The mean arterial blood pressure in the lungs is normally lower than in the rest of the body. It is 15 mmHg compared to 80-100 mmHg in the rest of the body. If the value rises above 20 mmHg, this is referred to as latent PAH. If the value is permanently above 25 mmHg, the clinical picture is classified as manifest pulmonary hypertension.
The first symptoms of failure usually occur from an arterial mean pressure of 30 mmHg. From a mean pressure of 50 mmHg, cardiac output decreases noticeably. Performance is reduced and circulatory collapse or even circulatory arrest may occur.
Pulmonary hypertension has a poor prognosis. It is therefore important to recognize and treat it in good time.
If pulmonary hypertension becomes chronic, the damage to the body can no longer be reversed. Only a transplant can then prevent a fatal outcome.
It is therefore important not to underestimate the symptoms and to seek medical advice too early rather than too late.
Pulmonary hypertension is caused by a constriction(narrowing) of the blood vessels in the lungs. The blood vessels are constricted by the muscle contractions in the vessel walls. This can be caused by
- too much blood flow in the pulmonary circulation or
- insufficient oxygen supply
be the cause.
In addition to acute pulmonary hypertension, there is also a chronic form. In the chronic variant, the pulmonary vessels undergo a transformation. Due to an increase in the vascular musculature, connective tissue gradually grows into the structure of the vessels, which develops from the musculature. This restricts the freedom of movement of the vessels. At this stage, pulmonary hypertension is irreversible.
Pulmonary hypertension usually occurs as a secondary disease of another clinical picture. Left heart failure in particular causes symptoms of PAH. The left heart is then no longer able to perform its pumping function at its usual level. This causes the blood to back up. This backlog then causes the vascular changes.
Pulmonary hypertension also frequently occurs in lung or heart diseases such as thrombosis or pulmonary embolism. Due to fluctuations in the composition of the blood count, blood clots can form on the pulmonary vessels and block the bloodstream(pulmonary embolism).
In most cases, a pulmonary embolism disappears without being noticed. In life-threatening cases, however, it causes a blockage in the pulmonary circulation and promotes pulmonary hypertension due to the congestion of blood in the lungs.
Pulmonary hypertension should be diagnosed early in order to improve the chances of recovery © Henrie | AdobeStock
Unfortunately, pulmonary hypertension does not manifest itself in a uniform set of symptoms. Different symptoms develop depending on the severity of the disease. Most cases of PAH are characterized by
- breathlessness,
- listlessness and
- fatigue.
In addition, dizziness and heart palpitations are among the most common physical symptoms of the disease. In addition, there are many other symptoms that do not occur in combination.
The following symptoms often indicate pulmonary hypertension:
- Shortness of breath and difficulty breathing,
- Circulatory problems/circulatory collapse,
- sudden loss of consciousness(syncope),
- fatigue and impaired general condition,
- angina pectoris,
- frequent coughing,
- Raynaud's syndrome (vascular spasms and poor circulation in the toes and fingers),
- cyanosis (bluish coloration of the mucous membranes or skin),
- peripheral edema (water retention in the interstitial tissue),
- Tightness in the chest,
- valvular insufficiency of the tricuspid valve (a heart valve that belongs to the group of leaflet valves).
The prognosis for pulmonary hypertension is usually poor. The survival rate over the next five years with a mean pressure of over 30 mmHg is only 30%. From a mean pressure of over 50 mmHg, it is even lower. Treatment of the clinical picture is difficult.
After an initial visit to the family doctor, the doctor usually refers the patient with a suspected case to a cardiologist (heart specialist). If the cardiologist identifies the cause as complications in the pulmonary circulation, the patient is then referred to a pulmonologist (lung specialist).
Acute pulmonary hypertension should be treated in such a way that it does not develop into a chronic form. Two treatment methods are usually combined. One treatment method consists of uncovering possible causes and combating them. A second treatment method aims to inhibit the progression of the disease.
In order to normalize the blood flow again, the resistance in the vessels must be reduced. Suitable drugs for this purpose include
- PDE-5 inhibitors,
- calcium antagonists or
- prostacyclin derivatives.
Those affected must follow strict rules to support the treatment of the disease. They must change their lifestyle and give up a number of things, including
- Long-term stress,
- physical exertion
- nicotine and
- alcohol.
Chronic PAH is irreversible and its symptoms can only be alleviated. Painkillers and medication are used to inhibit the symptoms.
A cure is only possible with the help of a lung or heart transplant using a donor organ.
