Pulmonary Hypertension

Pulmonary hypertension is a collective term for diseases characterised by increased vascular resistance and therefore increased blood pressure in the pulmonary circulation. The pulmonary circulation is the part of the circulatory system that carries blood from the heart to the lungs and back again. Pulmonary hypertension often results in right-sided heart failure - insufficient pumping capacity of the right ventricle. Pulmonary hypertension can be classified as primary or secondary. This is a simplified classification.

Primary pulmonary hypertension is considered idiopathic: the cause of the disease cannot be determined. There is no underlying disease mechanism (or it is not known), so the symptom itself-pulmonary hypertension-is regarded as the disease. However, this form of pulmonary hypertension is very rare. Various possible causes are discussed-including an increased release of vasoconstrictive (blood-vessel-constricting) factors and reduced production of relaxing (vasodilating) factors. However, the cause has not been sufficiently researched.

The secondary form of pulmonary hypertension is much more common. This occurs when pulmonary hypertension develops in the context of, or as a result of, an underlying condition. In this form, it therefore makes sense to treat the underlying condition and thereby also relieve or cure the pulmonary hypertension. Common underlying conditions in secondary pulmonary hypertension include, for example, chronic obstructive pulmonary disease (COPD), pulmonary embolism, pulmonary fibrosis, sarcoidosis, AIDS, adult respiratory distress syndrome (ARDS), sickle cell anemia, scleroderma, or a congenital heart defect.

In the chronic form of pulmonary hypertension, the problem is not a "physically controlled" narrowing of the lung vessels, but a remodeling of these vessels. The amount of vascular muscle increases and over time it is converted into connective tissue. The elasticity of the lung vessels decreases. The condition is irreversible. In addition, sclerosis (hardening) of the lung vessels occurs, which further worsens the situation. Lung ventilation no longer works optimally as the disease progresses. Adjustment in response to physical exertion is also no longer possible. This leads to a progressive reduction in oxygen uptake. The aforementioned heart failure develops as a result of the increased pulmonary arterial resistance: the heart's ability to pump blood gradually declines.

The physical capacity of patients affected by pulmonary hypertension is severely limited. Typical symptoms of pulmonary hypertension include dyspnea (shortness of breath). Exertional dyspnea-that is, shortness of breath during physical exertion-occurs in 60 to 100 percent of patients. Coughing and a "reduced general condition" are also typical symptoms of pulmonary hypertension. A "reduced general condition" means generally poor physical health that limits everyday activities. Other common symptoms and signs include circulatory disturbances up to and including "circulatory collapse" (syncope), angina pectoris ("chest tightness"), fatigue, peripheral edema, bluish discoloration of the skin and/or mucous membranes (cyanosis), clubbed fingers (digital clubbing), and Raynaud syndrome.

The mean blood pressure in the pulmonary artery is 12 to 16 mmHg in healthy people. Latent pulmonary hypertension is present at a pulmonary blood pressure of 21 to 24 mmHg at rest. Overt pulmonary hypertension is present when the pulmonary blood pressure at rest is above 25 mmHg. The first symptoms are felt at a mean pulmonary artery pressure of about 30 to 40 mmHg. Symptoms then typically become worse with exertion. At 50 to 70 mmHg, the heart's pumping ability steadily declines due to the increased pulmonary arterial resistance. Right heart strain increases. If untreated, this usually leads to right-sided heart failure and death from heart failure. Tricuspid valve insufficiency commonly occurs with pulmonary hypertension.

For diagnosis, a chest X-ray is usually taken. Echocardiography (ultrasound examination of the heart) with indirect measurement of pulmonary artery pressure, cardiac catheterization (direct measurement of pulmonary artery pressure), and a lung biopsy (if secondary pulmonary hypertension is suspected) are also part of the diagnostic work-up when pulmonary hypertension is suspected. Laboratory tests can provide indications of possible secondary heart failure.

Regarding treatment of the condition, any underlying disease should be treated in such a way as to prevent pulmonary hypertension from becoming chronic. If pulmonary hypertension becomes chronic, curative treatment is no longer possible. Treatment can then only be palliative. This palliative treatment involves medication or, in some cases, a transplant, either of the lungs alone or of the heart and lungs. Drug treatment usually consists of calcium channel blockers, prostacyclin derivatives (intravenously, subcutaneously or by inhalation), endothelin receptor antagonists and PDE5 inhibitors. If the right side of the heart is under strain, diuretics and digitalis preparations are also indicated. Long-term oxygen therapy is often used to relieve symptoms and improve quality of life.

Pulmonary hypertension is divided into four stages. The prognosis is often poor. If untreated, average life expectancy from the time of diagnosis is less than three years. With a mean pulmonary artery pressure over 30 mmHg, the 5-year survival rate is about 30 percent. If the mean pulmonary artery pressure is over 50 mmHg, the 5-year survival rate is around 10 percent.

• https://www.netdoktor.de/krankheiten/pulmonale-hypertonie/
• https://flexikon.doccheck.com/de/Pulmonale_Hypertonie

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