1. Congenital Heart Defects
MODULE F
Chapter Eleven
Cardiac System
Pages

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

5. Normal Cardiac Blood Flow

6. Ductus Arteriosus Fetal Circulation Component Closure secondary to:
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.

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.

15. ASD: Pathophysiology and Diagnosis
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.

20. VSD: Pathophysiology & Diagnosis
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.

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

25. Tetralogy of Fallot: Diagnosis and Treatment
Tet Spells
CXR: Boot-shaped Heart
Diagnosed with echocardiography.
Surgical correction.
Reparative or Palliative (Blalock-Taussig)

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

31. Transposition – Diagnosis and Treatment
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

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.

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 > lower

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

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.

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

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. 2 Coarctation of the aorta 3 Patent ductus arteriosus 4 Narrowed aorta
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