Early detection of pulmonary hypertension is important

By Nicola Menke Dec 12, 2011, 3:06 GMT

Berlin - Experiencing shortness of breath at the slightest exertion is not necessarily a sign of being out of shape.

It is also a possible symptom of many disorders, including arteriosclerosis and asthma as well as pulmonary hypertension, a rare disease that often remains undetected and can drastically lower life expectancy.

'Blood is pumped from the right ventricle of the heart through the pulmonary arteries (in the lungs), where it is enriched with oxygen. Then it flows into the left ventricle, which transports it into the body's circulatory system,' explained Ralf Ewert, head of the Pulmonology Department of Germany's Greifswald University Hospital, describing a normally functioning heart.

Ewert said the average pressure in the blood vessels between the right and left ventricles in a healthy person was about 15 millimetres of mercury (mmHg). A pressure of 25 mmHg or more is considered dangerous and known as pulmonary hypertension.

Pulmonary hypertension has a number of possible causes, on the basis of which it is divided into various groups.

'The disease model is idiopathic pulmonary arterial hypertension. There's a genetic predisposition to it, and it hasn't yet been linked to a definite trigger,' Ewert said.

Along with the primary form of the disease are several other, more common, forms, all of which are of a secondary or associated nature. For example, blood pressure in the lungs can become elevated as a result of chronic lung disorders such as smoker's bronchitis, connective tissue diseases such as scleroderma, and pulmonary embolisms.

Common to all forms are abnormal changes in the pulmonary arteries.

'Vasoconstriction occurs, which means the arteries narrow. In addition, blood clots increasingly form and the arterial walls restructure themselves and thicken, which causes further narrowing,' Ewert said. This makes it harder for blood to flow through the lungs, which raises pressure in the pulmonary arteries.

If pressure rises further, blood flow in the lungs is impaired and the body is supplied with insufficient oxygen.

'Fighting the high pressure, the right ventricle becomes increasingly overworked and its pumping performance gets weaker,' noted Michael Barczok, spokesman for the German Association of Pulmonologists. This, he said, could eventually lead to right ventricular insufficiency and heart failure.

Pulmonary hypertension manifests itself in very unspecific ways.

'The main symptom is shortness of breath not commensurate with the degree of physical exertion,' Barczok said.

Other symptoms are fatigue, circulatory problems and extreme weakness. 'At an advanced stage, there are sometimes additional symptoms such as fainting spells and chest pressure.'

Because these symptoms can also be caused by other diseases, pulmonary hypertension is often detected quite late.

'For many people who have it, there's a gap of several years between the first clinical symptoms and the diagnosis,' said Ekkehard Gruenig, head of the Pulmonary Hypertension Centre at the Heidelberg Thorax Clinic.

The insidiousness of the disease, its rareness and the undifferentiated symptoms make a diagnosis difficult. Gruenig said it was essential to heed the warning signs, however, and advised people who displayed them to visit a pulmonary hypertension centre.

'An untreated case of pulmonary hypertension can lead to death within a few years, and in six months if it has progressed to a very advanced stage,' Gruenig warned.

A variety of examinations help to make a diagnosis, including an electrocardiogram, a chest X-ray and an echocardiogram.

To make a definite diagnosis, a right heart catheterization is necessary, Ewert said. It is the only way to detect high blood pressure in the pulmonary vascular system.

Treatment depends on the form of the disease. When pulmonary hypertension has been caused by an illness, then the underlying illness is treated first. If the treatment proves effective, blood pressure drops somewhat. There are also many medications used to treat primary pulmonary hypertension.

'They only work symptomatically, though, and can't effect a cure,' Barczok said. Nevertheless, they substantially stabilize and prolong life expectancy in most cases, he added.

Besides drug therapy, a number of auxiliary measures can have a positive effect on the course of the disease, including the administration of pure oxygen and special breathing and exercise therapy.