Congenital Heart Defects

MODULE F
Chapter Eleven
Cardiac System
Pages 348 - 359
Objectives
Differentiate between the different
congenital heart diseases and state
whether they are cyanotic and acyanotic.
Describe the defect present with each
congenital heart disease.
Describe the surgical repair (if any) for
each congenital heart disease.
Cardiac Defects
Patent Ductus Arteriosus
Atrial Septal Defect
Ventricular Septal Defect
Tetralogy of Fallot
Transposition of the Great Arteries
Coarctation of the Aorta
Anomalous Venous Return
Truncus Arteriosus
Hypoplastic Left-Heart Syndrome

Web Sites
http://www.childrensheartinstitute.org/edu
cate/defects/defects.htm

Normal Cardiac Blood Flow
Ductus Arteriosus
Fetal Circulation Component
Connects Pulmonary Artery to Aorta
Shunts blood away from lungs
Maintained patent by presence of
prostaglandins
Closure secondary to:
Increase in PaO
2_

Decrease in level of prostaglandins
Patent Ductus Arteriosus
5-10% of all births (1 of 2000 live births)
80% of premature babies
2-3 times more common in females than
males.
5
th
or 6
th
most common congenital cardiac
defect.
Often associated with other defects.
May be desirable with some defects.
Morbidity/Mortality related to degree of
blood flow through PDA.
Pathophysiology - PDA
With a drop in pulmonary arterial pressure
(reduction in hypoxic pulmonary vascular
constriction), blood will flow through PDA.
LEFT TO RIGHT SHUNT
Increased pulmonary blood flow may lead to
pulmonary edema.
Reduced blood flow to all postductal organs
NEC
If pulmonary artery pressure rises above Aortic
pressure, blood will move in the other direction.
RIGHT TO LEFT SHUNT
Diagnosis - PDA
Loud grade I to grade III systolic murmur
at left sternal border.
Washing machine
Echocardiography
Treatment - PDA
Restrict fluids.
Diuretics
Prostaglandin Inhibitors - Indomethacin
Surgical closure (ligation).
Atrial Septal Defect
6-10% of all births (1 of 1500 live births)
2 times more common in females than males.
Types:
Ostium Secundum (at or about the Foramen Ovale)
Sinus Venous (at about the point where SVC and IVC
attach to Right Atrium)
In 1950 most children with ASD did not reach the
first grade. Today, first year surgery facilitates
normal growth and development.


ASD: Pathophysiology and
Diagnosis
Pathophysiology
Left to Right Shunt
Inefficient recirculation of good blood through
pulmonary arteries.
May not manifest symptoms and may be
found later in life.
If defect is significant, may cause problems
later in life due to inefficiencies.
Diagnosis
Murmur
Echocardiography
Treatment - ASD
Surgical closure.
Non-Surgical closure via cardiac
catheterization.
Ventricular Septal Defect
1% of all births (2 to 4 of 1000 live births)
Vast majority the hole is small.
In 1950, fatal. Today almost all VSD can be
closed successfully, even in small babies.
Lillehei was the first person in history to
correct both ASD and VSD on 8/31/54.

VSD: Pathophysiology & Diagnosis
Pathophysiology
May be isolated or associated with other
congenital cardiac defects.
With normal PVR:
LEFT TO RIGHT SHUNT
With elevated PVR (RDS):
RIGHT TO LEFT SHUNT
Diagnosis
Echocardiography
Treatment - VSD
Nothing if VSD is small.
With CHF or Failure to Thrive: Surgical
closure.
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Tetralogy of Fallot
1% of neonates.
Most common of the cyanotic cardiac diseases.
Mortality increases with age (1 year-old has a
25% mortality, 40 year-old has 95%).
In 1950, fatal. Today, less than 5% mortality with
children operated on in infancy, leading normal
lives.
Four Defects
Pulmonary Artery Stenosis (determinant factor related
to severity)
VSD (usually large)
Overriding Aorta
RV hypertrophy
Tetralogy of Fallot: Diagnosis and
Treatment
Tet Spells
CXR: Boot-shaped Heart
Diagnosed with echocardiography.
Surgical correction.
Reparative or Palliative (Blalock-Taussig)

Blalock-Taussig
Something
the Lord
Made.
Vivien
Thomas


Complete Transposition of the
Great Arteries
Second most common form (5-7%) of
congenital cardiac anomalies.
Aorta arises from RV and Pulmonary
Arteries from LV.
Without an abnormality, life would not
be possible.
ASD
VSD (30-40%)
PDA
Transposition Diagnosis and
Treatment
Diagnosis
Chest X-Ray: Egg on a
String
Echocardiography
Cardiac Catheterization (?)
Treatment
Balloon septostomy during
cardiac cath.
Rashkinds Procedure
Reestablish Foramen Ovale
Prostaglandin E
1
to keep
PDA open.
Surgical Correction
Jantene Operation
Coarctation of the Aorta
7% of congenital cardiac defects.
Constriction of the aorta.
Results in severely reduced blood flow.
Increased work on the heart leading to
CHF and cardiovascular collapse.
Location of narrowing determines the
clinical signs.
Usually associated with PDA, VSD and a
defective aortic valve.
Location of Coarctation
Pre-Ductal
Less common but more serious
Associated with VSD, PDA, Transposition
Post-Ductal
More common
Often associated with collateral circulation
beyond coarctation, which minimizes effect.
Diagnosed by a difference in blood pressure
between lower extremities and upper ones.
Pressure in upper extremities > lower
Coarctation Diagnosis and
Treatment
Diagnosis
Chest X-Ray
Echocardiography
Cardiac catheterization
Treatment
Support with inotropic agents (Dopamine).
Prostaglandins to maintain PDA.
Surgical repair

Anomalous Venous Return
Return of pulmonary venous blood to the
right atrium instead of the left.
ASD is present to sustain life.
Can also be partial.
Cyanosis usually present.
Diagnosed with echocardiography.
Surgical correction with reimplantation of
pulmonary veins.
Truncus Arteriosus
Defect in which one large vessel arises from
right and left heart over a large VSD.
Cyanosis is often present.
CHF common.
Diagnosed with echocardiography and
cardiac catheterization.
Surgery:
Separate pulmonary arteries from truncus.
Closure of VSD
Create valved connection between RV and
Pulmonary Artery
Repair of
Truncus
Arteriosus
Hypoplastic Left-Heart Syndrome
Several anomalies:
Coarctation of the aorta
Hypoplastic left ventricle
Aortic and mitral valve stenosis or atresia.
Cyanotic defect.
Right heart pumps blood to body through
PDA.
Closure of PDA results in hypotension,
shock, and death.
Maintain hypoxemia with normalized CO
2
levels.
40-40 Club

1 Patent
foramen
ovale
2 Coarctation
of the aorta
3 Patent ductus
arteriosus
4 Narrowed
aorta
5 Hypoplastic
left ventricle
6 Aortic atresia
Surgical Treatment of Hypoplastic Left
Heart Syndrome
Three separate
surgeries.
Norwood procedure
First few days after birth.
Glenn Shunt (Cavo
Pulmonary Connection)
3-9 months of age
Fontan Procedure
2 years of age
Less wait because of
damage from pulmonary
hypertension.
Stage I - Norwood Procedure
Stage II - Glenn Shunt
Stage III Fontan Procedure