signs and treatment
Pulmonary hypertension is the name for chronic high blood pressure in the lungs.
Normally, the pressure in the pulmonary artery is less than 20 mmHg (millimetres of mercury). If this value is 20 mmHg or more, this is referred to as pulmonary hypertension. There is no cure, but the disease can usually be treated with medication. If left untreated, it may lead to death.
Pulmonary hypertension (also known as PH) is high blood pressure in the lungs. This means that the blood pressure in the body’s pulmonary circulation is elevated. A distinction is made between pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH). PH includes various conditions in which the blood pressure in the blood vessels of the lungs is elevated. There may be different reasons for this. PAH is a specific form of PH that is caused by certain changes in the small blood vessels of the lungs, called pulmonary arteries.
When an artery in the lungs narrows, the heart must pump harder to transport blood to the lungs. As a result, the right half of the heart becomes enlarged while the left half is compressed. Due to the excessive strain on the right side of the heart, blood flow to the lungs is impaired. This also affects the oxygen uptake in the lungs, which means that the organs are no longer adequately supplied with oxygen. This may manifest itself in a decrease in physical performance and resilience.
Pulmonary hypertension is often difficult to diagnose. However, it is particularly important to detect PH early in order to prevent further progression of the disease. PH can usually be treated well with special medications.
The symptoms may vary depending on the degree of severity and the underlying disease. However, common symptoms are:
- Shortness of breath, especially after physical exertion
- Physical loss of performance
- A feeling of tightness in the chest
- Cardiac arrhythmias or palpitations
- Water retention (oedema) in the legs
In some cases, dizziness and even fainting may occur.
The doctor first performs a physical exam by listening to the patient’s heart and lungs. A blood sample is then taken and examined in more detail in the laboratory.
Using echocardiography, an ultrasound examination of the heart, the doctor checks the patient’s heart function and heart valves. Other tests that may be used include:
- Cardiac catheter examination of the right side of the heart to measure pulmonary arterial blood pressure.
- Chest X-rays to show dilated pulmonary arteries
- Electrocardiogram (ECG) – this provides information on the heart rhythm and rate
- Lung function test: measurement of lung volume (spirometry)
- Six-minute walk test (stress test)
- Imaging procedures: computed tomography (CT) or magnetic resonance imaging (MRI) to identify structural changes in the pulmonary arteries or abnormalities in the lung tissue. Scintigraphy – an examination using radioactive material to show tissue changes
- Blood tests to rule out other possible causes of PH and to identify specific markers for PAH or other forms of PH
There are different forms of PH, which have different causes. The classification of PH was developed by the World Health Organization (WHO) and divided into five groups:
- Pulmonary arterial hypertension (PAH): this includes various conditions in which the blood pressure in the pulmonary arteries is raised due to narrowing and stiffening of the blood vessels. Idiopathic PAH (without known cause), hereditary PAH (genetic) and PAH associated with certain diseases such as connective tissue disease, HIV infection or congenital heart disease also fall into this category.
- Pulmonary hypertension due to heart disease: this form occurs as a result of diseases of the left side of the heart that impair blood flow to the lungs. These include valvular heart disease, congenital heart defects, heart failure and pulmonary veno-occlusive disease.
- Pulmonary hypertension due to pulmonary disease and/or hypoxia: this form is caused by lung diseases such as chronic obstructive pulmonary disease (COPD), pulmonary fibrosis, pulmonary emphysema or sleep apnoea, which affect blood flow in the pulmonary arteries.
- Chronic thromboembolic pulmonary hypertension (CTEPH): this form of PH is caused by recurrent pulmonary embolisms when blood clots form in the pulmonary arteries. Chronic obstruction of blood flow leads to a permanent increase in blood pressure in the pulmonary arteries.
- Pulmonary hypertension with unclear or multifactorial mechanisms: this group includes PH cases where the exact cause cannot be clearly identified or where several factors contribute to the development of the disease.
In pulmonary hypertension, the blood vessels in the lungs are always narrowed. There are a variety of reasons why this happens. Diseases of the connective tissue or the lungs (COPD, asthma, pulmonary fibrosis and sleep apnoea) are possible causes. Pulmonary hypertension is also common in heart failure, especially if the left side of the heart is weakened. Genetic factors may also play a role, and certain medications can cause PH.
The doctor will first investigate the cause of the PH and treat it. Therapeutic measures usually include the administration of medications with a blood pressure-lowering or vasodilating effect such as calcium antagonists, prostanoids, phosphodiesterase (PDE) type 5 inhibitors, endothelin receptor antagonists or sGC stimulators.
In the case of a chronic pre-existing lung condition such as COPD, oxygen therapy for use at home is often the first remedy of choice – to alleviate shortness of breath.
If the drug-based treatment fails, a lung transplant is often the last possible therapeutic measure.
Pulmonary hypertension is incurable. However, treatment with drugs can improve quality of life and prolong life expectancy.
A healthy lifestyle is particularly important for those affected. Above all, this includes a balanced diet to provide the body with important vitamins and nutrients. In addition, consumption of alcohol and nicotine should be limited.
There is no need for people with PH to forgo exercise, but overexertion should be avoided as far as possible. If you experience shortness of breath, take a break. In addition to physical stress, the disease can also lead to psychological stress. Depression, loss of confidence and lack of motivation are common consequences. For this reason, sufferers should seek early support from relatives, friends or in a support group. They can also obtain further information from the Swiss Pulmonary Hypertension Society (SSPH) and the Swiss PH Association (SPHV).
Patientenorganisation für Betroffene:
- Deutsche Gesellschaft für Kardiologie, Herz- und Kreislaufforschung e.V.. Pocket-Leitlinie: Diagnostik und Therapie der pulmonalen Hypertonie (Stand: 2015), unter: https://leitlinien.dgk.org (Abrufdatum: 20.03.2022)
- S2k-Leitlinie der Deutschen Gesellschaft für Pädiatrische Kardiologie und Angeborene Herzfehler e.V. (DGPK). Pulmonale Hypertonie (Stand 2020), unter: https://register.awmf.org (Abrufdatum: 10.03.2023)
- Humbert, M. et al. 2022/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Developed by the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Endorsed by the International Society for Heart and Lung Transplantation (ISHLT) and the European Reference Network on rare respiratory diseases (ERN-LUNG), in: European Heart Journal, unter: https://academic.oup.com/eurheartj/article/43/38/3618/6673929 (Abrufdatum: 20.03.2022)
- USZ – Universitätsspital Zürich. Pulmonale Hypertonie, unter: https://www.usz.ch/krankheit/pulmonale-hypertonie (Abrufdatum: 10.03.2022)
- Stumpf M.J et al. Chronisch thromboembolische pulmonale Hypertonie, in: National Library of Medicine, 17.05.2021, unter: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8093596 (Abrufdatum: 10.03.2022)
- Swiss Society für Pulmonary Hypertension (SSPH). Definition of PH, unter: https://www.sgph.ch/de/definition.html (Abrufdatum: 10.03.2023)