Pulmonary hypertension is manifested by difficulty breathing, followed by chest pain. Until recently, patients with pulmonary hypertension lived on average for three years, in advanced cases - no more than 6 months. Currently, thanks to modern drugs, after a year of therapy, over 90% of people live in Poland. sick, after two years a little less. What are the symptoms of pulmonary hypertension? How are they treated?

Pulmonary hypertensionis a very serious, rare disease, often underdiagnosed, which reduces the chances of patients' survival. What are thesymptoms of pulmonary hypertension ? How istreatmentgoing? New drugs are very good news, but the condition is that the disease can be diagnosed quickly. And here's the problem. Because primary care physicians ignore the symptoms reported by patients.

Pulmonary hypertension - often misdiagnosed

- Stop your nervousness, and your heart will not be pounding so much - said Agnieszka Bartosiewicz from the Polish Association of People with Pulmonary Hypertension and their Friends, when she once again came to an internist concerned about her he alth condition. - I couldn't walk normally, everyone walked faster than me, I was getting out of breath after walking a few steps, I was still tired. The doctor suggested that I have neuroses and I'm fine.

- I was unable to complete the stress test. The doctor laughed at me saying it was a shame not to be fit at this age. Everyone prescribed me tranquilizers - adds Piotr Manikowski from the Polish Association of People with Pulmonary Hypertension and their Friends, who fell ill at the age of 28.

Pulmonary hypertension is a disease that affects the lungs and the heart and is characterized by high pressure in the arteries in the lungs. This increase in pressure is a consequence of changes in the walls of your blood vessel network (pulmonary arteries). They become thicker and less flexible, making it difficult for the right ventricle of the heart that is pumping blood into them. Pulmonary hypertension is a condition in which the mean pressure in the pulmonary artery exceeds 25mmHg.

- In Poland, we register about 5 cases per million people. The average age of patients is 36 years. You have to be aware that it is a very rare disease, very malignant, but it has a lotnon-specific symptoms such as fatigue, shortness of breath, swelling, fainting … Doctors diagnose neurosis, asthma, anemia, coronary artery disease, and even mental diseases, which delay in making a proper diagnosis and starting treatment - says Dr. Marcin Kurzyna from the Department of Internal Chest Diseases, Institute of Tuberculosis and Lung Diseases in Warsaw.

Pulmonary hypertension - symptoms

The first symptoms of pulmonary hypertension are constant fatigue (patients have difficulty walking even short distances, not to mention climbing stairs) and breathing difficulties, especially after exercise. In more advanced stages of the disease, dry cough and hoarseness, chest pain and hemoptysis appear. In patients, you can also notice a slightly blue tint to the lips and hands and coldness of the limbs.

- I was concerned because it took me 15 minutes to get to the first floor where I lived. After a few steps I had to stop and catch my breath. At the same time, my heart was beating as if it wanted to jump out - Agnieszka Bartosiewicz describes the beginnings of her illness. - The GPs sent me back with nothing until I was taken to the hospital. Here I heard that his heart is so broken that it is strange that I am still alive at all. I was directed to Warsaw, where I found wonderful doctors, I take medications, but I live a normal life and work. Piotr Manikowski blamed exhaustion on intense work and life in rush. A baby would be born to him in a few months. With bleeding from the digestive system, he was taken to hospital, he developed anemia after the examination. However, despite the treatment and better results, he felt worse and worse. Finally, he came to Warsaw, where he was diagnosed with idiopathic pulmonary hypertension. His body, however, did not respond to the drugs. - I was lying under oxygen, as long as an activity, such as combing my hair, was an enormous effort. I was sent to Vienna where I underwent my first lung transplant. After 3.5 years, I developed symptoms of rejection, a few months later I underwent a second transplant - he says. - At present I feel very well. Lives. I have two sons.

TNP can affect anyone, regardless of age (including children), but most often it occurs in young women. This disease is often associated with other conditions such as congenital heart disease, chronic liver disease, or connective tissue disease. In most cases, PAH cannot be diagnosed (then called idiopathic pulmonary arterial hypertension), and sometimes the condition may be hereditary.

Pulmonary hypertension - diagnosis

The first and basic test that can help diagnose the disease is the heart echo (assessing the structure of the heart andits functions and estimating systolic pressure in the pulmonary artery) and chest X-ray (typical for patients with NP is the expansion of the trunk and main branches of the pulmonary artery, and reduced blood flow through small pulmonary vessels, in advanced cases the heart is enlarged, specifically its right ventricle and right atrium). Then, among others, lung perfusion scintigraphy, computed tomography of the chest, arteriography of pulmonary arteries and hemodynamic tests.

Pulmonary hypertension - treatment

In recent years, thanks to the appearance of new drugs by 43 percent the mortality rate among patients was reduced. - Until recently, a patient could live for several years, on average 3. It is a deferred death sentence for a young man. In the case of advanced disease, these six months are already waiting for the sentence - says Dr. Marcin Kurzyna. - Until a few years ago, the basis of treatment, or rather alleviation of symptoms, was oxygen therapy and the use of anticoagulants, diuretics (for swelling), dilating blood vessels or lowering the heart rate. For several years, there have been three groups of new-generation preparations that act on the causes of the disease. These drugs are prostanoids, receptor antagonists for endothelin (the substance that determines the correct vascular tone) and the so-called phosphodiesterase type 5 inhibitors.

Surgical treatment is effective only in patients with chronic pulmonary thromboembolic hypertension - the condition is the presence of large thromboembolic material closing the pulmonary arteries, but in large pulmonary vessels. It is a very serious procedure performed in extracorporeal circulation in deep hypothermia. - Unfortunately, in 4 to 15 percent. in cases, the operation fails and the patient dies. In other cases, there is a complete recovery or a significant improvement in the patient's condition - explains Prof. Andrzej Biedermann, from the 1st Department of Cardiac Surgery, Institute of Cardiology in Warsaw. In some cases, in patients not responding to drug treatment, with advanced disease, the rescue is lung / lung or heart-lung transplant.

Category:

Respiratory system