In the 80s, the detection of pulmonary arterial hypertension (PAH) was a death sentence. The introduction of new drugs has made it a chronic disease for many patients, not a fatal one, it was reported on Thursday at a press conference.
Until recently, pulmonary hypertension was the most deadly disease, even more so than many malignancies such as colon cancer. Only patients suffering from lung cancer lived shorter. In the 80s, the detection of advanced PAH meant that the patient had a chance of survival for no more than 6 months. Other patients, even in the USA, lived on average no longer than about 3 years – said Dr. Marcin Kurzyna from the Institute of Tuberculosis and Lung Diseases in Warsaw.
Today, one year later, 90% of treatment in Poland is still alive. sick, and after two years – a little less. Many patients lead relatively active lives even after many years of treatment.
The prognosis of patients has been significantly improved by new treatment methods and new generation drugs that have been introduced in recent years. Until recently, only oxygen therapy and agents known for a long time, acting only on the symptoms of the disease, such as anticoagulants, diuretics, vasodilators, as well as increasing the contractility of the heart chambers and reducing the speed of the heart, have been used.
For several years now, there have been three groups of new-generation agents that target the causes of the disease. These are the so-called targeted therapies improve the function of the pulmonary artery endothelium. These are prostanoids, receptor antagonists for endothelin (the substance that determines the proper vascular tone) and the so-called phosphodiesterase type 5 inhibitors. These drugs after 3 months of taking as much as 40 percent. reduce the risk of death, as shown by a meta-analysis of 2009 studies conducted in 23, which included 3 sick – said Dr. Kurzyna.
Pulmonary arterial hypertension causes remodeling of the pulmonary vascular wall. The smooth muscles in the median membrane grow and the endothelium of the pulmonary artery and its branches enlarges. The walls of the pulmonary vessels are thickened and stiff, and their lumen is much smaller, which makes it difficult for blood to flow to the alveoli. In addition, as the disease progresses, clots form, which become fibrous and cause overgrowth of the vessels.
The right ventricle of the heart pumps blood with increasing difficulty and enlarges. Its walls thicken and stretch at the same time. The capacity of the ventricle decreases and it is unable to pump enough blood through the lungs during any exercise. The amount of blood that is pumped to other internal organs and body tissues during the heart’s contraction is also reduced.
The sick person feels worse and worse, begins to feel short of breath at the slightest effort. He is tired, weak and fainting. Edema of the lower limbs, angina chest pain, hemoptysis and ascites appear.
Initially, I only had trouble walking. Two months later I was no longer able to walk 5 meters without gasping for breath. It took 15 minutes to walk to the first floor where I lived, because I had to stop ten times to catch my breath. My heart was beating at a dizzying pace, recalled Agnieszka Bartosiewicz from the Polish Association of People with Pulmonary Hypertension and their Friends at the conference.
The doctor ignored her ailments. He said it’s nerves, he needs to relax and get physically fit. She started going to aerobics. The result was that she was hospitalized in the intensive care unit. When my hearts were examined, they were in such a state that they were surprised that I was still alive – recalled A. Bartosiewicz. Fortunately, it responds well to medications. She has disability group II, but has returned to work. He claims to lead almost normal lives.
Unfortunately, this is not an isolated incident. GPs rarely detect pulmonary hypertension. They often confuse them with other conditions such as asthma, pneumonia or bronchitis, coronary artery disease, neurosis, and chronic obstructive pulmonary disease.
The effect is that patients still see a specialist too late, after many years of illness. In some people, the changes are so advanced that the only salvation is lung transplantation.
Piotr Manikowski from the Polish Association of People with Pulmonary Hypertension is undergoing two lung transplants. He fell ill in 2005 at the age of 28. When he was first hospitalized, he had gastrointestinal bleeding, but was only diagnosed with anemia. Once again he found himself in the intensive care unit when he developed a pulmonary embolism. After the diagnosis of pulmonary hypertension, it turned out to be resistant to the effects of new drugs. The lungs were completely destroyed.
He underwent a lung transplant in Vienna, but complications arose after the operation. Postoperative wounds were infected and kidney failure occurred. After more than 3 years, the first symptoms of transplant rejection appeared. Six months later, I was once again in the intensive care unit. I thought there was no rescue for me this time. Then I suddenly got a chance for a second transplant. Today I feel great, I am full of energy and hope – said Piotr Manikowski.
The basic test for the detection of pulmonary arterial hypertension is echocardiography (using only ultrasound waves for testing). It is worth doing them when we suffer less from greater physical effort. A signal of the disease may be seemingly minor ailments, such as repeated coldness of the arms and legs and cyanosis (bluish discoloration of the lips, nose, ears, fingers and toes).
These symptoms may indicate a slower heart rate and a decrease in the amount of oxygen-rich blood reaching the tissues and organs. This is the last signal to detect the disease before serious complications appear.
Zbigniew Wojtasiński (PAP)