Congenital Heart Disease
Greg Gordon MD
American Society of Dentist Anesthesiologists
Baltimore, MD, May 3, 2012
Training for Career in
Pediatric Cardiac Anesthesia
Specific Fellowship: Rare
Suggested training (US & UK):
• Pediatric Anesthesia: 12 months
• Adult Cardiac Anesthesia: 6 months
• Pediatric Cardiac Anesthesia: 6 months
• Pediatric Critical Care: 6 months
Baum V & De Souza DG. Pediatric Anesthesia 17:407, 2007
White MC & Murphy TWG. Pediatric Anesthesia 17:421, 2007
Children & adults scheduled
for dental or oral surgery
and known to have CHD
Preop heart murmur:
Is it CHD?
Adults with CHD in US today
1,500,000
Growing 2% per year
Cahalan MK. Anesthetic Management of Patients with Heart Disease.
IARS 2003 Review Course Lectures
Andropolous, D. Anesthesia for the Patient with Congenital Heart Disease
For Noncardiac Surgery. ASA Refresher Course Lectures 2011
3 y/o with TOF
s/p right BTS
For dental restorations
•Turns blue with crying
•Scheduled to undergo cardiac repair
in 3 months
•SpO2 93
•Systolic ejection murmur
•Slight clubbing of fingers
•Hct 52
Tammy
5 year-old for dental work
Systolic murmur
VSD
Victor
Needs surgical closure
Cardiologist recommended dental restorations first
11 y/o with tricuspid atresia
s/p Fontan procedure
For lengthy oral surgery
with possible large blood loss
•Temporary BTS at age 3 weeks
•Modified Fontan at age 3 years
•Meds: digoxin, captopril
•SpO2 88 on RA, 98 in O2
•P 67, BP 99/42
•First degree AV block
Fran
26 y/o with D-TGA
s/p Mustard in infancy
Dental restorations
Developmental delay
Pacemaker
Travis
4 y/o D-TGA
s/p Jatene in infancy
Dental restorations
Very active
Keeps up with peers
Never any cyanosis
Tracy
Objectives
Participants will be able to more intelligently discuss:
• Newborn and infant heart and lungs
• Initial evaluation the child’s heart
• Pathophysiology of selected CHDs
• Anesthetic implications of CHD
Pediatric Anesthesia
Congenital Heart Disease
Lesson
Presentation
Quiz
greggordon.org
Fetal Circulation
Placenta (oxygenation) ->
Umbilical vein ->
Ductus venosus (liver bypass) –>
IVC ->
Foramen ovale (RV bypass) ->
Left atruim ->
Left ventricle –>
Ascending aorta (brain) ->
SVC ->
Right atrium ->
Right ventricle ->
Main pulmonary artery ->
Ductus arteriosus (lung bypass) ->
Descending aorta ->
Placenta
The Newborn Heart
Foramen Ovale
Functional closure first hours as LAP > RAP
Probe-patent
50% of 5-year-olds
25% of 20-year-olds
Paradoxical embolus
The Newborn Heart
Ventricular tissue
•Fewer myocytes
•Greater proportion of connective tissue
•Relative RVH
So:
•Decreased compliance
•More sensitive to preload
The Newborn Heart
•Near peak of Starling curve
•Stroke volume relatively fixed
Normally near peak of Starling curve
•C.O. relatively
heart
rate dependent
Stroke volume
relatively
fixed
C.O. relatively heart rate dependent
The Newborn Heart
++
Newborn myocardium derives relatively
high fraction of activator Ca from the
extracellular pool, so
Beware Ca channel blockers
The Preterm Infant Heart
More sensitive to depressant effects of inhaled agents
Decreased response to catecholamines
Relatively high PVR persists
Pulmonary vasculature more sensitive to vasoconstriction by:
Hypoxia
Acidosis
Hypercarbia
CHD Pearl
murmur in newborn =
benign disease
Initial evaluation of child’s heart
History:
To determine
Level of function
1.Well compensated with
no limitations
2. Some limitations
3. Poorly compensated with
severe limitations
CHF and/or cyanosis
Initial evaluation of child’s heart
History - cyanosis
•Turn blue?
•At rest?
•When crying?
•Passes out?
•Stops playing and squats
Initial evaluation of child’s heart
History - CHF
Run around like crazy?
Like sibs?
Or tends to be quiet, slow?
Infant – feeding behavior:
Slow to finish bottle?
Sweats when nursing?
Eyes puffy in the morning?
Initial evaluation of child’s heart
Physical exam
•Listen to heart first when/if infant quiet
(warm stethoscope)
•First concentrate on S1 and especially S2
Louder than normal?
Split normally?
•Systolic murmur:
Starts after or obscures S1?
•Diastolic murmur?
•Widely radiating murmur?
•Palpate liver
•BP in arm and leg
•Tongue - cyanosis
CHD Pearl
Sudden CHF in ‘healthy’ 10-day-old =
complicated coarct
General Approach to CHD Patient
1. Define cardiovascular pathology
2. Predict pathophysiology
3. Determine hemodynamic goals
4. Anticipate emergency treatments
Cahalan MK. Anesthetic Management of Patients with Heart Disease.
IARS 2003 Review Course Lectures
Recent Cardiologist Evaluation Needed?
Completely corrected,
Well compensated and stable:
Probably not
Complex and/or poorly compensated;
Cyanotic and/or single ventricle:
YES: Evaluation & ECHO within 3-6 mos
Don’t worry
Almost any anesthetic technic
may be used in any CHD patient
if
the anesthesiologist understands
•the pathophysiology of the lesion and
•the pharmacology of the drugs employed.
Normal Neonate
SVC
RA
m=2
1 week
60
PV
99
LA
m=4
65
RV
LV
30/3
MPA
30/12 m=18
65
65
80/5
99
99
Ao
80/50
Some basic definitions
physiologic
L to R shunt =
lungs to lungs shunt
Blood that is returning to the heart
from the lungs is recirculated back
to the lungs without going out to the
rest of the body.
Some basic definitions
physiologic
R to L shunt =
body to body shunt
Blood that is returning to the heart
from the body is recirculated directly
back to the body without going to the
lungs to be oxygenated.
Some basic definitions
effective pulmonary
blood flow=
body to lungs flow
Blood that is returning to the heart
from the body that is actually directed
to the lungs to be oxygenated.
Some basic definitions
Nonrestrictive VSD
VSD large enough that
pressure equalizes in the two ventricles
(no pressure gradient can be maintained)
LV pressure = RV pressure
Premature
1 week old
28 weeks EGA
SVC
RA
PV
LA
96
65
RV
LV
65/10
65/12
65
MPA
65/30
96
Ao
PDA
80
92
65/25
to R arm
& head
To L arm
MHMC PDA ligation
CHD Pearl
blue newborn +
no airway or breathing problem +
quiet heart =
decreased PBF lesion (TOF)
Tetralogy Of Fallot
Most common cyanotic lesion
NB: cyanosis plus quiet heart
Diminished pulmonary blood flow
Ao ejection click
Hypercyanotic “tet” spells
tachypnea, pallor, LOC, less murmur
Tammy
3 y/o with TOF s/p right BTS
1. Define cardiovascular pathology
2. Predict pathophysiology
3. Determine hemodynamic goals
Tammy
4. Anticipate emergency treatments
Tetralogy Of Fallot
Essentially a duality:
1. severe RVOT obstruction plus
2. nonrestrictive VSD
With anatomic consequences:
1. RVH
2. Overriding aorta
And physiologic consequences
1. R to L shunt
2. Diminished pulmonary blood flow
Tammy
Tetralogy of Fallot
SVC
40
96
RA
LA
RV
LV
m=5
m=4
85/6
MPA
15/10
40
40
85/5
85
50
Ao
85/45
Tetralogy Of Fallot
s/p
right BTS?
Blalock-Taussig Shunt
Tammy
Thomas-Blalock-Taussig Shunt
Vivien Thomas
Alfred Blalock
Helen Taussig
Vivien Thomas, Partners of the Heart, 1998 and
Something the Lord Made - Best Made-for-TV Movie, 2004
Thomas-Blalock-Tuassig
Dr. Blalock does the Blalock
(Johns Hopkins)
Systemic to Pulmonary Shunts
Tetralogy Of Fallot - Goals
Maintain adequate tissue oxygenation
1. Avoid increasing O2 demand
2.Maintain SVR, systemic BP
3.Minimize PVR
Maintain good hydration,
especially if polycythemic
Oral premed/induction
midazolam + ketamine
(0.6 mg/kg + 6 mg/kg)
Tammy
Tetralogy Of Fallot - Goals
Minimize PVR
Oxygen to FIO2 = 1
Mild hyperventilation
PaCO2 low 30’s
pH 7.45
Adequate anesthesia
Adequate analgesia
Normothermia, warm
Nitric oxide
Tammy
Tetralogy Of Fallot - Goals
Maintain SVR
Intravascular volume
Well hydrated
IV bolus prn
Maintain BP
Tammy
ketamine
phenylephrine
Free written board answer:
Speed of induction:
R->L shunt
• Inhalational: slower
• IV: faster
L->R shunt
• Inhalational: maybe faster
• IV: slower
But probably not clinically important
Tanner et al. Anesth Analg 64:101, 1985
Beware:
blunted chemoreceptor response to
Tammy
hypoxemia
Beware:
VD:VT may be 0.6
Tammy
And increase with
•start of mechanical ventilation
•too much PEEP
•hypovolemia
ETCO2 << PaCO2
VD/VT = (PaCO2 – ETCO2)/PaCO2
Tetralogy Of Fallot
Minimize R->L Shunt
MAINTAIN
SVR
•ketamine
•phenylephrine
Tammy
Tetralogy Of Fallot
Minimize RVOT obst & PVR
•oxygen
•beta blocker ready
Maybe:
•nitroglycerin
•phentolamine
•tolazoline
•prostaglandin E1
•nitric oxide
Tammy
Tetralogy Of Fallot
And of course:
•No Air in lines
Maybe no N2O
and
infective
endocarditis
prophylaxis
Tammy
Infective Endocarditis Prophylaxis
Infective endocarditis prophylaxis
for dental procedures is reasonable
only for patients with underlying
cardiac conditions associated with
the highest risk of adverse outcome
from infective endocarditis.
Wilson W, Taubert KA et al. AHA Guidelines. Prevention of
Infective Endocarditis. Circulation 116:1736-54, 2007
Infective Endocarditis Prophylaxis
Recommended
Unrepaired cyanotic CHD,
including palliative
shunts and conduits.
Circulation 116:1736, 2007
Infective Endocarditis Prophylaxis
Recommended
CHD completely repaired with
prosthetic material or device
less than 6 months ago.
Circulation 116:1736, 2007
Infective Endocarditis Prophylaxis
Recommended
Repaired CHD with
residual defect(s) at or near
a prosthetic patch or device.
Circulation 116:1736, 2007
Infective Endocarditis Prophylaxis
Recommended
Prosthetic material in a valve.
Previous infective endocarditis.
Valvulopathy after transplant.
Circulation 116:1736, 2007
Infective Endocarditis Prophylaxis
Recommended
For patients with the above conditions,
prophylaxis is reasonable for
all dental procedures that involve
manipulation of gingival tissue or
the apical region of teeth or
perforation of the oral mucosa.
Circulation 116:1736, 2007
Infectious Endocarditis Prophylaxis
NOT Recommended
Any form of CHD not listed above
Local injection -> noninfected tissue
Shedding deciduous teeth
Bleeding/trauma to lips, oral mucosa
Circulation 116:1736, 2007
Tetralogy Of Fallot
infective endocarditis prophylaxis
and
maintain
SVR
Tammy
Tetralogy Of Fallot
Treatment of Tet Spell
• 100% O2
• knee-chest position
• morphine 0.05-0.1 mg/kg
• crystalloid 15-30 ml/kg
• phenylephrine to increase systolic BP 20-40 mmHg
• beta blockade: propranolol 0.1 mg/kg or
esmolol 0.5 mg/kg and 50-300 mcg/kg/min
• ABG: NaHCO3 if necessary
• ECMO/surgery
DiNardo JA et al. in Davis PJ et al. Smith’s Anesthesia for Infants and Children, 8th ed. 2011
Schedule case early in the day
•Less fasting dehydration
•Less time of stress
•More time to monitor postop
•More support available
•Less team turnover
Schedule case WHERE?
•Well-compensated, no limitations,
not-complex:
Ambulatory center may be OK
•Not well-compensated, complex:
Center with CHD expertise &
backup available
5 year-old for dental work
Systolic murmur
VSD
Victor
Needs surgical closure
Cardiologist recommended dental restorations first
Newborn VSD
Most common lesion
2/3rds close spontaneously
Small VSD
Definite murmur
Will probably close
Large VSD
No murmur
No problems
Home with Mom
CHF symptoms by 4-8 weeks
VSD
nonrestrictive
SVC
98
60
96
RA
m=6
90/8
90/35
m=12
80
RV
MPA
LA
LV
90/10
94
88
94
Ao
90/60
Nonrestrictive VSD
L->R shunt
Pulmonary to System Flow Ratio
QP:QS =
SaO
2 – SvO2
__________
SpvO2 – SpaO2
=
94 - 60
_______
98 - 88
=
3.4:1
Victor
Nonrestrictive VSD - Goals
Maintain PVR
Normal ventilation
(paCO2 = 40’s)
FIO2 < 1
Lower SVR better
Major inhalational agents
Propofol, thiopental
Victor
Proper management of the physiologic
abnormalities is more important
than the choice of specific anesthetic
and pharmacologic approaches.
Nonrestrictive VSD - Goals
Of course:
•No Air in lines
Maybe no N2O
infective
endocarditis
prophylaxis?
Victor
NO longer recommended
Wilson W, Taubert KA et al. AHA Guidelines. Prevention of
Infective Endocarditis. Circulation 116:1736-54, 2007
Unrepaired nonrestrictive VSD ->
1. PVOD developing
2. Less L->R shunt
3. Less CHF
4. Less murmur
5. PVOD irreversible
6. R-L shunt
7. Less PBF
8. More cyanosis
Victor
Eisenmenger syndrome
11 y/o with tricuspid atresia
s/p Fontan procedure
Oral surgery, big blood loss?
•Temporary BTS at age 3 weeks
•Modified Fontan at age 3 years
•Meds: digoxin, captopril
•SpO2 88 on RA, 98 in O2
•P 67, BP 99/42
•First degree AV block
Fran
Tricuspid Atresia
3rd most common cyanotic CHD
1. TOF
2. TGA
Type IB most common
•Small VSD (and RV)
•PS
20% extracardiac abnormalities
•GI
•Musculoskeletal
Cyanosis
•Mixing in LA
•Decreased PBF
•Spells
Fran
Fontan procedure
Indicated to palliate:
Tricuspid atresia
Hypoplastic left heart syndrome
Double outlet right ventricle
Double inlet left ventricle
Unbalanced AV septal defect
Fontan physiology
Two defining features:
1. Single systemic ventricle
2. Pulmonary blood flow:
without pump!
Fontan procedure
Three main versions
Atriopulmonary connection (the original)
Total cavopulmonary connection (TCPC):
Intracardiac (lateral tunnel)
Extracardiac
Two stages:
Bidirectional Glenn shunt
Fontan completion
Modified
Bidirectional
Modified
Collaborate with cardiologist
Clarify
History
Pathophysiology
Risks
Status best possible?
Explain recent studies
Age 5 years
16/10
16/12
88/6
11 y/o with tricuspid atresia s/p Fontan procedure
Potential problems during surgery
Hypoxemia
1. Hypovolemia
2. Low PBF
CHF
1. Volume shifts
2. Anemia
3. Hypertension
Paradoxical embolus
Thrombosis
Vena cavae
RA
Pulmonary arteries
Fran
11 y/o with tricuspid atresia s/p Fontan procedure
Goals during surgery
Monitor RA pressure
•RA catheter
•Maintain starting pressure
Maintain systemic BP near baseline
Minimize myocardial depressants
NO AIR IN LINES
No N2O
Relatively high FIO2
Normal Hct
IE prophylaxis
Fran
11 y/o with tricuspid atresia s/p Fontan procedure
MAJOR GOAL
Maintain cardiac output and
transpulmonary gradient (TPG):
Adequate preload
Low PVR
Low intrathoracic pressure
Normal ventilation
Unobstructed PV return
Regular sinus rhythm
Low ventricular afterload
Normal ventricular funtion
Fran
Monitor RA Pressure
Right IJ?
16/10
Fran
16/12
88/6
CHD Pearl
blue newborn +
no airway or breathing problem +
hyperactive heart =
TGA
26 y/o with D-TGA
s/p Mustard in infancy
Dental restorations
Developmental delay
Pacemaker
Travis
TGA s/p Mustard
D-TGA, Transposition of the Great Arteries
Newborn: 75% no VSD
PGE1 to keep PDA
BAS prior to surgery
Travis
Older: Mustard or Senning
Younger: Jatene ASO
D-TGA
SVC
BAS
RA
RV
Ao
99
99
65
PDA
LA
LV
MPA
D-TGA
Mustard Procedure
SVC
PV
RV
Ao
LV
95
MPA
D-TGA + Mustard
RV systemic ventricle
RV failure
Tricuspid regurgitation
Ventricular arrhythmias
Sudden death
Atrial injury/scars
Atrial flutter/fib
Sick sinus syndrome
Travis
26 y/o TGA s/p Mustard, pacemaker
Poor RV function
Consider inotrope
Arrhythmias
Pacemaker
CIED practice advisory
Travis
CIED practice advisory
Preop:
What type CIED?
Pacer dependent?
Check function: interrogate device
EMI (e.g. Bovie) during procedure?
Reprogram to asynchronous mode?
Have backup pacing &
defibrillation equipment
immediately available
Practice Advisory for the Perioperative Management of Patients with Cardiac
Implantable Electronic Devices: Pacemakers and Implantable CardioverterDefibrillators. Anesthesiology 114:247-61, 2011
4 y/o D-TGA
s/p Jatene in infancy
For dental restorations
Very active
Keeps up with peers
Never any cyanosis
Tracy
D-TGA
SVC
RA
LA
RV
LV
Ao
MPA
D-TGA
SVC
RA
LA
RV
LV
MPA
Ao
4 y/o D-TGA s/p Jatene
Treat as normal, healthy child!
Tracy
Be happy!
For more cool stuff about CHD
check out the lesson and fun Quiz at
http://greggordon.org/edu/ped/chd1.htm
Now we can more intelligently discuss:
•Newborn and infant heart and lungs
• Initial evaluation the child’s heart
• Pathophysiology of selected CHD
• Anesthetic implications of CHD