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
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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 PaO2_
• 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.
• 5th or 6th 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.
http://64.143.41.32/news.html
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.
• Rashkind’s Procedure
• Reestablish Foramen Ovale
• Prostaglandin E1 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 CO2
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