What is pulmonary hypertension, which can lead to death within 3 years? Gachon University Hospital, Department of Cardiology, Prof. Wook-jin Chung

What is pulmonary hypertension, which can lead to death within 3 years? Gachon University Hospital, Department of Cardiology, Prof. Wook-jin Chung

Dec 13, 2024Ehotyshamull Joy

 

1. Definition of pulmonary hypertension

Is pulmonary hypertension a different disease than high blood pressure?

 

Hypertension and pulmonary hypertension are different conditions. Hypertension is a disease in which the blood pressure in the blood vessels leaving the heart rises above the highest blood pressure of 140 and the lowest blood pressure of 90 or higher, and when it occurs, the blood vessels and organs fail, and their function is impaired, resulting in death. Blood pressure measured by a blood pressure monitor is not directly related to circulatory blood pressure and pulmonary artery blood pressure.

 

In comparison, pulmonary hypertension is a disease in which blood pressure in the pulmonary arteries from the heart to the lungs rises.

Even a slight increase in the average pulmonary artery pressure of more than 20 mmHg is problematic. That is why pulmonary hypertension itself is more dangerous than high blood pressure that it damages the right ventricle of the lungs.

 

2. Pulmonary hypertension causes and age of onset

What causes pulmonary hypertension?

 

Pulmonary hypertension is a disease that began to emerge 30 years ago and is currently divided into five groups according to the World Health Organization (WHO). The causes and outbreaks of each group are as follows.

 

District 1

It accounts for about 2% of cases, and the cause of occurrence is idiopathic, hereditary, drug-induced, connective tissue disease, congenital, etc. Pulmonary arterial hypertension is one of these and is classified as a rare disease.

 

District 2

It is a very common classification, accounting for 65% of all pulmonary hypertension. It is caused by left-sided heart disease or heart failure.

 

District 3

It accounts for about 30% of cases and is caused by lung disease and hypoxemia. This includes pulmonary fibrosis.

 

District 4

It accounts for 3% of cases and is caused by the proliferation of pulmonary blood vessels after an acute pulmonary embolism*. It is a rare disease.

*Pulmonary embolism: A disease in which a blood clot blocks a blood vessel in the lungs

 

District 5

This is the case when it is caused by an unclear mechanism.

 

As such, pulmonary hypertension is a group of diseases that various departments such as cardiology, pulmonology, rheumatology, and pediatric cardiology treat together. Although the disease has not been discovered for a long time, there have been a number of papers in recent years, and treatments are actively being developed.

 

It is mainly characterized by women in their mid-40s, and it is believed that the cause is related to female hormones. In recent years, many studies have improved the survival rate, statistically allowing the patient to survive for about 10 more years.

 

3. Pulmonary Hypertension Treatment and Research Progress

What is the treatment for pulmonary hypertension?

 

The normal functioning of the right ventricle determines the well-being of life, so monitoring the symptoms and prognosis is important.

 

Currently, there are four pathways of therapy developed as targeted therapies that block specific signals that cause the cause. All four of these drugs have been proven to be effective in phase 2 and phase 3 clinical trials. Because the efficacy is good and the risk is low, the rarer the disease, the more it is recommended to participate in these clinical trials.

Originally, the TGF-b pathway was developed as the fourth pathway, and the pathway therapies 'sortartercept' and 'ralinepag' are currently being researched in Korea. Since there are studies that show that the combined treatment effect of targeted pathway therapy is strong, there are many patients who are being treated with all four pathways.

Recently, we are conducting precision treatment to analyze genome, transcriptome, metabolite, and proteome that goes beyond clinical practice with 'PHOENIKS', an in-depth phenotype analysis, to observe targets hidden in DNA. If biomarkers* are found in patients with pulmonary hypertension through this study, it is possible to increase the chances of early detection of pulmonary hypertension and increase the survival rate.

 

4. Precautions for pulmonary hypertension

Pulmonary hypertension, what do I need to know?

 

Pulmonary hypertension is dangerous because it takes a long time to be diagnosed, about 1.5 years, and the survival rate after diagnosis is very short, less than 3 years. In addition, the mode of death of pulmonary hypertension is sudden death in half of the cases and the other half is right heart failure. 60~80% of family members of patients are classified as potential patients.

 

That's why it's important to diagnose pulmonary hypertension early. The main symptoms are shortness of breath, chronic fatigue, edema, dizziness, and shortness of breath, which are easy to get buried in daily life, so it is recommended to be sensitive to your physical condition. If symptoms are felt, an echocardiogram should be performed, and both the patient and the primary care physician should be suspicious and pulmonary hypertension should be treated as soon as possible, which has a lower survival rate than any other cancer.

 

In addition, if there is an underlying heart-related disease such as a congenital heart defect, it is recommended to perform a right heart catheterization and echocardiogram periodically for surgical treatment or medication to prevent pulmonary hypertension.

 



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