Pulmonary arterial hypertension (PAH) is a vascular disease characterized by increased blood pressure in the pulmonary arteries and pulmonary arterioles, which reduces the efficiency of oxygen exchange in the lungs. By definition, the diagnosis of PAH requires mean pulmonary artery pressure greater than 20 mmHg and pulmonary vascular resistance greater than 3 Wood units on right heart catheterization. An increase in this pathological state may lead to right heart failure and even death. Many people do not know enough about PAH, so symptoms are often ignored in the early stages, delaying correct diagnosis.
According to the report, the five-year survival rate for pulmonary hypertension increased from 34% in the 1980s to 61% in 2010.
In the early stages, the symptoms of PAH may not be obvious. The most common symptom is dyspnea, which 98% of patients experience at the time of diagnosis. As the disease progresses, other symptoms such as fatigue, dizziness, heart palpitations, and chest pain may occur. Late signs include limb edema and ascites, which are signs of right heart failure.
The exact cause of PAH is still unknown. Approximately 39% to 46% of patients are diagnosed with idiopathic PAH, while other types of PAH are caused by genetics, drugs, or toxic substances. Variations in certain genes are related to the pathogenesis of the disease. For example, mutations in the BMPR2 gene are one of the main causes of hereditary PAH.
In the current classification system, PAH is divided into multiple types, including idiopathic, hereditary, and drug-induced.
The diagnosis of PAH mainly relies on cardiac ultrasound examination and right heart catheterization, which can accurately measure right heart pressure and pulmonary artery pressure. Other tests such as CT scans and cardiac magnetic resonance imaging can also help rule out other possible causes. During the diagnostic process, pulmonary hypertension caused by left heart disease, lung disease, or chronic blood clots also needs to be ruled out.
Treatment methods for PAH include supportive therapy and targeted therapy. Supportive therapy usually involves the use of diuretics to relieve fluid overload and oxygen therapy as needed. Targeted therapies include the use of drugs that directly target the pathological mechanisms causing hypertension, such as PDE5 inhibitors and endothelin receptor antagonists.
According to the latest research, combination therapy (that is, treatment targeting multiple pathological pathways) can significantly improve patient outcomes.
With advances in specialized treatments, five-year survival rates for patients with pulmonary hypertension have improved significantly. However, it is important to note that certain types of PAH, such as those associated with scleroderma, have a poor prognosis. Providing adequate medical and quality-of-life support is critical to overall patient survival.
Overall, the impact of PAH is far-reaching and complex, and may affect patients' quality of life, both physically and psychologically. In this case, as a member of society, how can we raise awareness and attention to this disease to improve the situation of patients?