Why did the lips turn blue? Experts explain pulmonary hypertension in detail

If the human body lives in a hypoxic condition for a long time, life will be threatened with death. However, there is a disease that suffers from hypoxia for a long time, and even the lips turn blue-purple due to long-term hypoxia, and they are called "blue lips", they are pulmonary hypertension patients.

May 5, 2024 is the 13th "World Pulmonary Hypertension Day", pulmonary hypertension is also known as "cancer in cardiovascular disease", but many people do not know about this disease, or even know its existence.

Clinical manifestations and diagnostic challenges of pulmonary hypertension

Xinhuanet: Could you please popularize what pulmonary hypertension is?

Liu Zhihong: In the past, when a patient said, "I'm sick," Western medicine usually classified the disease according to the organs. For example, diseases of the respiratory system are under the management of the respiratory department, and diseases of the cardiovascular system are under the management of the cardiovascular department. However, pulmonary hypertension is not confined to a single organ, but is an abnormal hemodynamic state. To measure pulmonary artery pressure, we need to place a catheter directly into the pulmonary artery. If the measured mean pulmonary artery pressure exceeds 20 mm Hg at sea level, then the patient is diagnosed with pulmonary hypertension. Pulmonary hypertension is very common in clinical practice, and once the pulmonary artery pressure is found to be elevated, it usually means that the disease has entered a more serious stage.

Pulmonary hypertension is a comprehensive disease with a relatively high incidence and multifaceted clinical impact. It affects the right side of the heart to drain blood, causing the patient to feel tired, short of breath when exerting or even short of breath with a little movement. In addition, due to the increased pressure, the pressure in the right atrium will also increase, and the return of blood will be blocked, which can lead to symptoms such as swelling of the lower limbs, multiple serous effusions, such as ascites and poor appetite. During a doctor's round, he or she may notice that the patient's jugular veins are full or distended, which are telltale symptoms of pulmonary hypertension.

Xinhuanet: What is the current diagnosis and treatment of pulmonary hypertension in mainland China?

Liu Zhihong: In the past, people often thought that pulmonary hypertension was a rare disease, but in fact, this mainly refers to the first major category of arterial pulmonary hypertension. Overall, pulmonary hypertension accounts for 1% of the global population. As the population ages, the prevalence can be as high as 10% in people over the age of 65. So, judging by this number, it is not a very rare disease.

Clinically, pulmonary hypertension is divided into five broad categories, one of which has a very poor prognosis. Diseases of the left heart system account for a large proportion of pulmonary hypertension, in addition, cardiovascular diseases and lung diseases are also numerous. In fact, as long as the underlying diseases are treated, such as respiratory problems, cardiovascular problems, etc., and the elevated pulmonary artery pressure is detected early, the pulmonary artery pressure can be effectively reduced. However, in the past, there was no drug treatment for arterial pulmonary hypertension, resulting in an average survival time of only 2.8 years in the absence of drugs, and the prognosis was extremely poor.

"Pulmonary hypertension" is not "hypertension", the emphasis is on early diagnosis and early treatment

Xinhuanet: The harm of pulmonary hypertension is so serious, what causes pulmonary hypertension? Is there a relationship between hypertension and pulmonary hypertension, and how to diagnose whether a patient has pulmonary hypertension?

Liu Zhihong: Hypertension is indeed very common in mainland China, and most of it is primary, that is, there is no obvious cause. However, there is a subset of high blood pressure that has a specific cause, which is different from the pulmonary hypertension we are discussing today. Pulmonary hypertension is actually an increase in pressure within the pulmonary blood vessels, which is significantly different from systemic hypertension in diagnosis and treatment.

The diagnosis of pulmonary hypertension is relatively inconvenient because it cannot be determined by a simple cuff blood pressure measurement. A catheter is usually required for qualitative confirmation or initial screening with ultrasound. Elevated pulmonary artery pressure, particularly due to pulmonary vascular resistance due to certain disorders, is secondary to overcoming resistance to maintain cardiac output. In this case, the treatment is not simple.

After pulmonary hypertension occurs, the right heart drainage is obstructed due to increased pulmonary artery pressure due to increased resistance and increased pulmonary vascular resistance. In cases such as acute pulmonary embolism, because the normal right heart can only overcome a certain range of systolic blood pressure, when the pulmonary artery pressure rises rapidly beyond this range, the blood in the right heart cannot reach the left heart through the pulmonary artery, resulting in a sharp drop in blood pressure.

For patients with multiple underlying medical conditions associated with pulmonary hypertension (e.g., heart disease, pulmonary disease, rheumatic immune disease, etc.), if symptoms such as fatigue, listlessness, and shortness of breath after activity have recently occurred, the presence of elevated pulmonary artery pressure should indeed be considered. These symptoms may indicate that heart function is affected and cardiac output is reduced.

For these patients, regular physical examinations, including electrocardiogram and chest x-ray, are recommended. If the ECG shows signs of right heart overload, such as right axis deviation, P wave abnormality, etc., or chest x-ray shows widening of the pulmonary artery and enlargement of the right heart, it may indicate that there is a problem with the right heart system. At this point, further ultrasonography should be performed to confirm the presence of pulmonary hypertension.

Especially for those who have had pulmonary embolism, unilateral leg swelling, lung disease, heart disease, or rheumatism immune disease, once symptoms such as shortness of breath appear, they should be alert to the possibility of pulmonary hypertension. Timely detection and treatment of pulmonary hypertension is of great significance for improving the quality of life and prognosis of patients.

Take multiple measures to improve the treatment of pulmonary hypertension

Xinhuanet: How should pulmonary hypertension be diagnosed and treated? What is the current efficacy of pulmonary hypertension treatment?

Liu Zhihong: The diagnosis and treatment of pulmonary hypertension and the efficacy vary due to individual differences, different etiologies, and timeliness of treatment.

1. Diagnosis

Right heart catheterization: right heart catheterization is the gold standard for confirming pulmonary hypertension. This method directly measures the pressure of various parts of the heart and the pulmonary artery through the insertion of a catheter through a vein, and can accurately understand the pressure of the pulmonary artery. Although this is an invasive test, it is highly accurate and necessary in patients with suspected pulmonary hypertension.

Cardiac ultrasound: Cardiac ultrasound is a non-invasive, convenient, and commonly used screening method. It can estimate pulmonary artery pressure by measuring the right heart systolic blood pressure. However, the accuracy of cardiac ultrasound is affected by a variety of factors, such as the size of the right atrium, the width of the inferior vena cava, etc., so there may be some errors.

2. Treatment and efficacy

Pharmacotherapy: Targeted drug therapy is one of the mainstay treatments for pulmonary hypertension. For arterial pulmonary hypertension, targeted drugs with three pathways are currently available in clinical practice, including endothelin pathway, nitric oxide pathway, and prostacyclin pathway. These drugs can effectively reduce the clinical symptoms and improve the prognosis of patients with arterial pulmonary hypertension.

Surgery: Surgery may be considered in patients who do not respond to medical therapy or who have severe disease. Surgical treatment includes interventional closure surgery, lung transplantation, etc. The efficacy of surgical treatment varies depending on the patient's specific condition and the type of surgery.

Other treatments: In addition to medical and surgical treatments, patients need adjuvant measures such as oxygen, increased exercise, and cold prevention. These measures can help patients relieve symptoms and improve their quality of life.

In general, the diagnosis and treatment of pulmonary hypertension needs to take into account the specific situation of the patient. For patients with suspected pulmonary hypertension, prompt medical attention and relevant tests are recommended to confirm the diagnosis. In terms of treatment, the appropriate treatment plan should be selected according to the specific condition of the patient, and the treatment plan should be reviewed and adjusted regularly.

Xinhuanet: If you want to effectively improve the long-term treatment effect of pulmonary hypertension, what should you pay attention to in the treatment?

Liu Zhihong: Pulmonary hypertension is indeed a complex disease, and its diagnosis and treatment need to consider a variety of factors. With regard to targeted drugs, they are primarily directed against arterial pulmonary hypertension (especially the first broad category), including idiopathic, heritable, and drug- or toxin-related PAHs.

When it comes to diagnosis, it is essential to determine the cause. For example, whether it is caused by congenital heart disease, rheumatic immune disease, or liver disease, etc. For those cases with no clear cause, we usually classify idiopathic pulmonary hypertension. In addition, genetic testing also plays an important role in the diagnostic process, especially for patients with PAH who have no clear cause. Genetic testing can not only help confirm the diagnosis, but also provide patients with a more personalized treatment plan.

In terms of treatment, the efficacy of the drug, side effects, and economic factors need to be considered during treatment. Patients with specific genetic mutations, such as biallelic mutations, may require a more intensive treatment strategy.

Acquired triggers are also very important. Even if you have a PAH-related gene, not everyone will get the disease. Environmental factors and lifestyle habits may become the "driving force" for the emergence of diseases. Therefore, for high-risk groups with a family history of PAH or gene carrier, in addition to genetic testing, it is also necessary to pay attention to maintaining good lifestyle habits and avoiding possible triggers.

In terms of fertility, for young people who carry PAH-related genes, through in vitro fertilization and genetic screening technology, embryos without gene mutations can be selected for transfer, so as to give birth to healthy babies. The development of this technology has brought new hope to such families.

In general, the diagnosis and treatment of pulmonary hypertension requires a comprehensive consideration of a variety of factors, including the etiology, genetic test results, the patient's lifestyle habits, and fertility needs. With the continuous advancement of medical technology, we believe that more effective treatments and strategies will emerge in the future to bring better quality of life to patients with pulmonary hypertension.

Xinhuanet: Could you please instruct the patient on how to carry out follow-up treatment after his condition is stabilized, and what should he pay attention to in his daily life?

Liu Zhihong: In view of the huge differences in the level of diagnosis and treatment of pulmonary arterial hypertension and the various difficulties faced by patients, we established the National Center for Cardiovascular Diseases and the Pulmonary Arterial Hypertension Specialty Alliance in 2022. Behind this important initiative is our in-depth analysis of national cardiovascular quality control data for three consecutive years. We found alarming differences in the level of diagnosis and treatment of pulmonary hypertension between regions and hospitals, and this imbalance clearly has a negative impact on patient outcomes and outcomes.

In order to gain a deeper understanding of the problem, we established the National Expert Committee on Cardiovascular Diseases and the Specialty Committee on Right Heart and Pulmonary Vascular Diseases in 2021. In the same year, we completed an extensive survey of patients and physicians. The results are staggering: nearly half of patients with the first major type of pulmonary hypertension need to travel across provinces, often from multiple hospitals to get a diagnosis. On average, the treatment of these patients is delayed by up to 2.6 years, which undoubtedly exacerbates their condition and leads to an extremely poor prognosis.

In the face of this situation, we know that we must act. Therefore, we emphasize the importance of early treatment and actively promote targeted drugs for pulmonary hypertension. These drugs are the result of clinical research in recent years, and they can effectively reduce pulmonary artery pressure and improve the patient's condition through a specific mechanism.

In terms of treatment strategy, we need to find the cause of the disease and treat it accordingly. For patients with the first major category of arterial pulmonary hypertension, because of their poor prognosis, even for low- and medium-risk patients, we recommend starting with a two-drug combination and two targeted drugs orally to stabilize the disease. For patients who are already at high risk, we will take more aggressive intensive treatment measures, including parenteral administration, to ensure that the drug can reach the lesion site quickly and effectively.

During the course of treatment, patients are also regularly recommended for follow-up examinations so that the treatment effect can be assessed and the treatment plan can be adjusted in a timely manner. If the patient's condition is stable and at low risk, we will maintain the original drug treatment and provide follow-up management and guidance through telemedicine and other means. This not only reduces the burden on patients, but also ensures that they receive continuous, effective treatment.

However, for some patients who do not respond well, we consider escalation. This may include adjusting the dose of medication, changing medications, or combining multiple medications. In some extreme cases, if the patient's condition is still not effectively controlled, we recommend that they undergo a transplant evaluation. While transplantation is not a necessary option, it does provide patients with a possible way out.

Overall, we are committed to improving the diagnosis and treatment and prognosis of patients with pulmonary hypertension through the establishment of specialized institutions, the promotion of targeted drugs, the development of personalized treatment plans, and the provision of continuous treatment management and guidance. We believe that these efforts will bring more hope and vitality to patients. At the same time, we also call on the whole society to pay attention to the serious disease of pulmonary arterial hypertension and provide support and help for more patients.

Source丨Xinhuanet