CONGENITAL HEART
DISEASE
BY CHOOLWE CHIBBANDULA MUDENDA
OVERVIEW OF THE HEART
INTRODUCTION
A congenital cardiac malformation occurs in about 1% of live
births. There is an overall male predominance, although some
individual lesions (e.g. atrial septal defect and persistent
ductus arteriosus) occur more commonly in females.
• As a result of improved medical and surgical management,
more children with congenital cardiac disease are surviving
into adolescence and adulthood.
• Thus there is a need for an increased awareness amongst
general physicians and cardiologists of the problems posed
by these individuals.
DEFINITION
• Congenital heart defect (CHD) may be defined as an
anatomic malformation of the heart or great vessels which
occurs during intrauterine development, irrespective of the
age at presentation
Congenital heart defects classified into acyanotic and
cyanotic
1. Acyanotic congenital heart defects
2. Cyanotic congenital heart defects
Acyanotic congenital heart
defect
• Includes the left to right shunt which results to an increase
to pulmonary blood flow.
1. Patent ductus ateriosus (PDS)
2. Ventricular septal defect(VSD)
3. Arteriole septal defect (ASD)
Cyanotic congenital heart
defects
• Includes right to left shunt meaning no oxygen in the blood it
consist of the 5 Ts
1. Tetralogy of Fallot
2. Transposition of great arteries
Cont..
• Tricuspid arteries
• Truncus arteriosus
• Total anomalous pulmonary venous return
Signs and symptoms
•Central cyanosis occurs due to left-to right shunting of blood.
•Pulmonary hypertension due to large left-to-right shunts.
•Clubbing of the fingers due to congenital cardiac conditions
associated with prolonged cyanosis
Cont..
• Growth retardation is common in children with cyanotic
heart disease
• Murmurs due to the defects
CAUSES
• Congenital heart disease is caused when something disrupts
the normal development of the heart.
• It is thought that the most cases occur when the heart’s
development is affected by some factors during about week
five of pregnancy (Colleen story & George krucik (2012))
RISK FACTORS
There are a number of things that can increase the chances of a
child having a congenital heart disease which include;
1.GENETIC CONDITIONS; several genetic health
conditions that a baby inherits from one or both parents can
cause congenital heart disease.
Cont..
• -DOWN’S SYNDROME; is the most widely known
genetic condition that can cause congenital heart
disease.
• In 90% of cases, this is a type of septal defect.
Cont…
• TURNER SYNDROME; a genetic disorder that only affect
females.
• It is estimated that 25% of children with Turner’s syndrome
will be born with congenital heart disease which is usually a
type of valve or artery narrowing problem.
Cont…
• NOONAN SYNDROME; a genetic disorder that can cause a wide range
of potential symptom;
• around 80% of children with Noonan syndrome will be born with
congenital heart disease and in about half of all cases, this will be
pulmonary stenosis
. MATERNAL DIABETES
•Women with diabetes who become pregnant are more likely
to give birth to a baby with heart defects. Most commonly
transposition of the great arteries.
ALCOHOL
• ALCOHOL, if a pregnant woman drinks too much alcohol
during pregnancy, it can have poisonous effects on the
tissues of the foetus. This is known as foetal alcohol
syndrome. Most commonly, ventricular or atrial septal
defect
RUBELLA
• A rubella infection can cause multiple birth defects including
congenital heart disease
MEDICATION
•MEDICATION; there are several medications linked to an
increased risk of babies being born with congenital heart
disease. These include;
•- Certain anti-seizure medications; such as benzodiazepines
and lithium.
Cont..
• - Certain acne medications; such as isotretinoin and topical retinoid .
• - ibuprofen; women who take ibuprofen during the first trimester of
pregnancy are likely to give birth to a baby with congenital heart
disease than the general population.
ACYANOTIC CONGENITAL HEART
DISEASE
Patent ductus ateriosus (PDS)
In patent ductus arteriosus, abnormal blood flow occurs
between the aorta and the pulmonary artery. is a medical
condition in which the ductus arteriosus fails to close after
birth:
CONT……
Symptoms are uncommon at birth and shortly thereafter, but
later in the first year of life there is often the onset of an
increased work of breathing and failure to gain weight at a
normal rate. With time, an uncorrected PDA usually leads to
pulmonary hypertension followed by right-sided heart failure
Signs and symptoms of PDA
• Dyspnea (shortness of breath)
• Tachycardia (a heart rate exceeding the normal resting rate
• Cardiomegaly (enlarged heart, reflecting ventricular dilation
and volume overload)
• Poor growth
Ventricular septal defect
• is an abnormal opening in the wall between the main
pumping chambers of the heart (the ventricles.
• When the heart beats, some of the blood in the left ventricle
(which has been enriched by oxygen from the lungs) flows
through the hole in the septum into the right ventricle.
• In the right ventricle, this oxygen-rich blood mixes with the
oxygen-poor blood and goes back to the lungs.
• The blood flowing through the hole creates an extra noise,
which is known as a heart murmur
Cont…
• Medium and large VSDs may cause noticeable symptoms.
Babies may have faster breathing and get tired when they try
to feed. They may start sweating or crying while feeding, and
may gain weight slowly.
Cont…
Signs and symptoms
Cyanosis- due to deoxygenated blood entering the systemic circulation
without passing through the lungs.
Digital clubbing- due to prolonged poor oxygen perfusion in the finger
Edema- due to backflow and congestion of blood in the lower limb
vessels hence causing extravasation of blood/fluids into the interstitial
space.
Growth retardation- due to reduced tissue perfusion
Cont..
Learning difficulties- due to reduced blood supply to the
brain
Syncope- due to reduced oxygenated blood flow to the brain
Dyspnea -due to blood congestion in the lungs
Fatigue - due to reduced oxygen tissue perfusion
MANAGEMENT OF CONGENITAL HEART DISEASE
INVESTIGATIONS
Chest x-ray It shows the enlarged heart and enlarged pulmonary artery
Electrocardiograph It shows the disturbance in the electrical conductivity
of the heart.
Echocardiograph It shows the dilated chambers of the heart and if they are
hypertrophied
Magnetic resonance imaging Shows an enlarged heart and the great vessels
• a cardiac catheterization: this provides information about the
heart's structures and the blood pressure and blood oxygen
levels in its chambers.
• This test usually is done for a VSD only when more information
is needed than the other tests can give. (It's sometimes also
used to close certain kinds of VSDs.)
TREATMENT
•Oxygen therapy-oxygen therapy is used to decrease work of breathing
by increasing alveolar oxygen tension and 5 to 8 litres per minute will be
administered when patient is dyspnoeic.
•Digitalis e.g., digoxin- which strengthens the contractility of the
ventricular muscles and slows down the conducting power of the
atrioventricular bundle thereby enabling ventricles to beat more
effectively.
Dosage; 0.25micograms orally per day. It may be administered
intramuscularly or intravenously when patient’s condition is life
threatening.
• Side effects- Digoxin slows down the pulse rate. You need to check
the pulse rate before administering
digoxin. If pulse rate is less than 60 beats per minute, do not
give this drug.
•Diuretics- for example, furosemide, can be given to help in
excretion of sodium and water.
dosage- 10 to 20mg once daily
•Anti-inflammatory- e.g. hydrocortisone 100mg tds intramuscularly/IV
can be given to relieve the inflammation
Analgesics-paracentamol 500mg used to relieve chest pain.
•Antibiotics for prophylaxis against infections. Metroninzole, 400mg
TDS for 5/7 days.
NURSING MANAGEMENT
Aims of management
To encourage compliance with drug regimens
To facilitate recovery from the illness
To provide emotional support
Cont..
Nutrition
• Balanced diet to promote good nutrition
• Determine likes and dislikes of the patient to promote good intake of
food.
• Daily weight to monitor the patient’s nutritional status.
Prevent infections
• Prevent exposure to communicable disease to prevent acquisition of
these infections.
Cont..
Immunization should be done if in a child to minimize the
acquisition of certain infections.
Hand washing be observed to promote hygiene
Give prophylactic medication
Reduce workload of the heart
Uninterrupted rest
Avoid unnecessary activities
Provide diversional activities
Prevent constipation
Relieve respiratory distress.
Observation
Monitor the vital signs to observe progress in the
patient’s condition. Vital signs like temperature to
detect hypothermia, pulse and blood pressure to
monitor the cardiac output and respirations to rule
out dyspnea.
Cont..
• Check for changes in cardiac rhythm or conduction
• Assess the patient’s cardiovascular status frequently so as to
watch for signs of left ventricular heart failure for example,
tachycardia, tachypnea, edema
• Monitor and maintain strict intake and output using the fluid
balance chart to avoid overloading the patient with fluids as
this can put further strain on the heart.
Psychological care
Explain all the stage of the management to allay anxiety
Allow the client to make decisions about the treatment
option they would prefer as this gives them autonomy
over their lives even in illness
Environment
The child will be nursed in acute bay near the nurses’ table
for easy observation.
Position
The patient is propped up in bed with the help of backrest or
cardiac table (on which the patient will lean).
COMPLICATIONS OF CONGENITAL HEART DISEASES
• CONGESTIVE HEART FAILURE
This may develop in children who have significant heart defect.
• STROKE
Can have stroke due to movement of an emboli which can block the
brain vessel resulting in little or no oxygen supply to that part of the
heart.
• IRREGULAR HEARTBEATS (ARRHYTHMIA)
Faulty heart signaling causes the heart to beat too fast ,
PREVENTION
Vaccination against rubella this may reduce the risk of having birth defects.
Control chronic conditions like diabetes which increases the risk of having birth
defects
Avoid harmful substances like herbal medicine as the side effects may not be
known
• Take multivitamin daily to reduce birth defects in the brain or spinal cord and may
reduce the risk of having birth defects.
Treatment of VSD
Heart Surgery
• Surgery usually is done within the first few weeks to months
of a child's life. The surgeon makes an incision in the chest
wall and a heart-lung machine will maintain circulation while
the surgeon closes the hole. The surgeon can stitch the hole
closed directly or, more commonly, will sew a patch of
manmade surgical material over it.
Cont..
Eventually, the tissue of the heart heals over the patch or
stitches. By 6 months after the surgery, tissue will completely
cover the hole.
Sometimes they recover own their own .
CONT…
Cardiac Catheterization
• Rarely, cardiologists may close some types of VSDs with
cardiac catheterization. They insert a thin, flexible tube (a
catheter) into a blood vessel in the child's leg that leads to
the heart. They guide the tube into the heart to make
measurements of blood flow, pressure, and oxygen levels in
the heart chambers.
CONT…
A special implant, shaped into two disks formed of flexible
wire mesh, is positioned into the hole in the septum. The
device is designed to flatten against the septum on both
sides to close and permanently seal the VSD.
medication
1. Lasix :diuretic that works by decreasing excess lung fluid caused by
the extra blood flow to the lungs. Furosemide may be given
anywhere from 1 to 4 times daily.
2. Digoxin: Afterload reduction, such as with angiotensin-converting
enzyme inhibition, may also affect clinical improvement, although
definitive studies in this setting are not available.
ARTERIOLE SEPTAL DEFECT
(ASD)
• During the embryologic development of the heart, a septum grow
toward the endocardium which divides the atrium.
• When they is failure of septum to grow they led to the arteriole septal
defect.
Atrial septal defects (ASDs) that aren't diagnosed until adulthood can
damage the heart and lungs, shortening life.
Cont….
• Many babies born with ASDs don't have symptoms. By the age of 30,
adults may notice
Signs and symptoms
shortness of breath
heart palpitations,
fatigue,
swelling in the legs
heart murmurs.
Cont….
CYANOTIC CONGENITAL HEART
DISEASE
• This happens when some of the systemic venous returns crosses from
right to left side of the heart and they return to the body without
going through the lungs ( right to left shunt)
• Cyanotic means no oxygen.
Cyanosis is categorized in two
Central cyanosis (blue mouth)
Peripheral (whole body)
The most common cyanotic congenital heart defects are five
Ts
Cont..
1. Tetralogy of Fallot
2. Transposition of great arteries
3. Tricuspid arteries
4. Truncus arteriosus
5. Total anomalous pulmonary venous return
TETRALOGY OF FALLOT
• This occurs as the babys heart grows and it the alteration of
blood flow to the lungs the rest of the body
TETRALOGY OF FALLOT
CHARACTERIZED IN FOUR
1. Right ventricular hypertrophy : this when blood from the
right ventricles to the left ventricle which will led to low
blood flow to the pulmonary then the pulmonary valve will
became narrow allowing fill blood to enter the pulmonary
artery.
Cont…
2. Aorta displacement : since there is no septum between the
ventricles and the aortic valve blood will mix .
The aorta is usually attached to the left lower heart chamber
but in tetralogy of fallot the aorta is in wrong place.
Cont…
3.Pulmonary stenosis : is the heart valve disorder .blood from
the heart pulmonary valve, this is when the opening is too
narrow leading to a reduction of blood to the lungs.
Narrowing of the valve is most present at birth.
4.Septal defects
This is when the septum of the ventricles have wall and blood
the right ventricle which is deoxygenated will mix with
oxygenated blood from the right ventricle which will led to
less blood to the pulmonary artery.
Signs and symptoms
Fingernail clubbing due to hypoxia
Inability to growth
Murmurs
Faint easily due to low oxygen level
Trouble in feeding
Treatment
Surgery
Shunt can be replaced
Drug such as Alprostrodil ( prostaglandin) which keeps the
ductus arteriosus open
TRANSPOSITION OF THE GREAT
ATERIES
• This is when aorta and the pulmonary artery switched.
Cont..
The left side it going to do has it wants and the right side too
with out communication.
Meaning the body will be receiving deoxygenated blood from
the heart and the tissue will lack oxygen for them to develop.
Cont.…
Signs and symptoms
Severe cyanosis
Respiratory breathing fast
Poor feeding
TREATMENT
Prostaglandin (Alprostrodil)
Arterial switch procedure
Ballon atrial septostomy
TRISCUPID ARTERIES
• Tricuspid atresia is a congenital (present at birth)
heart defect that occurs when the tricuspid valve
of the heart doesn’t form. The tricuspid valve is
normally between two chambers on the right
side of your heart, the right atrium (upper
chamber) and right ventricle (lower chamber).
CON..
• In tricuspid atresia, you have solid tissue instead of
the tricuspid valve.
• This sheet of tissue blocks blood flow from the right
atrium to the right ventricle, where blood would
normally go. Because of this blockage, the right
ventricle usually is small and underdeveloped.
Cont..
• The other valve on the right side between the
right ventricle and the lungs can also be
underdeveloped (pulmonary valve)
TRUNCUS ARTERIOSUS
That means it's a congenital heart defect.
In this condition, one large blood vessel leads out of the
heart, instead of two.
• Having only one large blood vessel means that oxygen-poor
and oxygen-rich blood mix.
Cont..
• This mixing reduces the amount of oxygen
delivered to the body.
• It usually increases the amount of blood flow into
the lungs too. The heart has to work harder to
adjust for the changes in blood flow.
Signs and symptoms
• Problems breathing
• Pounding heart
• Weak pulse
• bluish skin color
• Poor feeding
• Extreme sleepiness
prevention
• Get proper prenatal care
• Multivitamin and folic acid 400mg daily
• Don’t drink or smoke
• Measles vaccine
• Control sugar level
REFFERENCES
1.Cara T. Mai, Jennifer L. Isenburg, Mark A. Canfield,
Robert E. Meyer, Adolfo Correa, Clinton J. Alverson,
Philip J. Lupo, Tiffany Riehle‐Colarusso, Sook Ja Cho,
Deepa Aggarwal, Russell S. Kirby. National population‐
based estimates for major birth defects, 2010–2014.
BDR Oct 2019.