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Basic concepts to Understand
Congenital Heart Disease and their
Repair
p
Basic concepts to Understand
Congenital Heart Disease and their
R
Repair
i
• No Relationships to disclose
FRANCISCO J. GENSINI, MD
Assistant Professor of Surgery
Division of Congenital Cardiac Surgery
University of Rochester, SUNY Health Science Center and CROUSE
Medical Center
Normal Cardiac Anatomyy
• 4 cavities
• 2 sides with 2
cavities each
• Interatrial and
interventricular
septa
• 2 Atrioventricular
valves
• 2 Semilunar
valves
Normal Intra cardiac Blood Flow
Pulmonary
valve
Right
Left
Aortic valve
2
1
Interatrial
Septum
Tricuspid
valve
4
3
Mitral
valve
Inter
ventricular
S t
Septum
Right Heart
Left Heart
Orienting the Heart
Aorta
BASE
Right Atrium
Pulmonary Artery
Left
L ft Pulmonary
P l
veins
Right
Ventricle
Left Anterior
descending
coronary (LAD)
Left Ventricle
APEX
Surgical
g
view of the Heart
Ascending
Aorta
Right
Atrium
Atri
m
Cardiopulmonary Bypass Circuit
M i
Main
Pulmonary
artery
Reservoir
Oxygenator
Right
Ventricle
Bubble
Filter
Arterial
pump head
Heat Exchanger
Oxygen
Suction pumps
Cardioplegia
pump
Cardiopulmonary
p
y Bypass
yp
p
pump
p in the OR
Bubble Filter
Diagnosis:
Trans thoracic
Echo
Suction pumps
Venous
Reservoir
Oxygenator
Cardioplegia
C
di l i
pump
Arterial pump
Transesophageal
p g
Echo
Other Diagnostic tests
Cardiac
MRI
Cardiac Catheterization
Congenital Heart Disease
Most common Congenital Cardiac defects
1. Ventricular Septal Defect (VSD) : 20% isolated
50% with associated anomalies
Increased
Pulmonary
Blood Flow
Decreased
Pulmonary
Blood Flow
Other type of
Lesions
2. Patent Foramen Ovale (PFO) : 30%
3. Atrial Septal Defect (ASD) : 15% isolated
35-50% with associated anomalies
• PDA
• AP Window
4. Tetralogy of Fallot (TOF) : 8-10%
5 T
5.
Transposition
iti off Great
G t Arteries
A t i (TGA) : 8-10%
8 10%
• ASD
6. PDA: 5-10% (higher in prematures).
• VSD
• AVSD
7 Coarctation of the Aorta: 5
7.
5-8%
8%
8. Hypoplastic Left Heart Syndrome (HLHS) : 5%
9 Atrioventricular Septal Defect (AVSD) : 3-4%
9.
3 4%
•
Truncus
•
TAPVC
•
10. TOF-Pulmonary Atresia –MAPCAs: 2%
TGA + VSD
•
TA type C
Congenital Heart Disease
Pulmonary artery Banding
ASD
Increased
Pulmonary
Blood Flow
VSD
Decreased
Pulmonary
Blood Flow
Other type of
Lesions
• PDA
• TOF
• DORV
• AP Window
• LVOTO
• ASD
• TOF + PA +
MAPCAs
• VSD
• PA + IVS
• HLHS
• AVSD
• PV stenosis
• ALCAPA
PDA
Palliative and Repair
procedures for
Increased Pulmonary
circulation
•
•
Truncus
•
TAPVC
TGA + VSD
•
TA type C
• TA type A
•TGA+VSD+PS
• CoAo
Palliative procedures to increase
Pulmonary blood flow
Cyanotic Heart defects
Venous Blood is reaching the systemic circulation
• Tetralogy of Fallot (TOF)
• Transposition of Great Arteries (TGA)
• Tricuspid Atresia (TA)
• Truncus Arteriosus
• Total Anomalous Pulmonary venous return (TAPVR)
• Hypoplastic Left Heart syndrome (HLHS)
Left-to-Right Shunting Lesion:
Definition
• Communication
C
i ti b
between
t
left
l ft heart
h t structure
t t
and
d right
i ht
heart structure allowing blood flow (shunting) from the
left heart to the right
g heart
• No obstruction to right heart blood flow
• 2 well-formed ventricles
Left-to-Right
Left
to Right Shunt Pathophysiology:
Pulmonary Vascular Resistance
Neonatal Period :
-
High PVR
-
No pressure gradient
-
Minimal shunt
-
Asymptomatic
-
Absent murmur
PVR starts to decrease for following
g days
y ((2-6 weeks))
Left-to-Right Shunting Lesion:
Pathophysiology
• Decreasing PVR
– Increased shunting
g with
pulmonary over-circulation and
Left-to-Right Shunting Lesion:
Long-term Effects
• Chronic Volume Overload
– Arrhythmia
y
a
– Systolic / Diastolic Dysfunction
volume load (CHF)
– Audible murmur
– Tachypnea
yp
/ dyspnea
y p
• Pulmonary Vascular Disease
• Eisenmenger
Eisenmenger’s
s Syndrome
– Failure to thrive
Left-to-Right
g Shunting
g Lesion:
Timing of Surgical Repair
• Technical feasibility
• Size
• Co-morbidity
C
bidit
•
•
•
•
Symptomatic state: Shunt volume
Potential for spontaneous closure
Potential for irreversible pulmonary vascular disease
Potential for or presence of related pathophysiology
• Endocarditis risk
• Prolapse aortic leaflet with VSD
Calculation of Shunt Volume:
Fi k P
Fick
Principle
i i l
Shunt Volume = Pulmonary Blood flow (Qp)
Systemic Blood flow (Qs)
Qp =
VO2
PV sat - PA sat
Qs =
VO2
Ao sat - MVO2
Calculation of Shunt Volume: Fick Principle
p
Qp =
Qs =
I di ti
Indications
ffor Surgery:
S
Qp/Qs
Q /Q
VO2
PV sat - PA sat
VO2
Ao sat -MVO2
Qp/Qs = Ao sat - MVO2
• < 1.5 :1
No surgery
g y
• 1.5 - 2 :1
Consider surgery
• > 2 :1
Needs Surgical repair
PV sat – PA sat
Estimation of Shunt Magnitude
1. Chamber dilatation
– ASD:
RA and RV
– VSD:
LA and LV
– PDA:
LA and LV
– AVCD:
All
2. Flow velocity
P l
Pulmonary
V
Vascular
l Di
Disease
• Compensatory response to increased pulmonary blood
flow
• Progressive process: medial hypertrophy and intimal
hyperplasia: initially reversible
•
Eventually irreversible
• Rate of progression variable
• Lesion
• Genetic factors
Irreversible Pulmonary Vascular Disease
• Complete AVCD
9 -12 months
• Large VSD
1-2 years
• Large PDA
1-2 years
• ASD
30 - 40 years
Sec ASD
VSD
Technical
ec ca
Feasibility
Uncomplicated
U
co p ca ed
Mild
d to
o
moderately
complicated
Mildly
dy
complicated
Complicated
Co
p ca ed
Symptomatic
Sy
po a c
state
Usua y
Usually
Asymptomatic
So e es
Sometimes
symptomatic
sy
po a c
symptomatic
Symptomatic
Sy
po a c
Spontaneous
closure
Rare
Common <1
year of age
Common in
preterm
neonates
None
Irreversible
Pulmonary
vascular
disease
30-40 years
1-2 years
1-2 years
9-12 months
Earlier in
Down’s
syndrome
Risk
Endocarditis
None
Moderate
Rare
Moderate
Any age if
Sxs
2-4 years if
ASx
Any age if
Sxs
< 6 months
Earlier if Sxs
Age for repair 2-3 y old
Surgical
S i lA
Approaches
h ffor R
Repair
i
PDA
AVSD
Left sided Thoracotomy For PDA and
Coarctation repair
Patent Ductus Arteriosus (PDA)
Coarctation of the aorta and Patent Ductus
Arteriosus (PDA)
Recurrent
Laryngeal
Nerve
PDA
Isthmus
Descending
Aorta
Coarctation of the aorta and Patent Ductus
Arteriosus (PDA)
Coarctation of the Aorta repair
Coarctation of the Aorta repair
Coarctation of the Aorta repair
Midli sternotomy
Midline
t
t
iincision
i i
U i th
Using
the sternal
t
l saw
Sternal spreader - Anterior mediastinal
contents
t t exposed
d
Sternum is open
Pericardium opened
p
– Heart exposed
p
Ascending
Aorta
Right
Atrium
Atri
m
Ligation of Ligamentum arteriosum
(previously
(
i
l d
ductus
t arteriosus)
t i
)
M i
Main
Pulmonary
artery
Aorta
Right
Ventricle
Pulmonary
artery
Arterial cannulation into ascending
A t
Aorta
Venous Cannulation into Superior vena
cava
Connecting SVC to CPB machine
Cannulation of Inferior vena cava
Left ventricular vent placed and
cardioplegia needle placed in
ascending aorta
Aortic cross clamp applied to stop the
heart
h t
Slushed ice applied
pp
on the heart
Right atrium is opened to access the
i id off th
inside
the h
heartt
Repair of ventricular septal defect with
G
Gore-tex
t patch
t h
Resection of infundibular muscle
b dl tto relieve
bundles
li
RVOT obstruction
b t ti
Suture closing
g an atrial septal
p defect
Closing
g the right
g atrial incision
Surgery completed – Aortic clamp
removed
d tto restart
t t heart
h t function
f
ti
Both venous cannulae and LV vent
have
h
b
been removed
d
Hemostasis confirmed –
Sternal wires placed
Sternum is closed – wires tucked in
Skin is closed – sterile dressing to be
applied
li d