royal brompton hospital what is pulmonary hypertension? · 2020-05-28 · • pulmonary...

A lifetime of specialist care

What is pulmonaryhypertension?

Royal Brompton Hospital

Pulmonary hypertension_Patient Booklet 28/05/2020 10:55 Page 2

2

• What is pulmonary hypertension? 3

• What are the symptoms of PH? 4

• What causes PH? 5

• How is PH diagnosed? 5

• What are the treatments available? 6

• What to expect during your consultation and hospital visits 8

• Our team 9

• Shared care 11

• Useful contacts 12

• More information 13

Contents

This leaflet provides you with information about pulmonaryhypertension. It does not replace the need for personal advicefrom a qualified health professional. Please ask us if you haveany questions.


3

What is pulmonary hypertension?

You have been referred to Royal Brompton Hospital’spulmonary hypertension service as your doctor thinks there is a possibility that you may have pulmonary hypertension. Youneed to have some tests in order to confirm if this is the case.

This leaflet aims to provide you with some information aboutthis condition and about what to expect during your time in hospital.


Pulmonary hypertension (PH) is arare, serious heart and lungcondition where the pressure inthe pulmonary arteries is higherthan normal. The pulmonaryarteries are the blood vessels that carry blood from the heart to the lungs.

It may be referred to as “highblood pressure in the lungs”. It is important to know that PH isvery different to the morecommon type of “high bloodpressure” that is measured usingyour arm.

To understand PH it is useful tohave an understanding of thenormal flow of blood through theheart and lungs.

The right ventricle is one of thebig pumping chambers of the heart. It pumps blood that hasbeen circulated around the body through the pulmonaryarteries and into the lungs. In the lungs, the blood picks upoxygen and flows back to the left side of the heart, where it is pumped around the body, beginning the cycle again.


4

If the pressure in the pulmonary arteries becomes higher thannormal, the walls of the blood vessels may become thicker andless flexible.

Over a period of time the right ventricle also becomes thick andstiff and has to work harder to pump blood through the lungsand the heart. This means the heart eventually works lesseffectively. This can take several years.

What are the symptoms of PH?

The most common symptoms are:

• Shortness of breath

• Excessive tiredness / fatigue

• Chest pains

• Palpitations (a racing, thumping, or fluttering feeling in yourchest)

• Dizziness

• Fainting or near fainting episodes (known as syncope)

These symptoms can occur at rest or, more often, during periodsof physical exertion and have an impact on a person’s day-to-day life.


5


What causes PH?

There are many different types of PH. Sometimes the cause ofPH is unknown, and this is called idiopathic pulmonary arterialhypertension (IPAH).

More commonly, it is linked to other diseases. Some examples are:

• Heart diseases that you are born with (congenital heartdisease)

• Connective tissue disease such as systemic sclerosis

• Thromboembolic disease (blood clots in the lungs)

• Human immuno-deficiency virus (HIV)

In very rare cases it can be inherited.

How is PH diagnosed?

Patients should be referred to one of seven designatedspecialist centres in the United Kingdom. We are one of them.Information is shared between these centres and well thoughtout protocols (guidelines) have been developed to investigatepatients for PH.

Tests include:

• Blood tests

• Cardiopulmonary exercise – a test to see how your lungs,heart and muscles react together when you exercise ontreadmill or an exercise bicycle

• Chest X-ray

• Echocardiogram – an ultrasound scan of your heart. This scanshows the structure of the valves and chambers in the heartand shows how well your heart is working


6

• CT scan (computerised tomography) – a type of X-ray that gives detailed pictures of your lungs

• MRI (magnetic resonance imaging) – a scan using magnetsand radio waves to create images of the structure of yourheart and find out how well it is working

• Lung function – tests that measure how well your lungs areworking

• ECG (electrocardiogram) – a tracing of the electrical activity of your heart

• V/Q scan – a scan that measures ventilation (breathing) andperfusion (blood flow) in your lungs

• Sleep study – a test that measures your oxygen levels andbreathing overnight.

A confirmed diagnosis of PH is usually made by carrying out atest called a right heart catheterisation, also known as cardiaccatheterisation.

This test involves having a small injection to numb the skin inyour groin, arm or neck. The doctor makes a small cut andplaces the catheter (a thin flexible tube) under your skin andinto a blood vessel that goes to your heart. While the catheteris in place the doctor can see how well blood flows throughyour heart and measures pressures in the heart and lungs.When the test is over the tube is gently removed and pressureapplied to where the cut was made to stop any bleeding.

What are the treatments available?

There is no cure for most types of PH but treatment can begiven to manage your condition and help reduce your symptoms. The treatments you have depend on your typeof PH. If it is caused by another disease, then this must also betreated.


7


Depending on the cause of PH, you may have conventionaltherapy such as:

• Oxygen

• Warfarin (blood thinning medication)

• Diuretics (water tablets)

However, you may start on more specific therapies, such as:

• Sildenafil

• Tadalafil

• Riociguat

• Bosentan

• Ambrisentan

• Macitentan

• Selexipag

• Inhaled prostacyclin (Iloprost)

• Intravenous prostacyclin (Epoprostenol)

These disease-specific treatments called “pulmonaryvasodilators” help to relax the arteries in the lungs. This allowsblood to flow through these blood vessels more easily andreduces the blood pressure in your lungs. These treatments willbe discussed with you in more detail during your consultation.

In some cases of pulmonary hypertension, our specific therapiesmay not be suitable for you to take. If this is the case thereasons for this will be discussed with you.

If your PH is caused by thromboembolic disease (blood clots inthe lungs) you may be offered surgery to remove the blood


8

clots. This is called a pulmonary endarterectomy (PEA) and youwould be referred to Papworth Hospital in Cambridge. The staffat Papworth will meet with you and decide whether thistreatment is right for you.

What to expect during your consultation andhospital visits

Royal Brompton Hospital is part of the National PulmonaryHypertension Service and specialises in the diagnosis, care andtreatment of people with pulmonary hypertension.

The team consists of experienced consultants and clinical nursespecialists who will discuss your condition with you and supportyou through the diagnosis and treatment options.

It may be necessary for you to be admitted to the hospital forbetween two and four days. During this time you will havefurther tests carried out to confirm if you have pulmonaryhypertension. The results of your tests will be discussed withyou during this admission. Depending on the results of thetests, and if a diagnosis of PH is made, you may then starttreatment. After starting your treatment you will need regularreviews of your condition. This will be done in outpatient clinicsevery three to six months.


9


Our team

Pulmonary hypertension consultants

• Dr John Wort clinical lead for respiratoryand pulmonary hypertension

• Professor Kostas Dimopoulos cardiology, adult congenitalheart disease (ACHD) andpulmonary hypertension

• Dr Laura Price respiratory and pulmonaryhypertension

• Dr Aleksander Kempny cardiology, ACHD andpulmonary hypertension

• Dr Colm McCabe respiratory and pulmonaryhypertension

• Dr Aimee Brame respiratory, intensive therapyunit and pulmonaryhypertension

• Dr Bhaskar Mukherjee respiratory and pulmonaryhypertension

Associated consultants

• Professor Michael Gatzoulis cardiology, ACHD andpulmonary hypertension

• Dr Anna Pires ACHD and pulmonaryhypertension

• Dr Isma Rafiq ACHD and high risk pregnancy

• Dr Wei Li echocardiography

• Dr Simon Padley radiology


10

• Dr Thomas Semple radiology

• Dr Jayne Wood palliative care

• Dr Anna Reed transplant

• Dr Tessa Homfray genetics

Clinical nurse specialists and pharmacy

• Carl Harries pulmonary hypertensionclinical nurse specialist (CNS)

• Stuart Craig pulmonary hypertension CNS

• Deepa Lakhani pulmonary hypertensionpharmacist

• Deepti Ghelani pulmonary hypertensionpharmacy technician

Administration and research teams

• Sweeta Dhakan-Oakey service coordinator

• Selina Iuliano service coordinator

• Della Stokes data manager

• Margaret Hollis medical secretary

• Geraldine Faulkner inpatient and day casescheduler

• Rosa Da Costa research coordinator



Shared care

We have developed a shared care service with a number of NHShospital trusts (see list of trusts and health professionals below).Consultants from Royal Brompton regularly attend clinics atthese hospitals.

This shared care system makes it easier for patients to get adiagnosis and treatment. Offering services more locally alsomeans that patients do not have to travel as far forappointments.

Teams from Royal Brompton and the other shared care hospitaltrusts also meet regularly to discuss the care and managementof patients.

Shared care trusts and health professionals

St George’s University Hospital NHS Foundation TrustTooting – London

• Professor Madden lead consultant

• Nordita Bascon pulmonary hypertensionclinical nurse specialist (CNS)

University Hospital Southampton NHS Foundation Trust –Southampton

• Dr Samantha Fitzsimons lead consultant pulmonaryhypertension and congenitalheart disease

• Melanie Finch pulmonary hypertension CNS

• Dr Ahilanadan Dushianthan lead consultant (pulmonaryhypertension and respiratorymedicine)

• Michelle Ritchie pulmonary hypertension CNS

11


12

Oxford University Hospital NHS Foundation Trust (JohnRadcliffe) – Oxford

• Dr Elizabeth Orchard lead consultant ACHD

• Emma Weingart pulmonary hypertension CNS

Other centres

Royal Brompton’s teams also visit and run clinics at thesehospital trust locations:

• Surrey and Sussex Healthcare NHS Trust – Redhill, Surrey

• Royal Berkshire NHS Foundation Trust – Reading, Berkshire

• Frimley Health NHS Foundation Trust – Frimley, Surrey

Occasionally, you will also need to visit Royal Brompton for testsor treatments. We will tell you when that is necessary.

Useful contacts

For general enquiries and appointments, please contact thepulmonary hypertension secretary on 020 7352 8362.

For enquiries regarding a hospital admission, please contactcardiac bookings on 020 7351 8617.



13

Other useful contacts

• Royal Brompton & Harefield NHS Foundation Trust websitewww.rbht.nhs.uk/our-services/heart/pulmonary-hypertension-service

• Pulmonary Hypertension Association (PHA UK)www.phauk.org

• The Somerville Foundation (formerly Grown-up CongenitalHeart Patient’s Association)www.thesf.org.uk

For more information

If you would like any further information before youradmission, please contact the pulmonary hypertension clinicalnurse specialists on 020 7352 8121, extension 2156 or bleeps 1165 or 1170.


http://www.rbht.nhs.uk/our-services/heart/pulmonary-hypertension-13



http://www.phauk.org

http://www.thesf.org.uk

If you have concerns about any aspect of the service you havereceived in hospital and feel unable to talk to those peopleresponsible for your care, call the Patient Advice and LiaisonService (PALS) on:

• Royal Brompton Hospital – 020 7349 7715 • Harefield Hospital – 01895 826 572

You can also email [email protected]. This is a confidential service.

14


mailto:[email protected]

Royal Brompton HospitalSydney StreetLondonSW3 6NPTel: 0330 12 88121

Harefield HospitalHill End RoadHarefieldMiddlesexUB9 6JHTel: 0330 12 88121

Website: www.rbht.nhs.uk

Brosu� rteki bilginin Tu� rkçe tercu�mesi için tedavi goru� yoroldugunuz bolu�me bas vurunuz. Bolu�m personeli tercu�meningerçeklesmesini en kisa zamanda ayarlacaktir.

© Royal Brompton & Harefield NHS Foundation Trust May 2020


http://www.rbht.nhs.uk

royal brompton hospital what is pulmonary hypertension? · 2020-05-28 · • pulmonary...

Documents