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Congenital Cardiovascular

Classification of CHDs
1. Structural heart defects —due to
abnormal development of the heart
during the first 2 months after conception
2. Functional heart defects–
ex: congenital heart block
3. Positional heart defects —
ex: dextrocardia
• May occur:
– With Situs Inversus: carries
a slightly increased risk of
heart defects (~ 5 – 10%
associated with other

– Without Situs Inversus:

carries a greatly increased
risk of associated heart
defects (~95% associated
with other CHDs)

• Both conditions are

Situs Inversus
Classifications of
Structural Congenital Heart Defects
Increased Decreased Obstruction to
Pulmonary Pulmonary Systemic Blood
Blood Flow Blood Flow Flow
•PDA •Tetralogy of Fallot •Coarctation of the
•ASD (TOF) Aorta
•VSD •Transposition of the •Aortic Stenosis (AS)
•AV Canal Great Arteries (TGA) •Hypoplastic Left
•Total Anomalous •Pulmonary Stenosis Heart Syndrome
Pulmonary Venous (PS) (HLHS)
Return (TAVPR) •Pulmonary Atresia •Mitral Stenosis (MS)
•Truncus Arteriosis (PA)
•Tricuspid Atresia (TA)
Right to Left vs. Left to Right

• Right to left shunt: un-oxygenated blood is

shunted from the right side of the heart to the left
side, and then enters the systemic circulation.
• Left to right shunt: a portion of the oxygenated
blood is shunted from the left side of the heart to
the right side and enters the pulmonary
circulation, increasing the work load for the right
Cyanotic vs. Acyanotic
• Acyanotic (usually left to right shunts):
• Cyanotic (right to left shunts):
– TOF, Transposition of the Great Arteries, Hypoplastic
Left Heart
– O2 Sat less than 95%
– Child may have chronic hypoxia
– Caused by:
• Decreased pulmonary blood flow –and/or--
• Right-to-left shunting: de-oxygenated blood is shunted from the right side of the heart to the
left side without traveling though the pulmonary circulation, and blood ejected from the left
side of the heart to the systemic circulation is only partly oxygenated
Most Common Congenital Heart
These account for 85% of all
Atrioventricular Septal
Coarctation of the
9% Aorta
10% Tetralogy of Fallot
12% Transposition of the
10% Great Arteries
15% Ventricular Septal
All other congenital
heart defects
Some Statistics
• Most common birth defect – 30% of all
congenital birth defects
(36,000/yr in the United States)

• Most common cause of infant death for children

dying as the result of a birth defect

• In the US over 130,000 hospitalizations/year are

related to CHD
Etiology of CHD
• Unknown in most cases
• Incidence of CHD in children is slightly
increased if a sibling or parent has CHD
• Gender Factors
• Environmental Factors
• Genetic Factors
Gender Factors
• Occur equally among males and females,
– More common in males:
aortic stenosis, coarctation of the aorta

– More common in females:

Environmental Factors
• Maternal Infections:
– Rubella: PDA, pulmonary stenosis, VSD, ASD
• Maternal Drugs:
– Lithium: Tricuspid valve abnormalities, Ebstein’s Anomaly
– Thalidomide
– Possibly related to CHDs: Dilantin & Cocaine
– Alcohol abuse: VSD
• Maternal Disease:
– Diabetes: transportation of the great vessels, VSD, situs inversus,
single ventricle, hypoplastic left ventricle
– SLE: Congenital heart block
Genetic Factors
• Trisomy 21 (Down’s Syndrome):
A-V canal defects, VSD

• XO (Turner’s Syndrome):
coarctation of the aorta, aortic stenosis

• Osteogenesis Imperfecta:
Aortic incompetence

• Marfan Syndrome:
Aortic dilatation, aortic & mitral incompetence
The good news is--
• From 1991 – 2001 deaths related to CHD
declined 28% due to improvements in surgical
techniques and medical management
Prevention of CHD
• Not possible in most cases
• But -- there are actions a woman can take to
reduce her risk of having a child with CHD:
– Abstain from alcohol during pregnancy
– Be immunized against rubella before conception
– If diabetic, maintain tight control of blood sugars
– Folic acid 400 mcg/daily before conception may help
to prevent CHD (unproven)
– If there is a family history of CHD seek genetic
counseling prior to conception
Signs/Symptoms of CHD
• Murmurs
• Cyanosis –worsens with crying or other exertion
• Respiratory distress
• Signs of poor perfusion, such as slow capillary
refill, diminished peripheral pulses
• Fatigue – commonly observed during feedings in
newborns or during play in children
• Failure to thrive
Embryonic Heart Development

The heart develops in the embryo during

post-conception weeks 3 - 8
Beginning Development
• Early week 3 post-conception: heart begins as 2
endothelial tubes
• Mid-week 3 : endothelial tubes fuse to form a
tubular structure
• 28 days following conception: the single-
chambered heart begins pumping blood
Week 4
• Heart has:
– single outflow tract, the truncus arteriosus (divides to
form aorta & pulmonary veins)
– Single inflow tract, the sinus venosus (divides to form
the superior and inferior vena cavae)
– Single atrium
– Single ventricle
– AV canal begins to close
Weeks 5 - 7
Week 5 Week 7
• AV canal closure • Ventricular septum fully
complete developed
• Formation of atrial and • Coronary Sinus forms
ventricular septums • Outflow tracts (aorta &
• Heart growing rapidly, pulmonary truck) fully
and folds back on itself to separated
form its completed
anatomic shape
8 Weeks After Conception

• By the end of the 8th week after conception the

fetus has a fully developed 4-chambered heart
Fetal Circulation
• Before birth the placenta provides the
oxygen needed by the developing fetus—
the lungs receive only enough blood to
perfuse the lung tissues due to high
pulmonary vascular resistance & fetal
vascular shunts
Fetal Circulation
• Arterial blood in the fetus:
– enters the fetal circulation via the umbilical vein:
– passes through the ductus venosus and enters the
inferior vena cava
– flows into the right atrium and passes through the
foramen ovale into the left side of the heart
– passes from the right side of the heart, through the
ductus arteriosus to enter the systemic circulation,
bypassing the pulmonary circulation
Fetal Circulation
• Venous blood in the fetus:
– returns to the placenta through the 2 umbilical
After Birth
• Lungs distend with air and pulmonary
vascular resistance falls. Pulmonary
blood flow increases
• The foramen ovale and ductus venosus
usually close during the first day of life
• The ductus arteriosus usually closes
during the first 24 – 72 hours of life