Blood from SVC & IVC
Blood from SVC & IVC
Blood from SVC & IVC
Right Atrium
Tricuspid Valve
Blood from SVC & IVC
Right Atrium
Tricuspid Valve
Right Ventricle
Pulmonary Valve
Blood from SVC & IVC
Right Atrium
Tricuspid Valve
Right Ventricle
Pulmonary Valve
Main Pulmonary Artery, R& L
Branch Pulmonary Arteries
Blood from SVC & IVC
Right Atrium
Tricuspid Valve
Right Ventricle
Pulmonary Valve
Main Pulmonary Artery, R& L
Branch Pulmonary Arteries
Lungs- Gas Exchange
Lungs- Gas Exchange
Pulmonary Vein
Left Atrium
Mitral Valve
Left Ventricle
Lungs- Gas Exchange
Pulmonary Vein
Left Atrium
Mitral Valve
Left Ventricle
Aortic Valve
Ascending Aorta,
Descending Aorta
8 in 1000 infants- born with a congenital heart defect
25%- complex
1/3- will require surgical intervention
25%- have other associated congenital anomalies
90%- survive upto adulthood
Increasing pulmonary blood flow
Decreasing pulmonary blood flow
Obstruction to systemic blood flow
Left-to-Right Shunt
●
Description—Abnormal
opening between the
atria, allowing blood
from the higher pressure
left atrium to flow into
the lower pressure right
atrium
L atrial pressure
> R atrial
pressure
• Blood flows from
right to left
↑
oxygenated
blood into
the R side of
the heart
R ventricle
tolerates
flow of
blood
R atrial and
ventricle
enlargement
• (although
unusual)
If
unrepaired:
• Pulmonary
vascular
changes occur
after several
decades
Asymptomatic
• if the defect is small
• Many small ASDs will close
spontaneously in the first few years of
life
Dysrhythmias
• caused by atrial enlargement and
stretching of conduction fibers
Characteristic
Murmur
• Typically presents with a
systolic ejection murmur,
often described as a
"flow murmur," due to
increased blood flow
across the pulmonary
valve.
• May also have a fixed
split S2 (wide splitting of
the second heart sound).
Rales, congestion, tiring
with activity, frequent
RTI, or poor weight gain
Dilation of the right
atrium
pulmonary vascular
obstructive disease and
emboli formation
• pulmonary overcirculation,
• increased blood volume
• chronically increased
pulmonary blood flow
Asymptomatic
Symptomatic
• Not indicated for closure
• Diuretics- attempted to allow the
defect to close spontaneously
Non-surgical: Amplatzer Septal Occluder via Cardiac Catheterization
Post op- Low-dose
aspirin for 6 months
Surgical Treatment: Surgical patch closure
(pericardial patch or Dacron
patch)
Description—Abnormal
opening between the right
and left ventricles
Many VSDs (20%–60%)
close spontaneously
Spontaneous closure is most
likely to occur during the
first year of life in children
having small or moderate
defects.
• Higher pressure L
ventricle
• Resistance: Systemic
arterial circulation >
Pulmonary circulation
Left-to-right
shunting
↑ pulmonary
resistance
Hypertrophy
• R ventricles
• R atrium
Left heart
dilatation
Tachypnea, poor
feeding, or
failure to thrive
• Increase in pulmonary
blood flow
• increased pulmonary
blood flow
Application of Dacron
Patch with CPB
A congenital heart defect characterized by abnormalities
in the development of the septum between the atria and
ventricles of the heart.
In AVSD, septa are either partially or completely absent,
resulting in a large opening between the atria and
ventricles.
● AVSD is the most common type of CHD in children with
trisomy 21
●
AVSD can involve
various
combinations of
defects
Left-to-right shunting
• pulmonary blood flow
increases  Heart Failure
Complete
• with a large septal defect that includes both the atria and the
ventricles, and a single atrioventricular valve
Transitional
• with a small or moderate-sized ventricular septal defect in addition
to the atrial septal defect
Partial
• with an atrial septal defect but no ventricular septal defect, and the
common AV valve separated into a right- and a left-sided AV valve
Moderate to severe
Heart Failure
Murmur
Mild cyanosis
• rapid breathing, shortness of breath while
eating, poor weight gain, failure to thrive, and
diaphoresis by age 4 to 6 weeks
• Loud systolic
• Mixing of oxygen rich and oxygen poor blood,
Increases with crying
Medications
• furosemide, digoxin, and an ACE inhibitor like captopril or enalapril
Surgical Repair
• consists of closing the ASD and VSD and repairing the mitral and
tricuspid valves
• Children with Down syndrome- less than 3 months of age
• Children without Down syndrome – referred for repair by 5 or 6
months
Manageent
Pulmonary Artery Bands
• is a band surgically placed
around the pulmonary artery
that constricts it to decrease
some of the overall pulmonary
blood flow and prevent too
much pulmonary circulation
and long-term pulmonary
vascular changes.
●
●
Description—Failure of the
fetal ductus arteriosus (artery
connecting the aorta and
pulmonary artery) to close
within the first weeks of life.
left to right (from aorta to
pulmonary artery)
●
The ductus arteriosus is a normal
connection between the pulmonary
artery and aorta; it is necessary for
proper fetal circulation.
●
Two Mechanisms that Facilitate
Closure:
○ the rise in PaO2
○ decline in prostaglandin
concentration
left to right (from aorta to
pulmonary artery)
Murmur
• machinery-like murmur, second intercostal space, left
upper sternal border, or out to the left clavicular area,
rales, congestion, increased
WOB, difficulty feeding, or • increased pulmonary circulation
failure to thrive
Left heart dilation
Widened pulse pressure &
bounding pulses
• blood shunting to the lungs
• runoff of blood from the aorta to the pulmonary artery
cyclo-oxygenase (COX) inhibitor therapy (eg,
indomethacin, ibuprofen lysine) for preterm infants
• Given in three separate IV doses
Increase Caloric
intake- to help the
child gain weight
Furosemide, Fluid
Restriction- manage
increased blood flow
Transcatheter closure:
• the treatment of choice for
PDA in children > 1 year
IN SUMMARY
●
●
●
●
Left-to-right shunt
Increasing pulmonary blood flow
↑ pulmonary blood flow
○ Pulmonary edema (dyspnea)
○ Bronchopulmonary infections
Heart Failure
○ Periorbital edema, weight gain, oliguria
○ hepatomegaly
●
Description—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
Valvular Stenosis
• Most common type,
is usually caused by
malformed cusps that
result in a bicuspid
rather than tricuspid
valve or fusion of the
cusps
Supravalvular
stenosis
• occurs infrequently
• stenosis occurs when
there is narrowing or
constriction of the aorta
above the level of the
aortic valve.
Subvalvular
stenosis
• is a stricture
caused by a
fibrous ring below
a normal valve
Aortic
Stenosis
Left
Ventricular
Hypertrophy
• prominent
anatomic
consequence
Left
Ventricular
Failure
Interferes
with with
coronary
artery
perfusion:
myocardial
infarction
Increase
in Left
Atrial
Pressure
Pulmonary
Vascular
Congesti
on
Infant (critical)
Child
Systolic ejection
murmur
• decreased cardiac output with faint pulses,
hypotension, tachycardia, and poor feeding
• exercise intolerance, chest pain, and
dizziness when standing for a long period
• may or may not be present.
Balloon Angioplasty
• Narrowed valve is dilated
• The first intervention
Norwood procedure
• For newborns with
critical AS
Aortic valve replacement
• offers a good treatment option
and may lead to normalization
of left ventricular size and
function
●
Description—Narrowing at
the entrance to the
pulmonary artery. Resistance
to blood flow causes right
ventricular hypertrophy and
decreased pulmonary blood
flow.
●
●
is the extreme form of PS in
that there is total fusion of
the commissures and no
blood flows to the lungs.
The right ventricle may be
hypoplastic.
Right
ventricular
Hypertrophy
Pulmonary Stenosis
•Right ventricular
failure
Increase Right
Atrium
Pressure
Reopening of
Foramen
Ovale:
shunting of
unxygenated
blood into the
left atrium
Right sided HF
Asymptomatic
• mild narrowing
• Loud systolic ejection murmur
Cyanosis or HF
• At the upper left sternal border
Cardiomegaly
• Seen in chest radiography
Balloon
angioplasty
• to dilate the valve
Obstructive defects
•
impede blood flow out of the ventricles
obstruction
on the left
side of the
heart
results in
HF
severe
obstruction on
the right side
causes cyanosis
●
Description—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).
proximal to the defect
(upper extremities)
↑ pressure
distal to the defect (lower
extremities)↓ pressure
Upper
extremities
• high blood pressure and
bounding pulses in the arms
Lower
Extremities
• weak or absent femoral pulses,
and cool lower extremities with
lower blood pressure
systolic murmur
• heard along the left sternal border
and the left midscapular area.
dizziness,
headaches, fainting, • Older children
and epistaxis
Left ventricular
hypertension and
hypertrophy
• As the narrowing increases the
resistance to the left ventricle
hypertension
ruptured aorta
aortic aneurysm
stroke
●
Description—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
●
There is complete mixing of
unoxygenated and oxygenated
blood in the left side of the
heart, which results in systemic
desaturation, and varying
amounts of pulmonary
obstruction, which causes
decreased pulmonary blood
flow.
Continuous infusion of
prostaglandin
• For patients who depend on the patency
of the ductus arteriosus
Blalock-Taussig shunt (pulmonary–to–
systemic artery anastomosis)
Atrial septostomy
Pulmonary artery banding
●
Description—The classic
form includes four
defects: (1) ventricular
septal defect (VSD), (2)
pulmonic stenosis, (3)
overriding aorta, and (4)
right ventricular
hypertrophy
●
The alteration in hemodynamics varies
widely, depending primarily on the degree
of pulmonary stenosis, but also on the size
of the VSD and the pulmonary and systemic
resistance to flow.
- VSD is usually big - RV pressure = LV pressure
- Shunting will depend on: Pulmonary vs systemic vascular resistance
Pulmonary vascular
resistance > systemic
resistance
• Right-to-left shunt (RV 
Aorta)
• ↓ Blood Flow to the Lungs
Pulmonary vascular
resistance < systemic
resitance (Unrestricted
VSD or Variable
Pulmonary Stenosis)
• Left-to-right shunt (LV to RV)
• ↑ Blood flow to the lungs
Cyanosis
• progresses over the first year of life as the
pulmonary stenosis worsens
• Tet speels or blue spells (acute episodes of
hypoxia)
Murmur
• Systolic ejection murmur due to pulmonary
stenosis (harsh, crescendo-decrescendo,
heard best at the upper left sternal
border). May also have a holosystolic
murmur from the associated VSD.
●
Defects that cause decreased
pulmonary blood flow result in
cyanosis.
Place infant in knee-chest position
Employ a calm, comforting approach.
Administer 100% oxygen by face mask.
Give morphine subcutaneously or through existing
intravenous line.
Begin intravenous fluid replacement and volume
expansion, if needed.
•Repeat morphine administration.
●
Description—The
pulmonary artery leaves
the left ventricle, and the
aorta exits from the right
ventricle, with no
communication between
the systemic and
pulmonary circulations
Associated defects such as
must be present to permit
blood to enter the systemic
circulation or the pulmonary
circulation for mixing of
saturated and desaturated
blood.
Most common: foramen
Ovale
VSD increases the risk of
heart failure (HF)
large septal defects or a
• less cyanosis but have symptoms of HF
patent ductus arteriosus
with minimum
communication
• Severe Cyanosis
Tachypnea
Murmur
• may not be noted
Management
Intravenous prostaglandin E1
• At birth to maintain patency of the ductus
arteriosus and encourage mixing of blood
• SE: apnea ang hypotension (>2kg weight)
• infant to remain on room air, spontaneously
breathing
• Prepare ventillatory support
Balloon atrial septostomy
• patent foramen ovale (PFO)the opening will be enlarged
Jatene procedure
• includes cutting both the pulmonary artery and aorta
above their respective valves and switching the vessels
to the appropriate location.
• The final component of the surgical repair is removing
the coronary arteries from the base of the original aorta
and surgically placing them in an appropriate spot on
the new aortic root, now arising from the left ventricle.
Kawasaki disease (mucocutaneous lymph
node syndrome) is defined as an acute febrile
syndrome associated with generalized
vasculitis (inflammation of blood vessels)
affecting all blood vessels throughout the
body, including the coronary arteries
-unknown etiology
●
●
●
●
●
There is a genetic predisposition
Strong suggestion of an infectious precursor
Occurs more commonly in winter and spring, with
males affected more than females;
76% of children affected are less than 5 years
old
Diagnosis of exclusion
●
●
●
●
●
●
●
Prolonged fever (higher than 100.4°F
[39°C]) of 5 or more days
Four or more of the following symptoms:
Changes in hands and feet (erythema,
edema, peeling)
Polymorphous exanthema (diffuse
maculopapular rash of the trunk and
extremities)
Bilateral conjunctivitis without exudates
Changes in lips and mouth (erythema,
strawberry tongue, dry, cracked lips)
Cervical lymphadenopathy (larger than
1.5 cm in diameter, usually unilateral)
Thrombocytosis
leukocytosis,
elevated liver
enzymes,
elevated ESR
and CRP
mild anemia.
Acute Phase
• children are very irritable
and uncomfortable from the
fever and
• inflammatory process that
causes joint pain
Subacute Phase
• 10 days after
• Skin desquamates,
particularly on the palms
and soles.
• ↑ platelet count: stroke risk
• Aneurysm in a coronary
artery: ischemia and
infarction
●
●
IV fluids/ Antipyretics
high-dose intravenous immunoglobulin (IVIG) and highdose aspirin therapy
○ a reduction in the appearance of coronary artery
irregularities and aneurysms due to their antiinflammatory properties
●
One of the most important causes of cardiovascular
●
Morbidity and mortality among socially and economically
disadvantaged populations worldwide
●
Rheumatic fever is an autoimmune disease that occurs as a
reaction to a group A beta-hemolytic streptococcal infection,
specifically, a pharyngitis.
●
Inflammation from the immune response leads to inflammatory
lesions in the heart, blood vessels, brain, and joints.
●
Occurs as a reaction to a group A beta-hemolytic
streptococcal infection (pharyngitis)
●
Occurs most often in children 6 to 15 years of age, with a
peak incidence at 8 years
●
Genetically susceptible individuals
●
The sensitized child will eventually have a group A betahemolytic streptococcal pharyngitis that sets off an
unusually high antibody response
Erythema marginatum
Sydenham’s chorea
elevated
antistreptolysin-O
(ASO) titer
• recent streptococcal infection
ESR and CRP
• Inflammatory marker, elevated
ECG and CXR
• To evaluate the extent of cardiac
involvement.
•
•
•
•
•
Intramuscular (IM) or oral penicillin is
prescribed for a full 10-day course
Oral nonsteroidal anti-inflammatory
Phenobarbital and diazepam are both effective
in
Reducing the purposeless movements of the
chorea
Cardiologist consult for life