Pulmonary arterial hypertension (PAH) is a rare form of pulmonary hypertension that is genetically determined. It mainly affects young people. They are diagnosed in 4-5 patients per million people. Symptoms of pulmonary arterial hypertension - primarily breathlessness, shortness of breath and fatigue - appear when pulmonary vessels overgrow - blood cannot flow freely through them, which results in an increase in pressure in the pulmonary artery.

Pulmonary arterial hypertension (PAH)is a symptom complex caused by an abnormal increase in pressure in the pulmonary artery that supplies venous (deoxygenated) blood from the right ventricle to the pulmonary vessels where it is enriched into oxygen, or oxygenation.

About 1.1 thousand people suffer from pulmonary arterial hypertension in totalin Poland people , and in a year it is diagnosed in about 120 people. It mainly affects young people, but is now more and more often diagnosed in people over 60 years of age.Young women get sick more often- estimates indicate that in about 60 percent of cases. But PAH can appear at any age - in children, adults, and the elderly.

Pulmonary arterial hypertension is diagnosed whenpulmonary pressure values ​​ during haemodynamic testingexceeds 30 mmHg during exercise and 25 mmHg at rest.

In a properly functioning circulatory system, venous (deoxygenated) blood flows to the right ventricle of the heart. From there, it flows through the pulmonary artery to the lungs, where it is enriched with oxygen, i.e. oxygenation. Then, oxygenated blood flows from the lungs through four pulmonary veins to the heart, and then to the left atrium, from where it passes through the aorta to the cells of the entire body.

A patient with pulmonary arterial hypertension developsunfavorable changesin the structure of the pulmonary vascular walls, the pulmonary artery and its branches that lead blood from the right ventricle to the lungs. These changes consist of thickening their walls, which causesincreasing the resistance to the blood flowing through these vessels .

The right ventricle of the heart is no longer able to pump enough venous blood into the lungs. This causesan increase in blood pressurepumped out of the right ventricle into the pulmonary artery, which then reaches the changeddiseases of the pulmonary vessels.

Increased pulmonary pressure is a kind of a defensive reaction of the body - it is necessary for the blood to overcome the resistance. Otherwise, there would be pulmonary edema and death.

Pulmonary arterial hypertension (PAH) - causes

Idiopathic pulmonary arterial hypertension- is a rare disease whose causes are unknown. It is known that it is most often young people (under 35 years of age), 2/3 of which are women.

Hereditary pulmonary arterial hypertensionis a genetic disease.

Pulmonary thromboembolic hypertensionis a form of pulmonary hypertension caused by closure of the pulmonary artery and / or its branches by blood clots.

The following may contribute to the occurrence of the disease:

  • drugs and / or toxins, e.g. anorectics, i.e. drugs that suppress the appetite
  • autoimmune diseases
  • HIV infection
  • portal hypertension
  • congenital heart defect
  • schistosomiasis (exotic parasitic disease)
  • chronic hemolytic anemia
  • pulmonary vein occlusive disease
  • left heart disease

Pulmonary arterial hypertension can develop in the course of various lung diseases or chronic hypoxia, i.e. hypoxia of the body:

  • chronic obstructive pulmonary disease
  • interstitial lung disease
  • obstructive sleep apnea
  • alveolar hypoventilation
  • staying at high altitudes for a long time

Other causes of pulmonary arterial hypertension are:

  • hematological diseases
  • metabolic diseases, e.g. thyroid diseases
  • pressure through the tumor
  • fibrosing mediastinitis
  • chronic renal failure treated with dialysis

Pulmonary arterial hypertension (PAH) - symptoms

The first symptoms of pulmonary arterial hypertensionare related to the dysfunction of the right ventricle and appear in all forms of pulmonary hypertension:

  • shortness of breath, especially during exercise
  • feeling tired and generally weak
  • fainting - especially during exercise
  • pressure or pain in the chest, especially with exertion

Unfortunately, despite the fact thatare the main symptoms of PAH , they are not characteristic of this disease - they may appear in the course of many other cardiovascular and respiratory diseases, therefore the overall clinical picture of the patient is important.

In advancedstage of the diseaseless common symptoms may appear:

  • dry cough and hoarseness, which are caused by compression of the retrograde laryngeal nerve by the left branch of the dilated pulmonary artery
  • chest pain during exercise (angina pain) is the result of insufficient blood supply to the overgrown muscle of the right ventricle through the right coronary artery that nourishes the right ventricle
  • hemoptysis, which is a consequence of rupture of dilated bronchial arteries

During the physical examinationthe doctor may also notice other symptoms :

  • swollen ankles or legs
  • cool hands and feet
  • cyanosis (a bluish discoloration of the lips, nose, ears, fingers and toes)
  • ascites, i.e. increasing the amount of fluid in the abdominal cavity
  • widening of the jugular veins

Pulmonary arterial hypertension (PAH) - diagnosis

In the diagnosis of pulmonary arterial hypertension, a number of tests are performed, both non-invasive and invasive:

Non-invasive tests

  • Chest X-ray shows the widening of the pulmonary trunk and the main branches of the pulmonary artery;
  • an electrocardiogram (EKG) can reveal signs of right ventricular hypertrophy, right atrium or abnormal heart rhythm
  • echocardiographic examination (the so-called ECHO) is used to assess the structure and function of the heart

Invasive research

  • lung perfusion scintigraphy enables the diagnosis of pulmonary thromboembolic hypertension
  • spiral computed tomography of the chest (angio CT klp) visualizes in detail the pulmonary circulation, the pulmonary artery and its branches
  • pulmonary arteriography confirms or excludes the presence of thromboembolism in pulmonary arteries in patients with suspected thromboembolic pulmonary hypertension
  • Right heart catheterization is the only method that allows direct measurement of blood pressure in the pulmonary artery, inferior and superior vena cava. Based on this test, the final diagnosis is usually made

Pulmonary vessel reactivity testis also performed . The test involves inhaling a substance that has the ability to dilate the pulmonary arterioles. During the test, the following are performed: measurement of blood pressure and cardiac output.

An important element of the diagnosis of PAH is also the assessment of exercise capacity. It is performed during a 6-minute walk test.

If the above-mentioned tests do not answer the question of what causes pulmonary arterial hypertension, a number of other tests are performed, including gasometryarterial blood, NT-proBNP (a marker of heart failure) and spirometry.

Pulmonary hypertension does not have to be a fatal disease

Assessment of the severity of the disease

The stage of PAH is assessed using the four-stage NYHA classification, the functional class determines the further treatment process of the patient:

  • Class I - the disease does not limit physical activity; the patient does not feel fatigue, shortness of breath, chest pain or pre-syncope
  • Class II - the patient does not feel any discomfort while resting, but begins to feel worse during regular physical activity - he is tired, has shortness of breath, chest pains or pre-syncope
  • Class III - the patient does not feel any discomfort while resting, but must limit physical activity. Even with little effort, he feels fatigue, shortness of breath, chest pains or pre-syncope
  • Class IV - the patient is not able to perform any physical exertion, even when resting, may have symptoms of right ventricular failure, shortness of breath and fatigue.

Pulmonary arterial hypertension (PAH) - treatment

Treatment of pulmonary arterial hypertensionis divided intopharmacological , interventional andoperative . Medicines are usually given with sweat to prevent the symptoms of right ventricular failure and / or to relieve those already present. Thanks to pharmacotherapy, it is also possible to stop the pathological remodeling of pulmonary vessels.

In patients who do not respond to pharmacological treatment, invasive procedures are performed, thanks to which the pulmonary artery and its vessels can be examined without opening the patient's chest. Only as a last resort, surgical treatment is applied.

Drug treatment of pulmonary arterial hypertension

In pharmacological treatment, depending on the causes of hypertension, several groups of drugs are used:

  • anticoagulants (anticoagulants) reduce the viscosity of the blood and thus the risk of blood clots
  • Diuretics reduce swelling of the lower limbs, which is associated with failure of the right ventricle and fluid retention
  • drugs that increase the contractility of the heart chambers and reduce the speed of the heart (e.g. cardiac glycosides)
  • drugs that dilate the vessels, including pulmonary vessels, causing them to relax (calcium antagonists)

Another form of pharmacological treatment is targeted treatment, which consists in administering three groups of drugs: prostanoids, antagonistsendothelin receptors and phosphodiesterase type 5 inhibitors. These drugs act on endothelial cells that cover the inner wall of blood vessels, the function of which is impaired.

New drugs and therapies are constantly being developed - not only to prolong the life of patients, but also to make them a little more comfortable. Recently, an oral drug appeared in the group of drugs acting on the prostacyclin pathway (now they are prostacyclin derivatives), which is not a prostacyclin but a synthetic molecule that acts like this active substance. The new drug in the tablet may replace the substances in functional class III that are currently administered by inhalation, subcutaneous or intravenous infusion.

The last form of pharmacological treatment is oxygen therapy, which consists in administering oxygen from a concentrator through a mask.

Operative treatment of pulmonary arterial hypertension

The form of surgical treatment isatrial septostomy.This procedure creates an opening between the right and left atrium of the heart, thanks to which the right half of the heart is relieved. Then the arterial blood is saturated with oxygen. It is a procedure performed in patients with a very advanced form of pulmonary hypertension.

Surgical treatment of pulmonary arterial hypertension

Pulmonary endarterectomyis a cardiac surgery that can be performed in selected patients with chronic pulmonary thromboembolic hypertension. The operation is performed in extracorporeal circulation in deep hypothermia (body cooling). This means that during the procedure, the blood flow through the body is completely stopped for several minutes, which allows the doctor to remove clots from the pulmonary arteries.

In extreme cases, a lung or lung transplant with a heart is necessary. Only people who have failed conservative treatment are eligible for the procedure.

Worth knowing

5. May wasWorld Pulmonary Hypertension Day . This is a special day when we should especially look at the patients' problems and try to build a dialogue on the most urgent needs of patients in the light of new therapeutic solutions that increase the chances of improving the quality of life of patients.

A spot was prepared as part of the"Breathtaking Stories" campaignon pulmonary arterial hypertension. We encourage you to share the video and talk about this little-known disease to raise public awareness of the disease among our relatives, doctors and opinion leaders.

Category:

Cardiology