This increased pressure causes progressive damage to the heart and lungs.
When you exercise the heart beats more quickly to get more oxygen to the muscles. At the same time the pulmonary arteries expand to allow more blood through so that more oxygen can be carried to the muscles. They expand by stretching outwards slightly to create a larger inner area. In a person with PH, the walls of the pulmonary arteries are thicker, so are less able to stretch.
Because of this the heart has to work harder to pump blood to the lungs, especially during exercise. If the heart has to work harder than usual over a long period of time then it begins to work less effectively and damage occurs. PH affects many different types of people. In most people with PH, it is associated with another medical condition:
• portal hypertension
• connective tissue disease, eg systemic sclerosis
• HIV infection
• congenital heart disease
• sickle cell anaemia.
Some people develop PH with no known cause which is referred to as idiopathic PH and in some rare cases it can be inherited. Your stage of PH is classified as shown in the table below, depending upon when your symptoms occur.
WHO classification of PH: Class Description
1 No symptoms of any kind. Physical activity does not cause any symptoms
2 Comfortable at rest, but symptoms occur with ordinary physical activity
3 Comfortable at rest, but symptoms occur with less-than-ordinary effort (eg lifting the arms)
4 Symptoms while resting Treatment for PH can be split into three categories:
• Conventional therapy (often called background therapy), which can include the following
• Targeted therapy
• Calcium channel blockers
• Endothelin receptor antagonists
• Phosphodiesterase 5 inhibitors
• Pulmonary endarterectomy
• Atrial septostomy
• Transplant surgery
Most patients will have a regime involving a combination of background and targeted treatment which varies from person to person depending upon the cause of the PH and what stage they are at. Many patients will need oxygen therapy, although some only need it at night. Oxygen therapy increases the amount of oxygen in the blood and it can also help to relax the arteries in the lungs which leads to reducing the pressure in the pulmonary arteries.
Oxygen therapy can reduce tiredness and breathlessness in some people with PH and it can improve concentration and the ability to do everyday tasks. Having PH can make you tired and lethargic. This may make it more difficult to do ‘normal’, everyday things. Here are some tips from the PHA website from patients to help make life a little easier.
• Where possible, avoid bending, lifting or over-stretching when doing housework.
• Use a lighter vacuum cleaner and iron. • Do jobs sitting down where possible – a kitchen stool can really help. In bed
• If your duvet is heavy, consider buying a lighter one.
• Use extra pillows to raise your head and make it easier to breathe.
• If you use an oxygen concentrator, consider putting it outside the bedroom to avoid the noise disturbing you.
• After a bath, open windows before the house becomes too humid.
• Have chairs ready for places where you stand (eg, shaving or applying make-up).
• Slightly larger clothes can be less tiring to put on and take off.
• Put on a bathrobe straight after a bath or shower to avoid having to towel yourself dry.
• If bending down to put your shoes on is difficult, use a long-handled shoehorn.
Out and about
• Plan ahead to avoid having to rush.
• Consider asking for a wheelchair.
• If you drive, carry spare medication in the car.
References: http://www.phassociation.uk.com and http://www.who.int