Pediatric Cardiology

By
Hidayat khan
MSN, BSN, RN and DPHN
Fetal Circulation
Before birth, blood from the placenta, about
80% saturated with oxygen, returns to the fetus
by way of the umbilical vein.
On approaching the liver, most of this blood
flows through the ductus venosus directly into
the inferior vena cava, bypassing the liver.
After a short course in the inferior vena cava,
where placental blood mixes with deoxygenated
blood returning from the lower limbs, it enters
the right atrium.
 Fetal Circulation
Here it is guided toward the oval foramen by the
valve of the inferior vena cava, and most of the
blood passes directly into the left atrium.
From the left atrium, where it mixes with a small
amount of desaturated blood returning from the
lungs, blood enters the left ventricle and ascending
aorta.
Since the coronary and carotid arteries are the first
branches of the ascending aorta, the heart
musculature and the brain are supplied with well-
oxygenated blood.
Fetal Circulation
A small amount from the IVCInferior vena cava
 is prevented from entering the left atrium and
remains in the right atrium.
It mixes with desaturated blood returning from
the head and arms by way of the superior vena
cava.
Desaturated blood from the superior vena cava
flows by way of the right ventricle into the
pulmonary trunk.
Fetal Circulation
During fetal life, resistance in the pulmonary
vessels is high, such that most of this blood
passes directly through the ductus arteriosus
into the descending aorta, where it mixes with
blood from the proximal aorta.
After coursing through the descending aorta,
blood flows toward the placenta by way of the
two umbilical arteries.
The oxygen saturation in the umbilical arteries
is approximately 58%.
Fetal Circulation
N.B.
A. A small amount of blood enters the liver sinusoids and
mixes with blood from the portal circulation.
B. A sphincter mechanism in the ductus venosus, close to
the entrance of the umbilical vein, regulates flow of
umbilical blood through the liver sinusoids. This
sphincter closes when a uterine contraction renders the
venous return too high, preventing a sudden
overloading of the heart.
 During its course from the placenta to the organs of the
fetus, blood in the umbilical vein gradually loses its high
oxygen content as it mixes with desaturated blood.
Fetal Circulation
 Theoretically, mixing may occur in the following places :
1) In the liver by mixture with a small amount of blood
returning from the portal system.
2) In the inferior vena cava which carries deoxygenated blood
returning from the lower extremities, pelvis, and kidneys.
3) In the right atrium by mixture with blood returning from
the head and limbs.
4) In the left atrium by mixture with blood returning from
the lungs.
5) At the entrance of the ductus arteriosus into the
descending aorta.
Congenital heart disease (CHD)

Congenital heart disease is a defect in the structure of

the heart and
great vessels which is present at birth
• CHD are the main cause of defect-related deaths
• Incidence is 8-9/1000 live births
• More common in premature infants
• May be associated with a significant musculoskeletal defect
(e.g. diaphragmatic hernia, tracheo-oesophageal fistula,
imperforate anus)
 Congenital heart disease (CHD)
• Causes are multifactorial and
include maternal illness (
diabetes mellitus,
phenylketonuria, and
systemic lupus erythematosus),
maternal infections (Rubella),
drugs (lithium, thalidomide),
known teratogens, harmful
habits (alcohol, hydantoin)
and associations with
chromosomal abnormality or
other recognized patterns of
malformation or syndrome; Trisomy 18
100% have CHD
Systemic lupus erythematosus
 Systemic lupus erythematosus (SLE) is an autoimmune disease in which the body's
immune system mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys,
brain, and other organs .
hydantoin anticonvulsants; phenytoin and
fosphenytoin
 Recognition of Cyanosis

Central cyanosis
• noted in the trunk, tongue, mucous membranes
• due to reduced oxygen saturation

Peripheral cyanosis
• noted in the hands and feet, around mouth
• due to reduced local blood flow
 ASSESSMENT OF HEART DISORDERS IN
CHILDREN

History
Physical assessment
general appearance
pulse, blood
pressure, &
respirations
Acyanotic Congenital Heart Disease
Left-to-Right Shunt Lesions

Atrial Septal Defect (ASD)

Ventricular Septal Defect (VSD)
Atrioventricular Septal Defect (AV Canal)
Patent Ductus Arteriosus (PDA)
Atrial Septal Defect
ASD is an opening in the atrial septum permitting free
communication of blood between the atria. Seen in
10% of all CHD.
Atrial Septal Defect
Clinical Signs & Symptoms
Rarely presents with signs of CHF or other cardiovascular
symptoms.

• Most are asymptomatic but may have easy fatigability or

mild growth failure.

• Cyanosis does not occur unless pulmonary HTN is

present.
 Atrial Septal Defect pathophysiology
1. Oxygenated blood is shunted from
left to right side of the heart via
defect
2. A larger volume of blood
than normal must be
handled by the right side of
the heart hypertrophy
3. Extra blood then passes
through the pulmonary
artery into the lungs,
causing higher pressure
than normal in the blood
vessels in the lungs 
congestive heart failure
Treatment
Medical Management
Medications – digoxin

Cardiac Catheterizaton -
Amplatzer septal occluder

Open-heart Surgery
Atrial Septal Defect
Treatment:
Surgical or catherization laboratory closure is
generally recommended.

• Closure is performed electively between ages 2 & 5

yrs to avoid late complications.

• Surgical correction is done earlier in children w/ CHF

or significant Pulm HTN.
Treatment
Device Closure – Amplatzer septal occluder

During cardiac catheterization the occluder is placed in the

Defect
Cardiac Catheterization
Pre-care:
 History and Physical
 Lab work – EKG, ECHO cardiogram, CBC
 NPO
 Preprocedural teaching
  Post Care:
 Monitor vital signs
 Monitor extremity distal to the catheter instertion,
 Keep leg immobilized
 Vital signs
 Check for bleeding at insertion site
 Measure I&O
 Ventricular Septal Defect
VSD – is an abnormal opening in the ventricular
septum, which allows free communication between the
Rt & Lt ventricles. Accounts for 25% of CHD.
Ventricular Septal Defect
Hemodynamics

The left to right shunt occurs secondary to PVR

being < SVR, not the higher pressure in the LV.

This leads to elevated RV & pulmonary pressures

& volume hypertrophy of the RA & RV.
Ventricular Septal Defect
Clinical Signs & Symptoms

• Small - moderate VSD, 3-6mm, are usually

asymptomatic and 50% will close spontaneously
by age 2yrs.

• Moderate – large VSD, almost always have

symptoms and will require surgical repair.
Ventricular Septal Defect
Clinical Signs & Symptoms
• Prominent , Diastolic murmur.

• CHF, FTT, Respiratory infections, exercise intolerance.

Symptoms develop between 1 – 6
months
Ventricle Septal Defect pathophysiology
1. Oxygenated blood is shunted from
left to right side of the heart via
defect
2. A larger volume of blood
than normal must be
handled by the right side of
the heart hypertrophy
3. Extra blood then passes
through the pulmonary
artery into the lungs,
causing higher pressure
than normal in the blood
vessels in the lungs 
congestive heart failure
Ventricular Septal Defect
Treatment

• Small VSD - no surgical intervention, no

physical restrictions, just reassurance and
periodic follow-up and endocarditis prophylaxis.

• Symptomatic VSD - Medical treatment

initially with afterload reducers & diuretics.
Ventricular Septal Defect
Treatment

 Indications for Surgical Closure:

 Large VSD with medically uncontrolled symptomatology

& continued FTT.

 Ages 6-12 mo with large VSD & Pulm. HTN

Treatment
Surgical repair with a patch inserted
 Patent Ductus Arteriosus
PDA – Persistence of the normal fetal vessel that
joins the PA to the Aorta.
Normally closes in the 1st wk of life.

Accounts for 10% of all CHD, seen in 10% of

other congenital hrt lesions and can often play a
critical role in some lesions.
Female : Male ratio of 2:1

Often associated with coarctation & VSD.

Patent Ductus Arteriosus
Hemodynamics
As a result of higher aortic pressure, blood shunts
L to R through the ductus from Aorta to PA.

Extent of the shunt depends on size of the ductus

& PVR:SVR.

Small PDA, pressures in PA, RV, RA are normal.

 Patent Ductus Arteriosus
Clinical Signs & Symptoms
Small PDA’s are usually asymptomatic
Large PDA’s can result in symptoms of CHF,
growth restriction, FTT.
Bounding arterial pulses
Widened pulse pressure
Enlarged heart, prominent apical impulse
Classic continuous machinary systolic murmur
Mid-diastolic murmur at the apex
Patent Ductus Arteriosus pathophysiology
1. Blood shunts from
aorta (left) to the
pulmonary artery
(right)
2. Returns to the lungs
causing increase
pressure in the lung
3. Congestive heart
failure
 Patent Ductus Arteriosus
Treatment
Indomethacin, inhibitor of prostaglandin synthesis can
be used in premature infants.
PDA requires surgical or catheter closure.
Closure is required treatment heart failure & to prevent
pulmonary vascular disease.
Usually done by ligation & division or intra vascular
coil.
Mortality is < 1%
 Treatment for PDA
Surgery

Ligate the
ductus arteriosus
 Treatment for PDA
Cardiac Catheterization

Insert coil – tiny fibers

occlude the ductus
arteriosus when a
thrombus forms in the
mass of fabric and wire
Nursing Care:
Pre-op
Patient/parent teaching
Assess for infection
Obtain lab values for chart
Post-op
ABCs
Rest
Hydration/nutrition
Prevent complications
Discharge teaching
 Pulmonary Stenosis
Pulmonary Stenosis is obstruction
in the region of either the pulmonary
valve or the subpulmonary
ventricular outflow tract.

Accounts for 7-10% of all CHD.

Most cases are isolated lesions

Maybe biscuspid or fusion of 2 or

more leaflets.

Can present w/or w/o an intact

ventricular septum.
Pulmonary Stenosis
Hemodynamics
RV pressure hypertrophy  RV failure.
RV pressures maybe > systemic pressure.
Post-stenotic dilation of main PA.
W/intact septum & severe stenosis  R-L shunt 
cyanosis.
Cyanosis is indicative of Critical PS.
Pulmonary Stenosis
Clinical Signs & Symptoms
Depends on the severity of obstruction.
Asymptomatic w/ mild PS < 30mmHg.
Mod-severe: 30-60mmHg, > 60mmHg
Prominent jugular wave.
Split 2nd hrt sound w/ a delay
Heart failure & cyanosis seen in severe cases.
Pulmonary Stenosis
Treatment
Mild PS no intervention required, close follow-up.

Mod-severe – require relieve of stenosis.

Balloon valvuloplasty, treatment of choice.

Surgical valvotomy is also a consideration.

Pulmonic Stenosis
Treatment:
Medications – Prostaglandins to keep the PDA open
Cardiac Catheterization
 Baloon Valvuloplasty
Surgery
 Valvotomy
Aortic Stenosis
Aortic Stenosis is an obstruction to the outflow
from the left ventricle at or near the aortic valve
that causes a systolic pressure gradient of more
than 10mmHg. Accounts for 7% of CHD.
3 Types
Valvular – Most common.
Subvalvular(subaortic) – involves the left outflow
tract.
Supravalvular – involves the ascending aorta is the
least common.
 Aortic Stenosis
Hemodynamics
Pressure hypertrophy of the LV and LA
with obstruction to flow from the LV.
Mild AS 0-25mmHG
Moderate AS 25-50mmHg
Severe AS 50-75mmHg
Critical AS > 75mmHg
Aortic Stenosis
Clinical Signs & Symptoms
Mild AS may present with exercise intolerance,
easy fatigabiltity, but usually asymptomatic.
Moderate AS – Chest pain, dypsnea on exertion,
dizziness & syncope.
Severe AS – Weak pulses, left sided heart failure,
Sudden Death.
LV thrust at the Apex.
Systolic thrill @ rt base/suprasternal notch
 Aortic Stenosis
Treatment

Balloon valvuloplasty is the standard of treatment.

Aortic insufficiency & re-stenosis is likely after
surgery and may require valve replacement.

Activity should not be restricted in Mild AS.

Mod-severe AS, no competitive sports.

Coarctation of the Aorta
Coarctation- is narrowing of the aorta at varying
points anywhere from the transverse arch to the
iliac bifurcation.

98% of coarctations are juxtaductal

Male: Female ratio 3:1.

Accounts for 7 % of all CHD.

Coarctation of the Aorta
Hemodynamics
Obstruction of left ventricular outflow  pressure
hypertrophy of the LV.
 Coarctation of the Aorta
Clinical Signs & Symptoms
Classic signs of coarctation are diminution or absence of
femoral pulses.

Higher BP in the upper extremities as compared to the

lower extremities.

90% have systolic hypertension of the upper extremities.

Pulse discrepancy between rt & lt arms.

Coarctation of the Aorta
Clinical Signs & Symptoms
With severe coarc LE hypoperfusion, acidosis, HF and
shock.
Differential cyanosis if ductus is still open
Cardiomegaly, rib notching on X-ray.
absence of femoral pulses

1. Radial pulses full/bounding and femoral or

popliteal pulses weak or absent
2. Leg pains, fatigue

3. Nose bleeds
Coarctation of the Aorta
Coarctation of the Aorta
Treatment
With severe coarctation maintaining the ductus
with prostaglandin E is essential.

Surgical intervention, to prevent LV dysfunction.

Angioplasty is used by some centers.

Re-coarctation can occur, balloon angioplasty is

the procedure of choice.
Surgery for Coarctation of Aorta
1. Resect
narrow
area 2. Anastomosis
Cyanotic heart disease
Clinical Manifestations of Cyanotic Heart
Disease
chronic hypoxemia causes fatigue, clubbing, exertional
dyspnea, delayed milestones, tire easily with feeding,
reduced growth, CHF
hypercyanotic (hypoxic) spells: incr rate and depth of
respir, incr cyanosis, incr HR, pallor and poor
perfusion, agitation and irritability.
FIGURE 26–13 Clubbing of the fingers is one manifestation of a cyanotic defect in an older child. What neurologic signs may be associated with such a
defect?

© 2006 by Pearson Education, Inc.

Jane W. Ball and Ruth C. Bindler
Upper Saddle River, New Jersey 07458
Child Health Nursing: Partnering with Children & Families All rights reserved.
Cyanotic Spell
most signif prob to develop in infants and toddlers
with cyanotic heart disease
brought on by crying, feeding, exercise, warm bath, or
straining an intense or violent exertion to defecate
during a hypoxic spell, child will often squat knee to
chest to decrease venous return (by incr systemic
vascular resistance) from LE which decr CO and
relieves the cyanotic spell.
Tetralogy of Fallot
combination of four defects
pulmonary stenosis: degree determines severity
VSD
over-riding of the aorta
RVH
accounts for 10% of CHD
elevated R sided pressures: R to L shunt
xray: boot shaped heart d/t RVH
risk for metabolic acidosis and syncope.
Tetralogy of Fallot’s
Tetralogy of Fallot
 Four anomalies
 Pulmonary stenosis
 VSD
 Dextropositionof the aorta
 Hypertrophy of right
ventricle
Clinical manifestation of TOF
Symptoms are variable depending of degree of
obstruction
Cyanosis
 Digital clubbing and hyperpnea at rest are directly
related to the degree of cyanosis
Tachycardia
Mental retardation
Retarded growth and development
RV heave an upward movement (especially a rhythmical rising and falling)

Systolic ejection murmur is heard along the left sternal

border
Polycythemia
Paroxymal dyspnea
 Severe dyspnea on exertion
Squatting position for the relief of dyspnea caused
physical effort,
 “Blue” spells, paroxysmal hypercyanotic attacks –
infant becomes hyperpnea, restless, cyanosis increases,
gasping respirations, syncope
Hypercyanotic Spells/Blue Spells/Tet Spells

Clinical Manifestations
‫ ٭‬Most often occurs in morning after feedings,
defecation, or crying
‫ ٭‬Acute cyanosis
‫ ٭‬Hyperpnea
‫ ٭‬Inconsolable sad beyond comforting crying
‫ ٭‬Hypoxia which leads to acidosis
 Chest X-Ray

• Decreased
pulmonary vascular
marking

• “Boot-shaped
heart”
Treatment of TOF
total repair is done by 6 mo if cyanotic spells
surgery is not necessarily curative, but most have
improved quality of life and improved longevity
residual problems: arrhythmias and RV dysfunction
FIGURE 26–10 A child with a cyanotic heart defect squats (assumes a knee–chest
position) to relieve cyanotic spells.

© 2006 by Pearson Education, Inc.

Jane W. Ball and Ruth C. Bindler
Upper Saddle River, New Jersey 07458
Child Health Nursing: Partnering with Children & Families All rights reserved.
FIGURE 26–12 Place the infant who has a hypercyanotic spell in the knee–chest
position. This position increases systemic vascular resistance in the lower extremities.

© 2006 by Pearson Education, Inc.

Jane W. Ball and Ruth C. Bindler
Upper Saddle River, New Jersey 07458
Child Health Nursing: Partnering with Children & Families All rights reserved.
Surgical treatment
cardiac catheterization, which may include procedural
treatment in the cath lab
valve replacement
conduit a passage (a pipe or tunnel) through which water or electric wires can pass
placement
cardiac transplant
FIGURE 26–6 Interventional catheterization, balloon valvuloplasty to open the pulmonary valve.

© 2006 by Pearson Education, Inc.

Jane W. Ball and Ruth C. Bindler
Upper Saddle River, New Jersey 07458
Child Health Nursing: Partnering with Children & Families All rights reserved.
Treatment of the Child with TOF
Decrease cardiac workload
Prevention of intercurrent infection
Prevention of hemoconcentration
Surgical repair – palliative or corrective surgery
Balloon Dilatation
of Pulmonic Valve
Nursing Care:
Monitor VS
I&O
Medications
Position
Metabolic rest
Assess and document child/family
interactions
Parent teaching
 Transposition of Great Vessels
Aorta arises from the right
ventricle, and the pulmonary artery
artery arises from the left
ventricle - which is not
compatible with survival
unless there is a large defect aorta
present in ventricular or atrial
septum.
d-Transposition of the Great Arteries
Transposition of the Great Arteries
Pathophysiology
Cyanosis due to failure of delivery of pulmonary venous blood
to the systemic circulation
Two parallel circulations with no mixing
Open atrial septum (fossa ovalis) allows some left-to-right shunt,
enhanced by a left-to-right ductus arteriosus shunt
Presence of ventricular septal defect facilitates mixing
Transposition of the Great Arteries
Aorta from right ventricle, pulmonary artery from left
ventricle.
Cyanosis from birth, hypoxic spells sometimes
present.
Heart failure often present.
Cardiac enlargement and diminished pulmonary artery
segment on x-ray.
Transposition of the Great Arteries
Anatomic communication must exist between
pulmonary and systemic circulation, VSD, ASD, or
PDA.
Untreated, the vast majority of these infants would not
survive the neonatal period.
 Transposition of the Great Arteries
Clinical Manifestations

Cyanosis, tachypnea are most often recognized within the

1st hrs or days of life.
 Hypoxemia is usually moderate to severe, depending on
the degree of atrial level shunting and whether the ductus is
partially open or totally closed.
 Physical findings, other than cyanosis, may be remarkably
nonspecific.
Murmurs may be absent, or a soft systolic ejection
murmur may be noted at the midleft sternal border.
Transposition of the Great Arteries
Chest film
Oval-shaped heart
Narrow mediastinum
Normal or increased pulmonary vascular markings
 D-Transposition of the Great Arteries

This condition is a medical emergency,

and only early diagnosis and appropriate intervention can avert the
development of prolonged severe hypoxemia and acidosis,
which lead to death
 Treatment

When transposition is suspected, an infusion of prostaglandin E1

should be initiated immediately to maintain patency of the ductus
arteriosus and improve oxygenation.
 Endotracheal intubation

 Infants who remain severely hypoxic or acidotic despite

prostaglandin infusion should undergo Rashkind balloon atrial
septostomy
 A Rashkind atrial septostomy is also usually performed in all patients
in whom any significant delay in surgery is necessary.
 Preventing Birth Defects

 Stop smoking
 Avoid drinking alcohol while pregnant
 Take a daily vitamin containing folic acid
 Antenatal to make sure any medication (over-the-counter or
prescription) is safe to take during pregnancy
 Stop use of any illegal or "street" drugs
Nursing interventions pre and post cardiac
catheterization
Assessment pre-op for baselines
Assessment post-op:
Vital signs (which ones are priority?)
Extremities
Activity
Hydration
Medications
Comfort measures
Teaching after cardiac catheterization
Parental teaching
Watch for s/s of bleeding, bruising at site
Loss of sensation in foot on side of cath
When to call the physician
If any of above s/s noted within 1st 24 hrs
Rheumatic Fever
inflammatory connective tissue disorder that follows
initial infection by group A beta-hemolytic
streptococci
may lead to permanent mitral or aortic valve damage
migratory polyarthritis, subcutaneous nodules, fevers,
chorea movements.
Rheumatic fever
S/S
 Systolic murmur
 Chorea (sudden involuntary movement of the limbs)
 Macular rash on the trunk
 Swollen and tender joints, Subcutaneous nodules
 Positive ASO titer and increased ESR and C-reactive protein
Fever
Lethargy/general malaise
Anorexia
Splenomegaly
Retinal hemorrhages
Treatment for Rheumatic Fever
antibiotics to treat the strept infection: pcn,
erythromycin
Analgasic for joint pain and fever
monitored by cardiac echo (serial)
steroids for severe carditis with CHF
long term antibiotics until adulthood
1x/mo IM (Pen G)
 Bedrest
 Prognosis depends on how much heart involvement
Principles that apply to all cardiac
conditions:
Encourage normal growth and development
Counsel parents to avoid overprotection
Address parents’ concerns and anxieties
Educate parents about conditions, tests,
planned treatments, medications
Assist parents in developing ability to
assess child’s physical status