Congenital Heart Defects

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Congenital Heart Defects
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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.

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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

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Web Sites

• http//www.childrensheartinstitute.org/educate/def
  ects/defects.htm

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Normal Cardiac Blood Flow
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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

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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.

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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

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Diagnosis - PDA

• Loud grade I to grade III systolic murmur at left
  sternal border.
• Washing machine
• Echocardiography

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Treatment - PDA

• Restrict fluids.
• Diuretics
• Prostaglandin Inhibitors - Indomethacin
• Surgical closure (ligation).

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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.

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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

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Treatment - ASD

• Surgical closure.
• Non-Surgical closure via cardiac catheterization.

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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.

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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

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Treatment - VSD

• Nothing if VSD is small.
• With CHF or Failure to Thrive Surgical closure.

http//64.143.41.32/news.html
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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

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Tetralogy of Fallot Diagnosis and Treatment

• Tet Spells
• CXR Boot-shaped Heart
• Diagnosed with echocardiography.
• Surgical correction.
• Reparative or Palliative (Blalock-Taussig)

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Blalock-Taussig

• Something the Lord Made.
• Vivien Thomas

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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

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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 E1 to keep PDA open.
• Surgical Correction
• Jantene Operation

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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.

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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 gt lower

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Coarctation Diagnosis and Treatment

• Diagnosis
• Chest X-Ray
• Echocardiography
• Cardiac catheterization
• Treatment
• Support with inotropic agents (Dopamine).
• Prostaglandins to maintain PDA.
• Surgical repair

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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.

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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

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Repair of Truncus Arteriosus
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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

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• 1 Patent foramen ovale
• 2 Coarctation of the aorta
• 3 Patent ductus arteriosus
• 4 Narrowed aorta
• 5 Hypoplastic left ventricle
• 6 Aortic atresia

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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.

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Stage I - Norwood Procedure
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Stage II - Glenn Shunt
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Stage III Fontan Procedure