What is pulmonary hypertension?

(Explained by a layman)

Pulmonary hypertension is a disease that is as yet incurable and that can affect anybody at any age.

The human heart is a hollow muscle consisting of two pump chambers. The left half of the heart pumps the blood round the body under pressure (blood pressure), which is normally about 120/80 mmHg.

The right half of the heart pumps the blood through the lungs where it is enriched with oxygen. Here, the normal blood pressure is 25/15 mmHg. If the blood vessels in the lungs become constricted and the blood pressure rises, as a result of a wide variety of causes, such as inflammation, blood clots, heart defects, and also for unknown reasons, then the right half of the heart pushes out the blood against an increased resistance. The increased blood pressure causes enlargement of the pump chambers (right heart dilatation), severe cardiac insufficiency and, ultimately, right heart failure.

CHILDREN WITH PULMONARY HYPERTENSION …

…CANNOT:

Romp about, run, play or dance Because they do not have enough breath.
Cycle, roller skate, ski, ice skate or ride Because blood-thinning medications turn them into haemophiliacs and every bruise might be fatal.
Go to nursery, school, parties or events Because of the risk of infection. Every flu turns into a stay in hospital and reduces their life expectancy, which is very short in the first place.
Swim, bathe and splash about Because the life-extending treatment usually takes the form of a permanent infusion over 24 hours.

The disease can lead to death within 3-5 years if left untreated, so it is very important to get a diagnosis as early as possible. Breathlessness during exercise can in rare cases also mean pulmonary hypertension.



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