Anesthesia for Congenital Heart

Disease
ANDIKA A. L. W.
Introduction
Appropriate to distinguish between:

Congenital cardiac surgery
Non-cardiac surgery for patients with
congenital cardiac disease
Congenital Cardiac Surgery
General Principles:

Pre-operative management
Intra-operative Management
Cardiopulmonary bypass
Deep hypothermic arrest
Congenital Cardiac Surgery
Specific Considerations:

Simple procedures
Complex procedures
Closed procedures
Congenital Cardiac Surgery
Selected Specific Conditions/Procedures:
Patent Ductus (PDA)
Coarctation of the Aorta
Tricuspid atresia (TA)
Tetralogy of Fallot/Pulmonary Atresia
Transposition of the great vessels (TGV)
Hypoplastic Left Heart Syndrome (HLHS)
Congenital Cardiac Surgery
Preoperative Management
Preoperative evaluation
Wide spectrum of disease
Simple ASD to severe life-threatening HLHS
Remember psychological factors esp. family
Team oriented approach
Laboratory data ranges from minimal to very
extensive
Congenital Cardiac Surgery
Laboratory data
Cyanosis leads to polycythemia
May consider phlebotomy esp if no CPB
Leads to coagulation problems
Anemia may be relative and need transfusion
Newborn infant has immature systems
including renal/hepatic/coagulation
Hypoglycemia is much more common
Congenital Cardiac Surgery
Laboratory data:
Cardiac catheterization
Anatomic diagnosis
Saturation data shunt analysis
Pressure data gradients/diastolic function
Angiographic data systolic function/flow patterns
Prior surgery delineated line placement
Interventions in cath. Lab e.g. septoplasty or trial
of 100% oxygen
Congenital Cardiac Surgery
Premedication
Very varied practices
< 6 months 9
Atropine ( 0.01-0.02 mg/kg IM)
6-12 months
Pentobarbital (2-4mg/kg) po
Atropine and morphine (0.1-0.15 mg/kgIM)
> 12 months
Scopolamine (.015mg/kg IM up to .04mg max) or
morphine or midazolam (0.3 1.0 mg/kg), ketamine
(3mg/kg IM)
combinations are common

Congenital Cardiac Surgery
Preoperative medication
D/C diuretics and digoxin unless heart failure is
poorly controlled or digoxin is being used
primarily for rhythm
Continue inotropes
Continue prostaglandin infusions
Congenital Cardiac Surgery
Intraoperative Management
Physiologic Monitoring
Again very varied depending on the case
ECG, SpO2 x 2, ETCO2, precordial stethoscope,
NIBP are standard
A-lines +/- CVP (Atrial line placed intraop often
more valuable)
TEE
Congenital Cardiac Surgery
Intraoperative Management
Induction and Maintenance
Titrate to effect
Very dependent on age and cardiac reserve
Good cardiac reserve inhalational or IV
Neonates opiate-relaxant technique
Beware of PVR changes with inhalational induction
due to changes in PaO2, PaCO2, intrathoracic
pressure
Congenital Cardiac Surgery
Cardiopulmonary Bypass
Differences from adult
Lower temperatures (15-20 degrees C)
Lower perfusion pressure (20-30mmHg)
Very significant hemodilution (3-15 times greater)
Pump flows range from 200ml/kg/min to zero!
Different blood pH management (alpha-stat vs pH
stat)
Tendency to hypoglycemia
Cannula placement is much more critical

Congenital Cardiac Surgery
Deep hypothermic circulatory arrest
(DHCA)
Neonates and small infant usually < 10 kg
Oxygen consumption falls 2-2.5 times per 10
degree fall in temperature
Allows more controlled complex surgery in a
bloodless field
Often total CPB time is actually shortened by
this technique
Congenital Cardiac Surgery
Weaning from CPB
Heart assessed by direct visualization and right
or left atrial filling pressure, central cannula or
TEE
Pulse oximetry is also very helpful
Problems weaning are due to:
Inadequate repair,
pulmonary hypertension
And/or left or right ventricular dysfunction


Congenital Cardiac Surgery
Weaning from CPB
Problems weaning diagnosed by
Intraoperative cardiac catheterization
Echo-doppler
Leaving the operating room before correcting
the problem leads to a significant INCREASE
in morbidity
Congenital Cardiac Surgery
Anesthesia for simple open heart
procedures
Relatively straightforward repair
Uncomplicated hemodynamics
Uneventful post-op course anticipated
Examples:
ASD, VSD and some case of Tetralogy of Fallot
Usually involve CPB, sometimes DHCA
Congenital Cardiac Surgery
Anesthesia for simple procedures:
Inhalation induction usually well tolerated but
delayed if significant Right to Left shunt
Have agents available to maintain SVR
(phenylephrine) and reduce PVR (oxygen,
halothane and opioid)
Beware increases in PVR if high RVOT
obstruction
Congenital Cardiac Surgery
Anesthesia for simple procedures
Right atriotomy usually OK for most ASDs and
some membranous VSDs
Some VSDs need a ventricular incision which
may lead to
AV node dysfunction
mechanical ventricular dysfunction
Simple Fallots involve VSD repair and some
surgery to RVOT/pulmonic valve
Congenital Cardiac Surgery
Anesthesia for Complex Procedures:
Except for Left to Right shunt repairs and
uncomplicated Tetralogy, most procedures are
considered complex
Multiple defects
Complicated hemodynamics
Inhalation induction after pre medication still usual
technique unless very compromised child
Nasotracheal intubation often preferred
Arterial line placed
Congenital Cardiac Surgery
Anesthesia for closed heart operations,
performed without CPB
Corrective
PDA ligation
aortic coarctation repair
Non-corrective
Pulmonary banding
Blalock-Taussig shunt
Balloon Atrial Septostomies (Rashkind procedure)
(perfomed in the catheterization lab)

Congenital Cardiac Surgery
PATENT DUCTUS ARTERIOSUS
1/8000 live births, associated with prematurity
and female predominance of approx 3:1
Left to right shunt causes pulmonary edema
Occasionally right to left cause lower body
cyanosis
SpO2 probe on Right hand and lower limb
Confirms correct vessel ligated
Vagal reflex is pronounced by lung traction
Antibiotics required to prevent endocarditis

Congenital Cardiac Surgery
PATENT DUCTUS ARTERIOSUS
Usually left thoracotomy or thoracoscopy,
sometimes procedure performed in the NICU,
Lung retraction causes hypoxemia/hypercarbia
Air embolism, massive hemorrhage and
recurrent laryngeal nerve damage are possible
complications
LV overload can occur post-correction
Needs intercostal blocks or epidural for pain
Congenital Cardiac Surgery
COARCTATION OF THE AORTA
1/12,000 Male:female 2-5:1
Associated mitral and aortic valve disease
When severe, systemic perfusion depends on
right to left shunt across the PDA
Upper body hypertension may be severe
Perioperative paraplegia in 0.5%



Congenital Cardiac Surgery
COARCTATION OF THE AORTA
Left thoracotomy, may need DLT
Right radial a-line and lower NIBP or a-line
May need PGE
1
to keep open ductus arteriosus
Allow upper body hypertension on X-clamp
Nitroprusside may be necessary if pressure too
high but beware distal hypoperfusion and cord
ischemia
Congenital Cardiac Surgery
Tricuspid atresia
1/10,000 live births, third most common
cyanotic congenital heart disease
No connection between RA and RV
Maybe associated with transposition (TGA)
Obligatory flow through a PFO or ASD
If these are restricted then there is systemic
venous congestion
Congenital Cardiac Surgery
Tricuspid atresia
Palliative and definitive procedures are
applicable to any patient with univentricular
physiology
The single ventricle always becomes the
systemic ventricle
Initial palliative procedures either increase or
decrease pulmonary flow depending on the
lesions.
Congenital Cardiac Surgery
Tricuspid atresia
More than 70% patients are severely cyanosed
due to inadequate pulmonary flow through the
PDA
Need a systemic to pulmonary shunt
Variety used, most common Blalock-Taussig, this
RSC to RPA direct or via Gortex
Where pulmonary flow is high the PA is
banded
Assessment of RV function is important here
Congenital Cardiac Surgery
Tricuspid atresia
Definitive repair leads to a cavopulmonary
anastamosis (Fontan) and this is sometimes two
staged, i.e. Hemi-Fontan or Bidirectional Glenn
being the intermediary stage.
Patients present for BT shunt often on PGE1
Meticulous airway management is key to
maintain flow balances
Congenital Cardiac Surgery
Tetralogy of Fallot
1/5000 live births, risks of Tet Spells
Mortality in repair approx. 6%
Anatomy
RVOT Obstruction and RVH
Infundibular narrowing
Pulmonary stenosis and pulmonary hypoplasia
VSD (single or multiple)
Overriding aorta
LAD arises from RCA in 5% cases

Congenital Cardiac Surgery
Tetralogy of Fallot
Perioperative concerns
Increase in PVR or decrease in SVR leading to
Right to Left shunt
Tet Spells pre induction (crying/anxiety)
Polycythemia and bleeding
Air embolus
RV failure



Congenital Cardiac Surgery
Tetralogy of Fallot
Preoperative Preparation
Heavy premedication
Consider IM ketamine or inhalation induction
but get rapid control of airway.
Keep SVR up and PVR down, maintain heart
rate
Intraoperative TEE


Congenital Cardiac Surgery
Tetralogy of Fallot
Weaning from CPB, ratio RV:LV pressure
should be < 0.8
May need to keep PVR low with NTG,
milrinone, dobutamine phentolamine, PGE
1
May need RV inotrope post op
May need temporary pacing wire
Transposition of the Great Vessels
>5% congenital cardiac defects
Associated with VSD and LV outflow
obstruction
Without intervention, V. High mortality, 30%
1
st
week, 45% 1
st
mo. 90% 1
st
year.
Pre-op ductal patency may be life-sustaining
Congenital Cardiac Surgery
Congenital Cardiac Surgery
Transposition of the Great Vessels
Usual treatment is PGE
1
followed by a balloon
atrial septostomy (Rashkind) then either an
arterial switch (Jatene) or (often older child) an
atrial switch (Mustard)
Older child will be polycythemic, CHF is
frequently present
CVA is possible due to poor perfusion,
coagulopathy and hyperviscosity


Congenital Cardiac Surgery
Transposition of the Great Vessels
Periop
Maintain Cardiac output with adequate HR
Continue PGE
1
Reduce PVR and maintain SVR
Opioid/pancuronium technique
Blood loss may be significant

Congenital Cardiac Surgery
Transposition of the Great Vessels
Problems and concerns:
Atrial switch
Venous obstruction with low CO or SVC syndrome
Wide specturm of dysrhymias
RV dysfunction if Right ventriculostomy
Arterial switch
Ischemia due to kinking of reimplanted vessels or air
Inadequate LV function due to ischemia or low LV mass

Congenital Cardiac Surgery
Hypoplastic Left Heart Syndrome
Anatomy
Aortic atresia and LV and mitral hypoplasia
Systemic blood flow occurs across a PDA
Pathophysiology
As ductus closes there is severe systemic
hypoperfusion and acidosis

Congenital Cardiac Surgery
Hypoplastic Left Heart Syndrome
Two options:
Cardiac transplantation
2 or 3 stage procedure:
New aorta created from the pulmonary artery
Atrial defect is created to completely mix blood
Pulmonary flow improved by shunt e.g. BT
Later
Fontan (+/-preceded by a hemi Fontan)

Congenital Cardiac Surgery
Hypoplastic Left Heart Syndrome (HLHS)
Anesthetic Management
Maintain HR, preload and PGE
1
Balance SVR and PVR
Avoid too high PaO
2
May need CO
2
to avoid pulmonary over perfusion
and hence systemic hypo perfusion
Inotropic support may be necessary to support the
ventricular
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Congenital cardiac abnormalities are in 1%
live births
Rapid growth in corrective and palliative
procedures
Increasingly likely that anesthesiologists
will encounter these cases coming for non-
cardiac surgery
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Overview
Spectrum of disease
Management algorithms
Anatomy and implications of diseases
Cardiovascular factors
Antibiotics and anticoagulation
Specific problems
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Specific problems:
The cyanosed neonate
Tetralogy of Fallot
Obstetrics and congenital heart disease
Patients with a single ventricle
Recipients of a heart transplant
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Spectrum of disease:
Congenital heart disease NOT YET treated
Surgically corrected and symptom-free
Surgically corrected but with residual problems
Surgically palliated but stable
Surgically palliated but still with severe
symptoms or new problems
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Questions to ask:
Should the patient be referred for specialist
cardiology service before surgery?
Is entire procedure more suited to a specialist
center?
If we decide to proceed or are forced by an
emergent event to proceed what do we need to
know?
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Specific lesions, anatomic/pathophysiologic
considerations
VSD
L-R shunts and pulmonary overload pre-correction
Endocarditis and arrhythmias post-correction
ASD
L-R shunts and pulmonary overload pre-correction
Potential for paradoxical air embolus
Endocarditis and arrhythmias post-correction


Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
PDA
Late problems rare once corrected
Atrioventricular septal defect
Common in Downs syndrome
Can cause heart failure and pulmonary HTN
Mitral regurgitation may persist after correction
Endocarditis and arrhythmias post-correction


Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Hypoplastic Left Heart Syndrome
Before surgery pulmonary circulation needs a
patent duct
Three stage palliative surgery lead to single
ventricle and pulmonary flow through
cavopulmonary connections
Palliation may lead to heart failure and
arrhythmias
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Transposition of Great Vessels
Balloon septoplasty in first few hours of life
Arterial/atrial switch performed depending age
Residual risks of endocarditis, arrhythmias and
right ventricular (systemic) failure.


Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Pulmonary atresia
Treatment and outcome depend on a VSD
Repeat palliations and residual cyanosis
possible
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Tetralogy of Fallot
Variable cyanosis with potential for
hypercyanotic spells
Corrected by VSD closure and relief of RVOT
obstruction
Damage to pulmonary valve may leads to RV
failure.
Residual arrhythmia and endocarditis risk
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Cardiovascular impairment
Cyanosis
Indicates persistent abnormality
Associated with polycythemia altered hemostasis
Ensure hydration, maintain SVR and reduce PVR
Use high FiO2
Avoid sudden increase in oxygen requirement
Meticulous removal of air
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Cardiovascular impairment
Pulmonary disease
Associated pulmonary lesions, vascular rings,
compression form shunts, phrenic or RLN damage
Rising PVR can eventual lead to a Rt to Lt shunt
through an ASD or VSD, Eisenmengers syndrome
End-tidal CO
2
will frequently underestimate PaCO
2

due to reduced pulmonary flow and increased dead
space
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Cardiovascular impairment
Cardiac failure
Elective surgery should be postponed
In emergent situation, invasive monitoring is
mandatory and usually IPPV should be helpful
except where there are cavopulmonary shunts that
cause passive blood flow to the lungs
Arrhythmias
Common problems post correction
SVT, VT and complete heart block all seen

Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Antibiotics and anticoagulation
Endocarditis risk has to be assumed in all but
the most simple congenital cardiac lesions
especially if uncorrected
Generally speaking anticoagulated patients
should be switched from coumadin to heparin
closer to the time of surgery and then d/c
perioperatively
Emergent surgery, on balance, should call for
reversal of anticoagulation
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
The Cyanosed Neonate
Ductus closes functionally within 6hrs of birth
so ductal-dependent lesions will present early
They will need to have the duct opened
pharmacologically with prostaglandin
The neonate will deteriorate rapidly and
ventilation and transfer to a specialist center is
required
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Tetralogy of Fallot
VSD with RVOT obstruction
May live to adulthood, most corrected before
Principal sign is cyanosis
Hypercyanotic episodes (Tet Spells) occur when
child is distressed due to increased catecholamines
causing RVOT spasm and increased Rt to Lt shunt
Patient squatting or femoral pressure tend to
alleviate
Other therapy includes increasing SVR, fluid bolus,
morphine and Beta-blockers
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Obstetric intervention in the presence of
congenital heart disease
Maternal mortality is up to 30% if there is
pulmonary hypertension
If cyanotic lesions are present, 50% women
will suffer functional deterioration
Balancing SVR and PVR is key
GA for Cesarean section may be better option
for fixed cardiac output otherwise epidural
Anesthesia for Non-Cardiac Surgery in
Patients with Congenital Heart Disease
Patient with single ventricle
HLHS or pulmonary atresia with intact septum
Shunts inevitably cause some degree of
cyanosis (eg BT shunt)
As child grows pulmonary flow inadequate and
cavopulmonary shunts are needed (Fontan)
Spontaneous (negative pressure) ventilation
Morphology of ventricle determines outcome
Arrhythmias and ventricular failure are real
risks