Pulmonary hypertension, or pulmonary hypertension, is an increase in the mean pressure of the pulmonary artery to more than 20 mmHg. Pulmonary hypertension occurs in most cases secondarily as a complication of a primary underlying disease.
What is pulmonary hypertension?
Pulmonary hypertension (also pulmonary arterial hypertension or pulmonary hypertension) is a condition associated with an increase in mean pulmonary artery pressure (to more than 20 mmHg at rest) and vascular resistance, resulting in right heart failure in many cases. Characteristic symptoms of pulmonary hypertension include severely impaired exercise capacity, shortness of breath, fatigue, circulatory disturbances, angina pectoris, peripheral edema (water retention), cyanosis (blue discoloration of the skin), and Raynaud’s syndrome (reduced blood flow to the toes and fingers). In principle, a distinction is made between chronic and acute pulmonary hypertension. While acute pulmonary hypertension is characterized by temporary vasoconstriction in the pulmonary circulation, for example as a result of overload, chronic pulmonary hypertension leads in the long term to hypertrophy (increase in size) of the pulmonary vascular musculature, which later develops into connective tissue, and thus to sclerosis (hardening) and a loss of elasticity of the vessels. At this stage of pulmonary hypertension, oxygen uptake is permanently and irreversibly impaired.
Causes
Pulmonary hypertension often correlates with various underlying diseases. Thus, in many cases, pulmonary hypertension is due to primary diseases such as chronic obstructive pulmonary disease (COPD) resulting from heavy nicotine use, emphysema, pulmonary fibrosis, iterative pulmonary (micro)emboli (sudden pulmonary artery occlusion), asthma, AIDS, sleep apnea syndrome (cessation of breathing during sleep), sickle cell anemia, or congenital heart defects (secondary pulmonary hypertension). In rare cases, pulmonary hypertension occurs as an autonomic disease, with the specific cause usually remaining unexplained (idiopathic or primary pulmonary hypertension). However, it is known that individuals affected by primary pulmonary hypertension have increased secretion of certain vasoconstrictors (blood vessel constricting substances) such as endothelin or thromboxane and/or increased serotonin levels, while reduced synthesis of prostacyclin and nitric oxide can be observed. In addition, in certain individuals, pulmonary hypertension may also be drug-induced.
Symptoms, complaints, and signs
Pulmonary hypertension does not always cause symptoms at its onset. As the condition progresses, clear signs eventually appear, indicating serious disease. Initially, the insufficient oxygen supply to the lungs leads to a decrease in physical and mental performance. Furthermore, pulmonary hypertension leads to fatigue, tiredness and listlessness. Sufferers feel flabby and have unusual chest pain or swelling in the legs. Externally, bluish discolorations of the skin and lips also appear, along with feelings of numbness and coldness in the limbs. The lack of oxygen supply can cause circulatory problems such as dizziness, circulatory problems and palpitations. As the disease progresses, cardiac arrhythmias develop. These occasionally become noticeable as heart palpitations or reduced performance. The increasing strain on the heart can lead to right heart weakness, which reduces life expectancy and usually also limits the quality of life. If pulmonary hypertension progresses further, the patient may die from it. There is a risk of organ damage and heart attacks. In general, pulmonary hypertension favors the development of various diseases. Then there may be strokes, arthritis, ulcers and neurological disorders, which are also associated with symptoms and discomfort.
Diagnosis and course
Infographic on the anatomy and course of pulmonary hypertension . Click to enlarge. Radiographic examination of the chest (chest x-ray) provides initial evidence of pulmonary hypertension. The diagnosis is confirmed by a cardiac catheter examination or Doppler echocardiography, in the course of which the pulmonary arterial blood pressure can be determined.A value of 25 mmHg or more is considered manifest pulmonary hypertension, and a value between 21 and 24 mmHg is considered latent pulmonary hypertension. An elevated BNP value (Brain Natriuretic Peptide or natriuretic peptide type B) in the serum indicates additional heart failure. The exercise capacity of the individual affected can be assessed by a 6-minute walk test with measurement of pulmonary pressure. The prognosis is generally poor in pulmonary hypertension. If pulmonary hypertension exceeds 30 mmHg, the five-year survival rate is only 30 percent, and it worsens further if right heart failure is compromised. Untreated, pulmonary hypertension has a three-year life expectancy based on findings.
Complications
Pulmonary or pulmonary arterial hypertension (PAH) is divided into five different classes depending on the causation of the disease. Common to all five classes is that the disease, if left untreated, leads to significant complications and symptoms of disease that are usually not reversible. Early diagnosis with the consequence of early targeted therapy of the triggering underlying disease is most likely to offer a chance of cure. PAHs designated as primary or idiopathic, for which no causative factors are found, have in common that pulmonary hypertension is usually accompanied by an increased concentration of vasoconstrictors with a concomitant decreased concentration of hormones that cause vasodilation. If the symptoms of primary or idiopathic PAH are not successfully treated, serious complications gradually set in, the progression of which also depends on the severity of pulmonary hypertension. Arterial pulmonary blood pressure values greater than 25 mm Hg correspond to manifest PAH with poor prognosis. Pressures in the range of 21 and 24 mm HG represent latent pulmonary hypertension. If a complication of right heart failure, frequently observed in these cases, is added, the prognosis for survival is unfavorable unless the pathway is open for heart-lung transplantation. In addition to a noticeable drop in performance, heart failure is also indicated by certain laboratory values. Elevated BNP (brain natriuretic peptide) levels are classified as an indicator of heart failure.
When should you see a doctor?
This type of pulmonary should always be treated by a physician. If left untreated, it can lead to various complications that, in the worst cases, can be fatal for the affected person. Early treatment of this type of pulmonale always has a very positive effect on the further course of the disease. A doctor should be consulted if the affected person suffers from clear symptoms of an undersupply of oxygen. The skin may turn blue, and the affected person may also breathe quickly and become tired. Strenuous activities can no longer be carried out easily. Furthermore, disturbances in blood circulation also indicate this pulmonale and must be investigated if they do not disappear again on their own. It is not uncommon for those affected to suffer from dizziness or numbness over the entire body. Likewise, the risk of stroke is also significantly increased, so that the affected person is dependent on regular examinations. In the first instance, pulmonary can be detected by a general practitioner. Further treatment, however, always depends on the exact severity of the symptoms and is carried out by a specialist. In some circumstances, this may also limit the patient’s life expectancy.
Treatment and therapy
In most cases, therapeutic measures in pulmonary hypertension are aimed at treating the underlying disease that triggered the condition. In addition, the disease is treated with medication depending on the stage. For example, the endothelin receptor antagonist bosentan or the prostacyclin analog epoprostenol are recommended for stage III pulmonary hypertension. In some cases, sildenafil (PDE-5 inhibitor), iloprost (prostacyclin analog), and treprostinil and beraprost are also used. If stage IV disease is already present, epoprostenol is used primarily and bosentan, treprostinil, alpostadil and intravenous iloprost are used secondarily. If the vasoreactivity test is positive (response to vasodilator nitric oxide), calcium channel blockers such as diltiazem or nifedipine can be used.If necessary, an atrioseptostomy, in which an artificial connection is made between the two atria of the heart, may be indicated if there is no response to conservative measures. If pulmonary hypertension is already manifest, treatment can usually only be palliative (relief of symptoms) or transplantation (lung or heart-lung transplantation). For this reason, children with a congenital heart defect are operated on as early as possible to prevent the manifestation of pulmonary hypertension. In order to prevent intracardiac thrombosis, anticoagulation (blood clotting inhibition) is additionally indicated. In some cases, long-term oxygen therapy is also used to relieve symptoms, while diuretics and digitalis are used to treat right heart failure. In addition, nicotine abstinence is recommended in the presence of pulmonary hypertension, as well as weight reduction in cases of obesity.
Prevention
Pulmonary hypertension can be prevented to a certain extent. For example, consistent therapy of underlying diseases that may cause pulmonary hypertension reduces the risk of manifestation. Similarly, abstaining from nicotine use can prevent chronic obstructive pulmonary disease and, accordingly, pulmonary hypertension.
Follow-up
In pulmonary hypertension, both causal and symptomatic follow-up are provided. Causal follow-up is important to prevent chronicity of the disease. If chronic pulmonary hypertension occurs, only symptomatic treatment is possible. In some cases, transplantation of lungs or heart may be necessary. Due to pulmonary hypertension and oxygen deficiency, oxygen therapy is advised. This helps to alleviate the symptoms and increase the quality of life of the affected person. In addition, the pulmonary vascular resistance is lowered with the help of medication. Blood vessel dilating agents such as phosphodiesterase inhibitors are used for this purpose. Drugs are also prescribed to support the weakened heart. Diuretics or digitalis preparations are used here. After the disease, vigorous physical activity should be avoided, as this represents a risk factor for further damage to the heart or lungs. Furthermore, a healthy diet in combination with light exercise under professional guidance is important. Other approaches should include giving up nicotine and alcohol and avoiding obesity. Check-ups at regular intervals with a specialist are essential. These focus on checking pulmonary and heart failure and adjusting medications as necessary. The prognosis for pulmonary hypertension tends to be negative, but depends on various factors such as the precipitating cause and the heart’s ability to adapt to the increased pressure.
Here’s what you can do yourself
First of all, affected patients need to find out with their doctor how pulmonary hypertension, or pulmonary hypertension, came about. If there are underlying diseases that led to pulmonary hypertension, they must be treated. If the pulmonary hypertension is caused by medication, the medication should be changed. In any case, it is important to take this condition and its symptoms seriously, otherwise pulmonary hypertension has a poor prognosis. It is essential to take the medications prescribed by the physician. Smoking is taboo for patients with pulmonary hypertension. If possibilities allow, they should place their residence in rural areas with less fine dust pollution. The use of fine dust filters in the home can also provide relief for those affected. Since patients with pulmonary hypertension are prone to thromboembolism, they should be shown vein exercises that can prevent thrombosis. Alternating showers and treading water are also recommended measures. Patients should also avoid standing or sitting for too long, but instead should move as much as possible within the limits of their ability. An adequate supply of fluids in the form of two to three liters of water or herbal tea a day is recommended. The mineral water should contain little sodium. Self-help groups listed in the Pulmonary Hypertension Info Center (www.lungenhochdruck-infocenter.de/services/selbsthilfegruppen.html) also provide further assistance.