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Congenital Heart Defects

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Surgical Correction Jantene Operation Coarctation of the Aorta 7% of congenital cardiac defects. Constriction of the aorta. Results in severely reduced blood flow. – PowerPoint PPT presentation

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Title: Congenital Heart Defects


1
Congenital Heart Defects
2
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.

3
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

4
Web Sites
  • http//www.childrensheartinstitute.org/educate/def
    ects/defects.htm

5
Normal Cardiac Blood Flow
6
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

7
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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9
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

10
Diagnosis - PDA
  • Loud grade I to grade III systolic murmur at left
    sternal border.
  • Washing machine
  • Echocardiography

11
Treatment - PDA
  • Restrict fluids.
  • Diuretics
  • Prostaglandin Inhibitors - Indomethacin
  • Surgical closure (ligation).

12
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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15
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

16
Treatment - ASD
  • Surgical closure.
  • Non-Surgical closure via cardiac catheterization.

17
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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20
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

21
Treatment - VSD
  • Nothing if VSD is small.
  • With CHF or Failure to Thrive Surgical closure.

http//64.143.41.32/news.html
22
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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25
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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27
Blalock-Taussig
  • Something the Lord Made.
  • Vivien Thomas

28
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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31
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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33
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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35
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

36
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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38
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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40
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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42
Repair of Truncus Arteriosus
43
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

44
  • 1 Patent foramen ovale
  • 2 Coarctation of the aorta
  • 3 Patent ductus arteriosus
  • 4 Narrowed aorta
  • 5 Hypoplastic left ventricle
  • 6 Aortic atresia

45
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.

46
Stage I - Norwood Procedure
47
Stage II - Glenn Shunt
48
Stage III Fontan Procedure
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