Pulmonary hypertension

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Pulmonary hypertension (PH), also known as pulmonary arterial hypertension (PAH) or pulmonary hypertension, is a disease that causes the blood pressure in the pulmonary circulation to rise chronically (pulmonary hypertension). The blood vessels in the lungs are narrowed, which leads to increased resistance of the vessels and thus increased blood pressure. Pulmonary hypertension is usually caused by a chronic disease of the heart or lungs. Here you can find out how pulmonary hypertension can develop and how it is treated.

ICD codes for this disease: ICD codes are internationally recognized codes for medical diagnoses. They can be found, for example, in doctor's letters or on certificates of incapacity for work. I27

Pulmonary hypertension: description

Pulmonary hypertension (PH for short) is a disease in which the pulmonary vessels are narrowed due to various causes. This increases the blood pressure in the pulmonary circulation - this is also known as pulmonary hypertension.

The following happens in the pulmonary circulation, also known as the small circulation: The blood is transported from the heart to the lungs and back again. The oxygen-poor blood reaches the lungs from the right ventricle via the pulmonary trunk, which is divided into the right and left pulmonary arteries. There it is again enriched with oxygen.

The narrowed vessels in pulmonary hypertension increase the resistance in the lungs. This makes it difficult for the right ventricle to pump blood through the lungs. The result: the blood flow through the pulmonary arteries is disturbed and the blood pressure is increased, the right ventricle is increasingly overloaded. Further consequences can be a circulatory disorder in the lungs and poor oxygen uptake in the lungs. In the most severe cases, pulmonary hypertension leads to heart failure.

Pulmonary hypertension often does not cause symptoms at an early stage. Only when the disease progresses do symptoms appear. Since the body is no longer adequately supplied with oxygen in pulmonary hypertension, those affected are severely limited in their physical performance, quickly exhausted and complain, among other things, of symptoms such as shortness of breath. If untreated, pulmonary hypertension can be fatal.

In medicine, a distinction is made between primary and secondary pulmonary hypertension: In very rare cases pulmonary hypertension occurs as an independent disease (e.g. through heredity), then it is referred to as primary or idiopathic pulmonary hypertension (IPAH for short). As a rule, however, pulmonary hypertension is triggered by chronic diseases, certain medications or drugs.Then there is talk of secondary pulmonary hypertension.

Early detection of the underlying disease is particularly important in therapy so that the pulmonary hypertension does not progress any further. Pulmonary hypertension is usually treated with medication to reduce pulmonary hypertension.

Pulmonary hypertension: symptoms

With pulmonary hypertension, symptoms are often absent in the early stages of the disease. Only as the disease progresses do those affected perceive symptoms. The reduced oxygen supply to the lungs severely limits their performance. Typical symptoms of pulmonary hypertension are:

• Inefficiency
• Rapid fatigue
• shortness of breath
• dizziness
• Possibly sudden, brief loss of consciousness (syncope) during physical exertion
• Bluish discoloration of the skin and lips (cyanosis)
• Chest pain
• Swelling due to build-up of fluid in the tissues (edema), especially in the legs

On the one hand, the narrowed vessels impair the oxygen supply to the lungs, on the other hand, the heart needs more and more strength to pump the blood through the vessels. The heart has to beat faster and this results in an accelerated heart rate, which can also be felt in cardiac arrhythmias. The heart is increasingly stressed, especially the right atrium and the right ventricle are affected. Right heart failure (right heart failure) can develop.

Pulmonary hypertension: causes and risk factors

Pulmonary hypertension is defined as an increase in the mean blood pressure of the pulmonary artery, the so-called pulmonary arterial pressure (PAPm). In a healthy person, the mean pulmonary pressure at rest is below 20 mmHg. Pulmonary hypertension is present if the value rises to over 25 mmHg at rest and to over 30 mmHg during exercise.

The cause of pulmonary hypertension is a narrowing of the pulmonary vessels, which reduces the volume of the vessels and reduces blood flow. The reason for the vasoconstriction is an imbalance between certain messenger substances that regulate the vasodilation or vasoconstriction. The cause of this imbalance is so far unknown. Cells of the inner vascular wall (endothelial cells), the inner layer and the smooth vascular muscles of pulmonary arteries are increasingly exposed to vasoconstricting messenger substances (such as endothelin, serotonin, thromboxane). At the same time, vasodilating messenger substances (such as prostacyclin or nitric oxide) are present in reduced numbers. As a result, the vessels contract. The vasodilating substances also lead to increased cell growth, so that the vessel walls thicken and the vessels narrow more and more. These processes reduce the volume of the vessels: less blood can flow through them; at the same time, the lower volume also increases the resistance, which increases the pressure with which the blood flows through the vessels. This also increases the strain on the heart. Due to the higher resistance in the vessels, it has to use more and more force to pump blood into the pulmonary circulation. Over time, the right ventricle of the heart enlarges and the muscle in the heart wall thickens until the heart is no longer able to pump enough blood. This can lead to so-called right heart failure.

Doctors differentiate between primary and secondary pulmonary hypertension. Primary or idiopathic pulmonary hypertension (IPAH) occurs as an independent clinical picture without a known cause, so there is no underlying disease as the cause. IPAH can be inherited: If two or more family members are affected in a family, it is referred to as familial pulmonary hypertension (FPAH). However, both forms of pulmonary hypertension are extremely rare: the number of new cases of both forms combined is only one to three cases per million inhabitants per year.

Usually a certain underlying disease is the cause of pulmonary hypertension. Then there is talk of secondary pulmonary hypertension; pulmonary hypertension is therefore the result of the underlying disease. Especially patients with left heart failure or chronic lung diseases often suffer from pulmonary hypertension. Diseases that can cause pulmonary hypertension include:

• COPD (chronic obstructive pulmonary disease): It is the most common cause of pulmonary hypertension.
• Lung fibrosis: In this disease of the lung tissue, more connective tissue is formed, which among other things leads to reduced oxygen uptake.
• Connective tissue diseases such as the so-called CREST syndrome or scleroderma
• Pulmonary embolism (blockage of blood vessels in the lungs)
• HIV infection
• Left heart disease
• Liver disease
• Schistosomiasis (schistosomiasis): This worm-induced disease is a common cause of pulmonary hypertension, especially in South America.

Certain medications such as appetite suppressants (anorectics) and substance abuse are also considered risk factors that can cause pulmonary hypertension.

Depending on the cause, pulmonary hypertension is divided into five categories:

• Pulmonary arterial hypertension (PAH)
• Pulmonary hypertension in left heart disease
• Pulmonary hypertension in lung diseases and / or hypoxemia (low levels of oxygen in the arterial blood)
• Pulmonary hypertension due to chronic thrombotic and / or embolic diseases
• Pulmonary hypertension due to other unclassified diseases

Pulmonary hypertension: examinations and diagnosis

Pulmonary hypertension is often difficult to diagnose at the beginning because the symptoms are quite unspecific and also occur with other diseases. A detailed discussion of the medical history (anamnesis) and a physical examination provide important information. If pulmonary hypertension is suspected, doctors can use various methods to examine the heart and lungs:

• An ultrasound examination (echocardiography) of the heart is the most important examination method for pulmonary hypertension. This allows the doctor to examine the size and function of the heart and examine the heart wall and the movements of the heart valves. In addition, it is possible for him to display the blood flow inside the heart and to record the speed of the blood flow. In this way he can estimate an increased blood pressure in the pulmonary arteries (pulmonary arterial systolic pressure).
• With an X-ray examination of the chest, doctors can identify enlarged pulmonary arteries (pulmonary arteries). However, these can only be recognized at an advanced stage of the disease, so an X-ray examination in the initial stage of pulmonary hypertension is not very meaningful.
• An electrocardiogram (EKG) is also important. This method is used to measure the electrical activity of the heart: The doctor receives information on the heart rhythm and heart rate, for example. If pulmonary hypertension is present, characteristic changes can be seen in the ECG.
• A pulmonary function test is also done to diagnose pulmonary hypertension. The so-called spirometry is used to measure the volume of the lungs or breathing: In this examination, the patient breathes into a special device, the spirometer, which measures the amount of air flowing through. This examination is used to assess the severity, prognosis and course of pulmonary hypertension. It can also provide clues about the cause of pulmonary hypertension.
• To confirm the diagnosis of pulmonary hypertension and to determine the severity of the disease, a so-called right heart catheter examination is suitable. It can be used to measure pulmonary arterial blood pressure directly.

The so-called six-minute walk test provides information about the degree of physical resilience in pulmonary hypertension. It measures the distance a patient can cover in six minutes at a comfortable pace.

Once pulmonary hypertension has been diagnosed, its severity is divided into one of four severity levels:

• Class 1: No restriction on physical activity. Normal loads do not lead to discomfort.
• Class 2: Slight restriction of physical activity, no complaints at rest. Normal physical activity leads to increased breathlessness or tiredness, faintness, or chest pain.
• Class 3: Significant restrictions in physical activity, no complaints at rest. Light activities cause discomfort.
• Class 4: Patients with pulmonary hypertension of this severity cannot perform any physical exertion without symptoms. In addition, there is a right heart weakness, breathlessness and / or tiredness occur even at rest. The slightest activity leads to an exacerbation of the symptoms.

Pulmonary hypertension: treatment

Pulmonary hypertension is either treating the underlying disease that causes pulmonary hypertension or treating the symptoms it causes. Because a cure for pulmonary hypertension is not possible. The aim is to extend life expectancy as well as to improve physical resilience and quality of life.

Pulmonary hypertension is usually treated with medication. Depending on the cause of the disease, antihypertensive or vasodilating agents are used. These include, for example:

• High-dose calcium channel blockers: These drugs lower the blood pressure in the lungs, but are only used in patients with idiopathic pulmonary hypertension (IPAH). In addition, their effectiveness is previously tested with a right heart catheter.
• Prostacyclin derivatives (prostanoids): They are similar to the body's own messenger substance prostacyclin and have a vasodilating effect. These drugs are given either into a vein or through an inhaler.
• Phosphodiesterase (PDE) 5 inhibitors: Active ingredients in this group lower blood pressure in the pulmonary vessels.
• So-called endothelin receptor antagonists counteract the body's own messenger substance endothelin, which has a vasoconstricting effect.

If the cause is chronic obstructive pulmonary disease (COPD), the pulmonary hypertension can be treated with long-term oxygen therapy (with home ventilation with a mask) to improve the shortness of breath. If drug therapy for pulmonary hypertension is unsuccessful, a heart-lung transplant is often the last option.

There is no standard treatment for pulmonary hypertension - it depends on the underlying disease and the severity of the pulmonary hypertension. The therapy is therefore individually tailored to the patient.

Pulmonary hypertension: prevention

Since pulmonary hypertension occurs only in extremely rare cases as an independent disease, but as a consequence of chronic diseases, it is important to treat it at an early stage. This is the only way to prevent pulmonary hypertension. Regular check-ups with your doctor are therefore essential, especially if you already have a disease that is considered a risk factor for pulmonary hypertension.

People with pulmonary hypertension should avoid heavy physical exertion. This is especially true for strenuous sporting activities that are not supervised by a doctor, because they can lead to a further increase in pulmonary hypertension. However, physically supervised physical training can improve the condition of many patients and be a useful addition to therapy.

In addition, patients with pulmonary hypertension are not advised to travel to altitudes above 2,000 meters. because staying at such heights can worsen the condition. Air travel is therefore also a potential risk due to pulmonary hypertension.

Pulmonary hypertension: disease course and prognosis

Pulmonary hypertension is mostly due to chronic diseases of the lungs or heart. There is no cure for pulmonary hypertension - in the worst case it leads to right heart failure. Treatment can extend life expectancy and improve the general quality of life.

The prognosis for pulmonary hypertension also depends on how high the blood pressure in the lungs already is - the higher the arterial-pulmonary pressure (PAPm), the worse the so-called 5-year survival rate: the lower the PAPm -Values ​​above 30 mmHg. If there is no treatment at all, the average life expectancy after diagnosis is a maximum of three years.

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