Download Adult Congenital Heart Disease and Pulmonary Arterial Hypertension

Adult Congenital Heart
Disease and Pulmonary
Arterial Hypertension
Patient Information
WHAT IS CO N G EN I TA L H EART D I SEASE?
Congenital heart disease (CHD) refers to a defect of the heart
or major blood vessels (aorta, pulmonary artery, systemic veins
and pulmonary veins). The defect results when the heart or blood
vessels do not form normally. This happens while the fetus is in the
mother’s uterus (womb). There are many types of heart defects.
They include:
• Abnormal openings (communications) between heart chambers
• Abnormal openings (communications) between major blood vessels
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• Reversed position of heart chambers resulting in abnormal
connections between the upper and lower heart chambers
• Reversed position of major blood vessels resulting in abnormal
connections between the major blood vessels and the heart chambers
• Abnormal or missing heart valves
WHAT IS P UL MO N A RY A RTERI AL
H Y P E RTEN S I O N ( PA H ) ?
PAH refers to high blood pressure in the arteries (blood vessels) of
the lungs. This high pressure means that the right side of the heart
must work harder to pump blood into the lungs.
Many patients with CHD will develop some degree of PAH. Some
types of CHD that can lead to PAH include:
• Atrial septal defect (ASD): a hole in the wall (septum) between
the two upper chambers of the heart (atria)
• Ventricular septal defect (VSD): a hole in the wall (septum)
between the two lower chambers of the heart (ventricles)
• Patent ductus arteriosus (PDA): a small blood vessel (ductus
arteriosus) between the aorta and pulmonary artery that stays
open instead of closing after birth.
PAH may get worse over time. PAH can develop if you have an
open communication between the heart chambers. It can also
occur if you underwent repair of your CHD during childhood and
adulthood. The most severe form of PAH due to CHD is called
Eisenmenger syndrome.
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H OW DO E S E I S EN MEN G E R SYND ROM E
D EVELO P ?
The right side of the heart (an area of low blood pressure and
low oxygen) gets blood from the body and pumps it to the lungs
to pick up oxygen. The blood from the lungs then goes to the
left side of the heart (an area of high blood pressure and high
oxygen) and is pumped out to the body.
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Normally, the right side of the heart is separated from the left side
by a wall called the septum.
Normal Heart and Arteries
Ao Arteries
Normal Heart and
PA
LA
Ao
PA
RA
RA/LA = Right & Left Atrium RA
RV/LV = Right & Left Ventricle
Ao = Aorta
PA = Pulmonary Artery
RA/LA = Right
LA
LV
RV
LV
RV
& Left Atrium
RV/LV = Right & Left Ventricle
Ao = Aorta
PA = Pulmonary Artery
If there is a hole in the septum or an abnormal opening between
the major
vessels
(aorta
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artery) then
If there isblood
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with each
of the heart to the low pressure right side of the heart with each
heartheart
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left-to-right
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called a
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The shunt lets more blood than usual to flow into the blood
vessels of the lungs. Over time, the increased blood flow causes
a permanent change to the blood vessels in the lungs. The blood
vessels become thick and stiff. These changes lead to:
1. Problems with the exchange of oxygen between the blood
and the lungs
2. Even higher blood pressure in the lungs
As the pressure in the lungs increases, the right side of the heart
has to work harder to get blood to the lungs. This causes the right
side of the heart to become larger and the heart muscle to get
thicker. Over time, the blood pressure on the right side of the heart
gets higher than that on the left side and causes some oxygenpoor blood to flow to the left side of the heart across the opening.
Normal Heart and Arteries
WH
•
•
•
•
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Oxygen poor blood
crosses the defect to
enter the left side of
the heart – leading to
lower oxygen levels
in the blood going
to the body
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This
right-left shunt and results in oxygen-poor
RV/
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blood
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a result,
Artery pumped from the left side of the heart to the
body has a lower than normal oxygen level.
WHAT HA P P EN S W H E N OXYG EN LEV ELS
A RE LOW?
If there is a hole in the septum or an abnormal opening between
Your red
blood
cells (aorta
carry oxygen
to all partsartery)
of the then
body.
the major
blood
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and pulmonary
The
part
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some blood will flow or “shunt” from the high pressure left side
oxygen levels
are low,
theheart
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of thehemoglobin.
heart to theWhen
low pressure
right side
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eachby
increasing the number of red blood cells to carry more oxygen.
heart beat. This is called a left-to-right shunt.
Having more red blood cells than normal is called “secondary
erythrocytosis”.
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W HAT AR E T H E SY MP TO M S OF PAH ?
•
•
•
•
•
•
•
•
•
Shortness of breath or difficulty breathing (dyspnea)
Fatigue or feeling tired
Swelling of the feet, ankles or abdomen (edema)
Chest pain, heaviness, pressure, or tightness that may occur at
rest or with activity
Feeling dizzy, lightheaded or like passing out or actually losing
consciousness
Irregular, rapid or forceful heart beats (palpitations)
Hoarseness of the voice
Frequent cough
Coughing up blood (hemoptysis)
H OW IS PA H I N C H D D I AG NOSED?
A cardiologist or a respirologist with expertise in CHD and
PAH is the best person to diagnose PAH in persons with CHD.
This requires a complete medical history and physical exam.
Other tests which may be required before a diagnosis is made
include: blood work, electrocardiogram (ECG), chest x-ray,
echocardiogram, pulse oximetry, 6 minute walk test, Holter monitor,
CT scan, lung scan, MRI, and/or heart catheterization.
H OW IS PA H I N C H D T R E ATED?
There is no way to reverse the damage that has been done to the
blood vessels of the lungs. The goal of treatment is to manage
symptoms, stop the PAH from getting worse and prevent further
problems.
Medications used in the management of PAH in CHD differ
between patients. The medications used depend on the
severity of symptoms or problems that occur because of low
oxygen levels in the blood. Medications prescribed for PAH must
be closely monitored by the PAH or CHD clinic.
Ao
PA
W HAT SH O UL D I KN OW ABOUT LI V I NG
W ITH PAH I N C H D?
TELL
LA• Inc
REGULAR CHECK-UPS WITH THE SPECIALIST CLINIC ARE VERY
IMPORTANT. PAH in CHD is a complex and serious disease. It is
a life-long chronic condition that requires regular check-ups
with a specialist in PAH and CHD. Even if you feel fine, it is
important to go to all your check-ups. Problems may start
even before you notice symptoms. Early treatment prevents more
serious problems.
RA
RV
KNOW YOUR HEART CONDITION. Learn all that you can about PAH
and your CHD. Learn how to draw your CHD on a heart diagram.
Know your usual oxygen saturation level and blood pressure. Know
RA/LA Right & Left Atrium
the name =
of the medications you are taking, why you are taking
RV/LV
Right
& Le
ft often
Ventryou
them,
the=dose
and
how
icle take them. Many health care
Ao
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rta not experts and have limited experience in PAH or
providers
PA =Ask
CHD.
a copy
ofter
your
Pufor
lmon
ary Ar
y clinical report and carry it with you.
This report provides very helpful information if a new doctor needs
to take care of you in an emergency. Knowing your condition helps
to ensure you receive proper care.
WEAR A MEDICAL ALERT BRACELET OR NECKLACE THAT LISTS
PAH AND YOUR CHD. This ensures that emergency health care
providers know right away that you have PAH and CHD even if you
cannot speak for yourself.
•
•
•
•
•
•
•
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If there is a hole in the septum or an abnormal opening
THE NAME OF YOUR PAH AND CHD DOCTORS AND THEIR
the KNOW
major
blood vessels (aorta and pulmonary arte
CONTACT NUMBERS. Contact your PAH or CHD doctor or clinic if
youblood
have a serious
injury,
go to an emergency
department
some
will illness
floworor
“shunt”
from the
high pressure
or are admitted to hospital for any reason. If you cannot contact
of the
heart to the low pressure right side of the heart w
the doctor or clinic yourself, have a family member or friend call on
heart
beat.
your
behalf.This is called a left-to-right shunt.
LOW
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TELL YOUR DOCTOR ABOUT ANY OF THESE SYMPTOMS:
•
•
•
•
•
•
•
•
Increasing shortness of breath (dyspnea)
Dizziness, especially when standing or climbing stairs
Fainting (syncope)
Swollen ankles and legs (edema)
Chest pain, especially with activity
Frequent rapid or irregular heart beats (palpitations)
Coughing up blood (hemoptysis)
Fevers of unexplained origin
WHAT FAC TO R S CO UL D I M PACT
MY HE ALT H ?
DEHYDRATION
Dehydration is caused by a number of factors (e.g. diarrhea,
vomiting, fasting, fever, hot climate, air travel, bleeding). It may
limit exercise tolerance, increase the risk of stroke, or increase the
risk of irregular heart rhythms. Most patients should drink at least
1 to 1.5 liters of fluid daily. You may need to drink more when you
have a fever or when it is hot and humid. But you might also need
to drink less if you have heart failure. Talk to your doctor or nurse
about the right amount of fluid intake for you.
HEIR
nic if
ment
ntact
all on
LOW IRON LEVELS IN THE BLOOD
Low iron levels in the blood may limit exercise tolerance and
increase the risk of stroke. Your doctor may prescribe iron
supplements or ask you to eat foods rich in iron if you are low in iron.
BIRTH CONTROL AND PREGNANCY
Some birth control pills may increase your risk of blood clots and
stroke. Pregnancy in women with PAH and CHD can be very risky
for both the mother and fetus. Talk to your CHD or PAH doctor
about birth control and pregnancy.
HIGH ALTITUDES
SMO
Air at high levels contains less oxygen and you should avoid
altitudes higher than 2,500 meters. However, commercial
airplanes are usually safe because the air pressure in the cabin
is controlled. Talk to your doctor regarding the need for oxygen
during your flight.
Smo
It al
doct
ALCOHOL AND STREET DRUGS
You should avoid large amounts of alcohol. Do not take illicit street
drugs (e.g. marijuana, cocaine, heroin, ecstasy). They can cause life
threatening irregular heart rhythms.
NON
No
or E
befo
INT
INFECTIONS
You should
you should:
avoid
getting
infections.
To
avoid
infections
• Brush and floss your teeth daily and see your dentist at least once
a year.
• Ask your doctor if you need to take antibiotics to prevent
endocarditis, an infection in the blood that could damage the
heart. If you do, then you will need to take antibiotics before
dental work or other invasive procedures.
• See your doctor right away if you get an upper or lower
respiratory (lung) tract infection or have other signs of an
infection (e.g. infected cut).
• Avoid or limit contact with people who are ill with a cold or flu.
• See your family doctor for the seasonal flu vaccine every fall.
Make sure that you have had a pneumonia vaccine.
• Make sure your immunizations are up-to-date.
EXERCISE
In general, regular physical activity is good for people with PAH
and CHD. However, strenuous activity should be avoided. Talk to
your CHD or PAH doctor about which activities are safe for you.
Get your doctor’s approval before starting any exercise program.
If yo
you
Air
bub
lung
synd
byp
som
happ
be t
WH
•
•
•
•
Ea
Ma
Sta
Ha
SMOKING
void
rcial
abin
ygen
Smoking lowers the amount of available oxygen in the blood.
It also damages the lungs and heart. If you smoke, talk to your
doctor or nurse about ways to help you stop smoking.
NON-CARDIAC SURGERY
treet
e life
No surgical procedure is simple in a patient with PAH
or Eisenmenger syndrome. Talk with your CHD or PAH doctor
before having any surgery.
INTRAVENOUS (IV) LINES
tions
once
vent
the
efore
ower
f an
flu.
fall.
PAH
lk to
you.
am.
If you have a shunt with PAH or have Eisenmenger syndrome
you are at risk for air embolism with intravenous (IV) lines.
Air embolism refers to an air bubble in the blood. Normally, air
bubbles that accidentally get into an IV line are stopped in the
lungs. However, in the presence of a shunt or Eisenmenger
syndrome, the right to left shunt may allow the air embolism to
bypass the lung and travel directly to the brain. If this happens,
some areas of the brain may get less oxygen or a stroke may
happen if an artery becomes totally blocked. Special care should
be taken with IV lines to avoid air embolism.
WHAT C A N I D O TO KEEP H EALTH Y?
•
•
•
•
Eat a heart healthy diet
Maintain a healthy weight
Stay active
Have regular check-ups with your PAH and CHD clinics
WHAT WO R K C A N I D O?
Talk with your CHD or PAH doctor about the right type
of work for you. It is best to avoid jobs that require physical labor,
frequent traveling or long commutes.
WHE N SH O UL D I C A L L M Y PAH OR CH D
DO C TO R O R C L I N I C ?
• If you develop worse or new symptoms.
• Before starting any new medication. This includes birth
control pills, over-the-counter medications, herbal medications
or dietary supplements.
• If you get a severe cold or respiratory tract (lung) infection.
• If you get a fever with no clear cause.
• Before having any kind of surgery.
• Before starting an exercise program.
• Before starting a new diet.
• Before traveling or moving outside of your province.
FO C U S O N YO UR Q UA L I TY OF LI F E
It is important to know your condition and what you can do to keep
yourself in the best health possible. Talk to your doctor or nurse if
you have any questions. You can even ask them about what they
think your health will be like in the longer term. Don’t forget that
life isn’t only about your PAH and CHD. Focus on what you can do
and what you like to do in life.
The goal is to live as full and rich a life as possible.
NOTES
NOTES
pe
bor,
rth
ons
ep
e if
hey
hat
do
le.
This document was prepared by Doreen Fofonoff, MN, RN, CCN(C),
Clinical Nurse Specialist, Pacific Adult Congenital Heart Clinic
Vancouver, St-Paul’s Hospital, Vancouver, BC
With the valuable contribution of the following individuals:
Pamela Heggie, RN, BN, Clinic Nurse Coordinator, Northern Alberta
Adult Congenital Heart Clinic, University of Alberta Hospital,
Edmonton, AB
Yvonne Balon, RN, BN, MN, Nurse Clinician, Southern Alberta Adult
Congenital Heart Clinic, Peter Lougheed Hospital, Calgary, AB
Erwin Oechslin, MD, FRCPC, Director, Toronto Congenital Cardiac
Centre for Adults, Toronto General Hospital, Toronto, ON
Pictures courtesy of and used with permission of
James L Wilkinson, FRACP, FRCP, FACC, FRCPCH, FCSANZ
Professorial Fellow, Dept of Paediatrics, University of Melbourne
Senior Cardiologist, Royal Children’s Hospital, Melbourne, Australia
RESOURCES
For more information about congenital heart
defects go to:
Canadian Adult Congenital Health Network
www.cachnet.org/index.cgi
Canadian Congenital Heart Alliance (CCHA)
www.cchaforlife.org
For more information about pulmonary arterial
hypertension go to:
Pulmonary Hypertension
Association of Canada
www.phacanada.ca/index.php/en
Sponsored by an unrestricted educational grant from
Actelion Pharmaceuticals Canada Inc.
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