Pulmonary Hypertension

Pulmonary hypertension or pulmonary high pressure means an increase in the mean pressure of the arteria pulmonalis (pulmonary artery) to over 20 mmHg. In most cases, pulmonary hypertension occurs as a secondary complication of a primary underlying disease.

Pulmonary Hypertension

What is pulmonary hypertension?

Pulmonary hypertension (also pulmonary arterial hypertension or pulmonary hypertension) is a disease that is associated with an increase in mean pulmonary artery pressure (to more than 20 mmHg at rest) and vascular resistance, and in many cases leads to right heart failure. See topbbacolleges for Definitions of CMP.

Characteristic symptoms of pulmonary hypertension are severely restricted performance, shortness of breath, fatigue, circulatory disorders, angina pectoris, peripheral edema (water retention), cyanosis (blue discoloration of the skin) and Raynaud’s syndrome (reduced blood flow to toes and fingers).

In principle, a distinction is made between chronic and acute pulmonary hypertension. While acute pulmonary hypertension is characterized by a temporary vasoconstriction in the pulmonary circulation, for example as a result of overloading, chronic pulmonary hypertension leads to hypertrophy (increase in size) of the pulmonary vascular muscles in the long term, which later develops into connective tissue, and thus to sclerosing (hardening) as well as a loss of elasticity of the vessels. In this stage of pulmonary hypertension, oxygen uptake is permanently and irreversibly impaired.


Pulmonary hypertension often correlates with various underlying diseases.

In many cases, pulmonary hypertension is due to primary diseases such as chronic obstructive pulmonary disease (COPD) as a result of heavy nicotine consumption, pulmonary emphysema, pulmonary fibrosis, iterative pulmonary (micro)embolism (sudden pulmonary artery occlusion), asthma, AIDS, sleep apnea syndrome (stopping 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 those affected by primary pulmonary hypertension release increased levels of certain vasoconstrictors (substances that narrow blood vessels) such as endothelin or thromboxane and/or have an increased serotonin level, while at the same time a reduced synthesis of prostacyclin and nitrogen monoxide can be observed. In addition, pulmonary hypertension can also be drug-induced in certain people.

Symptoms, Ailments & Signs

Pulmonary hypertension does not always cause symptoms initially. Eventually, as the disease progresses, clear signs of a serious illness appear. The lack of oxygen supply to the lungs initially leads to a decrease in physical and mental performance. Furthermore, pulmonary hypertension leads to exhaustion, tiredness and listlessness.

People feel limp and have unusual chest pain or leg swelling. Externally, there are also bluish discolorations of the skin and lips, combined with numbness and cold feelings in the limbs. The lack of oxygen supply can cause circulatory problems such as dizziness, circulatory disorders and tachycardia.

Cardiac arrhythmias develop as the disease progresses. These are occasionally noticeable through heart palpitations or reduced performance. Due to the increasing strain on the heart, right heart weakness can develop, which reduces life expectancy and usually also restricts the quality of life.

If the pulmonary hypertension progresses further, the patient can die. There is a risk of organ damage and heart attacks. In general, pulmonary hypertension favors the development of various diseases. Then it can lead to strokes, osteoarthritis, ulcers and neurological disorders, which are also associated with symptoms and disorders.

Diagnosis & History

An X-ray examination of the chest (X-ray thorax) provides the first indications of pulmonary hypertension. The diagnosis is confirmed by a heart 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 and between 21 and 24 mmHg latent pulmonary hypertension. An increased BNP value (brain natriuretic peptide or natriuretic peptide type B) in the serum indicates additional cardiac insufficiency. The resilience of the individually affected person can be determined by a 6-minute walk test while measuring the pulmonary pressure. The prognosis for pulmonary hypertension is generally poor.

If the pulmonary hypertension is more than 30 mmHg, the five-year survival rate is only 30 percent and also worsens if the right heart failure develops. Untreated, pulmonary hypertension has a three-year life expectancy according to the findings.


Pulmonary or pulmonary arterial hypertension (PAH) is divided into five different classes depending on the cause of the disease. What all five classes have in common is that if left untreated, the disease leads to significant complications and symptoms that are usually not reversible. An early diagnosis with the consequence of an early targeted therapy of the triggering underlying disease offers the best chance of a cure.

The PAHs designated as primary or idiopathic, for which no triggering factors have been found, have in common that pulmonary hypertension is usually accompanied by an increased concentration of vasoconstrictors with a simultaneously reduced concentration of hormones that cause the vessels to dilate (vasodilation). If the symptoms of primary or idiopathic PAH are not treated successfully, serious complications gradually develop, the progression of which also depends on the severity of the pulmonary hypertension.

Arterial pulmonary blood pressure values ​​above 25 mm Hg correspond to manifest PAH with a poor prognosis. Pressure values ​​in the range of 21 and 24 mm HG are latent pulmonary hypertension. If right heart failure, which is frequently observed in these cases, is added as a complication, the prognosis for survival is poor unless the avenue for heart-lung transplantation is open.

In addition to a clearly noticeable drop in performance, cardiac insufficiency is also noticeable through certain laboratory values. Elevated BNP levels (brain natriuretic peptides) are classified as an indicator of cardiac insufficiency.

When should you go to the doctor?

This type of pulmonary disease should always be treated by a doctor. If left untreated, it can lead to various complications, which in the worst case can even be fatal for those affected. Early treatment of this type of pulmonary disease always has a very positive effect on the further course of the disease. A doctor should be consulted if the person concerned suffers from clear symptoms of an insufficient supply of oxygen. The skin can turn blue, and the person concerned also has to breathe quickly and becomes tired.

Strenuous activities can no longer be carried out easily. Furthermore, disorders of the blood flow indicate these pulmonaries and must be examined if they do not go away on their own. It is not uncommon for those affected to also suffer from dizziness or numbness all over their body. The risk of strokes is also significantly increased, so that those affected are dependent on regular examinations.

First and foremost, the pulmonary can be recognized by a general practitioner. However, further treatment always depends on the exact severity of the symptoms and is carried out by a specialist. This can also reduce the life expectancy of the patient.

Treatment & Therapy

In most cases, the therapeutic measures in the case of pulmonary hypertension are aimed at treating the underlying disease that caused it. In addition, depending on the stage, the disease is treated with medication.

For stage III pulmonary hypertension, the endothelin receptor antagonist bosentan and the prostacyclin analogue epoprostenol are recommended. In some cases, sildenafil (PDE-5 inhibitor), iloprost (prostacyclin analogue) as well as treprostinil and beraprost are also used. If stage IV of the disease is already present, epoprostenol is used primarily and bosentan, treprostinil, alpostadil and intravenous iloprost secondarily.

If the vasoreactivity test is positive (response to vasodilating nitric oxide), calcium channel blockers such as diltiazem or nifedipine can be used. If there is no response to conservative measures, an atrioseptostomy, in which an artificial connection is made between the two atria of the heart, may be indicated. If pulmonary hypertension is already manifest, it can usually only be treated palliatively (relief of the symptoms) or as part of a 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. To avoid intracardiac thrombosis, anticoagulation (blood clotting inhibition) is also 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, if pulmonary hypertension is present, smoking cessation and weight reduction if overweight are recommended.


Pulmonary hypertension can be partially prevented. Consistent therapy of underlying diseases that can cause pulmonary hypertension reduces the risk of manifestation. Likewise, abstaining from nicotine consumption can prevent chronic obstructive pulmonary diseases and, accordingly, pulmonary hypertension.


In the case of pulmonary hypertension, both causal and symptomatic follow-up care is carried out. Causal follow-up care is important to prevent the disease from becoming chronic. When chronic pulmonary hypertension occurs, only symptomatic treatment is possible. In some cases, a lung or heart transplant may be necessary.

Due to the pulmonary hypertension and lack of oxygen, oxygen therapy is recommended. This helps to relieve the symptoms and improve the quality of life of those affected. In addition, the pulmonary vascular resistance is reduced with the help of medication. Blood vessel dilating agents such as phosphodiesterase inhibitors are used for this.

Medicines are also prescribed to support the weakened heart. Diuretics or digitalis preparations are used here. Vigorous physical activity should be avoided after the illness, as this is a risk factor for further damage to the heart or lungs. Furthermore, a healthy diet in combination with light training under professional guidance is important.

Other approaches should include giving up nicotine and alcohol and avoiding being overweight. Check-ups at regular intervals by a specialist are essential. These focus on checking lung and heart failure and adjusting medication if necessary. The prognosis for pulmonary hypertension tends to be negative, but depends on various factors such as the triggering cause and the heart’s ability to adapt to the increased pressure conditions.

You can do that yourself

First of all, the affected patients have 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 disease and its symptoms seriously, otherwise pulmonary hypertension has a poor prognosis. It is imperative that you take the medication prescribed by your doctor. Smoking is taboo for patients with pulmonary hypertension. If the possibilities allow, they should relocate to rural areas with less fine dust pollution. The use of fine dust filters in the home can also bring relief to those affected.

Since patients with pulmonary hypertension are prone to thromboembolism, they should be shown vein exercises with which they can prevent thrombosis. Alternating showers and treading water are also recommended measures. Patients should also not stand or sit for too long, but instead move as much as possible within the limits of their ability. A sufficient supply of liquid in the form of two to three liters of water or herbal tea a day is recommended. The mineral water should contain little sodium.