- What are the causes of pulmonary hypertension in children?
- WHO-FC classification of pulmonary hypertension in children
- Treatment of pulmonary hypertension in children in Poland in 2022
- Methods of treating pulmonary hypertension in children
Pulmonary hypertension in children is a rare disease caused by heart defects or occurs as an idiopathic / inherited form. It is usually diagnosed in an advanced stage. How is the treatment of pulmonary hypertension in children in Poland going?
Pulmonary hypertension in children (PAH)is a huge challenge for pediatric cardiologists according to Dr. Małgorzata Żuk from the Department of Cardiology of the Memorial Institute of the Children's He alth Center. It isa rare disease that few patients suffer from . By definition, pulmonary hypertension is the elevated mean pressure in the pulmonary artery>25 mm Hg measured by invasive methods. How many children with pulmonary hypertension do we have in Poland?As the doctor noted, there are ten times less sick children with pulmonary hypertension than adults. Currently, the Polish database of pulmonary hypertension (BNP-PL) hasabout 90 little patients . The prevalence among the youngest is one-third lower than in adults. The causes of pulmonary hypertension in children can vary and the management depends on the correct diagnosis. According to the classification of the World He alth Organization (WHO), pulmonary arterial hypertension is most often diagnosed in the youngest patients. On the other hand, the available registers show thatheart defects constitute 50 percent. causes PH(Eng.Pulmonary Hypertension) . - Cardiovascular diseases, in particular heart defects and idiopathic pulmonary hypertension, are the main causes that we struggle with in patients. We are also not dealing with small patients with connective tissue diseases, which constitute a significant group of patients among adults - said Dr. Małgorzata Żuk during the online conference "Everyday breathless - what we should know about pulmonary hypertension". She added that in the Polish database of pulmonary hypertension (BNP-PL) "there are no data on thromboembolic pulmonary hypertension". Another cause of pulmonary hypertension in children isbronchopulmonary dysplasia , a disease that results from lung damage due to prematurity. - There are 19 percent of the database of the Children's Memorial He alth Institute. patients who have pulmonary hypertension associated with bronchopulmonary dysplasia. Ifwe will apply appropriate treatment to these patients, and other complications of prematurity will not lead to a bad course of the disease, we can cure these patients of pulmonary hypertension - explained Małgorzata Żuk, MD, PhD. As she emphasized, cardiologists in the treatment of pulmonary hypertension in children "are dealing with a growing organism." - We want a patient suffering from this disease to develop perfectly (interact with peers) and become an adult who can function independently if the development of the disease allows it - emphasized the cardiologist. Read also: Pulmonary arterial hypertension: causes, symptoms, tests and treatment Dr. Małgorzata Żuk, MD, Ph.D. presented at the online conference "Everyday breathless - what we should know about pulmonary hypertension" a detailed classification (WHO-FC) of pulmonary hypertension in children: Class I Children from the first days of life to 16 years of age:Normal motor and physical development, without restrictions on physical activity and symptoms of dyspnea, fatigue or fainting. Children aged 16 years and over:No physical activity restriction, no shortness of breath, fatigue, chest pain or pre-syncope. Class II Children from the first days of life to 2 years of age:delayed motor development. Lags behind milestones. Physical development according to your own percentile. Slight restrictions on physical activity, shortness of breath and fatigue. I feel comfortable at rest. Children from 2 years of age up to 16 years of age:physical development according to your own percentile. Slight limitations in physical activity, shortness of breath and fatigue during sports / physical education classes with peers. I feel comfortable at rest. No chest pains. Attendance at nursery / kindergarten / school is 75%. Children aged 16 and over:slight restrictions on physical activity. He feels comfortable at rest. Ordinary physical activity causes shortness of breath, fatigue, pain in the chestthoracic or presyncope. Class III a Children from the first days of life to 2 years of age:regression of motor development. Physical development stunted. Bad appetite. Significant reduction in physical performance, shortness of breath and fatigue. The child is quiet, reluctant to play, requires frequent naps. Feels comfortable at rest, less than normal activity, causes excessive fatigue, fainting, pre-syncope. Children from 2 years of age up to 16 years of age:significant limitation of physical capacity, development regression: stops climbing stairs, reluctant to play with friends. I feel comfortable at rest. Less than normal activity (e.g. dressing) causes excessive fatigue, fainting or chest pain. Attendance at nursery / kindergarten / school<50 proc. Children from the age of 16 : significant limitation of physical efficiency. I feel comfortable at rest. Less than normal activity causes excessive fatigue, presyncope, chest pain. Class III b Children from the first days of life to 16 years of age:3rd grade a + physical development significantly delayed. Bad appetite, requires supplementation / feeding. Significant limitation of physical capacity, development regression. He feels comfortable at rest, less than normal activity (e.g. dressing) causes excessive fatigue and fainting. Children from the first days of life to 2 years of age:quiet child, often napping. Children from 2 years of age up to 16 years of age:does not attend nursery / kindergarten / school. He moves around the house. It needs to be carried and the stroller is necessary for moving outside the home. Maintains relationships with peers. Children from the age of 16:significant limitation of physical efficiency. I feel comfortable at rest. Less than normal activity causes excessive fatigue, presyncope, chest pain. Class IV Children from the first days of life to 2 years of age:class III + cannot perform any activity without shortness of breath, fatigue or fainting. No interaction with the family. Symptoms of right ventricular failure and / or fainting. Children from 2 years of age up to 16 years of age:class III + cannot perform any activity without shortness of breath, fatigue, fainting or chest pain. No interaction with family and friends. Symptoms of right ventricular failure and / or fainting. He does not attend nursery / kindergarten / school. It needs to be worn and the stroller is necessary for moving outside the home. Children aged 16 and over:cannot do anything withoutshortness of breath, tiredness, fainting or chest pain. Fatigue at rest. Symptoms of right ventricular failure. Fainting, pre-syncope conditions. Below is a video for the rest of the article. In order to recognize and establish the etiology of pulmonary hypertension, a detailed diagnosis should be carried out. In assessing the stage of pulmonary hypertension disease, bothphysical development and interactions with peersare taken into account. Not only is the clinical examination important, but also the result of the 6-minute walk test. - This test in children is difficult to interpret. A three-year-old will walk differently, and a seventeen-year-old will walk differently - we have to compare the obtained result to the expected values. We generally do this by comparing the patient's test today with the next test, or today's test with the previous test, which tells us whether the patient is getting better or worse. However, one study is not decisive - emphasized Dr. Małgorzata Żuk, MD. Treatment of pulmonary hypertension in children is implemented on the basis ofassessment of the severity of the disease and the reactivity of pulmonary vessels , incl. therapeutic management including general recommendations, pharmacotherapy and possibly interventional treatment. Early detection of the disease significantly improves the quality of life for children, slows down the occurrence of complications and reduces mortality. Read also: Breathing problems in children - what to do? First aid for breathing disorders in children In recent years, new methods of treating pulmonary hypertension in children have been introduced, which are to extend the life of patients and improve their quality. The pulmonary hypertension drug program includes: 1) Monotherapy: 2) Combination treatment: According to Dr. Małgorzata Żuk, MD, “treating pulmonary hypertension in children” requires good access to he alth care ”. The doctor told the online conference "Everyday breathless - what we should know about pulmonary hypertension" that there are"patients who receive triple therapy and wait forlung transplant ” . Although transplant for adults is difficult to obtain, transplant for children is close to impossible. This thing has to change at some point - unless we get an artificial lung - she said.The treatment also includes bypass procedures so that patients survive until transplantation.- We helped some children with these procedures and they are not candidates for transplantation anymore - she added. What do parents have to do to get their child admitted to a pulmonary hypertension treatment center? They should visit a cardiologist with a small patient, who will write a referral for treatment to an appropriate clinic. Bibliography:What are the causes of pulmonary hypertension in children?
WHO-FC classification of pulmonary hypertension in children
Treatment of pulmonary hypertension in children in Poland in 2022
Methods of treating pulmonary hypertension in children