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Pulmonary hypertension is a disease the essence of which is an increase in blood pressure in the vessels supplying the lung tissue with oxygen and nutrients, i.e. in the pulmonary artery and its numerous branches. The causes of this ailment include, among others, left ventricular heart failure and chronic pulmonary embolism.

What is pulmonary hypertension?

Pulmonary hypertension is a disease characterized by an abnormal increase in pressure in the pulmonary artery. This condition is the result of a pulmonary embolism in which some arterial branches are closed by clots that have not been ‘uncovered’ during treatment of a past embolism. There may be times when the patient has no idea that he or she has had a pulmonary embolism because it was completely asymptomatic.

The leading symptom in patients with pulmonary hypertension is the deterioration of exercise tolerance – patients struggle faster, experience shortness of breath and chest pain. Sometimes they can also get hoarse. In the advanced stages of the disease, these ailments appear at rest, making it impossible for the patient to function normally. Pulmonary hypertension is diagnosed when the mean pulmonary artery pressure is ≥25 mmHg.

The causes of pulmonary hypertension

The etiology of pulmonary hypertension development includes:

  1. left ventricular heart failure,
  2. lung diseases such as chronic obstructive pulmonary disease, chronic obstructive sleep apnea, and interstitial lung disease
  3. pulmonary arterial hypertension due to e.g. heart defects where there is a connection between large and small circulation,
  4. chronic pulmonary embolism.

In the aforementioned diseases, various pathophysiological mechanisms cause a series of unfavorable structural and functional changes in the blood vessels of the lungs, causing the walls of arterioles to thicken, their chronic contraction and a reduction in the diameter of their lumen. This results in an increase in the so-called pulmonary resistance, so that the heart (its right ventricle to be exact) has to contract more and more to overcome this resistance and “push” blood through the narrowed vessels to the lungs. The requirement to work harder and harder can eventually lead to right heart failure with all its consequences.

Symptoms of pulmonary hypertension

In patients with pulmonary hypertension, the main symptom is severe fatigue and problems during exercise due to dyspnoea. The development of hypertension leads to:

  1. ascites (the presence of fluid in the abdominal cavity),
  2. swelling of the lower limbs,
  3. the formation of fluid in the pericardial sac and pleural cavity.

Especially congestion in the abdominal organs and the presence of fluid in them causes the patient to lose his appetite. Quite often, patients also complain of chest pain caused by right ventricular ischemia.

The advanced form of pulmonary hypertension may present as:

  1. fainting during exercise,
  2. bluish color of the skin,
  3. brown discoloration of the skin – especially on the lower limbs (changes after thrombosis), and sometimes even hardening of the skin.

Pulmonary hypertension – diagnosis

In the diagnosis of pulmonary hypertension, the medical history with the patient and the presence of the above-mentioned symptoms of the disease play an important role. In addition, it is necessary to perform additional tests to be XNUMX% sure that pulmonary hypertension has occurred.

The most frequently performed tests are presented below:

  1. echocardiography – or ultrasound of the heart, thanks to which the doctor can assess the size of the right ventricle and the width of the pulmonary artery;
  2. Chest X-ray – the examination reveals characteristic symptoms of the disease, e.g. hyperplasia of the ventricle and the right atrium, and dilatation of the pulmonary trunk;
  3. arterial blood gas – its task is to assess the oxygen level in arterial blood (usually it is low);
  4. lung perfusion scintigraphy – assesses the location of the isotope marker, which, with normal pulmonary vascularization, goes straight to the lungs (after intravenous administration); if there is an obstruction of the vessels – there are no tracer accumulation defects;
  5. spiral computed tomography of pulmonary arteries – allows to determine whether there are clots in the pulmonary artery and its branches;
  6. pulmonary arteriography – is an invasive test that involves catheterizing the pulmonary artery and applying a contrast agent to it. Then, a series of X-ray pictures is taken in the form of a film, thanks to which it is possible to assess the flow of contrast through the pulmonary vessels. There may be a lack of this flow when they have become obstructed. Pulmonary catheterization using a Swan and Ganz catheter may also be performed during this examination, or independently of arteriography. The purpose of this catheterization is to determine the pressure in the pulmonary artery and the heart cavities (a very invasive method).

Treatment of pulmonary hypertension

There is no single regimen in the treatment of patients with pulmonary arterial hypertension. Depending on the cause and stage of the disease, pharmacological, non-pharmacological and invasive treatments are used.

Non-pharmacological treatment of pulmonary hypertension primarily involves limiting, and preferably completely abandoning physical exertion. In addition, patients should reduce the amount of table salt consumed and excessive fluid intake. It is contraindicated to stay at high altitudes.

In pharmacological treatment, patients are administered anticoagulants, which are used in all cases of embolism, thrombosis and pulmonary hypertension. Oral anticoagulants are usually recommended, especially in pulmonary arterial hypertension after pulmonary embolism. Sometimes doctors prescribe drugs that inhibit the blood clotting system in the form of low molecular weight heparins (these are usually administered subcutaneously). Thanks to the intake of these preparations, it is possible to recanalize the pulmonary arteries and prevent the formation of another embolism and thrombosis. Note: There are times when this type of treatment must be used for the rest of the patient’s life.

Another method of pharmacological treatment of pulmonary hypertension is oxygen therapy. Oxygen is a very effective preparation that is usually used in home treatment, thanks to which the patient does not have to stay in the hospital. In addition, the preparations indicated in the treatment of pulmonary hypertension are cardiological and dehydrating preparations, which significantly improve the contractility of the heart muscle. Vasodilators also work well and are helpful without significantly affecting the condition of the pulmonary arteries.

Invasive methods of pulmonary hypertension treatment include:

  1. surgical removal of clots from the pulmonary artery and its branches called pulmonary endarterectomy; during the procedure, the clots attached to the inner membrane of the pulmonary arteries are removed; it is a long and complicated procedure performed in extracorporeal circulation;
  2. percutaneous balloon pulmonary artery plastic surgery – during the procedure, the narrowed branches of the pulmonary arteries expand; this method is performed mainly in patients who, for various reasons, cannot undergo surgery; balloon plastic surgery can only be performed in qualified centers with trained personnel;
  3. lung or heart and lung transplant – is performed in patients with advanced disease.

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