‘ The Pedi-Cardiac Lecture ’
Part 2
Pediatric Cardiovascular Disorders
Jerry Carley MSN, MA, RN, CNE
Concept Map:
Pediatric Cardiac Conditions
Distribution of Congenital Heart Defects
by anatomical location

PDA
PDA
Persistent Ductus Arteriosus






PDA
Incidence 10% of all reported CHDs
One of the most common benign defects
Ductus normally closes within hours of birth
Connection between the pulmonary artery
(low pressure) and aorta (high pressure)
High risk for pulmonary hypertension
Pulmonary Artery
Ductus Arteriosus
Fetal Structure,
Connecting
Aorta
Function
Allows Blood to
Bypass Lungs
Blood from (R))
Ventricle
Pulmonary
Artery
Reenters Aorta
Tachypnea
Dyspnea
Effects / Symptoms
Pulmonary
Hypertension
Cardiomegaly
Spontaneous
Closure
Usually by
2 years
Medication
Indomethacin
(Indocin)
Ibuprofen
(Motrin)
Surgery
Heart
Catheterization
Closed Heart
Surgery
Tires
Easily
Bounding
Pulse
Heart Failure
Treatments
FTT
Difficulty
Feeding
Murmurs
Nursing Care
Infective
Endocarditis
Recurrent
Pneumonia
Diagnosis and Treatment

Diagnosis by


Chest x-ray – enlarged heart and dilated
pulmonary artery
Echo-cardiogram – show the opening between
pulmonary artery and aorta
Treatment



Indomethocin (Indocin) given po – constricts
the muscle in the wall of the PDA and
promotes closure
Cardiac Catheterization – coil is placed in the
open duct and acts like a plug
Closed heart surgery – small incision made
between ribs on left hand side and PDA is
ligated or tied and cut
ASD
Atrial Septal Defect






ASD
10% of defects
Blood in left atrium flows into right atrium
Pulmonary hypertension
Reduced blood volume in systemic circulation
If left untreated may lead to pulmonary
hypertension, congestive heart failure or
stroke as an adult.
Lower Pressure
In ® Atrium
Oxygenated blood
From lungs shunted
To ® Atrium from
(L) d/t ASD
Pathophysiology
Short Stature
Heat Murmur
Effects / Symptoms
Dyspnea
Pulmonary
Hypertension
Cardiomegaly
Treatments
Patching
Large ASDs:
Surgical
Closure
Arrhythmia
Suturing
Heart
Catheterization
Nursing Care
Transcatheter
Balloon
R
L
Blood recirculates
back to the lungs
Via pulmonary arteries
ASD
Right
Left
Diagnosis and Treatment


Diagnosis: heart murmur may be heard in the
pulmonary valve area because the heart is
forcing an unusually large amount of blood
through a normal sized valve.
Echocardiogram is the primary method used
to diagnose the defect – it can show the hole
and its size and any enlargement of the right
atrium and ventricle in response to the extra
work they are doing.
Treatment



Surgical closure of the atrial septal defect
After closure in childhood the heart size will
return to normal over a period of four to six
months.
No restrictions to physical activity post
closure

VSD
Ventricular Septal Defect






VSD
30% of congenital heart defects
Opening in the ventricular septum
Left-to-right shunt
Right ventricular hypertrophy
Deficient systemic blood flow
Pathophysiology
Hole in Ventricular
Septum
R
L
Shunting
Increased Pulmonary
Flow & Pressure
Frequently seen
With other anomalies,
e.g., TOF
Tachypnea
Tachycardia
Effects / Symptoms
Enlarged
Pulmonary
Arteries
Eventually, will
become
R
L
Shunt if Not Treated !
Pulmonary
Hypertension
Dyspnea
Paleness
Pulmonary
Edema
FTT
Treatments
Digoxin
Sweating
While
Feeding
Diuretics
Cardiomegaly
Medication
Captopril
(ACE Inhibitor)
Murmurs
Nursing Care
Heart
Catheterization
Surgery
Open-Heart
Septal
Plasty
Recurrent
Pneumonia
Congestive
Heart
Failure
VSD



Small holes generally are asymptomatic
Medium to moderate holes will cause
problems when the pressure in the right side
of the heart decreases and blood will start to
flow to the path of least resistance (from the
left ventricle through the VSD to the right
ventricle and into the lungs)
This will generally lead to CHF
Diagnosis and Treatment




Diagnosis – heart murmur – clinical pearl a
louder murmur may indicate a smaller hole
due to the force that is needed for the blood
to get through the hole.
Electrocardiogram – to see if there is a strain
on the heart
Chest x-ray – size of heart
Echocardiogram – shows size of the hole and
size of heart chambers
Treatment VSD




CHF: diuretics of help get rid of extra fluid in
the lungs
Digoxin if additional force needed to squeeze
the heart
FTT or failure to grow may need higher
calorie concentration
Will need prophylactic antibiotics before
dental procedures if defect is not repaired
Surgical Repair



Over a period of years the vessels in the
lungs will develop thicker walls – the pressure
in the lungs will increase and pulmonary
vascular disease
If pressure in the lungs becomes too high the
un-oxygenated blood with cross over to the
left side of the heart and un-oxygenated
blood with enter the circulatory
system.(Becomes a Right
Left Shunt)
If the large VSD is repaired these changes
will not occur.

COA
Coarctation of Aorta





COA
7 % of defects
Congenital narrowing of the descending aorta
80% have aortic-valve anomalies
Difference in BP in arms and legs (severe
obstruction)
Diagnosis and Treatment




In 50% the narrowing is not severe enough to
cause symptoms in the first days of life.
When the Ductus Arteriosis closes a higher
resistance develops and heart failure can
develop.
Pulses in the groin and leg will be diminished
Echocardiogram will show the defect in the
aorta
Treatment





Prostaglandin may be given to keep the DA open to
reduce the pressure changes
The most common repair is resection of the
narrowed area with re-anastomosis of the two ends
Surgical complications – kidney damage due to
clamping off of blood flow during surgery
High blood pressure post surgery – may need to be
on antihypertensives
Antibiotic prophylactic need due to possible aortic
valve abnormalities.

PS
Pulmonary Stenosis





PS
7% of defects
Obstruction of blood flow from right ventricle
Hypertrophy of right ventricle
If severe cyanosis due to right-to-left shunt
Pulmonary Valvular Stenosis



In pulmonary valvar stenosis the pulmonary
valve leads to narrowing and obstruction
between the right ventricle and the pulmonary
artery.
Thickened tissue become less pliable and
increases the obstruction
Right ventricle must work harder to eject
blood into the pulmonary artery.
Pathophysiology
Abnormality of
Pulmonary Valve
Leaflets
Leakage of
Pulmonary Valve
When closed
Sometimes part of
DiGeorge Syndrome
Asymptomatic
(usually)
® Ventricular
Hypertrophy
Effects / Symptoms
Dilated
Pulmonary
Artery
Potential ®
Ventricular
Failure
S/S
®
Heart
Failure
Pulmonary
Hypertension
Dyspnea
Treatments
Feeding
Problems
FTT
Tires
Easily
Surgery
Transcatheter
Balloon
Stenting
Usually by
2 years
Indomethacin
(Indocin)
Ibuprofen
(Motrin)
Heart
Catheterization
Nursing Care
Diagnosis and Treatment




Diagnosis: heart murmur is heard – clicking
sound when the thickened valve snaps to an
open position.
Electrocardiogram would be normal
Echocardiogram most important non-invasive
test to detect and evaluate pulmonary
stenosis
Cardiac Catheterization – to measure
pressures and measure the stenosis
Treatment


Cardiac Catheterization to dilate the valve
and open up the obstruction.
Open- heart procedure would only needed for
more complex valve anomaly.

TOF
Tetralogy of Fallot (TOF)


6% of all CHD defects
Most common cardiac malformation
responsible for cyanosis in a child over 1 year
1. Narrowing
of the Pulmonic
Valve
3. Displacement of
Aorta over ventricular
septal defect
4. Ventricular
Septal
defect
2. Thickening of
Right Ventricular
Wall
Right
Left
Pulmonic Valve
Narrowing
Pathophysiology
(R) Ventricle
Hypertrophy
Displacement of
Aorta
R
L
Ventricular Septal
Defect (VSD)
Tachypnea
Effects / Symptoms
Dyspnea
Difficulty
Feeding
Central
CYANOSIS
Tires
Easily
FTT
Heart Failure
Tet
Spells
Cardiomegaly
Treatments
Harsh Systolic
Ejection Murmur
Surgery
Close
VSD
Relieve ®
Ventricular
Outflow
Nursing Care
Finger
Clubbing
Usually
SelfLimiting
TOF

Four Components




VSD
Pulmonary stenosis – narrowing of pulmonary
valve
Overriding of the aorta – aortic valve is enlarged
and appears to arise from both the left and right
ventricles instead of the left ventricle
Hypertrophy of right ventricle – thickening of the
muscular walls because of the right ventricle
pumping at high pressure
Clinical Manifestations





Dependent on degree of right ventricular
outflow obstruction.
Right-to-left shunt
Clubbing of digits
“tet” spells - ‘hyper-cyanotic episodes’
treated by flexing knees forward and upward
Severe irritability due to low oxygen levels
Children with T.O.F. exhibit cyanosis
during episodes of crying or exertion.
Knee-chest Position
Nurse puts infant in knee-chest
position.
Child with a cyanotic heart
defect squats (assumes a knee-chest position) to relieve
cyanotic spells. (“tet spells “)
Diagnosis




Cyanosis (central)
Oxygen will have little effect on the cyanosis
Loud heart murmur
Echocardiogram – demonstrates the four
defects characteristic of tetralogy
Treatment



If oxygen levels are extremely low prostaglandins
may be administered IV to keep the PDA open
Complete repair is done when the infant is about 6
months of age
Correction includes




Closure of the VSD with dacron patch
The narrowed pulmonary valve is enlarged
Coronary arteries will be repaired
Hypertrophy of right heart should remodel within a few
months when pressure in right side is reduced
Long Term Outcomes




Leaky pulmonic valve that can lead to
pulmonary insufficiency
Arrhythmias after surgery
Heart block – occasionally a pacemaker is
necessary
Periodic echocardiogram and exercise stress
test or Holter monitor evaluation

End of Part 2