CONGENITAL HEART DISEASES

Presented by Sharoon rufan RN, BSN

 Objectives
 At the end of this presentation the students will be able to
 Explain Fetal circulation

 Elaborate Congenital malformations of heart

 Define Rheumatic heart disease

 understand pharmacological management and Nursing Care

approaches while dealing with clients having congenital

heart diseases
 Fetal circulation
 Fetal circulation refers to the unique circulatory system
present in an unborn baby (fetus) during pregnancy. The
fetal circulatory system is adapted to allow the exchange of
nutrients, oxygen, and waste products between the fetus
and the mother while the baby is developing in the womb. It
differs significantly from the circulatory system present after
birth, as certain structures and pathways are not needed
until the baby starts breathing and obtaining oxygen on its
own.
 Important structures of fetal circulation
 Placenta: The placenta is an organ that develops during pregnancy
and acts as a bridge between the mother's blood supply and the
fetus. Oxygen and nutrients are exchanged through the placental
membrane, while waste products are eliminated from the fetal
bloodstream.
 Umbilical Cord: The umbilical cord connects the fetus to the

placenta. It contains two umbilical arteries that carry deoxygenated

blood from the fetus to the placenta and one umbilical vein that
carries oxygenated blood from the placenta to the fetus.
 continue...
 Foramen Ovale: In the fetal heart, there is a small opening called
the foramen ovale between the two atria (upper chambers). This
opening allows some of the oxygen-rich blood returning from the
placenta to bypass the fetal lungs and flow directly into the left
atrium.
 Ductus Arteriosus: Another important fetal adaptation is the ductus

arteriosus, a blood vessel that connects the pulmonary artery

(leading to the lungs) to the aorta (leading to the body's systemic
circulation). This allows most of the blood from the right ventricle to
bypass the lungs and flow directly into the systemic circulation.
 continue...
 Ductus Venosus: This vessel shunts a portion of the
oxygenated blood from the umbilical vein directly into the
inferior vena cava, bypassing the liver. This helps supply
oxygen and nutrients to the developing fetal tissues.
continue...
 Changes after birth
 After birth, several changes occur as the baby begins to breathe air
and adapt to life outside the womb:

 First Breath: When the baby takes its first breath, the lungs expand,
and pulmonary blood flow increases. This decrease in resistance in
the pulmonary circulation causes the closure of the ductus
arteriosus.
 continue...
 Closure of Foramen Ovale: As blood flows into the lungs,
pressure in the left atrium increases, leading to the closure
of the foramen ovale. This prevents blood from shunting
directly between the atria.
 Closure of Ductus Venosus: With the umbilical cord

clamped and cut after birth, blood flow through the ductus
venosus ceases. This vessel will eventually close and become
a ligament within a few days to weeks.
 Congenital heart diseases
 Congenital heart diseases (CHDs) are a group of
structural or functional abnormalities in the heart that
are present at birth. These conditions can affect the
heart's chambers, valves, blood vessels, and electrical
conduction system. these diseases vary in severity,
ranging from mild conditions that might not require
treatment to severe defects that can be life-
threatening and necessitate medical intervention.
Types of congenital heart diseases
 1.Acynotic (a.increased pulmonary blood flow)

 1.Atrial Septal Defect (ASD):

An opening in the wall
(septum) between the heart's
upper chambers (atria). This
can lead to abnormal blood
flow between the atria.
Types of the atrial septal defect
 Ostium primum (ASD 1): Opening at lower end of
septum; may be associated with mitral valve
abnormalities
 Ostium secundum (ASD 2): Opening near center of

septum
 Sinus venosus defect: Opening near junction of superior

vena cava and right atrium.

 Pathophysiology

left atrial pressure slightly exceeds right atrial pressure, blood
flows from the left to the right atrium, causing an increased flow
of oxygenated blood into the right side of the heart. Despite the
low pressure difference, a high rate of flow can still occur because
of low pulmonary vascular resistance and the greater
distensibility of the right atrium, which further reduces flow
resistance. This volume is well tolerated by the right ventricle
because it is delivered under much lower pressure than with a
VSD. Although there is right atrial and ventricular enlargement,
cardiac failure is unusual in an uncomplicated ASD.
 Surgical treatment
 Surgical patch closure (pericardial patch or Dacron patch) is done for
moderate to large defects. Open repair with cardiopulmonary bypass is
usually performed before school age.
2.Ventricular Septal Defect (VSD)
 Ventricular Septal Defect
(VSD): A hole in the septum
between the heart's lower
chambers (ventricles), allowing
blood to flow between them.
 Pathophysiology
 Because of the higher pressure within the left ventricle and because
the systemic arterial circulation offers more resistance than the
pulmonary circulation, blood flows through the defect into the
pulmonary artery. The increased blood volume is pumped into the
lungs, which may eventually result in increased pulmonary vascular
resistance. Increased pressure in the right ventricle as a result of left-
to-right shunting and pulmonary resistance causes the muscle to
hypertrophy. If the right ventricle is unable to accommodate the
increased workload, the right atrium may also enlarge as it attempts to
overcome the resistance offered by incomplete right ventricular
emptying.
 Surgical management
 Complete repair (procedure of choice): Small defects are repaired with
sutures. Large defects usually require that a knitted Dacron patch be
sewn over the opening.
3.Atrioventricular Canal Defect
 Incomplete fusion of the
endocardial cushions. Consists
of a low ASD that is continuous
with a high VSD and clefts of
the mitral and tricuspid valves,
which create a large central AV
valve that allows blood to flow
between all four chambers of
the heart
 Pathophysiology
 The alterations in hemodynamics depend on the severity of the
defect and the child's pulmonary vascular resistance. Immediately
after birth, while the newborn's pulmonary vascular resistance is high,
there is minimum shunting of blood through the defect. When this
resistance falls, left-to-right shunting occurs, and pulmonary blood
flow increases. The resultant pulmonary vascular engorgement
predisposes the child to development of HF.
 4.Patent Ductus Arteriosus (PDA)
 Patent Ductus Arteriosus (PDA):
Failure of the fetal ductus
arteriosus (artery connecting the
aorta and pulmonary artery) to
close within the first weeks of life.
The continued patency of this
vessel allows blood to flow from
the higher pressure aorta to the
lower pressure pulmonary artery,
which causes a left- to-right
shunt.
 Pathophysiology
 The hemodynamic consequences of PDA depend on the size of the
ductus and the pulmonary vascular resistance. At birth, the resistance in
the pulmonary and systemic circulations is almost identical so that the
resistance in the aorta and pulmonary artery is equalized. As the
systemic pressure comes to exceed the pulmonary pressure, blood
begins to shunt from the aorta across the duct to the pulmonary artery
(left-to-right shunt). The additional blood is recirculated through the
lungs and returned to the left atrium and left ventricle. The effects of
this altered circulation are increased workload on the left side of the
heart, increased pulmonary vascular congestion and possibly resistance,
and potentially increased right ventricular pressure and hypertrophy.
 Medical management
 Administration of indomethacin (a prostaglandin inhibitor) has proved
successful in closing a PDA in preterm infants and some newborns.
1.Acynotic (b. obstruction to the blood flow to the ventricles)

 1.Coarctation of the Aorta:

Localized narrowing near the
insertion of the ductus
arteriosus, which results in
increased pressure proximal to
the defect (head and upper
extremities) and decreased
pressure distal to the
obstruction (body and lower
extremities).
 Clinical manifestations
 The patient may have high BP and bounding pulses in the arms, weak
or absent femoral pulses, and cool lower extremities with lower BP.
There are signs of HF in infants. In infants with critical coarctation, the
hemodynamic condition may deteriorate rapidly with severe acidosis
and hypotension.
 Surgical treatment
 Repair is by resection of the coarcted portion with an end-to-end
anastomosis of the aorta or enlargement of the constricted section
using a graft of prosthetic material or a portion of the left subclavian
artery.
2.Aortic Stenosis
 Narrowing or stricture of the aortic
valve, causing resistance to blood
flow in the left ventricle, decreased
cardiac output, left ventricular
hypertrophy, and pulmonary
vascular congestion. The prominent
anatomic consequence of AS is the
hypertrophy of the left ventricular
wall, which eventually leads to
increased end-diastolic pressure,
resulting in pulmonary venous and
pulmonary arterial hypertension.
 Pathophysiology
 A stricture in the aortic outflow tract causes resistance to ejection of
blood from the left ventricle. The extra workload on the left ventricle
causes hypertrophy. If left ventricular failure develops, left atrial
pressure will increase; this causes increased pressure in the
pulmonary veins, which results in pulmonary vascular congestion
(pulmonary edema).
 Clinical manifestations:
 Newborns with critical AS demonstrate signs of decreased cardiac
output with faint pulses, hypotension, tachycardia, and poor feeding.
Children show signs of exercise intolerance, chest pain, and dizziness
when standing for a long period.
 Surgical treatment
 Balloon dilation in the catheterization laboratory is the first-line
procedure.
 Aortic valve replacement
 3.Pulmonic Stenosis
 Narrowing at the entrance to
the pulmonary artery.
Resistance to blood flow
causes right ventricular
hypertrophy and decreased
pulmonary blood flow.
 Pathophysiology
 When PS is present, resistance to blood flow causes right ventricular
hypertrophy. If right ventricular failure develops, right atrial pressure
will increase, and this may result in reopening of the foramen ovale,
shunting of unoxygenated blood into the left atrium, and systemic
cyanosis. If PS is severe, HF occurs.
 Surgical treatment
 In infants, transventricular (closed) valvotomy (Brock procedure) is
the surgical treatment.
2.cynotic (Decreased pulmonary blood flow)
1.Tetralogy of Fallot: The classic
form includes four defects: (1)
Ventricular Septal Defect, (2)
Pulmonary Stenosis, (3)
overriding aorta, and (4) right
ventricular hypertrophy.
 Pathophysiology
 The alteration in hemodynamics varies widely, depending primarily on the
degree of PS but also on the size of the VSD and the pulmonary and
systemic resistance to flow. Because the VSD is usually large, pressures may
be equal in the right and left ventricles. Therefore, the shunt direction
depends on the difference between pulmonary and systemic vascular
resistance. If pulmonary vascular resistance is higher than systemic
resistance, the shunt is from right to left. If systemic resistance is higher
than pulmonary resistance, the shunt is from left to right. PS decreases
blood flow to the lungs and consequently the amount of oxygenated blood
that returns to the left side of the heart. Depending on the position of the
aorta, blood from both ventricles may be distributed systemically.
 Clinical manifestations
 Some infants may be acutely cyanotic at birth; others have mild
cyanosis that progresses over the first year of life as the PS worsens.
There is a characteristic systolic murmur that is often moderate in
intensity. There may be acute episodes of cyanosis and hypoxia, called
blue spells or tet spells. Anoxic spells occur when the infant's oxygen
requirements exceed the blood supply, usually during crying or after
feeding.
 Surgical treatment
 Complete repair: Elective repair is usually performed in the first year
of life. Indications for repair include increasing cyanosis and the
development of hypercyanotic spells.
 Tricuspid Atresia
 The tricuspid valve fails to
develop; consequently there is
no communication from the
right atrium to the right
ventricle. Blood flows through
an ASD or a patent foramen
ovale to the left side of the
heart and through a VSD to the
right ventricle and out to the
lungs.
 Pathophysiology
 At birth, the presence of a patent foramen ovale (or other atrial septal
opening) is required to permit blood flow across the septum into the
left atrium; the PDA allows blood flow to the pulmonary artery into the
lungs for oxygenation. A VSD allows a modest amount of blood to enter
the right ventricle and pulmonary artery for oxygenation. Pulmonary
blood flow usually is diminished.
 Clinical manifestations
 Cyanosis is usually seen in the newborn period. There may be
tachycardia and dyspnea. Older children have signs of chronic
hypoxemia with clubbing.
 Surgical treatment
 Palliative treatment is the placement of a shunt (pulmonary–to–
systemic artery anastomosis) to increase blood flow to the lungs. If the
ASD is small, an atrial septostomy is performed during cardiac
catheterization.
2.Cynotic( mixed defects)
 1.Transposition of the Great
Vessels: The pulmonary artery
leaves the left ventricle, and
the aorta exits from the right
ventricle with no
communication between the
systemic and pulmonary
circulations.
 Pathophysiology
 Associated defects, such as septal defects or PDA, must be present to
permit blood to enter the systemic circulation or the pulmonary
circulation for mixing of saturated and desaturated blood.
 Clinical manifestations
 These depend on the type and size of the associated defects.
Newborns with minimum communication are severely cyanotic and
have depressed function at birth. Those with large septal defects or a
PDA may be less cyanotic but have symptoms of HF. Heart sounds
vary according to the type of defect present. Cardiomegaly is usually
evident a few weeks after birth.
 Therapeutic management
 The administration of IV prostaglandin E1 may be initiated to keep
the ductus arteriosus open to temporarily increase blood mixing and
provide an oxygen saturation of 75% or to maintain cardiac output.
During cardiac catheterization or under echocardiographic guidance,
a balloon atrial septostomy (Rashkind procedure) may also be
performed to increase mixing by opening the atrial septum.
 Rheumatic heart disease
 Rheumatic heart disease (RHD) is a serious condition that
can result from untreated or inadequately treated
streptococcal throat infections, particularly streptococcal
pharyngitis (strep throat). It is an inflammatory disease
that affects the heart and its valves, often leading to
permanent damage.
 Causes
 RHD is caused by an autoimmune response to an infection with
group A Streptococcus bacteria. When the body's immune system
reacts to the streptococcal infection, it can also mistakenly attack and
damage the heart valves and other parts of the heart.
 Sign and Symptoms
 : The symptoms of RHD can vary widely and may include shortness of
breath, fatigue, chest pain, palpitations, and swollen ankles or legs.
In severe cases, RHD can lead to heart failure and other
complications.

 Valve Damage: RHD most commonly affects the heart valves,

particularly the mitral valve. The inflammation can cause scarring,
thickening, and deformities in the valves, which can disrupt blood
flow through the heart.
 Diagnostics
 Diagnosis of RHD involves a combination of medical history, physical
examination, and diagnostic tests such as echocardiography (ultrasound of
the heart), electrocardiography (ECG), and sometimes cardiac
catheterization.
 Prevention
 The primary prevention of RHD involves promptly and adequately
treating streptococcal infections, particularly strep throat, with
appropriate antibiotics to prevent the development of acute
rheumatic fever (ARF), which is a precursor to RHD. In some
cases, long-term antibiotic prophylaxis might be recommended
for individuals at high risk of developing RHD
 Treatment
 Treatment for RHD focuses on managing the symptoms, preventing
further damage, and addressing complications. Medications such as
antibiotics (for ongoing prophylaxis), anti-inflammatory drugs, and
medications to manage heart failure might be prescribed. In cases
of severe valve damage, surgical interventions such as valve repair
or replacement might be necessary.
 Nursing management
 Assess and record heart rate, respiratory rate, blood pressure (BP),
and any signs or symptoms of decreased cardiac output every 2 to 4
hours and as necessary.
 Administer cardiac drugs on schedule. Assess and record any side
effects or any signs and symptoms of toxicity. Follow hospital
protocol for administration.
 Keep accurate record of intake and output.
 Weigh infant on same scale at same time of day as previously.
Document results and compare to previous weight.
 continue...
 Administer diuretics on schedule. Assess and record effectiveness
and any side effects noted.
 Offer small, frequent feedings to infant's tolerance.
 Organize nursing care to allow infant uninterrupted rest.
Refrences
 Hawes, H., & Scotchmer, C. (1993). What children need to know and
pass on about child development Children for health London: The
Child to Child Trust, UNICEEF.
 Wong, D. L. (2005). Whaley and Wong’s nursing care of infants and

 children, St. Louis: Mosby.

Thank you!!!