Congenital Heart Disease

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


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
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
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 1
st
year
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
Ventricular Septal Defect III
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
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
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
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
IVCs entrances to the heart
Atrial Septal Defect III
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
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.
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
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
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
Eisenmengers Syndrome.
This results in flow of
desaturated blood to the lower
extremities.


Picture: www.lpch.org
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.
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

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.

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
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.
Interrupted Aortic Arch III
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
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
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
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
Tetralogy of Fallot
Anatomic Defects
Ventricular septal
defect
Overriding Aorta
Pulmonary artery
stenosis
Right ventricular
hypertrophy







Picture: www.lpch.org

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
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.
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
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.
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%.
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

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

Tricuspid Atresia
Hypoplastic Left Heart
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
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
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

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?
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 patients functional capacity?
Any recent echos or EKGs?
Patients with a history of ASD or VSD
repairs are prone to arrhythmias and
endocarditis