NCM 102 – MS1

CONGENITAL HEART DISORDERS

CONGENITAL HEART DISORDERS

STRUCTURE AND FUNCTION OF THE HEART (Anatomy & Physiology)

A. Structure:
I. Heart - composed of 4 chambers with a septum in between the right and left chambers.
1. Right atrium - upper chamber
2. Left atrium - upper chamber
3. Right ventricle - lower chamber
4. Left ventricle - lower chamber

II. Great vessels

1. Arteries - coronary, aorta, brachiocephalic, subclavian and pulmonary.
2. Veins - IVC, SVC, and pulmonary.

III. 3 Tissue layers of the heart

1. Myocardium
2. Endocardium
3. Pericardium - has 2 layers:
a. Serous pericardium, parietal layer
b. Serous pericardium, visceral layer
*Pericardial space - space between the 2 pericardial layers which contains pericardial fluid to
allow frictionless
movement of the heart muscles.

IV. Heart valves (4)

1. Pulmonic valve
2. Aortic valve
3. Mitral valve
4. Tricuspid valve

V. Electrical conduction system

1. Sinoatrial (SA) node
2. Atrioventricular (AV) node
3. Atrioventricular bundle (bundle of His)
4. Purkinje fibers

B. Function: The CV system’s basic function is to pump oxygenated blood to tissues and to
remove metabolic waste products from the tissues.
I. Cardiac cycle - involves sequential contraction (systole) and relaxation (diastole) of the
atria and ventricles.

Atrial contraction - will eject blood into the ventricles

↓
Atrial relaxation
↓
Ventricular contraction - will eject blood into lungs via the pulmonary artery and
↓ to the systemic circulation via the aorta
↓
Blood returns to the right atrium completing the cycle
- from the systemic circulation via SVC and IVC
- from the pulmonary circulation via Pulmonary artery

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II. Cardiac output (CO) - is the volume of blood ejected by the ventricles during a given period
(1 minute)
- varies as the metabolic needs of body tissues changes (ex: exercise)
- CO = Heart rate x Stroke volume

C. Fetal and Postnatal circulation

1. Fetal heart development - begins during the first month of gestation; at 21 days AOG begins
beating and blood starts
to circulate.
nd th
2. Between 2 and 7 weeks AOG -the primitive heart starts to develop the 4 chambers and
great arteries.
3. During gestation, the lungs are nonfunctional . Fetal oxygenation occurs via the placenta.
4. Key structures in the fetal circulation include:
a. Foramen ovale - opening between the atria that allows blood to flow from the right directly
to the left atrium.
b. Ductus arteriosus - a conduit between the pulmonary artery and the aorta that shunts blood
away from the
pulmonary circulation.
5. Important circulatory changes during transition to extrauterine life:
a. Inspired O2 dilates pulmonary vessels, decreasing pulmonary vascular resistance and
increasing pulmonary blood
flow, which facilitates lung expansion.
b. Foramen ovale closes functionally soon after birth from compression of the atrial septum.

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PHYSICAL ASSESSMENT

I. Health history - Description of symptoms including onset, duration, location and

precipitation.
- Cardinal s/sx like feeding problems, respiratory difficulties and chronic
fatigue.
- Prenatal risk factors like maternal use of medications, tobacco or alcohol.
- Personal risk factors such as chromosomal abnormalities, prematurity,
infections.
- Family risk factors include congenital heart disease, M.I. before 55 years of
age or sudden
death of unknown cause.

II. Physical examination

1. Vital signs - blood pressure, cardiac rate, respiratory rate, temperature

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2. Inspection - cyanosis, periorbital/peripheral edema, engorged neck veins, clubbing of fingers

or toes,
Abdominal distention.
3. Palpation - precordium for PMI, thrills, lift and heaves.
- abdomen for hepatomegally and splenomegally.
- extremities for pitting edema.
4. Auscultation - anterior chest for heart sounds and murmurs.
- posterior chest for adventitious lung sounds.

III. Diagnostic Procedures

1. Complete blood count (CBC) - to assess a compensatory increase in Hemoglobin and
hematocrit levels and
erythrocyte count (polycythemia).
2. Pulse oximetry - identifies the child’s O2 saturation level.
3. Coagulation studies - to identify abnormalities in hemostasis (decreased platelet, clotting
factors)
4. ECG - evaluation of cardiac function
5. CXR - heart size and distribution of pulmonary blood flow
6. Echocardiography - more detailed assessment of heart structure and function
7. Cardiac catheterization - assessment of anatomic abnormalities
8. Angiography - radiographic visualization of the heart and its blood vessels with a contrast
medium

IV. Therapeutic Management

Medications used to treat cardiovascular disorders
1. Potent vasopressors - dopamine, dobutamine, epinephrine and isoproterenol
2. ACE Inhibitors - enalapril, captopril
3. Beta-adrenergic blockers - propranolol
4. Diuretics - furosemide, hydrochlorothiazide
5. Antiarrhythmics - digoxin
6. Antibiotics - penicillin, sulfadiazine
7. Anti-inflammatory - aspirin, corticosteroids

V. Nursing Management
1. Promote adequate cardiac output and oxygenation
2. Promote adequate nutrition
3. Prevent infection
4. Provide preoperative and postoperative care
Congenital Heart Diseases (CHD)
Overview:
1. CHD’s are structural defects of the heart, great vessels or both that are present from birth.
2. Incidence - 5 to 8 / 1,000 live births.
3. Children with CHD are more likely to have associated defects such as TEF and chromosomal
defects.
4. CHD’s are divided into 2 major classifications each with different variations and defects.

Classification:
I. Acyanotic Defects
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1. Increased Pulmonary blood flow

a. ASD – Atrial septal defect
b. VSD – Ventricular septal defect
c. PDA – Patent ductus arteriosus
d. AVC – Atrioventricular canal defect
2. Obstruction to blood flow from ventricles (Obstructive defects)
a. CoA – Coarctation of the aorta
b. AS – Aortic stenosis
c. PS – Pulmonic stenosis

II. Cyanotic Defects

1. Decreased Pulmonary blood flow
a. TOF – Tetralogy of Fallot
b. Tricuspid atresia
2. Mixed blood flow (Mixed defects)
a. TGA/TGV – Transposition of the great arteries/vessels
b. TAPVC – Total anomalous pulmonary venous connection
c. TA – Truncus arteriosus
d. HLHS – Hypoplastic left heart syndrome

Etiology:
1. More than 90% of CHD cases - cause is unknown
2. Factors associated with increased incidence of CHD include:
- fetal and maternal infection during the 1st trimester, especially rubella (German measles).
- maternal alcoholism
- maternal use of other drugs with teratogenic effects
- maternal age >40 y.o.
- maternal dietary deficiencies
- maternal insulin-dependent induced diabetes
- sibling with CHD
- parent with CHD
- chromosomal abnormality such as Down syndrome

ASD – Atrial Septal Defect: Abnormal opening between the 2 atria, allowing blood from the
higher pressure left atrium
into the low pressure right atrium resulting in ineffective pumping
of the heart, thus
increasing the risk of heart failure. Unknown cause.

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There are 3 types:

1. ASD-2 or Ostium secundum - Most common type of ASD
- Opening occurs near center of septum between the right
and left atrium.
- A variant of this type is called patent foramen ovale (PFO)
and is very small.
- Non-surgical treatment; maybe closed using devices

during cardiac catheterization.

Normal heart ASD-2

2. ASD-1 or Ostium primum - Next most common type, commonly occurs with Down’s
syndrome.
- Opening occurs at the lower end of septum
- May be associated with a mitral valve defect known as
mitral valve cleft (a slit-like or
elongated hole at one of the leaflets (anterior leaflet) that
form the mitral valve.
- May require repair or rarely MV replacement.
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Normal heart ASD-1

3. Sinus venosus ASD - The least common, often has an abnormal pulmonary vein connection
associated with it.
- Four pulmonary veins, two from the right lung and two from the left
lung, normally return red
blood to the left atrium.
- A pulmonary vein from the right lung will be abnormally connected
to the right atrium instead
of the left atrium. This is called an anomalous pulmonary vein.
- Opening is near the junction of SVC (superior vena cava) & RA (right
atrium) or upper portion
of the atrial septum.
- Maybe associated with a partial anomalous pulmonary venous
connection.
- Treatment requires patch placement, so the anomalous right
pulmonary venous return is
directed to the left atrium.

Clinical Manifestations: Assymptomatic

Fatigue on exertion
May develop CHF (congestive heart failure)
Presence of characteristic murmur
Surgical Treatment: Surgical Dacron patch closure of moderate to large defects similar to
closure of VSD’s.
1. The surgical approach to the atrial septal defect is somewhat dependent upon its location.
2. Open repair with CPB (cardiopulmonary bypass) before school age (preschool or early school
age).
3. In general, three surgical approaches may be undertaken:
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CONGENITAL HEART DISORDERS

>median sternotomy (midline sternal-splitting incision)

>right thoracotomy (going between the ribs on the right side)
>submammary (under the breast tissue on the right front of the chest)
Complications: Physical underdevelopment
Respiratory infections
Heart failure
Atrial arrhythmias
Mitral valve prolapse.

VSD – Ventricular Septal Defect: Most common CHD.

Abnormal opening between the right and left ventricles.
Frequently associated with other defects: PS, TGV,PDA,
COA and other atrial defects.
Spontaneous closure occurs in about 20-60% of cases
st
during the 1 year of life in
children having small or moderate defects.

The septum is a wall that separates the heart's left and right sides. Septal defects are sometimes
called a "hole" in the heart. A defect between the heart's two lower chambers (the ventricles) is
called a ventricular septal defect (VSD).
Pathophysiology:
When there is a large opening between the ventricles,
↓
A large amount of oxygen-rich (red) blood from the heart's left side is forced through the defect
into the right side.
↓
Then it's pumped back to the lungs, even though it's already been refreshed with oxygen.
↓
This is inefficient, because already-oxygenated blood displaces blood that needs oxygen.
↓
This means the heart, which must pump more blood, may enlarge from the added work.
↓
High blood pressure may occur in the lungs' blood vessels because more blood is there.
↓
Over time, this increased pulmonary hypertension may permanently damage the blood vessel
walls.

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CONGENITAL HEART DISORDERS

Clinical Manifestations: CHF (congestive heart failure) is common

Presence of a characteristic murmur
Risk for Bacterial endocarditis and Pulmonary vascular
obstructive disease.
Eisenmenger syndrome may develop in severe cases.
Surgical Treatment:
1. Palliative – Pulmonary artery banding to decrease pulmonary blood flow. Common
approach in the past.
2. Complete repair (procedure of choice) – Performed via cardiopulmonary bypass. The repair
is generally approached
thru the right atrium and tricuspid valve. Post-op complications include residual VSD and
conduction disturbances.
a. Small defect – repaired with a purse-string approach.
b. Large defect – usually require a knitted Dacron patch sewn over the opening.
Nonsurgical treatment:
Device closure during cardiac catheterization – has a high operative risk.
PDA – Patent Ductus Arteriosus: 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, causing a
left-to-right shunt.

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Clinical Manifestations: May be assymptomatic or may show signs of CHF.

Characteristic machinery-like murmur .
Widened pulse pressure and bounding pulse.
Risk for bacterial endocarditis and pulmonary vascular
obstructive disease.
Medical Management: Indomethacin (prostaglandin inhibitor)
Surgical Management: Surgical division or ligation of the patent vessel via a left thoracotomy.
Visual-assisted thoracoscopic surgery (VATS) – a newer technique
that uses a thoracoscope
and instruments placed thru 3 small incisions to place a clip on the
ductus.
Nonsurgical treatment: Use of coils to occlude the PDA during catheterization.

AVC – Atrioventricular Canal Defect: Incomplete fusion of endocardial cushions.

Consists of a low atrial septal defect that is continuous
with a high ventricular
septal defect and clefts of the mitral and tricuspid
valves, creating a large central
atrioventricular (AV) valve that allows blood to flow
between all 4 chambers of the
heart. It is the most common defect in children with
Down syndrome.

Clinical Manifestations: Moderate to severe CHF

Characteristic murmur
Mild cyanosis that increases with crying
High risk for developing pulmonary vascular obstructive disease
Surgical Treatment:
1. Palliative – Pulmonary artery banding
2. Complete repair – Patch closure of septal defects and reconstruction of AV valve tissue.
If MV defect is severe,
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a valve replacement may be needed.

COA – Coarctation of the Aorta: Localized narrowing near the insertion of the ductus
arteriosus, resulting in:
1. increased pressure proximal to the defect (head & upper
extremities)
2. decreased pressure distal to the obstruction (body &
lower extremities)
There are 3 types of CoA according to location:
a. Preductal - proximal to the insertion of the ductus
arteriosus
b. Postductal - distal to the ductus arteriosus
c. Juxtaductal - at the insertion of the ductus arteriosus

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Clinical Manifestations: High BP and bounding pulses in arms

Weak or absent femoral pulses
Cool lower extremities with lower BP
Signs of CHF in infants
Risk for HPN, ruptured aorta, aortic aneurysm or stroke

Surgical Treatment:
1. Resection of the coarcted portion with end-to-end anastomosis of the aorta
2. Enlargement of the constricted section using a graft of prosthetic material or a portion of
the left subclavian artery.
3. Percutaneous balloon angioplasty – effective for residual post-op coarctation
gradients.
Note: Because the defect is outside the heart and pericardium, CPB is not required and a
thoracotomy incision is used.
Post-op HPN > 160mmHG – treat with I.V. Na Nitroprusside or Amrinone followed by
oral medications such as
captopril, hydralazine and/or propranolol.
Elective surgery within the first 2 years of life is advised
Nonsurgical Treatment: Balloon angioplasty – has a high restenosis rate in infants <7 months
of age.

AS – Aortic Stenosis: Narrowing or stricture of the aortic valve, causing:

1. Resistance to blood flow in the left ventricle
2. Decreased cardiac output
3. Left ventricular hypertrophy
4. Pulmonary vascular congestion

Types of AS:
1. Valvular AS – most common type, caused by malformed cusps resulting in a bicuspid rather
than tricuspid valve.
Sx tx: Aortic valvotomy; Ross procedure; Extended aortic root replacement
Non Sx tx: Dilating narrowed valve with balloon angioplasty in cath.lab.

2. Subvalvular AS – a stricture caused by a fibrous ring below a normal valve.

Sx tx: Konno procedure

3. Supravalvular AS – occurs infrequently.

Clinical Manifestations:
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1. Infants with severe defects

a. Decreased cardiac output with faint pulses
b. Hypotension
c. Tachycardia
d. Poor feeding
2. Children
a. Exercise intolerance
b. Chest pain and dizziness when standing for a long period
3. Characteristic murmur
4. Risk for bacterial endocarditis, coronary insufficiency and ventricular dysfunction

PS – Pulmonic Stenosis: Narrowing at the entrance of the pulmonary artery.

Resistance to blood flow causes RVH and decreased pulmonary
blood flow.

Clinical Manifestations: May be assymptomatic

Mild cyanosis
CHF, murmur
Cardiomegally on CXR
Surgical treatment:
1. Infants – Brock procedure or transventricular (closed) valvotomy
2. Children – Pulmonary valvotomy with CPB
Nonsurgical treatment: Balloon angioplasty in cath.lab.

TOF – Tetralogy of Fallot: The classic form includes four defects:

1. VSD
2. PS
3. Overriding aorta
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4. RVH
Clinical Manifestations:
1. Infants – acute episodes of hypercyanosis and hypoxia (blue/tet spells). Knee-chest
position. Characteristic murmur is
present.
2. Children – clubbing of fingers; squatting (serves to decrease the return of poorly
oxygenated blood from the lower
extremities and to increase systemic vascular resistance which increases
pulmonary blood flow and eases
respiratory effort) and poor growth.

Surgical treatment:
1. Palliative shunt – for infants who cannot undergo primary repair. Blalock-Taussig or B-T
Shunt is the preferred
procedure which provides blood flow to the pulmonary arteries from the left or right
subclavian artery.
2. Complete repair – performed in the first year of life. It involves closure of the VSD and
resection of the infundibular
stenosis, with a pericardial patch to enlarge the right ventricular outflow tract. This requires a
median sternotomy
and cardiopulmonary bypass.

Tricuspid Atresia: Failure of the tricuspid valve to develop.

There is no communication between the RA and RV. It is often associated
with PS and TGV.
There is complete mixing of unoxygenated and oxygenated blood in the left
side of the heart,resulting
in systemic desaturation and pulmonary obstruction causing decreased
pulmonary blood flow.
Patients are at risk of bacterial endocarditis , brain abscess and stroke

Clinical Manifestations:
1. Newborn – cyanosis, tachycardia and dyspnea
2. Children – chronic hypoxemia with clubbing

Surgical Treatment:
1. Palliative – placement of a shunt (Pulmonary-to-systemic artery anastomosis)
2. Pulmonary artery banding – to lessen blood flow to the lungs
3. Bidirectional Glenn Shunt (Cavopulmonary anastomosis) – performed at 6-9 months as a 2nd
stage.
4. Modified Fontan procedure – separates oxygenated and unoxygenated blood inside the heart
and eliminates excess
volume load on the ventricle but does not restore normal anatomy or hemodynamics.
5. Complete Fontan procedure – conduit between right atrium and pulmonary artery.

Note: Absence of a tricuspid valve is incompatible with life unless there is presence of ASD, VSD,
or PDA.

TGA/TGV – Transposition of the Great Arteries/Vessels: The pulmonary artery leaves the
left ventricle and the aorta

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exits from the right ventricle

with no communication between
the systemic and pulmonary
circulations.

Therapeutic Management: To provide intracardiac mixing – administer I.V. PGE1 (Prostaglandin

E-1).
Rashkind procedure (Balloon atrial septostomy) may be done
during cardiac catheterization
to increase mixing and maintain cardiac output for a longer
period.

Surgical treatment:
1. Arterial switch procedure – done during 1st week of life, to reestablish normal circulation with
the left ventricle acting as the systemic pump.
2. Intraarterial baffle repairs – done in the 1st year of life using the Senning and Mustard
procedures.
a. Senning procedure - diverts venous blood to MV and pulmonary venous blood to the
tricuspid valve using the
patients atrial septum.
b. Mustard procedure - same as Sennings but a prosthetic is used instead of the patients
atrial septum.
3. Rastelli procedure – operative choice in infants with TGA, VSD and severe PS. Involves closure
of the VSD with a baffle
directing left ventricular blood through the VSD into the aorta. The pulmonic valve is then
closed and a conduit is
placed from the right ventricle to the pulmonary artery creating a physiologically normal
circulation. Disadvantage of
procedure is that it requires multiple conduit replacement as the child grows.

TAPVC – Total Anomalous Pulmonary Venous Connection: Rare defect.

Characterized by failure of
pulmonary veins to join the left
atrium.
Instead, the pulmonary veins
are abnormally connected to
the systemic venous circuit
via the right atrium or various
veins draining toward the
right atrium such as the SVC.
Clinical Manifestations: Cyanosis and CHF
Surgical Treatment: Corrective repair in early infancy

TA – Truncus Arteriosus: Single vessel that overrides both ventricles.

This is due to the failure of normal septation and division of the
embryonic bulbar trunk into
the pulmonary artery and the aorta.

Clinical Manifestations: Moderate to severe CHF

Cyanosis
Poor growth
Activity intolerance
Murmur

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Risk of brain abscess.

Surgical Treatment: Modified Rastelli Procedure – done in the 1st few months of life. Closure of
VSD so TA receives outflow from LV, excision of pulmonary artery from aorta and attach it to the
RV by means of homograft.

HLHS – Hypoplastic Left Heart Syndrome: Underdevelopment of left side of the heart
resulting in a hypoplastic left
ventricle and aortic atresia.
Most blood from the left atrium flows across
the patent foramen ovale to
the right atrium, to the right ventricle, and
out the pulmonary artery.
The descending aorta receives blood from
the patent ductus arteriosus
supplying systemic blood flow.
Clinical Manifestations: Cyanosis
CHF
Usually fatal in the 1st month of life without intervention.

Therapeutic Management: Prostaglandin-E1 infusion (PGE-1)

Surgical Treatment: Several-staged approach.

1. First stage: Norwood procedure – Anastomosis of the main pulmonary artery to the aorta to
create a new aorta, shunt
placement and creation of a large ASD.
2. Second stage: Bidirectional Glenn Shunt – done at 6-9 months of age to relieve cyanosis and
to reduce volume load on
the right ventricle.
3. Third stage: Modified Fontan procedure – to separate oxygenated from unoxygenated blood
and to eliminate excess
volume load on ventricles.

Heart transplantation – best option for newborns.

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