The document discusses various types of congenital heart defects including cyanotic and acyanotic defects, summarizing key features such as symptoms, physical exam findings, treatments, and anesthetic considerations for septal defects, atrial septal defects, patent ductus arteriosus, interrupted aortic arch, tetralogy of Fallot, and single ventricle physiology. It provides an overview of important congenital heart conditions from an anesthesiology perspective.

1. Congenital Heart Disease
Conrad Cheung, MD
Marie Sankaran, MD
Department of Anesthesiology
Boston University Medical Center
Faculty Advisor: Elena Brasoveanu, MD
March 9, 2006

2. Classification
 Acyanotic
– Interrupted Aortic Arch
– Aortic Stenosis
– Ventricular Septal Defect
– Atrial Septal Defect
 Cyanotic Defects
– Hypoplastic Ventricle
– Pulmonary Stenosis
– Tetralogy of Fallot
– D transposition of the great vessels
– Tricuspid atresia

3. Plan
 We will discuss few topics that are important from
the anesthesiologist point of view
– Septal Defects
– Atrial Septal Defects
– Patent Ductus Arteriosus
– Interrupted Aortic Arch
– Tetralogy of Fallot
– Single ventricle

4. The Cyanotic Neonate
 Ductus Arteriosus closes within 6 hours of birth so
any congenital heart disease that relies on it for
perfusion of oxygenated blood will present early
 To prevent death from inadequate oxygenation,
the duct has to be maintained open with
prostaglandines
 Any neonate with a congenital heart disorder that
relies on an open ductus arteriosus for
oxygenation will deteriorate rapidly and requires
immediate transfer to a specialty center

5. Ventricular Septal Defect I
 Defect in the septum separating the left and right
ventricles
 Most common type of congenital heart disease
accounting for 21% of all cases
 Can occur singly or in multiples anywhere along
the ventricular septum
 Small defects often close spontaneously in the first
2 years of life while large defects require surgical
repair within the 1st year

6. Ventricular Septal Defect II
 Prevalence equal between boys and girls
 Due to increased pressures in the left
ventricle, left to right shunting of
oxygenated blood occurs
 With the increased pulmonary blood flow,
pulmonary hypertension can occur with
large defects

7. Ventricular Septal Defect III

8. Symptoms of Ventricular Septal
Defects
 Rapid breathing
 Irritability
 Excessive Sweating
 Poor weight gain
 Congestive Heart Failure, usually within 6
to 8 weeks of life if defect is large
 Pulmonary Hypertension if defect is large

9. Treatments for Ventricular Septal
Defects
 Lasix and Aldactone to decrease symptoms
of CHF
 Digoxin to increase effectiveness of
myocardial function
 If surgery needed, patching or suturing the
defect can be done
 Mortality from surgery is low

10. Atrial Septal Defect I
 Defect in the septum separating the left and
right atria
 Accounts for 5-10% of congenital heart
disease
 Twice as frequent in girls versus boys
 Three types of atrial septal defects

11. Atrial Septal Defect II
 Ostium Primum: Defect located in the lower
part of septum near tricuspid valve which
separates the right atrium and right ventricle
 Ostium Secundum: Defect located near
center of atria septum (most common
accounting for 50-70% of atrial defect)
 Sinus Venosus: Located near the SVC or
IVC’s entrances to the heart

12. Atrial Septal Defect III

13. Atrial Septal Defect IV
 Due to increased pressures, there is left to
right shunting of oxygenated blood
 If large defect, can cause enlarged right
atria, right ventricle, and pulmonary artery
resulting in abnormal arrhythmias
 CHF can occur if left untreated till
adulthood

14. Symptoms of Atrial Septal Defects
 Slender build
 Heart murmur resulting from increased
blood flow through pulmonary valve
 Usually no significant exercise restriction
unless defect is large.
 SOB or palpitations are possible.

15. Treatment of Atrial Septal Defects
 If defect is small (less than 2mm), will
usually resolves spontaneously
 If defect is large, surgical correction is
needed
 Minimally invasive procedures available
 Transcatheter devices, such as a septal
occluder may be used

16. Patent Ductus Arteriosus
 The ductus arteriosus
connects the pulmonary
artery to the descending
aorta during fetal life.
 PDA results when the
ductus fails to close after
birth.
Picture: www.lpch.org

17. Patent Ductus Arteriosus
Pathophysiology:
– Blood flows from aorta to the
pulmonary artery, creating a
left to right shunt, resulting in
left atrium and ventricle
overload.
– Increased pulmonary blood
flow can result in pulmonary
hypertension and reversal of
the shunt, which is known as
Eisenmenger’s Syndrome.
This results in flow of
desaturated blood to the lower
extremities.
Picture: www.lpch.org

18. Patent Ductus Arteriosus
 Symptoms:
– Children with small patent ductus are usually
asymptomatic.
– Large left to right shunts develop symptoms of
congestive heart failure such as tachypnea,
tachycardia, poor feeding and slow growth
 Physical exam:
– Continuous murmur heard best at the left sternal
border.

19. Patent Ductus Arteriosus
 Lab Studies:
– CXR: enlarged cardiac silhouette secondary to left atrial
and ventricular enlargement with prominent pulmonary
vascular markings.
– EKG: left atrial enlargement, LVH
– ECHO: doppler flow through the ductus
 Treatment:
– Surgical division or ligation of the PDA

20. Patent Ductus Arteriosus
 Anesthetic Management:
-Maintain high arterial pressures as low diastolic pressure
and pulmonary steal can result in decreased organ
perfusion.
 Concerns After Repair:
– Rarely, recanalization or incomplete ligation of the
ductus occur.
– Post repair, these patients can be treated as healthy
patients and do not require endocarditis prophylaxis.

21. Interrupted Aortic Arch I
 Relatively rare occurs in 2 cases per
100,000 live births
 Consists of 2 different defects: divided
aortic arch and ventricular septal defect
 Divided Aortic arch results in continued
blood flow to the upper extremities, but
none to the lower extremities

22. Interrupted Aortic Arch II
 Lower extremities oxygenated due to the
combination of patent ductus arteriosus and
ventricular septal defect
 PDA connects lower portion of the aortic to
the pulmonary artery
 VSD allows travel of oxygenated blood to
the right ventricle which in turns travels to
the pulmonary artery.

23. Interrupted Aortic Arch III

24. Symptoms of Interrupted Aortic Arch I
 Often discovered 3-4 days after birth when
the patent ductus arteriosus closes
 Symptoms of shock develops very rapidly
as no oxygenated blood flows to the lower
extremities
 Rapid breathing, clammy sweating, and
poor feeding often develops during the first
week

25. Symptoms of Interrupted Aortic Arch II
 Most babies born at term with normal
length and weight
 Heart murmur usually heard
 Liver may be enlarged
 Left arm/leg pulses may be diminished or
absent
 Echocardiogram for diagnosis

26. Treatment of Interrupted Aortic Arch
 Before surgery, try to keep the PDA open to
provide oxygenated blood to the lower
extremities
 Surgery to suture together the two ends of
the aorta, patch the VSD, and ligate the
PDA
 85-90% patients survive the hospital stay

27. Long Term Prospects for Interrupted
Aortic Arch
 Patients are at increased risk for subacute
bacterial endocarditis requiring antibiotics
before surgery and dental work
 Restriction from vigorous or competitive
sports. Emphasis placed on child to self-
limit their level of exertion

28. Tetralogy of Fallot
 Anatomic Defects
– Ventricular septal
defect
– Overriding Aorta
– Pulmonary artery
stenosis
– Right ventricular
hypertrophy
Picture: www.lpch.org

29. Tetralogy of Fallot
 Pathophysiology:
Increased resistance by the
pulmonary stenosis
causes deoxygenated
systemic venous return
to be diverted from RV,
through VSD to the
overriding aorta and
systemic circulation 
systemic hypoxemia
and cyanosis
Picture: www.lpch.org

30. Tetralogy of Fallot
 Symptoms:
– Dyspnea on exertion or when crying
– Tet spells: irritability, cyanosis, hyperventilation
and sometimes syncope or convulsions due to
cerebral hypoxemia.
– Patients learn to alleviate symptoms by squatting
which increases systemic resistance and decreases
the right-to-left shunt and directs more blood to the
pulmonary circulation.

31. Tetralogy of Fallot
 Physical exam:
– Clubbing of the fingers and toes
– Systolic ejection murmur heard at the upper left sternal
border created by turbulent blood flow through stenotic
RV outflow tract
 Lab Studies:
– CXR: prominent RV
– EKG: RVH, right axis deviation
– ECHO: displays and quantifies extent of RV outflow
tract obstruction

32. Tetralogy of Fallot
 Treatment:
– Surgical closure of the VSD and enlargement of the pulmonary
outflow tract
 Anesthetic Management:
-The goal is to control the magnitude of the right to left intracardiac shunt,
which is increased by:
1) Decreased SVR
2) Increased PVR
3) Increased myocardial contractility
-Patient given beta blockers for prophylaxis against Tet spells
-Inhalation induction can be employed in an attempt to avoid Tet spells
while placing an intravenous line.
-Physiologic monitoring includes standard ASA monitors and an arterial
line. Echocardiography and EEG may also be employed.

33. Tetralogy of Fallot
 Concerns After Surgical Repair:
– Endocarditis prophylaxis
– Residual VSD secondary to incomplete closure
– Residual RV outflow tract obstruction
– Chronic pulmonary valve regurgitation results in a large volume
load on the right ventricle that can lead to cardiomegaly and
increased incidence of arrhythmias.
– Right ventriculotomy during the repair leads to scarring which
increases the risk of dysrhythmias and conduction abnormalities.
 Overall incidence of sudden death in TOF patients after
surgical repair is about 0.3%.

34. Single Ventricle Physiology
 Group of congenital heart disease
 Instead of 2 separate ventricles, there is
essentially 1 ventricle pumping blood to the
aortic and pulmonary artery
 Complete mixing of oxygenated and
deoxygenated blood occurs

35. Examples of Single Ventricle
Congenital Processes
 Tricuspid atresia: Narrowed pulmonary
arteries. At Birth patent PDA allows
sufficient oxygenation to occur, however
after closure of PDA cyanosis results
 Hypoplastic Left heart: Blood flow to the
body severely restricted. Once PDA close,
patient has profound shock

36. Tricuspid Atresia

37. Hypoplastic Left Heart

38. Treatment of Tricuspid Atresia
 Blalock-Taussig shunt placed to form a
conduit between pulmonary artery and aorta
to maintain oxygenation
 Prior to surgery, maintain PDA patency

39. Treatment of Hypoplastic Left Heart
 Keep PDA patent prior to procedure to
prevent patient from going into shock
 Aorta is formed from the base of the
pulmonary artery and narrowed aorta
 Modified Blalock-Taussig shunt formed to
maintain adequate oxygenation

40. Long Term outlook of Single Ventricle
 Activity limitations
 Prophylactic antibiotics prior to surgery and
dental work
 Life-long checkups and medications
 Prone to rhythm disturbances, fluid
retention, and increased risk of CHF

41. Anesthetic Considerations for Non-Cardiac
Surgery in Patients with Congential Heart
Disease
 Has the heart defect been corrected?
 If so, what kind of follow-up has the patient
had?
 Should the surgery be done at a specialized
center with expertise in congential heart
disease?
 Will a cardiology consult be needed to help
plan the anesthetic management?

42. Anesthetic Considerations for Non-Cardiac
Surgery in Patients with Congential Heart
Disease II
 Pre-op the patient as you would any other
with special emphasis on the heart
 What is the patient’s functional capacity?
 Any recent echos or EKGs?
 Patients with a history of ASD or VSD
repairs are prone to arrhythmias and
endocarditis