AR SYSTEM

AND ITS
DISORDER IN
CHILDREN

Presented by:Prerna sharma

M.Sc Nursing,3rd Semester

INTRODUCTION
The

circulatory system is composed of the heart

and blood vessels, including arteries, veins, and
capillaries. Our bodies actually have two
circulatory systems: The pulmonary circulation
is a short loop from the heart to the lungs and
back again, and the systemic circulation (the
system we usually think of as our circulatory
system) sends blood from the heart to all the
other parts of our bodies and back again.

THE HEART
The

heart is the key organ in the circulatory

system.
As a hollow, muscular pump, its main function is
to propel blood throughout the body.
It usually beats from 60 to 100 times per
minute, but can go much faster when it needs to.
It beats about 100,000 times a day, more than 30
million times per year, and about 2.5 billion
times in a 70-year lifetime.

ANATOMY
The

upper part of the heart is made up of the other

two chambers of the heart, called the right and left
atria (pronounced: AY-tree-uh).
The right and left atria receive the blood entering
the heart. A wall called the interatrial (pronounced:
in-tur-AY-tree-ul)septum divides the atria, and
they're separated from the ventricles by
the atrioventricular (pronounced: AY-tree-oh-venTRIK-yoo-lur) valves.
The tricuspid valve separates the right atrium from
the right ventricle, and the mitral (pronounced: MYtrul) valve separates the left atrium and the left
ventricle.

CONTD
Two

other heart valves separate the ventricles

and the large blood vessels that carry blood
leaving the heart.
These valves are called the pulmonic valve,
which separates the right ventricle from
the pulmonary artery leading to the lungs, and
the aortic valve, which separates the left
ventricle from the aorta, the body's largest blood
vessel.

CONGENITAL HEART DISEASE

It

is a structural malformation of heart

or great vessel present at birth which
may not be detected at the time of
birth; CHD may represent an isolated
heart disease or a combination of
defect.

ETIOLOGY: It

results either from abnormal embryonic

development or persistence of fetal sturucture
beyond the time of normal involution.
A fetal and maternal infection occurring first
trimester: Rubella infection
Teratogenic effect of drug
Alcohol taken during pregnancy
Maternal dietary deficiency
Genetic defect(trisomes e.g. down syndrome)
Maternal anomalies.

CLASSIFICATION OF COMMON
CONGENITAL HEART DISEASE

ACYANOTIC HEART
DISEASE:Left to right shunt

Obstructive lesions

1. Ventricular septal defect(VSD)

1. Aortic valvular stenosis(AVS)

1. Arterial septal defect(ASD)

1. Pulmonary stenosis(PS)

1. Patent ductus arteriosis(PDA)

1. Co-arcation of aorta

CYANOTIC HEART
DISEASE
Decreased
pulmonary blood
flow

Increased pulmonary
blood flow

1. Tetrology of
fallot(TOF)

1. Transposition of great
vessels

1. Tricuspid atresia

1. Total anomalous
pulmonary venous
connection

ACYANOTIC HEART DISEASE:LEFT TO RIGHT SHUNT

The

three common left to right shunt are

VSD,ASD AND PDA. VSD and PDA
present in infancy while ASD present in
later childhood. These lesions are present
and characterized by:
Absence of cyanosis
Recurrent respiratory infection
Congestive heart failure
Cardiomegaly
Increased risk of infective endocarditis

VENTRICULAR SEPTAL
DEFECT(VSD)
An

abnormal opening in the septum

between the right and left ventricle found
in 25% of all congenital heart defects. It
may vary in size from very small defect
either the membraneous and vascular
portion of the ventricle system
90% are Located in the membraneous part
of the ventricular septum with variable
extension into the muscular septum.

HEMODYNAMICS:

The pressure in left ventricle is greater in right ventricle this promotes the flow of
oxygenated blood from the left ventricle to this right ventricle shunt. This results in
increased right ventricular pressure and increased blood flow in right ventricle.

The flow of blood from left ventricle to right ventricles starts early in systole.

When the defect is restrictive, a high pressure gradient is maintained between the two
ventricles throughout the systole.

The murmur starts early masking the first sound and continues throughout the systole
with almost the same intensity appearing a pansystolic murmur on auscultation. As a
thrill.

Pulmonary artery and lungs as the disease progress long standing pulmonary arterial
hypertension may result in increased pulmonary resistance.

These results in diminished blood flow to the blood flow through the pulmonary
artery and veins and markedly in this right ventricle pressure there by the right
ventricle. Pressure raised by left ventricle pressure.[ventricular pressure lower than
aortic pressure].

The increased blood volume in left atrium may result in left atrial enlargement.passing
through a normal mitral valve results in delayed diastolic murmur at the apex.

CONTD
Since left ventricle has two outlets, the aortic valve
allowing forward flow and the VSD result in backward
leak, since the ejection into the right ventricle and
pulmonary artery is increased because of left to right
shunt.
At this stage, the direction of shunt is reversed to right to
left shunt. Cyanosis appears due to mixing and
hypoxaemia. This condition results in eisenmenger
complex. This indicates poor prognosis.

Fig 2 comparison of normal heart and VSD

CLINICAL MANIFESTATION

Small VSD usually closes spontaneously but higher VSD develop

symptoms at 2-3 months of age. Premature babies with VSD can become
symptomatic even earlier. i.e.

Palpitation

Frequent chest infection

Hyperkinetic with a systolic thrill at left sterna border.

Heart size is moderately enlarged with a left ventricular type apex.

Murmur{the first and second sound is marked by pansystolic murmur which

may be heard by left sterna border and third sound may be heard at apex of
heart}

Initially in ECG right ventricular hypertrophy is diagnosed.

Weight gain

Failure to thrive

Tachycardia

Tachypnea

Feeding problems

Exsertional dyspnea

Seating excessive

Child is pallor and suffers from respiratory tract syndrome.

COMPLICATION
Pulmonary

artery hypertension or aortic

regurgitation due to prolapsed of the right
coronary or non coronary cusp of aortic
valve
Infective endocarditis.
Pulmonary stenosis due to hypertrophy of
right ventricular infundibulum.

 Diagnosis

DIAGNOSTIC EVALUATION

is confirmed by cardiac catheterizationan abnormal communication between ventricles and

again increased blood oxygenation and pressure in
right ventricle.
Echocardiography shows increased left atrial and
ventricular size as well as exaggerated mitral
motion.
Infundibulus stenosis.
Chest Xray ranges from normal to cardiomegaly
with associated pulmonary artery enlargement and
increased pulmonary vascular marking.
ECG is normal or show biventricular hypertrophy.
On auscultation pansystolic murmur heart beat at the
lower sternum (4th intercostals space)

MANAGEMENT
If

it is small defect symptomatic

treatment is conservative because
spontaneously closure occurs between
1-2 yrs of age.
If it is larger medical management is
done.

MEDICAL MANAGEMENT
Administration

of diuretic and digoxin

Control of anemia and infection
If not treated by medical management; open
heart surgery and cardio pulmonary bypass.
Small systemic defect is closed with a purse
string suture while larger defect needs a
knitted dacroam patch swing over a opening.
Surgery is contraindicated in patient with
eisenmenger complex.
After surgery heart size returns to normal and
murmur and thrill disappear.

SURGICAL MANAGEMENT
Surgery

is indicated if: Congestive cardiac failure occurs in

infancy
Left to right shunt is large.
If there is associated pulmonary stenosis,
pulmonary atrerial hypertension or aortic
regurgitation

Fig 3 surgical management

OPERATIVE TREATMENT
Consists

in closure of VSD with the use of a

patch.the operation is performed through the
right atrium. Surgery can be done as early as
diagnosed and also if medical management is not
helpful.
Catheter closure of VSD is best suited for
muscular defects in relatively older children.
Device closure is also a new technique for VSD
closure but require expertise.
Complication os surgery:-complete heart
block,bifascicular block and residual VSD.

ARTERIAL SEPTUM DEFECT

ASD

is an abnormal opening between the

septum of left and right atrium. ASD
accounts for 10% of congenital defect.

Fig 4 Normal V/S Atrial Septum Defect

TYPES

Ostium secundium typeASD:-

The defect is located at the center of the atrial septum the site of foramen
ovale.

Ostium primumASD:-

There is a large gap at the base of atrial septum and associated with deformity
of mitral and tricuspid valve or a small or high VSD.

Fossa ovalis ASD-

They are located in the central portion of atrial septum, in the position of
foramen ovale. These defects are amenable to closure in catheterization in
laboratory.

Sinus venosusASD-

They are located at junction of superior vena cava and right atrium. These
defects do not have a superior margin because superior vena cava straddles
the defect.these defect is associated with anomalous drainage of one or
more right pulmonary veins.

Coronary sinus ASD-

An unroofed coronary sinus is a rare communication between the coronary

sinus and left atrium, which produces feature same as of other types ASD.

HEMODYNAMICS
The

pressure in left atrium is greater than the right

atrium and promotes the flow of oxygenated blood
from left to right atrium.
Left to right shunt increased blood flows from the
right atrium to right ventricle resulting in volume
overload of right ventricle and its dilation.
Overload of right ventricle results in prolong time
required for emptying due to which hang out
interval result and low resistance in pulmonary
circulation occur.
As a result blood flow increase in the pulmonary
circulation but pulmonary hypertension is rare
complication.

Fig 5 Hemodynamics in ASD

CLINICAL MANIFESTATION
ASD

generally asymptomatic even

when defected is large.
Recurrent pneumonitis, mild activity
intolerance and recurrent.
Chest infection, cardiac enlargement
is mild to moderate.

DIAGNOSTIC EVALUATION
On

auscultation first sound , s2 is quietly split

Chest X-ray reveal enlargement of pulmonary
artery, right arterial and right ventricular
enlargement and increase in vascular marking of
lung and a relatively small aortic shadow and
plethoric lung fields.
ECG shows right ventricular volume overload.
Cardiac catheterization shows defect in arterial
septum, higher pressure on right side of chest.
Echocardiogram shows increased size of right
ventricle with paradoxical ventricular septal
motion.

MANAGEMENT
Spontaneous

closure occurs in patient with small

ASD i.e. less than 6mm.
Open heart surgery done in affected child before
going to school even no symptoms present.
For small purse string closure done by stitching
around opening and pulling it closes.
If surgery not done during childhood
pulmonary hypertension, cardiac failure,
arterial arrhythmia make operation more
dangerous in adult
If defect is larger a knitted clacron patch is used
to occult the aperature.

Fig 6 atrial septal defect closure using an expanding device.

PATENT DUCTUS
ARTERIOSIS(PDA)
PDA is

the persistence of a fetal

connection between pulmonary artery
and aorta resulting in a left to right
shunt. More common in females than
males.

Fig 7 normal v/s PDA

HEMODYNAMICS

During fetal life the ductus arteriosis allow most of the ventricular blood to
bypass by non direction lung by direct in blood from pulmonary artery to aiota.

After birth with the initiation of respiration the ductus arteriosis is no longer
needed.

It should functionally closed within several hrs after birth (24-72hrs) and
anatomically within several days after birth.

Degenerative changes occur in ductus arteriosis and it cause become fibrous

connective tissue(ligamentum arteriosis)

If ductus arteriosis remain patent, oxygenated blood from the higher systemic
cercube flows to the lower pressure pulmonary cercube flow through PDA.

This cause left to right shunt.{in premature infants ductus arteriosis may remain
open because of lower oxygen level resulting from respiratory distress after
birth}

*Because of transfer of blood from aiota to pulmonary artery through the ductus
systemic flow is compromised. The volume blood that the heart must pump
meet demands of the peripheral tissue is increased. On the other side a greater
volume burden is placed on the lungs and hence on the left side of the heart.

Fig 7 Normal V/S Abnormal Circulation

CLINICAL MANIFESTATION
Children

may develop symptoms around 6-12 wks of

life.
Older children present effort intolerance, easy
fatiguability, dyspnea on exertion
Increase respiratory infection
Frequent chest infection
Heart rate per over 150/mt, gallop rhythm due to
rapid filling of ventricle.
Large defect may be associated with congestive heart
failure and pulmonary hypertension.
Pulse is high volume and bounding pulse pressure is
wide.

ASSESSMENT OF SEVERITY

Larger

the heart size the larger the left to

right shunt.
Absence of third sound and delayed
diastolic murmur indicates small left to
right shunt.
Presence of third sound indicate moderate
left to right shunt whereas an audible
delayed diastolic murmur suggest a large
left to right shunt.
Wider the pulse pressure larger the shunt.

DIAGNOSTIC EVALUATION
Chest

Xray reveals cardiomegaly and left

ventricle enlargement.
Aorta is prominent, lung feels plethoric
Easy to reveal left ventricular hypertrophy.
On auscultation continuous murmur is felt
on the left intraclavicular area.
A thrill may be palpable.
Echocardiography shows atrial
enlargement. Ascending aorta and aortic
knuckle are prominent.

Fig 8 Abnormal Blood Flow

DIFFERENTIAL DIAGNOSIS
Coronary

arteriovenous fistula
Ruptured sinus of valsalva fistulae into
right side
Systemic arteriovenous fistula over the
chest.
Pulmonary arterialvenous fistula
Bronchial collateral murmur

MANAGEMENT
Medical management: Indomethazine or ibuprofen may be used to close the
ductus in small new born preterm newborn; if diagnosis
is made within 2 wks of life.
Indomethzine causes inhibition of prostaglandin
synthesis 12-18 hrs after medication, improvement in left
or right shunt.
Dose is 0.2 mg/l kg/dose, orally, every 12-24 hr for three
doses(second and third dose is at 0.1 mg/kg/dose for <48
hr old and 0.25 mg/kg/dose for>7 days old)
Side effect of indomethazine:- it reduces amount of
albumin bound serum bilirubin and may develop
hyperbilirubinemia also a danger of transient and
hyponetremia.

SURGICAL MANAGEMENT
Consist of ligation of PDA, now a days device coil
closure of PDA is possible without need of
surgery in children more than 5 kg.
Note: - since the right to left shunt through the
PDA flows down the descending aorta, cyanosis is
present in toes but not in fingers. This is called
differential cyanosis and is a characteristic of
PDA with pulmonary arterial hypertension and
right to left shunt.

Fig 8 surgical repair of PDA

OBSTRUCTIVE LESIONS
These

lesions are also acyanoti heart

disease which may be right sided
(pulmonary stenosis) or left sided (aiortic
stenosis), co-arcation of stenosis.

AIORTIC STENOSIS
There

is obstruction to the left

ventricle outflow tract at the level of
aiortic valve.
This is the most common form of
aiortic stenosis. Other being
hypertrophic subvascular stenosis,
supravascular stenosis.

Fig 9 aortic valve stenosis

HEMODYNAMICS

Valve obstruction is overcome by raising the systolic pressure of the

left ventricle. This cause hypertrophy of left ventricle.
Ventricular hypertrophy cause rise in left ventricular diastolic
pressure.
Blood flows from the left ventricle to the obstructed aortic valves
into the aorta. This results in increased left ventricular pressure to
overcome the resistance offered by obstructed valve.
Myocardial ischemia may occur in late stages as a result of the
hypertrophied left ventricle and the amount of oxygen that can be
supplied to the myocardium.
Left ventricle may fail resulting in pulmonary edema.
The disorder rarely presence in neonatal period or during evidence
with evidence of decreased cardiac output like fainting, peripheral
pulses, pallor, cool skin.
In older children it present as chest pain, dyspnea, syncope attack,
exercise intolerance.
Complication include:- congestive heart failure and bacterial
endocarditis

Fig 10 pathophysiology of aortic stenosis

CLINICAL FEATURES
Dyspnea

on exertion
May have history of angina on effort and
syncope.
Cardiac size is normal unless left
ventricular failure is present.
Systolic thrill may be palpable at the
second right interspace, suprasternal notch
and carotid arteries.

DIAGNOSIS
Pulse

is low in volume and there is thrill in

the super sterna notch.
Chest X-ray detects dilating ascending
aorta and varying degree of left ventricular
enlargement.
ECG reveals normal and left ventricular
hypertrophy.

MANAGEMENT
If

heart failure occurs in infancy

management is carried out by surgical
intervention.
Surgical management consist of balloon
dilatation, aiotic valvotomy or aiortic
valve replacement.
[valvotomy is done to divide the fused
cusps if the stenosis is at the valvular
level, supravalvular or subaiortic stenosis
is corrected through the excision of the
obstructive tissue.]

PULMONARY STENOSIS
There

is an obstruction to flow of
blood from right ventricle to lungs. It
accounts for 8% of congenital heart
defect.
90% of the obstruction occurs at the
level of pulmonary valve other
outside subvavlvular and
supravalvular pulmonary stenosis.

CONTD..

Infundibular stenosis,there is a muscular or fibrous stenosis,

valvular stenosis caused by the failure of involution. This
obstruction may close to pulmonary artery or venoid. If pressure
from other valvular or infundibular obstruction greatly increased
blood back up in the left atrium resulting present foramen ovale in
neonate allow blood to be shunted in left atrium.
If the right ventricle cannot eject necessary amount of
venous blood in pulmonary circulation, systemic if patent
ductus arteriosus. Pressure blood shunted in aiota to
pulmonary artery and then lungs partially compensating
with obstruction with severe defect persistence is to return
of blood to heart and congestive heart failure.

Fig 12 Normal Heart V/S Pulmonary Stenosis

CONTD..
Infundibular

stenosis can be
distinguished from pulmonary stenosis
by: absence of click
absence of post stenotic dilatation
Relatively lower point of maximum
intensity of systolic murmur in 3-4 th left
interspace

HEMODYNAMICS
Blood

flows from the right ventricle

through obstructive pulmonary valve into
right ventricle pressure increases to
maintain normal cardiac output and right
ventricular hypertrophy occurs in several
cases.
Right sided ventricular failure occurs in
severe cases.

CLINICAL MANIFESTATION
Child

is generally asymptomatic.
Poor exercise tolerance; child become fatigue
with exertional dysphagia, precordial pain.
Presence of murmur in mild stenosis, cyanosis
and cardiac failure may occur with the severe
defect with severe symptoms of severe stenosis.
Complication-anoxic spell in infant, bacterial
endocarditis, congestive heart failure and sudden
death.

Fig 13 Complication of Pulmonary Stenosis

DIAGNOSTIC EVALUATION
Auscultation

reveals systolic ejection,

murmur over pulmonary area.
Chest X-ray reveals enlargement of
ventricle and main pulmonary artery.
ECG shows right ventricular
hypertrophy.

MANAGEMENT
Pre

congestive heart failure is present, surgery is

done like valvotomy, ballon dilatation of
pulmonary obstruction.
Open heart surgery is needed because right
ventricle centred to reach this structure.
If obstruction at ventricular level leaflet of valve
are separated by incision at fused commisure.
If stenosis is infundibular , resection of excess
muscular fibrous tissue is done, if there is ASD it
is closed.
Balloon pulmonary valvuloplasty is the
treatment of choice for isolated valvar
pulmonary stenosis.

COARCTATION OF AORTA.
It

is a narrowing or constriction of
aiorta at any point most commonly
the constriction is located distal to
origin of the left subclavin artery in
the vignity of the ductus arteriosis.

Fig 14 Coarctation of Aorta

HEMODYNAMICS

In fetal life the portion of the aorta distal to the left subclavian and
before the portion where the ductus arteriosus join is called Isthmus. At
birth, isthmus is the narrowest part of the aorta.
Following closure of ductus arteriosus, the descending aorta must
receive its total supply from left ventricle via ascending aorta.
The exact mechanism for the production of systemic hypertension in
coarcation is not known.
The narrowing of aorta obstructs the blood flow through constricted
segment of aorta thus increasing left ventricle pressure and work load.
Collateral vessel develops arise in chiefly between the branches of
subclavin and intercostals artery by passing the coarcation segment of
aorta and subclavin circulation to the lower extremity.
Palpable collaterals are also felt at medial and inferior angel of scapula.
Because of the decompression of the upper segment by collaterals, the
resting blood pressure in upper extremity may even be normal, but rises
on exercise.

CLINICAL MANIFESTATION
Children

are usually asymptomatic in early year.

Growth and development is normal.
Sometime they develop occasional fatigue,
headache,dizziness, nose bleeding and leg cramps.
Femoral pulse absent or greatly reduces. Carotid
pulse is bounding, Blood Pressure reduce
hypertension in upper extremity and diminished in
lower extremity.

CONTD.
Severe

lesions present in infancy with symptoms

of poor feeding growth failure, tachypnea,
peripheral edema, acidosis, low cardiac output
and severe congestive heart failure.
Claudication, pain and weakness of legs and
dyspnea on running.
Systolic thrill may be palpable in suprasternal
notch.

Fig 15 clinical symptom of coarctation of aorta

DIAGNOSTIC EVALUATION
Reversal

of normal blood pressure determination

in arms and legs.
Chest X-ray shows a prominent aorta, rib
notching is common, findings in children older
than 6 years.
ECG shows varying degree of ventricular
hypertrophy.st and t waves changing below the
age of 15 yrs suggest additional aortic stenosis.

MANAGEMENT
Either

surgical or non surgical ballon dilatation or

stunting of coarcation depends on age.
Control of congestive heart failure by an appropriate
drug.
Removal of narrowed portion of aorta with
anastomosis at the end.
Prostaglandlin E1 is used to maintain a ductal
patency prior to surgery in first few weaks of life.
In some cases the graft of transplanted aorta is
inserted.
Age 3-6yrs of age if child condition promotes, not
during infancy.

Fig 16 anastomosis

CYANOTIC HEART DISEASE

Right to left shunt is in two groups:Decreased pulmonary blood flow: Tetralogy of fallot(TOF)
Tricuspid atresia

Increased pulmonary blood flow: Transposition of great vessel

Total anomalous pulmonary venous return.

TETRALOGY OF FALLOT
It

account for 6-10% congenital heart

disease and consist of four abnormality: Pulmonary stenosis
Ventricular septum defect
Overriding of aorta(dextra position of
aorta)
Right ventricular hypertrophy.

Fig 17 Tetralogy Of Fallot

HEMODYNAMICS
Due

to pulmonary stenosis there is obstruction of

blood flow from the right ventricle to pulmonary
artery. Pressure thus rises in the right ventricle
increases. So as to become equal to the ventricle.
Deoxygenated blood is shunted from right
ventricle through the VSD to left ventricle
directly into aorta (right to left shunt)

Fig 17 Hemodynamic Of TOF

CLINICAL MANIFESTATION
Clinical

features depends upon primarily on

degree of pulmonary stenosis and right ventricle
outflow obstruction(many infant with this defect
are not cyanosed at birth they develop cyanosis
as stenosis become relatively more severly)
Cyanosis initially observed while crying and
only on exertion then later even at rest.
In some children infundubular stenosis
may be minimal, so that cyanosis never
develop pink tetrology

CONTD..
Hypercyanotic

spells(hypoxic or anoxic spell)It is characterized by episodes of intend cyanosis

that occur predominantly in morning after
awakening from sleeping, during or after
defecation during or immediately following
feeding. Child start crying,get cyanosed and may
have convulsionor become unconsciousness.
Cyanosis can be seen in mucous membrane, lips,
mouth, pharynx and toe nail.

Fig 18 Child with Cyanosis

CONTD
Clubbing of fingers, toe nails occur by 2-4 yr of age.
Exercise cause severe dyspnea.
Infant take knee chest position rather than expanding their
extremities when they lie down.
Child assumes squatting position to increase pulmonary blood
flow squatting act by increasing systemic resistance and
defecating blood flow to relatively low resistance pulmonary
circuit.
Infant becomes cyanotic and gasp for breath, convulsion or
hemeparesis even death may present.
Slow weight gain, failure to thrive, associated with dyspnea on
exertion.

CONTD
Short episodes of unconsciousness followed by sleep
may persist.
Murmur shortens and cyanosis increased with increasing
severity of right ventricular outflow tract obstruction.

COMPLICATION
Infective

endocarditis
Cerebrovascular accidents
Paradoxical embolism
Anoxic infarction due to anoxic spell.
Brain abscess Polychythemia develops because of body
attempts to compensate for unoxygenated blood.
The resulting increase viscosity of the blood
causes slowing of the circulation and possibly
thrombophlebitis, embolism and cerebrovascular
defect may occur.

DIAGNOSTIC EVALUATION
Cyanosis,

clubbing, polychythemia
Chest X ray reveals heart size is normal and
shape like shoe(boot shaped heart)
Pulmonary segment appear small and concave.
Lung margin is diminished
ECG reveals right axis deviation and left
ventricular hypertrophy.
Lab investigation shows degree of
polychythemia, arterial oxygen desaturation.

MEDICAL MANAGEMENT
Child

should be treated for cyanosis and hypoxic

spell.
Oral B-blocker helps to prevent cyanotic spells.
Maximally tolerated dose of propranol ranging
from 0.5-1.5 mg/kg/dose should be administered.
Treat for dehydration and anemia.
Iron supplementation.
Treatment for congestive heart failure
vigorously.

SURGICAL MANAGEMENT
Palliative

shunt this is used to infant who cannot

undergo primary repair. In this palliative
procedure to increase pulmonary blood flow and
increased oxygen saturation may be performed.
The types of surgery depend upon condition nof
child and on the severity of right ventricular
outflow obstruction.

BLALOCK TAUSING SHUNT

Preferred

procedure is blalock tausing or

modified blalock tausing shunt.
Artificial ductus is created by anastomosis of
branch of aorta(subclavian artery) to pulmonary
artery. This shunt provide blood flow to the
pulmonary artery from the left to right
subclavian artery.
A side to side anastomosis of presenting aorta
and right pulmonary artery in neonate(water
stent shunt)

Fig 19 patch repair

CONTD..
Anastomosis of upper descending aorta and left pulmonary
artery(potts procedure): The particular blood vessel i.e. used to create a systemic
pulmonary anastomosis depends on the childs size because
one i.e too large may result in congestive heart failure.

Fig 20 Anstomosis

CONTD
Mustard

procedure is definitive repair by arterial

switch operation or by redirecting blood flow.
Complete repair(elective surgery); it includes
closure of VSD and resection of infundibular
stenosis with a pericardial patch to enlarge the right
ventricular outflow tract. The procedure requires a
medial sternotomy and a use of cardio pulmonary
bypass.
Corrective surgery at an early stage prevent children
from continuing to be crippled by defect even after
previous surgery has been a success.

TRICUSPID ATRESIA
Failure

of tricuspid valve to develop, so that there is

no communication from right atrium to right
ventricle.
In this condition blood flows through on ASD or a
patent foramen ovale, to the left side of the heart and
through a VSD to the right ventricle and out to
lungs.
It is associated with pulmonary stenosis and
transposition of great artery.
There is complete mixing of unoxygentaed and
oxygenated blood in left side of heart resulting in
systemic desaturation and pulmonary obstruction
which result in decreased pulmonary blood flow.

Fig 21 Normal Heart V/S Tricuspid Atresia

CLINICAL MANIFESTATION
Left

ventricular type of apical pulse

Prominent large wave in jugular venous
pulse
Enlarged liver
Cyanosis of lip, skin ,nails
Tachycardia
Dyspnea or laboured breathing
Cool calmy skin
Chronic hypoxiemia with clubbing.

DIAGNOSTIC EVALUATION
Chest

X-ray
ECG left ventricular hypertrophy.
Cardiac catheterization

PATHOPHYSIOLOGY
At

birth presence of patent foramen ovale is

required to permit blood flow across the septum
into the left atrium.
The PDA allows blood to flow to pulmonary
artery into the lungs for oxygenation.
VSD allows some amount of blood to enter right
ventricle and pulmonary artery for oxygenation.
In this condition blood flow is decreased.

Fig 22 changes in tricuspid atresia

MANAGEMENT
In

neonate whose pulmonary blood flows

depend upon the patency of ductus
arteriosis in continuous infusion of
prostaglandlin-E, started at 0.1 mg/kg of
body weight/min until surgical treatment
not done.

SURGICAL MANAGEMENT
Palliative

treatment is the placement of the

shunt(pulmonary tube systemic artery
anastomosis): To decrease blood flow to lungs.
If ASD is small than atrial septostomy is done
during cardiac catheterisation.
A direction glnn shunt(cardio pulmonary
anastomosis) may be performed at 6-7 month at
the 2nd stage.

Fig 23 surgical repair

MODIFIED FONTAN
PROCEDURE
In

this procedure systemic venous return directed to

the lungs without ventricular pump through
surgical connection between right atrium and
pulmonary artery.
A fenestration(opening) in the right atrial baffle is
sometimes done to relieve pressure.
The patient must have normal ventricular pressure
and low pulmonary vascular procedure to be
successful.
The modified fanton procedure separate
oxygenation and unoxygentaed blood inside the
heart and eliminate the excess volume load on
ventricle but does not restore normal antomy.

Fig 24 fontan procedure

TRANSPOSITION OF GREAT
VESSEL(TGA)
TGA occurs

when the pulmonary

arteries originate from the left
ventricle and aorta originates from the
right ventricles.

HEMODYNAMICS
This

result in two separate circulations the

right heart manages the systemic circulation
and left heart manages the pulmonary
circulation.
This is just opposite of normal circulation.
Aorta carries unoxygenated blood to the
systemic circulation and pulmonary circuit
circulation carries oxygenated blood back to
lungs. Pulmonary vessels return back to
atrium.

Fig 25. Normal heart V/S transposition of great vessel

CONTD
Two

circulatory systems exists one pulmonary and

one systemic to sustain life there must be an
accompanying and unoxygenated blood between
the two circulations.
Mixing of oxygenated and unoxygenated blood
through one or more of the following shunt
through ASD, VSD and PDA(these existing lesions
provide a means for mixing venous and atrterial
blood.PDA crosses functionally and anatomically
in neonatal period.)
ASD(foramen ovale) VSD remain open causing
increased blood flow through lung.

Fig 26 changes in transposition of great vessel

CLINICAL MANIFESTATION
These

manifestations are influenced by extent of

intercirculatory mixing. These children present with
cyanosis and unable to suck.
Rapid breathing and hypoxemia within first weak of life.
Murmur sound present
Clubbing of fingers and toes
Easy fatiguability
Slow weight gain
Metabolic acidosis
Failure to thrive
Hypopnea, tachypnea
Cardiomegaly.

COMPLICATION
Infective

endocarditis
Brain abscess
Cerebro vascular accident due to
thrombosis
Severe hypoxia.

DIAGNOSTIC EVALUATION
Chest

X-ray shows cardiomegaly with a

typical egg on site. The right upper lung
fields appear more plethoric than other
areas.cardiomegaly present.
Pulmonary vascular markings are
increased
ECG shows right axis deviation and right
or bi-ventricular hypertrophy.

MEDICAL MANAGEMENT
Treating

CHF vigorously palliative and

corrective procedure
Prostaglandlin E1 can help to reduce cyanosis in
selected cases by keeping the PDA open
Palliative procedure This include the non surgical technique of
enlarging of existing foramen ovale or ASD so
that interarterial mixing of blood can occur by
pulling a balloon through the defect. Balloon
septostomy during cardiac catheterization
RASKIN PROCEDURE.

SURGICAL TECHNIQUE
Palliative

creating atrial septum defect

(blalock hanlon operation)
Pulmonary artery bending done to decrease
pulmonary hypertension to presence of
ventricular septum defect.

CONTD

Surgical creation of ductus arteriosus if pulmonary stenosis and

VSD exist. Total creation or definitive repair by atrial stich
operation or by redirecting the blood flow by mustard or
senning procedure.in this a new arterial septum is created from
pericardium or with a prosthesis with result that two separate
function correct atria are formed. Systemic venous
deoxygenated blood returning to right atrium is redirected to
mitral valve into the left ventricle and into the pulmonary artery
and to lung oxygenated blood that returns to its ventricle is
redirect to tricuspid valve to right atrium and then into the
systemic circulation to the aorta this procedure reverse the
function of aorta but does not transplant the transverse position.

Fig 27 senning operation

CONTD
Arterial

switch operation is now established as

treatment of choice in TGA in which pulmonary
artery and aorta are transected.
Systemic improvement after surgeries i.e.
exercise tolerance improves, cyanosis and
clubbing disappear.

Fig 28 Arterial Switch Operation

TOTAL ANOMALOUS
PULMONARY VENOUS
CONNECTION (TAPVC)
All

the pulmonary veins instead of joining

the left atrium are connected anomalously
to result in the total pulmonary venous
blood reaching the right atrium. The
anatomical classification of TAPVC is into
supracardiac, cardiac, ifracardiac and
mixed varieties.

Fig 29 normal V/S TAPVC

HEMODYNAMICS

TAPVC results in pulmonary venous blood reaching the right

atrium, which also receives the systemic venous blood.
This results in almost complete mixing of the two venous returns.
The blood flow to the left atrium is the right to left through a
patient foramen ovale or atrial septal defect.
The oxygen saturation of the blood in the pulmonary artery is
often identical to that in the aorta because of mixing of blood in
right atrium.
Physiologically TAPVC cann be divided into { patient with
pulmonary venous obstruction and patient without pulmonary
venous obstruction}
Pulmonary venous obstruction results in pulmonary arterial
hypertension and as well as restriction in blood flow.
In absence of pulmonary obstruction blood flow is large and
result in cardiac failure between 4-10 weak of age.

Fig 30 changes in TAPVC

CLINICAL FEATURE
Cyanosis
Failure

to thrive
Cardiomegaly
Hyperkinetic precordium normal or accentuated
pulmonic component. A grade two to four
pulmonary ejection systolic murmur and a
tricuspid flow murmur.tricuspid regurgitation

DIAGNOSTIC INVESTIGATION
ECG

shows right axis deviation and right

ventricular hypertrophy.
X-ray of obstructive TAPVC shows
normal heart size with severe pulmonary
venous hypertension resulting in ground
glass appearance of lungs very much like
hyaline membrane disease.

MANAGEMENT
Operation

is indicated as soon as possible

in 80% cases of infants die within first 3
months of life without surgical help.
Obstructed TAPVC need surgery in short
notice.
Long time recovery time is needed.
These patients are prone to develop
pulmonary hypertensive crisis in the
postoperative period.

ACUTE RHEUMATIC FEVER

Acute

rheumatic fever (ARF); is the

leading cause of acquired heart disease in
pediatric patient. The school age children
are more susceptible to disease more
common in children related to poor socio
economic status.
Rheumatic fever is a group of bodily
response to streptococcus.

ETIOLOGY
This

is an immunological disorder caused by

damage to the heart by antibodies. These antibodies
are produced in response way primary infection to
throat or skin by a group of b-hemolytic
streptococci.
History of sore throat may present.
Following untreated throat streptococcal sore throat
is in latent period last from 10 days-7 weak. During
this period antibodies formed to streptococcal
antigen. These antigens have capacity to reactive
with connective tissue.
This antigen antibody reaction result in rheumatic
fever.

Fig 32 Causes Of Acute Rheumatic Fever

PATHOPHYSIOLOGY
During

acute stage there is exudates influence

reaction in connective tissue of joints, heart and skin.
There is edema and lymphatic infiltration
All layers of heart are affected.
In joints there is edema, articular and periarticular
infiltration of synovial membrane and effusion.
Histopathology aschaff body which is pathogenic of
rheumatic disease chacterized by large
multinucleated.
Cells arranged in pervascular area around and
avascular fibrinoid material.

CLINICAL FEATURES(MAJOR
CLINICAL MANIFESTATION)
Fever(range

102-1040F)
Polyarthritis i.e. joints shows limited range of
motion, inflammation , tenderness,
swelling(generally in large
joints,knees,wrist,elbow,shoulder and hip)
Other chacteristics:-Migrating and reversible
nature of progress helps to differentiate from
other of arthritis.

Fig 34 clinical feature of acute rheumatic fever

CONTD..
Carditis-

Inflammatory hemorrhagic blue lesion

known as aschaff body or nodules present in
interstitial tissue of the heart in response of rheumatic
myocarditis, vegetation also form as result of
myocarditis.
The vegetation occurs mainly on the mitral aiortic
valve and become scar, when fibrous area healed.
Carditis often seems as CHF, cardiomegaly,
pericardiac effusion, pericarditis.Sign and symptomsTachycardia, Murmur,Prediastolic gallop, Precardia
friction rub, Precardial pain.

CONTD
Stenosis-

stenosis of the leaflets (valves) or cusp

occurs because of their fusing together. This
process causing obstruction to the fl;ow of blood
to left ventricle for aiorta or both valves. Become
scarded that may cannot completely close
causing a back flow or regurgitation (valvular
insufficiency) when the valve closed.

CONTD
Chorea(syndenhens

chorea)- saint witus danee

children have involuntary, sudden random
movement of extremity, emotional liability, sleep
disturbance and severe muscle weakness, facial
grimaces(involuntary)
Erythema marginatum-Rash is reddish; lesions are
non pruritic and appear as undifferentiated macule
on trunk and inner aspect of extremity. These
macule join together in a connective pattern giving
chicken wire appearance on skin.

CONTD
Subcutaneous

nodules- These are present on

elbow, knees, ankles, over scalp, occiput over
long prominence and skin. Careful observation
of prominence is must because the nodules of
rheumatic fever are seen than palpated.

MINOR COMPLICATION
Low grade fever(high in afternoon)
Weakness, fatigue, weight loss, epistaxis with no
known cause and abdominal pain.
Prolonged PR interval in ECG

COURSE OF DISEASE
Cardiac

involvement may result in permanent

damage to valves causing long lasting heart
lesion aortic or mitral regurgitation, mitral
stenosis, tricuspid regurgitation i.e. classified
and referred as rheumatic heart disease.

DIAGNOSTIC EVALUATION
ASO(antistreptolysin

Otitre):ASO titer is antibody reaction to streptococcal infection.

Children over 5 years of age a titre of 333 todd unit is
considered indicative other test can be used to determine
serologic evidence of streptococcus infection.
CRP(C-reactive protein):CRP is absent in sera of normal individual. One positive
culture is firm, 3 throat swabs at varying time interval for
confirm of group A, B-hemolytic streptococci. These
cultures should be taken atleast 2 before starting antibiotic
therapy.
Throat culture.
ESR

ESSENTIAL CRITERIA
Increase

ASO titer
Scearlet fever
Positive throat culture

MEDICAL MANAGEMENT
Focus

of treatment is: Suppression of acute inflammatory process.

Eradication of streptococcal infection.
Prevention of disease recurrence.

MANAGEMENT
Start

steroid and aspirin: If heart involvement is present prednisolone 1.5

mg/kg/day in 3 divided doses is given for 3
weeks. Taper the dose by 5 mg/wk and
discontinue after 12 wks simultaneously start.
Start aspirin 100 mg/kg/day in 3 divided doses in
uncompleted Rh Fever and before 12 weeks.
Always give aspirin after meal to avoid gastritis
and watch for side effect.

CONTD
Penicillin: Give

proclaimed penicillin 4 lakhs unit IM BD for

10 days to avoid streptococci.
Start benzoteine penicillin 12 lakhs IM or IV on 8th
day of proclaimed penicillin therapy. These
infections are painful. If the child is sensitive to
penicillin erythromycin therapy to reduce
streptococcal infection.
Steroid therapy may result in cushingoid features,
hurshitism, and increased susceptibility to
infection.

NURSING MANAGEMENT
Bed

rest: Assist child and parent to understood need for

amount of bed rest i.e. complete bed rest till the
rheumatic activity subsides. Exertion can aggrevate
symptoms. Bed rest to be continued from 2-3 wks
to 2-3 months very strictly, if cardiac involvement
is there.
Diet: Restrict salt intake only if feature of CHF present
otherwise normal diet rich in iron prevent anemia.
Due to fever child may be anorexic. Encourage
fluid intake to prevent dehydration. Over feeding
has to be avoided.

CONTD
Accident

prevention: Warm and cool alternative day to affect joint to

reduce swelling and inflammation to reduce
pain.
Bed cradle to minimize pressure on affected
joints.
Use side rails to prevent falling, padded side of
bed or chair to prevent injury; when involuntary
jerking and violent movement occurs.
Clothing according to weather.

CONTD
If

child develops chorea and behavioral changes

occur learning difficulty should be discussed
with teacher, classmates.
Monitor daily weight, intake output chart,
resting and sleeping heart rate in absence of
febrile state. Nurse must encourage parent and
child continue follow up care even when
condition of child is improved.

PREVENTION
Primary

prevention: Diagnosis and treat all cases of sore throat by

penicillin or erythromycin for 10 days to prevent
Rh fever.
Secondary prevention: After Rh fever occur start child on 3wkly
benzathine penicillin. Continue for atleast 5 yrs
if there is no heart involvement. Treatment need
to be taken lifelong if cardiac involvement is
there, this prevent recurrence of Rh fever.

RHEUMATIC HEART DISEASE

Rheumatic

heart disease is permanent damage to the

heart following rheumatic fever. It can lead to heart
failure and sometimes the need for cardiac surgery.
Rheumatic heart disease is the most common form
of heart disease in children in the world.
A heart valve acts like a one-way door. It makes
sure that blood pumped by the heart flows in one
direction. When the heart is damaged, the heart
valves are unable to function adequately.

SYMPTOMS
People

who get rheumatic heart disease sometimes

end up very sick because the blood stops flowing
the right way, making them tired and short of
breath
Chest pain
Heart palpitations
Breathlessness on exertion
Breathing problems when lying down
Waking from sleep with the need to sit or stand up
Swelling
Fainting

MITRAL REGURGITATION (MR)

This

occurs when the mitral valve allows

reversal of blood flow from the left
ventricle (LV) to the left atrium.

PATHOPHYSIOLOGY
Normal blood flow from the left atrium to the LV and,
subsequently, to the systemic circulation, is altered in mitral
regurgitation.
In the presence of mitral regurgitation, blood flows antegrade
from the LV into the aorta, and the regurgitant volume flows
retrograde from the LV into the left atrium.
This causes a proportionate increase in LV ejection volume. The
regurgitant fraction reenters the LV, producing left ventricular
volume overload.
The LV compensates via the Frank-Starling mechanism,
resulting in a greater ventricular stroke volume.

CONTD
The volume of the regurgitant fraction depends on
several factors, including size of the orifice allowing
regurgitation and the pressure gradient between the left
ventricle and left atrium.
This volume also depends on ventricular systolic
pressure; therefore, the regurgitant volume increases in
situations that increase afterload, such as hypertension or
aortic stenosis.

COMPLICATIONS
In

patients with mechanical prostheses, too much

warfarin may result in excessive bleeding,
whereas insufficient anticoagulation may lead to
thromboembolism.

OPTIMAL MEDICAL THERAPY

Diuretics

are also helpful in decreasing the total

volume and may alleviate the pulmonary edema and
congestion that may be present.
Digoxin is useful in patients with left heart failure
because it allows the heart to pump more efficiently.
Vasodilators, such as nitroprusside, are very
effective; however, preexisting hypotension may be
exacerbated.
Anticoagulation may be needed if diminished left
ventricular function or atrial fibrillation or evidence
of thromboembolism.

CONTD
Inotropic agents may improve systolic blood pressure.
Intra-aortic balloon counterpulsation or immediate
surgical intervention (valvuloplasty) may be necessary in
severe cases.
In patients who stabilize but remain symptomatic, early
semielective surgery should be considered to reduce the
risk of irreversible ventricular dysfunction.
Patients who become asymptomatic with medical
therapy can be treated in the same manner as those with
chronic mitral regurgitation.

SURGICAL CARE

Transcatheter management, mitral valve clips may be a therapeutic

option.
Surgical repair of the regurgitant mitral valve can be classified into 3
major groups depending on the leaflet motion, namely, normal,
prolapsing, and restricted.
If the mitral annulus is dilated, an annuloplasty may be successful in
alleviating the degree of regurgitation. The annuloplasty may involve
the use of a ring prosthesis. In younger patients (in whom restriction
of valve growth is undesirable), resection of a portion of the leaflet
and annular plication may be performed.
Shortening of the chordae and/or papillary muscles may repair
prolapsed leaflets. Lengthening may be required in cases where there
are short chordae
Mitral regurgitation with restricted leaflet motion is observed in
parachute and hammock valves and, along with a valvuloplasty, can
be improved by incising the valve leaflets at an appropriate location.

CONTD
Quadrilateral resection of a prolapsing leaflet with sliding
annuloplasty may be helpful in cases with mitral valve
prolapsed
E-to-E mitral valve repair is help in some cases in which an
opposition suture in placed in the center of the anterior and
posterior leaflets producing a double orifice
Mitral valve replacement is the final option in the treatment of
mitral regurgitation. The choice of which valve to use
(mechanical vs bioprosthesis) can be difficult.
Bioprosthetic valves resolve the anticoagulation issue but raise
problems of their own. Bioprostheses may degenerate rapidly
and may become calcified and dysfunctional as early as 6
months after insertion.

MITRAL STENOSIS
Mitral

stenosis is a disorder in which the mitral

valve does not fully open. This restricts the flow
of blood.

CAUSES
Mitral

stenosis means that the valve cannot open

enough. As a result, less blood flows to the body. The
upper heart chamber swells as pressure builds up.
Blood and fluid may then collect in the lung tissue
(pulmonary edema), making it hard to breathe.
The valve problems develop 5 - 10 years or more
after having rheumatic fever. Symptoms may not
show up for even longer.
Children may be born with mitral stenosis
(congenital) or other birth defects involving the heart
that cause mitral stenosis.
Mitral stenosis may run in families.

SYMPTOMS

Chest discomfort that increases with activity and extends to the arm,
neck, jaw or other areas (This is rare)
Cough, possibly with bloody phlegm
Difficulty breathing during or after exercise, or when lying flat;
Waking up due to breathing problems (This is the most common
symptom)
Fatigue
Frequent respiratory infections, such as bronchitis
Feeling of pounding heart beat (palpitations)
Swelling of feet or ankles
In infants and children, symptoms may be present from birth (congenital).
It will almost always develop within the first 2 years of life. Symptoms
include:
Cough
Poor feeding, or sweating when feeding
Poor growth
Shortness of breath

EXAMS AND TESTS

The health care provider will listen to the heart and lungs with a
stethoscope. A murmur, snap, or other abnormal heart sound may
be heard. The typical murmur is a rumbling sound that is heard
over the heart during the resting phase of the heartbeat. The sound
often gets louder just before the heart begins to contract.
The exam may also reveal an irregular heartbeat or lung
congestion. Blood pressure is most often normal.
Narrowing or blockage of the valve or swelling of the upper heart
chambers may be seen on:
Chest x-ray
CT scan of the heart
Echocardiogram
ECG (electrocardiogram)
MRI of the heart
Transesophageal echocardiogram (TEE)

TREATMENT

Diuretics (water pills)

Nitrates, beta-blockers
Calcium channel blockers
ACE inhibitors
Angiotensin receptor blockers (ARBs)
Digoxin
Anticoagulants (blood thinners) are used to prevent blood clots
from forming and traveling to other parts of the body.
Antibiotics may be used in some cases of mitral stenosis.
People who have had rheumatic fever may need long-term
preventive treatment with the antibiotic penicillin.
In the past, most patients with heart valve problems were given
antibiotics before dental work or invasive procedures, such as
colonoscopy. The antibiotics were given to prevent an infection
of the damaged heart valve.

SURGICAL TREATMENT
Percutaneous mitral balloon valvotomy (also called
valvuloplasty). During this procedure, a tube (catheter) is
inserted into a vein, usually in the leg. It is threaded up
into the heart. A balloon on the tip of the catheter is
inflated, widening the mitral valve and improving blood
flow. This procedure may be tried instead of surgery in
people with a less damaged mitral valve.
Surgery to repair or replace the mitral valve.
Replacement valves can be made from different
materials. Some may last for decades, and others can
wear out and need to be replaced.

(PROGNOSIS)

The outcome varies. The disorder may be mild,

without symptoms, or may be more severe and
become disabling over time. Complications may
be severe or life-threatening. In most cases,
mitral stenosis can be controlled with treatment
and improved with valvuloplasty or surgery.

POSSIBLE COMPLICATIONS
Atrial

fibrillation and atrial flutter

Blood clots to the brain (stroke), intestines,
kidneys, or other areas
Congestive heart failure
Pulmonary edema
Pulmonary hypertension

TRICUSPID
REGURGITATION
The

valve is leaky or doesn't close tight

enough, causing blood to leak backwards
across the valve

Fig 35 Tricuspid Regurgitation

ETIOLOGY
Infection,

such as rheumatic fever or infective endocarditis

A dilated right ventricle, causing the annulus (a ring of
tough fibrous tissue which is attached to and supports the
leaflets of the valve) of the tricuspid valve to enlarge
Increased pressure through the tricuspid valve (seen with
pulmonary hypertension)
Less common causes include congenital defects, trauma,
carcinoid heart disease, tumor, tricuspid valve prolapse,
Ebstein's anomaly, systemic lupus, and trauma.
Tricuspid valve disease, if caused by rheumatic fever, is
often combined with mitral and/or aortic valve disease.

SYMPTOMS MAY INCLUDE:

Irregular

heart rhythm (atrial fibrillation)

Easily tired (fatigue)
A fluttering discomfort in the neck
With severe disease, heart failure
symptoms (right abdominal pain,
shortness of breath, swelling in the legs or
abdomen, cold skin)

HOW IS TRICUSPID VALVE

DISEASE DIAGNOSED?
Tricuspid valve disease may first be diagnosed during a
physical exam. The doctor will often hear a murmur
(abnormal blood flow through the valve). Other signs your
doctor may find are an irregular pulse and a fluttering or
abnormal pulsation in your neck (jugular vein).
Tests used to diagnose valve disease may include:
Electrocardiography (ECG)
Chest X-ray
Echocardiography
Transesophageal echocardiography
Cardiac Catheterization (cardiac cath or angiogram)
Radionuclide scans

Fig 36 Lanyard and template handle assists with placement of ring

SURGICAL MANAGEMENT

Tricuspid Valve Repair

When valve disease is severe, it may be necessary
to repair or replace the diseased valve. Tricuspid
valve repair using an annuloplasty ring is the
preferred surgical approach for tricuspid
regurgitation and may be performed for primary
tricuspid disease or for combined cases with other
valve surgery (mitral, aortic).

Fig 37 Anatomically correct design conforms to the 3-D tricuspid valve opening.

AORTIC REGURGITATION
Aortic

regurgitation in children is usually found

in association with a bicuspid aortic valve.
The aortic valve connects the left ventricle to the
aorta. As the ventricle starts to contract to pump
blood to the body, pressure builds. This increase
in pressure causes the aortic valve to open,
allowing blood to pass from the left ventricle
into the aorta. As the heart then starts to relax,
pressure falls and the valve closes.

CONTD
Aortic

regurgitation refers to a leak of blood

backwards from the aorta into the left ventricle
because of inadequate or incomplete closure of
the aortic valve. Aortic regurgitation is also
called aortic valve insufficiency, or simply an
aortic valve leak.

CAUSES

Aortic regurgitation in children is usually

the result of abicuspid aortic valve. Normally
the aortic valve typically has 3 separate pieces or
leaflets. A bicuspid aortic valve is a valve which
has only two leaflets instead of the normal three.
Typically 2 out of the 3 leaflets have fused
together during development in the womb.

CONTD.

Aortic regurgitation can also happen in children who

have enlarged aortas from any cause. As the aorta
enlarges, it may actually pull the valve leaflets apart.
This can lead to an open space in the middle of the valve
where blood can then flow backwards into the left
ventricle while the valve is trying to close. Enlarged
aortas are often seen in the setting of Marfan syndrome
or other connective tissue disorders.

CONTD

Aortic regurgitation in children can also be seen in the

setting of a ventricular septal defect. VSDs that are
positioned very close to the aortic valve may create a jet
effect that can actually distort and deform the valve.
Over time, this may result in the development of aortic
regurgitationy

CONTD

Less common causes of aortic regurgitation include

infections and other inflammatory conditions. Rarely
bacteria may attack the aortic valve causing endocarditis.
The valve gets destroyed or damaged by the infection
and therefore does not work properly.

SYMPTOMS

Aortic regurgitation is a potential problem in that it creates an

extra workload or stress on the heart.
The left ventricle is forced to pump the normal amount of blood
coming forward into it, plus any additional blood that leaks
backwards.
Over time, this can creates an extra burden and workload on the
heart that may tire it out over the course of several years. This is
usually only a problem with a significant degree of aortic
regurgitation.
Symptoms caused by aortic regurgitation are variable. Many
children are identified by the presence of an asymptomatic
heart murmur.
Other children may present with symptoms of fatigue or
decreasing exercise tolerance.
Symptoms such aschest pain,palpitations, orsyncopeare
unusual in the setting of aortic regurgitation.
Infants with aortic valve insufficiency may haverapid breathing,

TREATMENT
When

aortic regurgitation is significant, it usually

must be addressed by surgery.
Medication may assist the heart in tolerating the
extra workload but does not do anything to decrease
or lessen the degree of leaking in most cases.
The most common indication for surgery in pediatric
patients is the development of symptoms or
evidence of a progressively enlarging heart size.
Surgery usually involves completely replacing the
aortic valve, although in some instances the valve
may be able to be successfully repaired without
needing to be replaced.

NURSING MANAGEMENT OF
A CHILD WITH HEART
DISORDER

NURSING ASSESSMENT
Complete thorough history of child and family should be taken.
Child growth and development pattern, exercise intolerance.
Observe childs skin, mucous membrane for colour and temperature
change.
Extremities-Are extremities cold? is their difference present between
upper and lower extremity, check quality of pulse in all limbs, check
edema in extremity.
Observe for clubbing increase in soft tissue around terminal phlenges
of finger especially thumb nail. This may occur in cyanotic children one
year of age.
Observe for any chest deformity and precordial pulse.
Auscultate apical pulse rate for full one minute.
Determine cardiac rhythm or any change
Record vital signs
Look for any other congenital defect, abnormal fascia or relevant history.

RELIEVING RESPIRATORY DISTRESS

Determine the degree of respiratory distress.

Observe pattern of regularity of respiratory pattern.
Observe for nasal flaring, listen for grunting and observe any
response to change of positioning.
Raise the head end upto 45o degree angle to increase the pressure of
viscera from the diaphragm and increase lung volume.
Feed slowly allowing frequent resting period. To reduce the risk of
aspiration.
Observe for abdominal distension which may increase respiratory
difficulty, if distension present inserts NG tube for the relief.
Suction nose and mouth if the child is not able cough out the
secretion.
Provide oxygen therapy as required.
Administer diuretics as ordered to reduce lung congestion, monitor
intake output chart; monitor effectiveness, specific gravity of urine
and restrict fluid as required.

IMPROVING CARDIAC OUTPUT

Organize

nursing care to provide period of

uninterrupted rest.
Prevent excessive crying.
Provide diversion activities.
Proper medication should be given as per
prescription.
Avoid excessive temperature.
Prevent constipation.

IMPROVING OXYGENATION AND ACTIVITY

TOLERANCE
Provide

oxygen therapy.
Observe child response to oxygen.
Observe child response while weaned off
from oxygen.

PROVIDE ADEQUATE NUTRITION

Feed

slowly in semierect position.

Burp infants after each ouns to decrease
compression of stomach on heart and lungs.
Provide small and frequent feed.
Monitor strict intake and output chart.
Monitor daily weight.
Proper diet chart should be maintained to follow
high nutritive value.

PREVENT THE INFECTION

Prevent

exposure to communicable disease

including exposure to children with upper
respiratory infection.
Ensure that child is completely immunized.
Prevent cold stress.
Report any symptoms of fever, diarrhoea and
vomiting.
Be specific that child receive prophylactic
antibiotics for endocarditis before any dental
procedure or urinary instrumentation.

REDUCE FEAR AND ANXIETY OF

PARENTS AS WELL AS CHILD
Prepare

child and parent for corrective surgery.

Refer family to appropriate resources concerned
with the financial or emotion aspect of caring
child with CHD.

SUMMARY

The human heart is an astonishing organ. A muscle

only about the size of your fist, it sits just to the left of
the center of your chest contracting and relaxing to
pump bloodroughly five liters of it a minute
throughout your body. It is an involuntary muscle.
commonly called heart disease (though, more accurately
known as coronary artery disease) is, interestingly enough,
not a disease of the heart at all. Its a disease of the large
arteries outside the heart that supply the smaller vessels that
feed the heart muscle with blood rich in nutrients and oxygen
that the heart needs to keep working. Coronary arteries, the
large arteries carrying blood to the heart muscle, are like the
huge pipes that carry water from a reservoir to a big city, to
be distributed to streets, individual houses, and then specific
faucets before being carried away again through drains.