What’s Pulmonary Hypertension ?
Pulmonary hypertension (PH) is a disease in which the lung vessels are constricted due to different causes. This causes the blood pressure increases in the pulmonary circulation which is called ‘’ pulmonary hypertension’’.
The blood is transported back from the heart to the lungs. Deoxygenated blood passes from the right ventricle via the pulmonary trunk, which divides into the right and left pulmonary artery to the lungs. There, it is again enriched with oxygen
The resistance in the lungs is increased through the narrowed vessels in pulmonary hypertension. This makes difficult the right chamber of the heart to pump blood through the lungs. As a result, the blood flow through the arteries of the lungs are disturbed and blood pressure is elevated and right ventricle becomes overloaded. Further consequences can be a circulatory disorder of the lungs and a deterioration of the oxygen uptake in the lungs. In the most severe case of pulmonary hypertension leads to heart failure.
In an early stage of pulmonary hypertension often causes no symptoms. Symptoms occur when disease progresses.Because , body will no longer be supplied in the case of pulmonary hypertension with sufficient oxygen and sufferers will be severely limited in their physical performance, quickly exhausted and complain among other symptoms such as shortness of breath.It pulmonary hypertension is not treated , pulmonary hypertension becomes fatal.
In medicine, we distinguish between the primary and secondary pulmonary hypertension: In very rare cases, pulmonary hypertension occurs as a distinct disease (e.g., by inheritance), then it is referred to as primary or idiopathic pulmonary hypertension ( IPAH).
If trigger of a pulmonary hypertension are diseases or certain drugs, then it is called’’secondary pulmonary hypertension.’’.
Pulmonary Hypertension Symptoms
Pulmonary hypertension symptoms often does not exist in an early phase of the disease.There can be complaints , if disease is progressive. Due to the reduced oxygen supply to the lungs, they are restricted in their efficiency. Typical symptoms of pulmonary hypertension are:
- Performance weakness
- Rapid onset of fatigue
- Shortness of breath
- Brief loss of consciousness (syncope) during physical exertion
- Bluish discoloration of the skin and lips (cyanosis)
- Chest pain
- Swelling due to fluid deposits in tissues (edema), especially in the legs
Due to narrowed vessels, oxygen supply to the lungs worsens. On the other hand, heart requires more and more energy to pump the blood through the vessels. The heart has to beat faster and this results heart rate increase. The heart is burdened, especially the right atrium and the right ventricle are affected. It may develop right heart failure.
Pulmonary Hypertension Causes and Risk Factors
A pulmonary hypertension is defined as the increase in average blood pressure in the pulmonary artery. In a healthy person, the value for the pulmonary pressure is under 20 mmHg.If pulmonary pressure is present value rises above 25 mmHg.
The cause of a pulmonary hypertension is a narrowing of the pulmonary vessels which decreases the volume of vessels and reduces the blood flow. The reason for the vasoconstriction is an imbalance of certain neurotransmitters that regulate the vasodilatation and vasoconstriction. The cause of this imbalance is unknown.
Doctors distinguish primary from the secondary pulmonary hypertension. The primary or idiopathic pulmonary hypertension (IPAH) acts as an independent disease with known cause.Thus, there is no underlying disease as a cause. IPAH can be inherited.
Both forms of pulmonary hypertension can be seen together rarely: Number of new cases of both forms together are seen one to three cases per million population per year.
If a specific underlying disease is the cause of pulmonary hypertension , then it is called ‘’secondary pulmonary hypertension’’ by the doctors. . Especially patients with left heart failure or chronic lung disease often suffer from pulmonary hypertension.
Diseases that can lead to pulmonary hypertension are :
- COPD (chronic obstructive pulmonary disease)which is the most common cause of pulmonary hypertension.
- Pulmonary fibrosis
- Connective tissue disorders such as crest syndrome or scleroderma
- Pulmonary embolism (blockages of blood vessels in the lungs)
- HIV infection
- Left heart disease
- Liver disease
- Schistosomiasis (bilharzia):This is the most common cause of pulmonary hypertension which is caused by worms in South America.
Depending on the cause, pulmonary hypertension is divided into five categories:
- Pulmonary arterial hypertension (PAH)
- Pulmonary hypertension associated with left heart diseases
- Pulmonary hypertension associated with lung diseases and / or hypoxemia (decreased content of oxygen in the arterial blood)
- Pulmonary hypertension due to chronic thrombotic and / or embolic disease
- Pulmonary hypertension due to other not classified diseases
Pulmonary Hypertension Diagnosis and Investigations
The pulmonary hypertension is often difficult to diagnose because the symptoms are fairly nonspecific and occur also in other diseases at the beginning. A detailed discussion on the medical history (anamnesis) and a physical examination provide important information.
If there is a suspicion of pulmonary hypertension , doctors examine heart and lungs with several methods:
An ultrasound (echocardiography) of the heart is the most important method of analysis in a pulmonary hypertension. Physician enables to examine size,function of heart,heart wall and movements of the heart valves. In addition,It is possible for doctors to see flow of blood in the heart and to detect the velocity of blood flow.Doctor also can estimate increased blood pressure in the pulmonary arteries.
With an X-ray examination of the chest will allow doctor to recognize advanced pulmonary arteries. But this can only be seen in an advanced stage of the disease.
Electrocardiogram (EKG) is also important. Using this method, the electrical activity of the heart is measured: The doctor receives, for example information about the heart rhythm and heart rate.
Lung function test is aslo conducted for the diagnosis of pulmonary hypertension. Spirometry measures to the lung and respiratory volume: In this study, the patient breathes in a special device, the spirometer, which measures the amount of air flowing through. This analysis is used to assess severity, prognosis and progression of pulmonary hypertension. In addition, they may provide clues to the cause of pulmonary hypertension.
To confirm the diagnosis pulmonary hypertension and to determine the severity of the disease:Right heart catheter examination is appropriate. It can be used directly measure the pulmonary arterial blood pressure.
Pulmonary hypertension is divided its severity in for levels :
- No limitation in physical activity.
- Slight limitation in physical activity,normal physical activity leads to increased breathlessness or fatigue, dizzy spells or chest pain.
- Significant limitations in physical activity,light activities provoke complaints.
- Right heart weakness exists, breathlessness and / or fatigue already occur at rest. Lowest activities lead to an increase in symptoms.
Pulmonary Hypertension Treatment
In the case of a pulmonary hypertension,It is either treated the underlying disease that leads to pulmonary hypertension or it is treated to releive symptoms. As a cure of pulmonary hypertension is not possible. The aim is to extend the life expectancy and quality of life.
Treatment of pulmonary hypertension is usually with medications. Hypotensive or vasodilating agents are used depending on the cause of the disease. These include for example:
- High-dose calcium channel blockers
- Prostacyclin derivatives (prostanoids)
- Phosphodiesterase (PDE) 5 inhibitors
If underlying cause of pulmonary hypertension is chronic obstructive pulmonary disease (COPD), patient can be treated with oxygen therapy. In this case drug therapy of pulmonary hypertension does not lead to success, a heart-lung transplant is often the last treatment option.
There is no uniform treatment in pulmonary hypertension – it depends on the respective underlying disease and the severity of pulmonary hypertension. The treatment is individually tailored to the patient.
How to Prevent Pulmonary Hypertension
Pulmonary hypertension occurs only in extremely rare cases.This is a distinct disease which can result chronic diseases. It is important to treat these at an early stage.
Regular check-UPS at the doctor are essential, especially if you already have a disease that is considered to be a risk factor for pulmonary hypertension.
People with pulmonary hypertension should avoid heavy physical efforts. This is especially true for strenuous sporting activities which are not supervised by a doctor, because they can lead to a further increase of pulmonary hypertension. However, a physician-supervised physical training can improve the condition of many patients and a useful supplement to your therapy.
In addition, patients who suffer from pulmonary hypertension must not travel at heights of over 2,000 meters.Because stay in such heights can worsen the condition.Therefore , air travel is potential risk people who are suffering from pulmonary hypertension.
Pulmonary Hypertension Disease Progression and Prognosis
The pulmonary hypertension is usually chronic disease of the lungs or the heart. A pulmonary hypertension is not curable – at worst, it leads to a right heart failure. A treatment can prolong life expectancy and increase the overall quality of life.