Download Pulmonary arterial hypertension (PAH) with congenital heart

PAH–CHD Information Booklet Contents 20/12/13
Pulmonary arterial hypertension (PAH) with congenital heart disease (CHD) /
Eisenmenger's syndrome
What it means for you
Acknowledgements
This booklet is a joint project between The Somerville Foundation and Actelion Pharmaceuticals
UK Ltd. Funding was provided by Actelion Pharmaceuticals UK Ltd.
We are grateful to our committee of experts who helped write this booklet:

Kostas Dimopoulos, Consultant Cardiologist, Royal Brompton, London

Linda Griffiths, Adult Congenital Heart Specialist Nurse, Manchester Royal Infirmary
We would also like to thank the people with PAH–CHD who shared their experiences. Their
insights supported the development of this booklet.
1
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
Introduction
This booklet provides information to help you understand pulmonary arterial hypertension (PAH)
with congenital heart disease (CHD). This includes how the conditions are linked and their effect
on you.
In this booklet, you can find out about:

How the heart and lungs work together

Pulmonary arterial hypertension with congenital heart disease (PAH–CHD)

Diagnosing and managing PAH–CHD

Practical advice for lifestyle, travel and work
By knowing more about your condition, you can work together with your medical team to help
manage your condition and reduce the impact that it has on your life.
Table of contents
2

How do the heart and lungs work?
3

What is congenital heart disease (CHD)?
5

What is pulmonary arterial hypertension (PAH)?
8

Diagnosing and managing PAH–CHD
10

Living with PAH–CHD
15

Working and PAH–CHD
19

Travelling and PAH–CHD
20

Relationships and PAH–CHD
22

Getting support
23

What to do in an emergency
26
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
How do the heart and the lungs work?
To understand how pulmonary arterial hypertension with congenital heart disease (PAH–CHD)
affects you, it is useful to know how the heart and lungs work together to supply your body with
oxygen.
Your body needs oxygen to work properly and carry out essential functions. You get oxygen from
the air you breathe. Your lungs breathe in air and oxygen is absorbed by your blood. The heart
pumps the blood around your body to deliver oxygen to all your cells. The heart also pumps the
blood back through the lungs to remove carbon dioxide and collect more oxygen.
The heart uses four chambers to do this:

Two collecting chambers at the top – called the right and left atriums

Two pumping chambers at the bottom – called the right and left ventricles
The right side of the heart pumps blood that has used up its oxygen (de-oxygenated or ‘blue’
blood) back to the lungs where it absorbs more oxygen. The blood, which is now full of oxygen
(oxygenated or ‘red’ blood), flows to the left side of the heart, which pumps it back around the
body.
The left side of the heart pumps blood at high pressure. The right side of the heart works under
lower pressure as it only has to pump the blood through the lungs.
Normally blue and red blood does not mix.
3
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
Right side
Left side
[Simplified diagram to be redrawn similar to this with labels for heart, lungs, veins, arteries, right
side, left side and arrows to show direction of blood flow. Ensure that pulmonary artery, pulmonary
vein and pulmonary capillaries are shown on diagram]
4
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
What is congenital heart disease?
Some people are born with a problem in the structure of the heart – this is known as congenital
heart disease (CHD). Congenital means that it is present at birth.
Congenital heart problems can mean that there is a ‘hole’ in the heart. The ‘hole’ can allow blood to
flow from one chamber to another. Many people can have a small ‘hole’ in the heart and have no
effects. A ‘hole’ in the heart allowing blood to mix is also known as a shunt.
For some people, a ‘hole’ in the heart can affect how much oxygen there is in the blood going to
their body. If the ‘hole’ is in the heart wall between the left side and right side of the wall, it means
blue blood and red blood can mix. This happens when blue blood moves to the left side of your
heart and mixes with red blood.
5
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
[PULL OUT BOX]
What is a shunt?
A shunt is a ‘hole’ in the heart which allows blue blood and red blood to mix.
Shunts are described as:

Left-to-right (red-to-blue) or

Right-to-left (blue-to-red) or

Bidirectional (flowing both ways)
[Diagram to be simplified and adapted. Additional aspects to be included: Ventricular septal
defect (left-to-right shunt) and patent ductus arteriosus (PDA) (usually left-to-right shunt)]
Symptoms of CHD
People born with CHD can experience a number of symptoms, which may include:
6

Chest pain

Shortness of breath

Blue-coloured skin, particularly on the fingers, toes and lips (called cyanosis – see box)

Getting tired easily, particularly after exerting yourself
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
[PULL OUT BOX]
What is cyanosis?
Blue blood contains less oxygen than red blood. If blue blood shunts to the left and mixes with
red blood, less oxygen is delivered to the body.
As a result, the body makes more haemoglobin to help your blood carry more oxygen. Your
blood also becomes thicker as a result. This is known as cyanosis.
How does cyanosis this affect my health?

You may notice a blue colour to the skin, particularly on the fingers, toes and lips

You may be more prone to headaches
It is rare for people to experience major side effects from cyanosis, so procedures to remove
blood and decrease the amount of haemoglobin in the blood are discouraged.
Types of CHD
CHD can be more complex than just a simple ‘hole’. For example:

You may have a large or small ‘hole’ in the heart. Over the years, this may have allowed
too much blood to flow to the lungs

You may have developed abnormal blood vessels between your lungs and your heart,
which resulted in too much blood flowing to the lungs

7
Sometimes a ‘hole’ is left after your heart has been repaired
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
What is pulmonary arterial hypertension?
Pulmonary arterial hypertension (PAH) is a condition in which blood is pumped through the lungs at
high pressure. The high pressure makes it difficult for blood to go through the lungs to capture
oxygen. This can leave you feeling more tired than usual.
I have congenital heart disease (CHD) – why do I also have PAH?
PAH can be caused by a variety of diseases, including CHD. Not all CHD will result in PAH.
“Because I have always been so focused on my heart condition, it took me a
long time to understand the fact that I have a heart and a lung condition...”
The most common reason for PAH to develop in patients with CHD is a ‘hole’ (or shunt) in the
heart. PAH can also be caused by an abnormal connection between the large blood vessels.
A ‘hole’ in your heart can cause too much blood to flow to the lungs. The blood vessels in the lungs
may react by becoming thicker and narrower. The heart (in particular the right pumping chamber)
has to pump harder. Over time, this can lead to a strain on the heart, which may fail.
PAH may also develop or persist immediately after an operation to repair a heart defect, or may
arise many years later. Finally, you may only have a small ‘hole’ in your heart but still develop PAH
– the reasons for this are not clear.
What are the symptoms of PAH in patients with CHD?
Many people remain very well for many years. The onset of symptoms tends to be gradual and can
vary from person to person.
The symptoms of PAH are similar to those of CHD. You may notice the following:

You have gradually become more tired, breathless and less able to do things that you used
to be able to do easily

You may notice a build-up of fluid around your stomach and ankles

You may develop cyanosis (see Page [7] to find out more about cyanosis)

You may start feeling dizzy when you exert yourself

You may feel your heart racing, going too slow or beating quite irregularly – this can result
in feeling dizzy or fainting

8
You may experience chest pain or discomfort, especially when you are more active
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
What are the different types of PAH–CHD?
There are several types of PAH–CHD. They vary depending on the cause and the severity. They
also have different symptoms.
“When I was diagnosed, I just wanted to know the meaning of Eisenmenger’s
syndrome...”
The four groups of PAH–CHD
A
Eisenmenger's

Very high blood pressure in the lungs
syndrome

Right-to-left shunt: blue blood flowing through to the left side of
the heart, leading to cyanosis at rest or when you exert
yourself
B
PAH with left-to-

Mild-to-moderate increase in blood pressure in the lungs
right shunt

Left-to-right shunt: red blood flowing through to the right side of
the heart
C

No cyanosis at rest
PAH with small

High blood pressure in the lungs, not clearly due to the ‘hole’
‘hole’

Right-to-left shunt: blue blood flowing through to the left side of
the heart, leading to cyanosis at rest or when you exert
yourself
D
PAH after

closing a ‘hole’
Blood pressure in the lungs remaining high or increasing after
closure of a ‘hole’

Usually no cyanosis at rest, unless the ‘hole’ persists
Different types of PAH–CHD can have different treatments. Please speak to your doctor treating
your PAH and/or CHD about which group you belong to and what this means for you.
9
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
Diagnosing and managing PAH–CHD
Many patients with PAH–CHD are managed at specialist pulmonary hypertension centres. These
centres may share a location or work closely together with adult congenital heart disease (ACHD)
centres. You may have heard these referred to as grown-up congenital heart disease (GUCH)
centres. You may be cared for by a hospital with a shared care arrangement between the two
centres, or be looked after by a specialist congenital heart disease team.
To find specialist centres near you, visit:

ACHD centres: www.thesf.org.uk/help-advice/specialist-centres.aspx

PAH centres: www.phassociation.uk.com/treatment_for_ph/ph_specialist_centres.php
How is pulmonary arterial hypertension (PAH) diagnosed?
If you have congenital heart disease (CHD), your doctor will routinely carry out tests to see if you
have PAH. You doctor will assess the function of your heart using an:

Electrocardiogram (ECG) – this measures the electrical activity of the heart

Echocardiogram (heart ultrasound) – this measures the shape and size of the heart and
can provide information on PAH
[include images of ECG and echocardiogram]
Your doctor might suggest cardiac catheterisation. During this procedure, a very thin, soft tube
called a ‘catheter’ is used to measure the blood pressures in the heart and lungs. This procedure is
usually done under a local anaesthetic. The catheter is guided into the heart through a blood
vessel in the leg, neck or arm. Cardiac catheterisation is the most reliable way of finding out
whether someone has PAH. It can also measure how severe PAH is and which treatments might
be effective.
How is PAH assessed?
To help make the best treatment decisions for you, you and your doctor will need to think about
how much PAH affects your everyday life.
The World Health Organization (WHO) has developed a system to grade PAH. Your doctor will use
it to assess your condition and the effect that it has on your life. You may find that your Class
changes over time.
10
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
Functional classification of PAH from the WHO
WHO Class I

There are no symptoms of PAH with exercise or at rest

You do not notice breathlessness or chest pain, or feel like
you are going to faint, when you undertake ordinary physical
activities (such as walking, simple household chores,
shopping etc)
WHO Class II

Your physical activity is slightly limited

Ordinary physical activity makes you feel breathless,
fatigued, chest pain or like you are going to faint, but you feel
comfortable at rest
WHO Class III

You physical activity is noticeably limited

Less than ordinary activity causes breathlessness, or fatigue,
chest pain or near-fainting, but you feel comfortable at test
WHO Class IV

You cannot undertake any physical activity without
experiencing symptoms

There are signs of right heart failure

You may feel breathlessness and/or fatigued even at rest

Your discomfort is increased by any physical activity
How is PAH treated?
You may be given treatment to lower the blood pressure in your lungs. This should improve your
ability to be active without becoming very breathless or tired.
There are different types of medication. You should discuss with your consultant or specialist nurse
which treatment is likely to work well for you and any potential side effects that it may cause.
The different medications can be taken in the form of:

Tablets, by inhaling medication from a special piece of equipment (inhaled nebuliser) or

A mini-pump to administer the medicine straight into the bloodstream (intravenous infusion)
You may also need extra oxygen. [Include images of nebuliser and minipump]
11
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
The types of medication that may be prescribed to treat PAH are:

Phosophodiesterase type 5 (PDE5) inhibitors (tablets): sildenafil or tadalafil

Endothelin receptor antagonists (ERA) (tablets): bosentan or ambrisentan

Prostanoids (inhaled or delivered into the bloodstream): epoprostenol (intravenous),
iloprost (nebulised)
Treatment usually starts with tablets or a combination of tablets. They should improve your PAH
symptoms and help keep your condition stable.
Over time, you may need to change how you take your medication. Additional tablets or adding
inhaled or injectable therapy may help you to control your condition.
How often should I see my doctor?
Everyone with PAH should see a specialist approximately every 3–6 months. This is especially
important if you are taking medication.
You should stay in close contact with your healthcare team between your clinic appointments.
Contact your specialist nurse if you have any concerns.
If your condition worsens, contact your specialist nurse immediately.
How can I prepare for a clinic visit?
It can be helpful to keep a diary. You can keep track of the changes in your symptoms and the
amount of activity you can do.
“I write everything down in a diary and I also write how I feel at the end of every
day. I mention anything that stands out at my appointments when my doctor
says ‘How are you?’”
A diary can help you check how your symptoms have changed over time. This will help you to see
any changes in how you feel and what you can do over a period of weeks and months. Keep track
of any differences between the amount of activity you can do compared with your friends or family
and how this has changed over time.
As you fill in your diary, make a note of any questions you have. Your appointments are a good
time for you to discuss any queries or concerns you may have about your condition or treatment
with your doctor or nurse.
12
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
What will happen during a clinic visit?
During your clinic visit, your doctor may perform some tests. This may include an echocardiogram
(an ultrasound of the heart), electrocardiogram (ECG), blood tests and an exercise test. They will
use the results to see if they need to change any aspect of your care.
Your doctor may also ask questions about your daily life and how you have been since your last
visit. Even if you are asked a general question, try to give specific examples using every everyday
activity, such as:

The distance you are able to walk

The number of stairs/steps you can climb

Washing, cleaning, cooking, gardening etc

Sports and exercise

Playing with children

Coping with work

Shopping
Try using everyday activities and landmarks to check your progress. For example, “I was able to
walk from the bus stop to home without stopping – now I have to stop halfway to rest”.
You can bring your diary with you to help you remember how your symptoms have changed since
your last clinic visit.
How will I know if my treatment is working?
Once treatment for PAH has been started, it is important that you take it regularly, as agreed with
your healthcare team.
You may not notice any changes straight away. It can take up to 3–4 months before you know
whether a treatment is working for you. It is important to give your treatment time to have a
noticeable effect.
Are everyday activities, such as walking, climbing stairs or shopping, becoming more difficult? If
they are, perhaps your treatment is working as well as it used to. Keep in mind that you may notice
small changes in your symptoms during periods of intense cold or very warm weather. In addition,
chest infections can make symptoms worse for a short or even a longer time, as people with PAH
may find it more difficult to recover. Speak to your healthcare team about having an annual flu and
pneumonia vaccination.
13
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
If you feel concerned about how well your treatment is working, or if you feel like your symptoms
are getting worse, talk with your PAH and ACHD specialist doctors, who will examine you, perform
tests and talk with you about the best option. If your treatment is not working well, you may be able
to change to a different treatment or add another treatment to your existing one (this is known as
combination therapy).
It is important to fully understand any changes to your treatment, so don’t be afraid to ask
questions and contact your team at any point.
You should not stop taking your treatment without first talking to your doctor or specialist nurse. If
you are experiencing significant side effects, you should discuss this with your doctor or specialist
nurse as soon as possible.
Will I eventually need transplantation?
Some PAH–CHD patients may be referred for transplantation. The availability of effective drugs to
treat PAH has delayed the need for transplantation. If transplantation is required, a heart–lung
transplantation is often required for patients with PAH–CHD.
14
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
Living with PAH–CHD
It’s important for you and your family to understand your condition, so you can manage your health
and its impact upon your life in the best way possible. In this section, we will discuss how
pulmonary arterial hypertension with congenital heart failure (PAH–CHD) can affect your daily life,
and ways that you can minimise its impact.
Understanding the condition
PAH–CHD is not a very common condition, and your family and friends may find it difficult to
understand it. Some healthcare professionals outside of your specialist team may not be very
familiar with your condition. You might find it useful to share this booklet with them.
It is important that you know the name of your condition, your treatment and who to contact in an
emergency (see What to do in an emergency on Page [25]).
It is useful to carry copies of up-to-date clinical letters with your diagnosis, current status and
treatments. Some centres may provide a Patient Passport to hold important information about your
condition. Alternatively, you may wish to use the Personal Health Passport also available on The
Somerville Foundation website (www.thesf.org.uk).
PAH–CHD and learning disabilities
People with Down’s syndrome are more likely to have complex cardiac problems, and so are at
risk of developing PAH–CHD. If you are caring for someone with a learning disability, the
healthcare team will be able to help you and the person you are caring for to understand the
condition.
Day-to-day life with PAH–CHD
Life with PAH–CHD differs from person to person and from day to day. You will probably have days
when you can carry out most everyday activities, such as going for a walk or shopping. At other
times, you might have little energy or feel unwell.
“On a good day I can just do normal things...”
“On a bad day I just don’t feel right. I have no get up and go, whatsoever...”
Build in additional time to keep active when you can, and balance time for activities with time for
15
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
rest. Your PAH therapies are there to improve your ability to cope with physical activity and so
make it easier for you to perform ordinary activities.
If you have had PAH since childhood, you might underestimate its impact on your life in terms of
limiting your day-to-day activities. Discuss with your specialist doctor the different options available
to help control your PAH symptoms and help you with your daily activities.
Spending time talking with friends and family can be an important part of feeling positive. Online
patient support groups can be a good way to get in touch with other people with PAH–CHD or
similar conditions, and to share experiences and support one another. You can find out about
different patient support groups available on Page [23].
Although these groups may be a good source of support, your healthcare team will be the most
accurate source of information relating to your condition.
Finding out about activities or groups in your local area could be a helpful option for you. When you
go to your appointments, look out for information around the clinic, or just ask.
Do I have to change what I eat and drink?
Eating a healthy balanced diet is a good idea for everybody, and even more so if you have PAH–
CHD. What you eat can make a big difference to the health of your heart.
Do talk to your PAH and ACHD healthcare teams about your diet. Some helpful suggestions to
build into your daily diet and shown below:
Aspects of your diet to consider
Advice for eating well
Make sure you eat plenty of foods that are high
 Eat fresh vegetables and fruit
in fibre
 Choose wholegrain breads and cereals
Try to avoid fatty meats
 Choose lean chicken, fish and pulses
Limit saturated fats
 Drink skimmed or low-fat milk
 Use vegetable oil instead of butter where
you can
Avoid hydrogenated oils (trans fats)
 Trans fats are found in margarine, chips and
fast food
16
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
Be careful about how much salt you eat or put
 Don’t add salt to your food during cooking
on your food
 Smoked meats, snacks and processed foods
can contain high amounts of salt
Don’t drink too much coffee
 Try drinks with little or no caffeine such as
herbal tea (although some herbal teas
contain caffeine, so check the label)
Avoid or reduce alcohol intake
 One or two alchoholic drinks are the current
recommended maximum daily intake
 It is best not to binge drink
Smoking and PAH-CHD
It is best not to start smoking or to give up as early as possible. Not smoking is particulary
important if you are waiting for surgery, as it can increase your risks of complications.
Speak to your GP or practice nurse if you would like to find out about the support available to help
you quit smoking.
Am I at risk of developing a heart infection?
Endocarditis is an infection of the inner lining of the heart. People with CHD are at risk of
developing endocarditis, whether or not they have had an operation to repair their congenital heart
defect. It is relatively rare but can have serious consequences if not treated as soon as possible.
Please discuss endocarditis with your healthcare team during your visits.
Endocarditis can develop quite slowly over a number of weeks or months, or it can develop quite
quickly. You may feel generally unwell, with night sweats, low-grade fever, general aches and
pains, or lack of appetite. A fever develops in most cases. Please contact your team immediately if
you experience any of the above.
You should take steps to reduce your risk of developing endocarditis:

Make sure you keep your teeth and gums clean and healthy. Be careful not to eat too many
sugary foods, which can lead to tooth decay. Visit your dentist for regular check-ups, and
let your dentist know that you have PAH–CHD and may be at a greater risk of developing
endocarditis, so that they can appropriately care for you dental health

17
Alert any health professional who may need to give you treatment or perform a procedure
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13

There may be a risk of infection with body piercing or tattooing. Before deciding to get a
piercing or tattoo, please speak to your healthcare team
18
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
Working and PAH–CHD
Most people with pulmonary arterial hypertension with congenital heart failure (PAH–CHD) are able
to work, although they may not be able to carry out some jobs that require a lot of physical activity,
such as heavy lifting. What you are able to do depends on the severity of your disease. If you are
not sure, talk to your PAH and CHD healthcare team about what is manageable and safe.
“If I have a busy day, I just know that I need to get rest the day before – it is
critical...”
Your employer has a legal obligation to make reasonable adjustments to both your job role and
your workplace. For example, they may need to provide special equipment, or adjust your working
hours or duties.
You may find that larger employers are better able to support you with any changes you need. If
you are a member of a trade union or trade association, they should be able to help with any
issues. Always answer questions from your employer or prospective employer to the best of your
knowledge.
Check if you have any legal cover through your household insurance, as some policies include
cover for medical and employment issues. You can also contact The Somerville Foundation
(www.thesf.org.uk) if you cannot obtain free legal advice through any other means.
For more information regarding employee rights and preparing for job interviews, visit:
19

www.gov.uk/rights-disabled-person/employment

www.gov.uk/looking-for-work-if-disabled/looking-for-a-job

www.dls.org.uk
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
Travelling and PAH–CHD
Most people who have pulmonary arterial hypertension with congenital heart disease (PAH–CHD)
are able to go on holiday in the UK or abroad when they are stable and well. Make sure you
choose activities that are suitable for you.
You should get comprehensive medical insurance before travelling abroad. You will need to tell
them about your condition to ensure that your insurance is valid. You may have to speak to several
insurance providers to get the cover you need. Make sure you plan your insurance well in advance
of your holiday.
If you are having trouble finding insurance, it may be helpful to discuss any difficulties on The
Somerville Foundation, British Heart Foundation (BHF) or Pulmonary Hypertension Association UK
(PHA UK) forums:

www.thesf.org.uk/help-advice/resources

www.bhf.org.uk

www.phassociation.uk.com/forum
Flying and PAH–CHD
People with PAH–CHD should not necessarily feel that they cannot use commercial flights.
If you use oxygen, you may also require it during the flight. This is quite easy to arrange through
some airlines and may be free of charge. It is always best to check with your airline first. You may
also need to check with your travel providers about permission to take your oxygen on trains,
ships, coaches or aircraft, and there may be restrictions on the size of the cylinder you can take.
Oxygen concentrators are smaller than oxygen cylinders, and may be a better option for travel.
It’s important to move around regularly and drink plenty of water to ensure that you remain well
hydrated, both during flights and in warm weather.
Be careful in airports:

If you need to walk long distances to your gate, you can ask for assistance (e.g.
wheelchairs or other transport). Arrange this well in advance of your travel day

Do not carry heavy luggage

Remember to keep well hydrated in the airport – this is particularly important if you cannot
take water with you through airport security
20
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
Some tips for taking medicines on airplanes:

Carry medicines in their original containers

Always carry your medicines in your hand luggage, rather than check-in luggage

If you need to carry syringes, make sure you have a letter from your doctor

In case of queries, it may be useful to take a copy of your prescription
Take care during long bus, train and car journeys:

Pull over, stand up or move around about every 1–2 hours

Ask your doctor about whether you need to take aspirin or another treatment to reduce the
risk of deep vein thrombosis (DVT) for long journeys (i.e. over 3–5 hours)

There are also leg exercises you can do in your seat to reduce the risk of DVT
Obtaining oxygen while on holiday
If you use long-term oxygen and are travelling within the UK, you should be able to have your
oxygen prescription available at your destination. Discuss your oxygen requirements with your
oxygen supplier at least 2 weeks before you travel.
If you are travelling abroad, your oxygen requirements need to be organised separately, and you
may be charged. Speak to your oxygen supplier at least 3 weeks before your travel.
Different oxygen suppliers have service teams available who may be able to help arrange oxygen
at your destination. Contact your oxygen supplier to find out more.
21
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
Relationships and PAH–CHD
Just because you have pulmonary arterial hypertension with congenital heart disease (PAH–CHD)
doesn’t mean that you can’t have a healthy love life. Through open communication with your
partner about how you feel and what you desire, you can enhance your feelings of trust and
confidence, and talk through any issues or worries you may have.
If breathlessness and fatigue are restricting your love life, try having sex when you’re feeling at
your best – perhaps in the morning or early afternoon. You might consider finding time for sex
shortly after taking your medication. This is when the positive effects of your treatment are most
noticeable.
Some sexual positions may be difficult or uncomfortable for people with PAH–CHD. It is important
that you avoid positions that put weight on your chest, so that you can breathe freely. Be open and
honest with your partner, and you can find positions and techniques that you both enjoy.
Pregnancy and contraception
Pregnancy can be dangerous for women with PAH–CHD. It can put strain on the heart and lungs,
and some of the treatments you may be taking may be harmful to a developing baby.
The use of contraception is therefore very important, so ask your ACHD or PAH healthcare team
where you can get appropriate contraception and which methods are suitable for you.
Looking after your family
Caring for children can be quite physically demanding and you may find it difficult to keep up with
them. There are many people with PAH–CHD who manage to care for their family with few
problems, providing they get enough support from friends and family and tailor activities to suit
their abilities.
22
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
Getting support
There may be different ways you can obtain support, including financial and travel support, and
support from other people with the same condition.
During your appointments, don’t forget to ask about local services and activities that are available
in your area.
Financial support
There is a range of allowances available from the government to support people with health issues
and their carers. Some people who have pulmonary arterial hypertension with congenital heart
disease (PAH–CHD) may be eligible for this kind of financial support.
Find out more about UK disability benefits here: www.gov.uk/browse/benefits/disability
OR you can talk to an adviser about which benefits you might be entitled to on
0800 882 200
OR you can fill in an online form to find out more about benefits here: www.gov.uk/benefits-adviser
In Northern Ireland you can get advice about benefits from Disability Action here:
www.disabilityaction.org
OR apply for benefits online at: www.nidirect.gov.uk/index/information-and-services/people-withdisabilities/health-and-support.htm
Travel support for hospital appointments
There are travel cost support schemes run by healthcare services in Great Britain and Northern
Ireland. There may be transport organised by your hospital for outpatient appointments. Keep in
mind that travel support for hospital appointments may not extend to companions.
For England, Scotland and Wales see:
www.nhs.uk/nhsengland/healthcosts/pages/travelcosts.aspx
OR telephone: 0300 330 1343
In Northern Ireland see:
www.nidirect.gov.uk/hospital-travel-costs-scheme
23
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
Patient support groups
The Somerville
Supporting people with a
www.thesf.org.uk
Foundation
congenital heart condition
Tel: 0800 854759
offering help and advice. Forum
Email: [email protected]
for people with a congenital
Facebook:
heart condition
www.facebook.com/thesomervillefoun
dation
Twitter: @_thesf
Pulmonary
UK charity for people with
www.phassociation.uk.com
Hypertension
pulmonary hypertension.
Tel: 01709 761450
Association UK
Provides information, organises
Email: [email protected]
events and funds research
Twitter: @PHA_UK
Pulmonary
Online forum for people with
www.phassociation.uk.com/forum/def
Hypertension
pulmonary hypertension and
ault.asp
Association UK
their family and friends
forum
Pulmonary
An association for people living
www.pulmonaryhypertension.ie
Hypertension
in the Republic of Ireland who
Tel: (01) 8034420/4423
Association Ireland
have pulmonary hypertension.
Email: [email protected]
Organises an annual conference
Twitter: @pha_ireland
and has a newsletter
Down’s Heart Group
Offers support to people with
www.dhg.org.uk
Down syndrome who have heart
Tel: 0844 288 4800
conditions and their families
Email: [email protected]
Facebook:
www.facebook.com/groups/downshea
rtgroup
British Heart
Help and advice on heart
www.bhf.org.uk
Foundation
disease and an online forum to
Tel: 0300 330 3322
share experiences
Email: [email protected]
Facebook: www.facebook.com/bhf
Yheart
24
Information, games and advice
www.yheart.net
for young people with heart
Facebook:
conditions
www.facebook.com/yheartfan
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
Twitter: @Yheart_Tweets
Irish Heart
Provides information and
www.irishheart.ie
Foundation
advocacy for people with heart
Tel: 1890 432 787
disease and their families
Facebook:
www.facebook.com/irishheartfoundati
on
Twitter: @Irishheart_ie
“Because my condition is so rare, I can’t meet anyone with the same problem
where I live so I asked on the forum if anyone had the same as me and I now talk
to the person I met regularly...”
25
Date of preparation: December 2013; PAH 13/0024
PAH–CHD Information Booklet Contents 20/12/13
What to do in an emergency
Before any emergencies arise, make a clear plan with your team on exactly what you should do in
an emergency. They will be able to tell you what to look out for, given your individual condition and
the medication you are taking.
Keep a copy of the latest letter containing your clinical information with you, along with an up-todate list of your current medication and contact details of your specialist teams. You may also wish
to carry your Patient Passport if you have been supplied one by your healthcare team, or a
Personal Health Passport which is available on The Somerville Foundation website
(www.thesf.org.uk).
In some areas of the country it is possible to register your details with your local ambulance
service. This means that when they receive a 999 call from your address they know that you have
a complex condition and that you are normally cared for by a specialist team at a specific hospital.
The ambulance staff then have the option of taking you to that hospital rather than your local A&E.
Please speak to your specialist team about this.
Your emergency contact numbers [to appear on the inside front, inside back or back cover]
Make a note of your emergency contact numbers below.
Emergency pulmonary arterial
Name:
hypertension (PAH) contact
Clinic:
details
Phone number:
Emergency congenital heart
Name:
disease (CHD) contact details
Clinic:
Phone number:
General practitioner (GP)
GP name:
contact details
Practice name:
Phone number:
26
Date of preparation: December 2013; PAH 13/0024