Xinhua Online Hai May 5 (Wang Kunshuo) May 5 is the World Pulmonary Hypertension Day, and idiopathic pulmonary hypertension is a rare disease. Since the beginning of this year, from the tax reduction of drugs for rare diseases to the "2019 National Medical Insurance Drug List Adjustment Work Plan", it is proposed that drugs for rare diseases should be included in the priority scope, and drugs for rare diseases have attracted much attention. Recently, Ge Junbo, academician of China Academy of Sciences and director of the Department of Cardiology of Zhongshan Hospital affiliated to Fudan University, said in an interview with Xinhuanet that although pulmonary hypertension is a rare disease of "minority", it has a high degree of malignancy and the patient is very painful. The average life expectancy after diagnosis is less than three years. On the eve of World Pulmonary Hypertension Day, Ge Junbo called on doctors, patients and the whole society to pay more attention to pulmonary hypertension and intervene and treat it in time.
The diagnosis of pulmonary hypertension is complicated and the misdiagnosis rate is high.
At present, there is no accurate statistics of pulmonary hypertension in China. According to foreign data, the incidence of idiopathic pulmonary hypertension is about 2-5 cases/million people/year.
"The incidence of pulmonary hypertension is not high, which can be described as a minority, but it is a malignant disease, with young patients in the majority. Once diagnosed, the average life expectancy is less than three years, which is even worse than some malignant tumors ‘ Evil ’ . In addition, patients with pulmonary hypertension are very painful and their quality of life is very low. " Ge Junbo said.
Dyspnea, shortness of breath, holding your breath and coughing up blood are common symptoms of pulmonary hypertension. Many patients have blue-purple lips due to long-term hypoxia, and daily walking is also a painful torture for them.
It is understood that pulmonary hypertension is divided into idiopathic (primary) pulmonary hypertension and secondary pulmonary hypertension, of which the former has not yet found a clear cause, and this part of pulmonary hypertension with no cause is classified as a rare disease. There are many causes for secondary pulmonary hypertension, and congenital heart disease is the most common cause. In addition, pulmonary embolism, left heart failure, valvular disease, chronic obstructive pulmonary disease and rheumatic immune system diseases are all induced causes.
Due to the low social awareness rate, the rate of missed diagnosis and misdiagnosis of pulmonary hypertension is relatively high. Many patients delay the precious treatment opportunity in the long diagnosis process, and the disease is not diagnosed until the later stage, resulting in unsatisfactory treatment effect and high mortality.
Ge Junbo said that one of the reasons why the diagnosis of pulmonary hypertension is difficult is that the diagnosis is complicated. Right cardiac catheterization is the gold standard for the diagnosis of pulmonary hypertension, and it is an invasive examination. However, for many primary hospitals, it is not qualified for catheterization, and some medical staff also lack the awareness of identifying pulmonary hypertension, resulting in a high rate of misdiagnosis and missed diagnosis.
"The significance of World Pulmonary Hypertension Day is also to appeal to the whole society to pay attention to this disease and improve the awareness rate, especially for medical staff, who should have the awareness of identification, early diagnosis and early treatment." Ge Junbo said.
Although pulmonary hypertension is highly malignant, it can’t be cured at present, but targeted therapy drugs can obviously improve symptoms and prolong patients’ lives. With the improvement of diagnosis and treatment technology of pulmonary hypertension in recent years, and in order to better improve the prognosis of patients, the treatment strategy of pulmonary hypertension has evolved from single drug therapy to early initial combined therapy. The 2018 World Congress on Pulmonary Hypertension, the guidelines of the European Society of Cardiology (ESC) and the 2018 China Guidelines on Diagnosis and Treatment of Pulmonary Hypertension all suggested that patients with pulmonary hypertension should start combined therapy. However, the related treatment drugs are expensive and have not yet entered the national medical insurance coverage. Only a few provinces and cities have been included in local medical insurance, and many patients have to bear high treatment costs. Ge Junbo said: "After the targeted drugs for pulmonary hypertension are applied to clinic, the treatment situation has been greatly improved. The five-year survival rate of patients has increased from about 20% to more than 50%, but the main problem is the high cost." Ge Junbo said.
Fortunately, with the implementation of a series of policies to support rare diseases and the price reduction of pharmaceutical companies, the burden of patients with idiopathic pulmonary hypertension has been decreasing. Since March 1st this year, 21 drugs for rare diseases have been officially reduced in tax, among which amritan, a targeted drug for treating pulmonary hypertension, is among the 21 drugs for rare diseases.
Treatment of acute myocardial infarction needs to adopt "three-complete model"
If pulmonary hypertension is a "minority" disease in cardiovascular diseases, then myocardial infarction is a "mass" cardiovascular disease.
Ge Junbo said that in recent years, the incidence of cardiovascular diseases has shown an explosive growth trend, and diseases related to atherosclerosis have continued to increase, manifested as stroke and myocardial infarction. "A group of diseases, mainly atherosclerosis, cause ischemia of target organs, leading to panvascular diseases including heart failure, chronic heart failure, arrhythmia and peripheral vascular diseases." He said.
Ge Junbo recalled that when he went to college more than 40 years ago, the Department of Cardiology was not independent from the Department of Internal Medicine, and there were not so many cardiovascular patients. Later, with the increase of patients and the progress of technology, the Department of Cardiology gradually became independent.
So, why has cardiovascular disease shown an "explosive" growth in recent years? Ge Junbo believes that the main reason is that people’s lifestyle has changed fundamentally, and the disease spectrum has changed accordingly, with more and more hypertension, hyperlipidemia, hyperglycemia and obesity, and smoking has not been effectively controlled, leading to the growth of diseases represented by atherosclerosis. In addition, the extension of life expectancy has caused more elderly people to develop diseases. "In the past, life expectancy was short, and many people died of malnutrition, infection and other reasons before they lived to get cardiovascular disease. The aging of the population is an important cause of the growth of cardiovascular diseases. " Ge Junbo said.
For myocardial infarction, the most important thing is timely treatment. Ge Junbo said that over the years, media publicity and mass education have made people know the harm of myocardial infarction and how to treat it. In 2014, under the leadership of the Chinese Medical Doctor Association and the Cardiovascular Branch of the Chinese Medical Association, November 20th of each year was designated as the "Myocardial Infarction Treatment Day", and the "1120" plan was launched. Its implication is: the golden treatment time for myocardial infarction is 120 minutes, and irreversible myocardial necrosis will occur after 120 minutes; The emergency number is 120, which is homophonic to remind the public to call 120 for help.
According to Ge Junbo, statistics show that very few patients with myocardial infarction call 120, most of them are family members driving and taking public transportation, which delays the treatment time, so many patients with myocardial infarction died before they arrived at the hospital. "If you can arrive at a hospital with rescue conditions in time, the success rate of rescue is over 97%. But if the patient comes too late and the heart is irreversibly necrotic, it will be difficult to treat. So this is why we have been calling for social participation, whole-process management and global coverage ‘ Three-mode ’ The reason is that just relying on the hospital to wait for the patient can’t solve the problem. " Ge Junbo said.
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