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The mystery of pulmonary hypertension: Why are its early symptoms so difficult to detect?
Pulmonary arterial hypertension (PAH) is a serious health condition in which pressure in the arteries located in the lungs becomes abnormally high, leading to potential complications that can be life-threatening. Although the diagnostic criteria for PAH are clear, many patients often do not realize its presence in the early stages, which makes the early detection of this disease more complicated.
The diagnosis of pulmonary hypertension requires a mean pulmonary artery pressure greater than 20 mmHg and must be confirmed by right cardiac catheterization.
According to the research, early symptoms tend to be non-specific, such as dyspnea (i.e. shortness of breath) and fatigue, which are similar to those of many other diseases. Therefore, when patients experience these problems, they often do not immediately associate them with possible early signs of PAH.
It is reported that 98% of PAH patients experience dyspnea at diagnosis, which is the most common symptom.
In addition, patients may experience dizziness, chest pain, and palpitations, which are symptoms that may go unnoticed. As the disease progresses, late signs such as swelling of the legs and other parts of the body (edema) and accumulation of fluid in the abdomen (ascites) may develop, but are usually not noticed until the disease is already quite advanced.
Classification and causes of pulmonary hypertension
Pulmonary hypertension can be divided into several categories based on its cause, of which idiopathic pulmonary hypertension and hereditary pulmonary hypertension are the most common. According to the World Health Organization, pulmonary hypertension is classified as Group 1. Diseases in this group may result from genetic factors, environmental toxins, or specific illnesses, among other things.
Recent literature shows that more than half of PAH cases are considered idiopathic, drug-induced, or hereditary.
Other causes include comorbidities with chemotherapy drugs, illegal drugs (such as methamphetamine), immune system disorders (such as sclerosis and lupus erythematosus), and unique risks for pregnant women. These factors make the diagnosis and treatment of PAH more complicated.
Pathology of pulmonary hypertension
The pathological changes of pulmonary hypertension are manifested in the imbalance between proliferation and apoptosis of endothelial cells. This leads to intimal thickening, smooth muscle cell proliferation, and arterial remodeling, ultimately causing sclerosis of the pulmonary arteries and arterioles. This process leads to an increase in blood flow resistance, followed by adaptive changes in the right ventricle. Over time, the right ventricle becomes unable to cope with this increased pressure, eventually leading to heart failure.
Persistent pulmonary hypertension leads to compensatory hyperplasia of the right ventricle, which becomes maladaptive in the long term as the disease progresses.
Diagnostic Methods and Treatment
Diagnosis of pulmonary hypertension requires right heart catheterization, which is the gold standard for confirmation. Before this, doctors usually do a screening test, such as an echocardiogram, to estimate pulmonary artery pressure. After diagnosis, treatment will vary depending on the cause, but the main goal is to improve the patient's quality of life and prolong life.
Current treatments include therapies targeting different pathological pathways, such as PDE5 inhibitors and antagonists of endogenous acetaminophen.
In more serious cases, doctors usually consider dual-drug therapy, which treats two or more pathological pathways at the same time. Such therapy can significantly improve the patient's survival rate.
Prognosis for PAH patients
In the past, the 5-year survival rate for patients with pulmonary hypertension was only 34%, but since the 2000s, with the advancement of specific therapies, this figure has risen to 86% in 2010. However, patients with inherited PAH have a worse prognosis, with more rapid disease progression and a poorer response to therapy.
Studies have shown that the mortality rate of pulmonary hypertension is higher among pregnant women, and women with a history of this disease are advised to make adequate preparations before planning pregnancy.
Today, research on this disease is still ongoing, and scientists are exploring new treatments to improve patients' quality of life and survival rates.
The early symptoms of pulmonary hypertension are so subtle and easy to be ignored. To what extent should we be alert to fight this terrible disease?
