High level review of congenital heart lesions; recommendations for anesthetic management of adults with congenital heart disease for noncardiac surgery

1. Caring for the
Adult with
Congenital Hear t
Disease
S a r a h B e t h H a r t l a g e , M D M S

2. C o n g e n i t a l H e a r t D i s e a s e ( C H D ) :
L i f e l o n g c o n s e q u e n c e o f a
s t r u c t u r a l a b n o r m a l i t y o f t h e
h e a r t , w h i c h w a s p r e s e n t a t
b i r t h .

3. CHD is…
• Common
– The most common birth anomaly – 1 in 100 live births
– 40,000 infants with CHD born each year in the US
– 2-3 million adults living with CHD in the US
• Costly
– Annual direct hospital cost for CHD exceeds $6 billion
– Accounts for 15% of the total cost of all hospitalization for children and adolescents (age
20)
– One in three CHD children is on Medicaid
• Crucial
– There is no cure
– 25% require life-saving interventions in the first 6 months of life
– 1 year survival rate is 95%; 18 year survival rate is 85%
– Survivors require lifelong, complex, interdisciplinary medical care
Pediatric Congenital Heart Association, Section for Research and Advocacy

4. Anomaly Estimated Prevalence / 10000 Live Births
Left to Right Shunting Anomalies
Ventricular Septal Defects (all) 17.6 – 44.8
Atrial Septal Defects (all) 3.7 – 10.6
Atrioventricular Septal Defects (all) 2.4 – 4.0
Cyanotic Anomalies
Tetralogy of Fallot* 2.9 – 5.8
Transposition of the Great Arteries* 2.3 – 3.9
Truncus Arteriosus* 0.6 – 1.4
Total Anomalous Pulmonary Venous Return* 0.6 – 1.2
Tricuspid Atresia* 0.2 – 1.2
Ebstein’s Anomaly* 0.4 – 1.6
Single Ventricle Complex* 0.5 – 1.4
Heterotaxy Syndrome 0.8 – 1.7
Left Heart Obstructive Anomalies
Coarctation of the Aorta* 2.9 – 4.9
Valvular Aortic Stenosis 1.6 – 3.9
Hypoplastic Left Heart Syndrome* 1.5 – 2.8
Right Heart Obstructive Anomalies
Valvular Pulmonic Stenosis 3.6 – 8.4
Pulmonary Atresia* 0.8 – 1.5
Totals
All Critical CHD* 10.8 – 15.3
All CHD 60.2 – 105.7
CDC Center for Birth Defect
Surveillance

5. • Differental pulse oximetry screening for critical congenital heart disease is a part of
the required newborn screening in most states (including Kentucky)
• Many newborns with innocent murmurs are referred for cardiology follow up and
echocardiography
• Most cases of CHD are nonsurgical and many resolve as the child grows
Kentucky Cabinet for Health and Family Services

6. Brief Historical Perspective
1938
Dr. Robert Gross
performs the first
successful PDA closure
Dr. Helen Taussig and
Dr. Alfred Blalock
perform the first “Blue
Baby” pulmonary-to-
systemic shunt.
1944
1946
Drs. Crafoord and Nylin
performs the first
successful repair of
coarctation of the aorta
1973
Dr. Barratt-Boyes
combines CPB,
circulatory arrest, and
deep hypothermia,
making intracardiac
repair possible even on
very young infants.
Drs. Muller and
Dammann pioneer the
use of pulmonary
artery banding for
palliation of left-to-right
shunts.
1952
1954
Dr. C Walton Lillehei
performs the first
successful repair of
intracardiac repair
(VSD) using controlled
cross-circulation
Drs. Coceani and Olley
establish a protocol for
use of Prostaglandin E1
for duct-dependent
defects
1978
Dr. Mustard performs
the first successful
arterial switch
operation for correction
of TGA
1963
1983
First successful staged
repair of HLHS –
Norwood/Glenn/Fonta
n procedures in series.
Dr. Lock and Rao
advance ability of
transcatheter closure of
septal defects
1993
2016
Successful hybrid stage
I/II combination for
HLHS (followed by
Fontan).
Extracardiac Repair
/ Palliation Era Intracardiac Repair Era Stage Compression/
Minimally Invasive
Era
Freedom RM, Lock J, Bricker T. Pediatric Cardiology and Cardiovascular Surgery: 1950-2000. Circulation. 200 Nov;102:lv-58-lv-68.

7. Initial Successes
30 year follow-up on 106 patients
• Survival
– 8 deaths in first 10 years
– 12 deaths in years 11-20
– 1 death in years 21-30
• Causes of death: sudden cardiac death(5),
accidental (4), congestive heart failure (2),
reoperation (2), suicide (2), endocarditis (2),
other (4)
• Quality of life
– 58 attended postsecondary education
• 34 bachelor’s degrees, 5 master’s degrees,
2 MDs, 2 PhDs, 1 JD
– 40 successfully had progeny
• 6 children had cardiac anomalies
Lillehei CW, Varco RL, Cohen M, Warden HE, Gott VL, DeWall RA, Patton C, Moller JH. The first open
heart corrections of tetraology of Fallot. A 26-31 year follow-up of 106 patients. Annals of Surgery. 1986
Oct;204(4):490-502.

8. I d o n ’ t d o p e d i a t r i c s
o r c a r d i a c c a s e s … w h y
s h o u l d I c a r e ?

9. Knowledge and Attitudes of Anesthesia
Providers about Noncardiac Surgery in Adults
with Congenital Heart Disease
KNOWLEDGE ASSESSMENT
• 20 questions with answer choices “True”, “False”, “Don’t
Know”
• 1 point for correct answers, 0 points for incorrect or
Don’t Know
• Ranged from 0-19 with median 7
• Scores varied by fellowship training
– Cardiac – median 11, P = 0.005
– Pediatrics – median 12, P = 0.001
– No difference for critical care, obstetrics, regional, or
pain
• Scores varied proportional to frequency of performing
cardiopulmonary bypass cases, as well as to frequency
of caring for patients under 2 years of age
• No difference by gender or number of years removed
from residency
• Variance by topic – excellent responses RE Down
syndrome / AV canal, etc
ATTITUDE ASSESSMENT
• 14 questions with 10-point Likert Scale (“I feel…”)
• 10 multiple choice questions
– Situational (“I would…”)
– Demographic
• Likert scores were 5-6 for comfort managing complex
lesion ACHD patients, managing pacemaker/AICD
dependent patients, and using TEE to assess ACHD
patients
– Differed by frequency of performing
cardiopulmonary bypass cases (7-9; P = 0.001)
– No difference by other fellowships, gender,
experience, etc
• Comfort managing a parturient with ACHD were
much lower but not significant (1-4; P = 0.09)
• Willingness to proceed for elective noncardiac cases:
– Those without cardiac fellowship or frequent CPB
cases – 93% (simple lesion), 39% (moderate lesion),
13% (complex lesion)Maxwell BG, Williams GD, Ramamoorthy C. Knowledge and Attitudes of Anesthesia Providers about
Noncardiac Surgery in Adults with Congenital Heart Disease. Congenital Heart Disease. 2014

10. Perioperative Outcomes of Major
Noncardiac Surgery in Adults with
Congenital Heart Disease
• Matched cohort study using the Nationwide
Inpatient Sample database
– Study cohort 10,004 ACHD patients
– Matched cohort 37, 581 patients
– Code-based data definitions (Major Surgery
= Procedure Class 4 based on ICD-9)
– Exclusions: patients <18, cardiac surgery,
obstetrics, unspecified anomaly, missing
matching data
• ACHD admissions represent 0.11% of all
admissions from 2002-2009, with a
statistically significant increase over time (P <
0.001)
• Major noncardiac surgery represented 8.4%
of all ACHD admissions, with a statistically
significant increase over time (P < 0.001)Maxwell BG, Wong JK, Kin C, Lobato RL. Perioperative Outcomes of Major Noncardiac Surgery in
Adults with Congenital Heart Disease. Anesthesiology. 2013 Oct;119(4):762-769.

11. Perioperative Outcomes of Major
Noncardiac Surgery in Adults with
Congenital Heart Disease
Maxwell BG, Wong JK, Kin C, Lobato RL. Perioperative Outcomes of Major Noncardiac Surgery in
Adults with Congenital Heart Disease. Anesthesiology. 2013 Oct;119(4):762-769.

12. The Adult with CHD
• Patients with Adult Congenital Heart Disease (ACHD) should be viewed as a
continuum
• In nearly all cases, ACHD should be viewed as a systemic condition with associated
multiorgan dysfunction to varying degrees
s/p non-
anatomic
correction
s/p anatomic
correction
s/p palliation
procedure
unrepaired
Moderate DiseaseMild Disease Severe Disease
Cannesson M, Earing MG, Collange V, Kersten JR. Anesthesia for Noncardiac Surgery in Adults with
Congenital Heart Disease. Anesthesiology. 2009 Aug;111:432-40.

13. Longterm Sequelae of CHD
CARDIAC
• Pulmonary hypertension
• Ventricular dysfunction
• Dysrhythmia / conduction anomaly
• Residual shunt
• Valvular lesions
• Hypertension
• Aneurysms
EXTRACARDIAC
• Secondary erythrocytosis
• Cholelithiasis
• Nephrolithiasis
• Developmental delays
• CNS abnormalities (seizure, stroke)
• Auditory or visual loss
• Restrictive or obstructive chronic lung
disease
Galli KK, Myers LB, Nicholson SC. Anesthesia for Adult Patients with Congenital Heart Disease
Undergoing Noncardiac Surgery. CHOP CHD Symposium, 2015.

14. The
Univentricular
Heart

15. Staged Palliation Procedures
• Stage 0 – PDA kept open with prostaglandins
• Stage I – Norwood or Sano (first week of life)
– Atrial septectomy
– Rerouting the proximal pulmonary artery to the aorta
– Creation of BT pulmonary/systemic shunt (subclavian to distal PA vs ventricle to PA)
Nayak S, Booker PD. The Fontan Circulation. Continuing Education in Anesthesia, Critical Care, and
Pain. 2008 Feb;8(1):26-30.

16. Staged Palliation Procedures
• Stage II – Bidirectional Glenn (3-6 months)
– Removal of BT shunt
– Rerouting the SVC to the main PA
– Closure of atrial septectomy
Nayak S, Booker PD. The Fontan Circulation. Continuing Education in Anesthesia, Critical Care, and
Pain. 2008 Feb;8(1):26-30.

17. Staged Palliation Procedures
• Stage III – Fontan (2-5 years)
– Rerouting the IVC to the PA
– Creating a fenestrated baffle in the RA to completely separate the pulmonary and
systemic circulation
Nayak S, Booker PD. The Fontan Circulation. Continuing Education in Anesthesia, Critical Care, and
Pain. 2008 Feb;8(1):26-30.

18. • The success of the Fontan heart is primarily related to PVR, tricuspid valve function,
and maintenance of sinus rhythm
• Pulmonary flow is nonpulsatile and there is stagnation in the RA  requires lifelong
anticoagulation
• Most have an EF >50% and SpO2 >90% at rest
• Nearly half have some degree of cirrhosis, and almost all have at least NYHA Class I
heart failure
• About 30% of survivors require cardiac transplantation
Nayak S, Booker PD. The Fontan Circulation. Continuing Education in Anesthesia, Critical Care, and
Pain. 2008 Feb;8(1):26-30.

19. Anesthetic
Management

20. Preoperative Evaluation
• Establish the specific anomaly and historical course
– Note the patient’s baseline level of functioning, including BP, SpO2, hematocrit
– Recent EKG (always), echo (often), and catheterization (sometimes)
• Minimize NPO time (most are preload dependent) – should be the first case of the day
• Home medications
– Antimicrobials – continue
– Anticoagulants/antiplatelets – continue (but may transition to shorter acting rx for titration)
– Cardiac medications including B-blockers, antiarrhthmics, and ACEi – continue
• Things to consider when planning your anesthetic:
– Associated anomalies
– Intubation history
– Vascular access history
– Opioid tolerance
– Medication list
– Surgical factors: laparoscopy, need for Trendelenburg positioning, one lung ventilation
Jooste E, Machovec K. Anesthesia for adults with congenital heart disease undergoing noncardiac

21. Intraoperative Management
• Monitors
– Traditional 5 lead EKG monitoring preferred (even if dextrocardic)
– Invasive BP monitoring usually indicated
– Central venous catheter if needed – always under ultrasound
– Optional monitors
• TEE
• Cerebral oximetry / near infrared spectroscopy
• Induction
– Propofol (0.5-2mg/kg), slowly titrated to effect
– For patients with shunting or severely impaired ventricular function, consider ketamine
(0.5-2mg/kg) induction (usually in combination with propofol)
– For those who will likely require postoperative ventilation, consider high dose opioid
(fentanyl 7-10mcg/kg)
Galli KK, Myers LB, Nicholson SC. Anesthesia for Adult Patients with Congenital Heart Disease
Undergoing Noncardiac Surgery. CHOP CHD Symposium, 2015.

22. Intraoperative Management
• Maintenance
– Typically inhalational (1 MAC does not alter Qp/Qs)
– If TIVA indicated for other reasons, recommendation is propofol (50-150mcg/kg/min) with
adjuncts to lower necessary dose and reduce myocardial depression
• Fentanyl 1-2mcg/kg/hr
• Remifentanil 0.05-0.3mcg/kg/min
• Ketamine 5mcg/kg/min
• Dexmedetomidine 0.2-1mcg/kg/hr
• Fluid management – adequate preload must be maintained and significant volume
resuscitation may be required; some suggest use of colloids but no solid evidence of
superiority
• Ventilation management
– Negative pressure ventilation preferred where feasible
– Avoid hypoxia and hypercarbia (increases PVR); some advocate for mild hyperventilation
• Vasopressor selection is based on the individual patient and their degree of cardiac
dysfunction
Galli KK, Myers LB, Nicholson SC. Anesthesia for Adult Patients with Congenital Heart Disease
Undergoing Noncardiac Surgery. CHOP CHD Symposium, 2015.

23. Postoperative Management
• Extubate awake, possibly on dexmedetomidine infusion
• Treat postoperative pain in PACU to avoid sympathetic stimulation
• Encourage adequate respiratory stimulation – head up, incentive spirometry, etc
• Patient must remain on continuous pulse oximetry
Galli KK, Myers LB, Nicholson SC. Anesthesia for Adult Patients with Congenital Heart Disease
Undergoing Noncardiac Surgery. CHOP CHD Symposium, 2015.

24. A note on regional…
• ACHD is not a contraindication to regional anesthesia
• Neuraxial anesthesia also an acceptable choice
– Consensus: prefer epidural to spinal
– Can be slowly titrated to effect rather than abrupt onset of spinal
– Still likely to require invasive monitoring and hemodynamic support
• Many patients are on anticoagulation that may complicate the above
Galli KK, Myers LB, Nicholson SC. Anesthesia for Adult Patients with Congenital Heart Disease
Undergoing Noncardiac Surgery. CHOP CHD Symposium, 2015.

25. Recommendations
for Specific
Defects

26. CHD: Atrial Septal Defect
• Pathophysiology
– Left  Right shunt
• Areas of Concern
– If unrepaired
• Paradoxical emboli
– If repaired
• Atrial fibrilliation
• Anesthetic Pearls
– De-air all intravenous lines

27. CHD: Ventricular Septal Defect
• Pathophysiology
– Left  Right shunt; can progress to Right 
Left
– May be associated with other anomalies
• Areas of Concern
– If unrepaired
• High risk of pulmonary hypertension
(higher for larger anomalies)
• Risk of endocarditis, subpulmonic
obstruction, subaortic obstruction, aortic
regurgitation
• Right ventricular failure
– If repaired
• Arrhythmia (including complete heart
block)
• Persistent pulmonary hypertension
• Anesthetic Pearls
– Manage L  R shunt
– Maintain pulmonary blood flow if R  L
shunt
– Manage pacemaker

28. CHD: Coarctation of the Aorta
• Pathophysiology
– LV pressure overload  hypertrophy
– Aortic branch collaterals
– 50-80% associated with bicuspid aortic
valve
• Areas of Concern
– If unrepaired
• BP gradient between upper and lower
limbs
• LVH, systemic HTN
– If repaired
• Premature CAD
• Risk of bleeding with future thoracic
surgery
• Anesthetic Pearls
• BP inaccurate in L arm (if subclavian
angioplasty)
• Prone to postoperative hypertension
• Avoid tachycardia, hypotension

29. CHD: D-Transposition of Great Arteries
• Pathophysiology
– Pulmonary artery arises from LV
– Aorta arises from RV
– Abnormal coronary artery anatomy
– Often associated with VSD/ASD/PDA/pulmonic
stenosis/coarctation of the aorta
• Areas of Concern
– If unrepaired
• Survivable only if associated with VSD/ASD/PDA
• Profound hypoxia
– If repaired
• Sinus node dysfunction
• Myocardial ischemia
• Ascending aortic aneurysm
• Anesthetic Pearls
• Recommend preoperative cardiac evaluation to
include functional status and coronary imaging
• Maintain pulmonary blood flow
• May require pacing or cardioversion

30. CHD: Tetralogy of Fallot
• Pathophysiology
– Pulmonic stenosis
– RV hypertrophy, VSD
– Overriding aortic root
• Areas of Concern
– If unrepaired
• R L shunt, cyanosis
• Rare. Death before age 25.
– If palliated
• Chronic LV overload
• Pulmonary HTN
– If repaired
• Sinus node and AV node dysfunction
• Ascending aortic aneurysm
• LV dysfunction
• May have persistent pulmonary HTN, RV failure
• Anesthetic Pearls
• Manage pacemaker / have external pacing available
• Maintain pulmonary blood flow
• Maintain systemic blood pressure

31. CHD: Univentricular Heart
• Pathophysiology
– Double inlet
– Absence of AV connection
– Single well-developed ventricle
• Areas of Concern
– If unrepaired
• Rare.
– If repaired
• Dysrhythmias
• Heart failure
• Hepatic dysfunction
• Thromboemboli
• Restrictive lung disease
• Anesthetic Pearls
• Right radial arterial pressure inaccurate
• Manage dysrhythmias
• Maintain low pulmonary vascular resistance
• Maintain adequate preload
• Replace coagulation factors

32. The Parturient
with ACHD

33. High Risk Pregnancy
• Normal physiologic changes of pregnancy may be difficult for ACHD patients to tolerate
– Increased cardiac output
– Decreased systemic vascular resistance
– Increased oxygen requirements
• The desire to have children may cause some women to minimize their symptoms
• Risk of mortality or severe morbidity is high for some groups
– Low risk (<1%)
• Most with anatomic repairs
• Uncomplicated LR shunts
• Mitral valve prolapse, bicuspid aortic valve, other mild valvular lesions
– Intermediate risk (1-5%)
• Univentricular heart
• Unrepaired cyanotic lesions
• Mitral stenosis (all), aortic stenosis (mild/moderate), pulmonary stenosis (severe)
– High risk (>5%)
• NYHA class III-IV
• Severe aortic stenosis
• Severe ventricular dysfunction
• Pulmonary hypertension (30-50%)
Dob DP, Yentis SM. Practical management of the parturient with congenital heart disease. International
Journal of Obstetric Anesthesia. 2006 July;15(137-144).

34. Peripartum Management
• Monitoring
– Continuous EKG, pulse oximetry, NIBP for all
– Invasive BP for intermediate – high risk patients
– Sufficient peripheral venous access vs Central access if likely need for vasopressors
• Analgesia for vaginal delivery
– Early labor epidural with very slow titration recommended
– Consideration must be given to anticoagulation
• Anesthesia for Cesarean delivery
– Recommend slow epidural titration if possible
– GA may be indicated if patient is likely to have complications (eg, need for cardioversion) or is
at risk from drop in SVR that accompanies neuraxial techniques (may exacerbate shunting)
Dob DP, Yentis SM. Practical management of the parturient with congenital heart disease. International
Journal of Obstetric Anesthesia. 2006 July;15(137-144).

35. Peripartum Complications
• Hemorrhage
– Reduced ability to compensate
– Always cross-match and transfuse if needed
• Pulmonary edema
– Common after autotransfusion
– Some protocols recommend small dose of furosemide at time of delivery
• Uterine atony
– Oxytocin may cause profound tachycardia and hypotension, should never be bolused in ACHD
– Ergotamine causes pulmonary vasoconstriction and may precipitate pulmonary hypertensive
crisis
– Carbaprost may cause hypertension and pulmonary edema
– Misoprostol is generally well tolerated
– Recommendation: misoprostol (buccal/vaginal/rectal), slow infusion of oxytocin, manual
massage
• Embolism
– Recall risk for paradoxical embolus in patients with septal defects
Dob DP, Yentis SM. Practical management of the parturient with congenital heart disease. International
Journal of Obstetric Anesthesia. 2006 July;15(137-144).

36. In Summary…

37. Remember…
• The number of patients with Adult Congenital Heart Disease increases each year
• Transitional and Adult Congenital Cardiology is an emerging field
• ACHD is a continuum and ranges from mild to life-threatening
• In general, keep the patient normo-everything
– Hypoxia and hypercarbia are particularly important to avoid (can exacerbate Pulmonary
HTN)
– Hypotension and arrhythmia can also be disastrous

38. General Anesthetic Pearls
• A thorough, multidisciplinary preoperative evaluation is necessary
– For anatomically repaired lesions, no major contraindication to proceeding
– For others, low threshold for echo, stress, or cath
• Be wary of pulmonary hypertension, always be ready to pace, and consider embolic
risk
• In most repaired ACHD patients, Right Radial arterial line is a bad idea…SpO2
measurement may be compromised as well
– Except Coarctation (then it’s the Left)!
• Patients without complete anatomic repairs will require EKG and SpO2 monitoring
overnight postoperatively
• For elective cases in patients with palliated or unrepaired anomalies, consider transfer
to a center with CPB / ECMO capability

39. And finally…
ASK FOR HELP
• The patient
• The family
• The primary doctor
• Your colleagues

40. Adult Congenital Cardiology
• Adult CHD is an emerging field with a
growing need
• Typically Med/Peds residency followed
by one or more fellowships
• The first board certification exam will
be offered in October 2017
• Norton Children’s Hospital Heart
Center, Dr. Craig Alexander

41. References
• Cannesson M, Earing MG, Collange V, Kersten JR. Anesthesia for Noncardiac Surgery in Adults with Congenital Heart Disease. Anesthesiology. 2009
Aug;111:432-40.
• CDC Center for Birth Defects, http://www.cdc.gov/ncbddd/birthdefects/index.html
• Dob DP, Yentis SM. Practical management of the parturient with congenital heart disease. International Journal of Obstetric Anesthesia. 2006 July;15(137-
144).
• Freedom RM, Lock J, Bricker T. Pediatric Cardiology and Cardiovascular Surgery: 1950-2000. Circulation. 200 Nov;102:lv-58-lv-68.
• Galli KK, Myers LB, Nicholson SC. Anesthesia for Adult Patients with Congenital Heart Disease Undergoing Noncardiac Surgery. CHOP CHD Symposium,
2015.
• Gottlieb EA, Andropoulos DB. Anesthesia for the patient with congenital heart disease presenting for noncardiac surgery. Current Opinion in
Anaesthesiology, 2013 June;26(3):318-326.
• Jooste E, Machovec K. Anesthesia for adults with congenital heart disease undergoing noncardiac surgery. UpToDate. July 2016.
• Khoshnood, B, Lelong N, Houyel L, Thieulin AC, Jouannic JM, Magnier S, Delezoide AL, Magny JF, Rambaud C, Bonnet D, Goffinet F. Prevalence, timing of
diagnosis, and mortality of newborns with congenital heart defects: a population-based study. Heart. 2012 Nov;98(22):1667-73.
• Lillehei CW, Varco RL, Cohen M, Warden HE, Gott VL, DeWall RA, Patton C, Moller JH. The first open heart corrections of tetraology of Fallot. A 26-31 year
follow-up of 106 patients. Annals of Surgery. 1986 Oct;204(4):490-502.
• Lillehei CW. Chapter 1: Historical Development of Cardiopulmonary Bypass in Minnesota. In Cardiopulmonary Bypass: Principles and Practice, 2nd Edition.
Eds: Gravlee GP, Davis RF, Kurusz M, Utley JR. Lippincott Williams & Wilkins, 2000.
• Lovell AT. Anaesthetic implications of grown-up congenital heart disease. British Journal of Anesthesia. 2004 July;93(1):129-39.
• Maxwell BG, Williams GD, Ramamoorthy C. Knowledge and Attitudes of Anesthesia Providers about Noncardiac Surgery in Adults with Congenital Heart
Disease. Congenital Heart Disease. 2014 Jan;9(1):45-53.
• Maxwell BG, Wong JK, Kin C, Lobato RL. Perioperative Outcomes of Major Noncardiac Surgery in Adults with Congenital Heart Disease. Anesthesiology.
2013 Oct;119(4):762-769.
• Nayak S, Booker PD. The Fontan Circulation. Continuing Education in Anesthesia, Critical Care, and Pain. 2008 Feb;8(1):26-30.
• Pediatric Congenital Heart Association, http://www.conqueringchd.org
• Pike NA, Evangelista LS, Doering LV, Koniak-Griffin D, Lewis AL, Child JS. Clinical Profile of the Adolescent/Adult Fontan Survivor. Congenital Heart Disease.
2011 Jan;6(1):9-17.
• Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A. Prevalence of Congenital Heart Defects in Metropolitan Atlanta 1998-2005. Journal of
Pediatrics, 2008 Dec; 153(6)807-813.

42. Questions?

43. O n a
p e r s o n a l
n o t e . .