PAH (where the cause is unknown), heritable PAH (caused by genetic mutations), PAH associated with connective tissue diseases (like scleroderma or lupus), PAH associated with congenital heart defects, and PAH associated with HIV infection or certain drugs and toxins. Understanding the specific subtype of PAH is crucial for guiding treatment strategies and predicting prognosis.
The symptoms of PAH are often non-specific and can develop gradually, making early diagnosis challenging. Common symptoms include shortness of breath (dyspnea), particularly during exertion, fatigue, chest pain or pressure, dizziness or fainting spells (syncope), swelling in the ankles and legs (edema), and a racing heartbeat (palpitations). As the condition progresses, these symptoms tend to worsen and can occur even at rest, significantly impacting the patient's quality of life and functional capacity. Due to the insidious onset and overlap with symptoms of other cardiopulmonary conditions, a thorough diagnostic workup is essential for accurate identification of PAH.
Diagnosing PAH typically involves a combination of clinical evaluation, non-invasive tests, and ultimately a right heart catheterization. Non-invasive tests may include an echocardiogram (ultrasound of the heart) to assess right ventricular function and estimate pulmonary artery pressure, pulmonary function tests to evaluate lung function, blood tests to look for underlying conditions, a chest X-ray or CT scan to visualize the lungs and heart, and a ventilation-perfusion (V/Q) scan to rule out chronic thromboembolic pulmonary hypertension (CTEPH), another group of PH. However, the gold standard for diagnosing PAH and assessing the severity of pulmonary hypertension is a right heart catheterization. This procedure involves inserting a thin catheter into a vein and guiding it to the right side of the heart and into the pulmonary arteries to directly measure the pressures within these vessels.
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