Tetralogy of Fallot

and
Anesthesia

Josh LeVee, Kyle Wescott, and Emily Wolters

What is Tetralogy of
Fallot?
Four Defects
1. Ventricular Septal Defect
(VSD)
2. Pulmonary stenosis
3. Coarctation of Aorta
4. Right Ventricular
Hypertrophy
Image courtesy of
https://www.cdc.gov/ncbddd/heartdefects/tetralogyoffallot.html
Comparison with Normal Heart

Tetralogy_of_Fallot_svg by Alexandra Almonacid is licensed under CC BY-SA 3.0

 SIGNS & SYMPTOMS:
Mild to moderate disease: Severe forms:
1. Cyanosis during the first few 1. Profound cyanosis
months of life occurs rapidly
2. Some may remain 2. Hyperpnea
asymptomatic and present with 3. May lose consciousness
heart failure later in life 4. Stroke
5. Seizures
6. Death

(Steppan & Maxwell, 2018)

 Hypercyanotic Attacks (aka: Tet
Spells) ● Children become hypercyanotic
during:
○ Feeding
○ Crying
○ Overstimulation
● Most commonly seen in 2-4
months of age
● “Pink” Tets are rare, but can
Image from https://medlineplus.gov/ency/images/ency/fullsize/18134.jpg
occur:
(Mayo Clinic Staff, 2017)
○ L-to-R shunt of VSD
 Hypercyanotic Attacks (aka: Tet
Spells)
● Mechanisms contributing to
attacks:
○ Increased sympathetic
activity = vasoconstriction
at pulmonary infundibulum
○ Decreased pH
○ Decreased pO2
(Haque, Smith, & Animasahun, 2016)

Original image
Treatment of Hypercyanotic Attacks (Tet
(Not in OR)
Spells)
● Knee-chest “squat” position
○ Increases SVR
● Supplemental Oxygen
● Keep child calm
(Mayo Clinic Staff, 2017)
Surgical Repairs

1. Surgical repair of VSD

2. Surgical repair/replacement of pulmonary valve
(Mayo Clinic Staff, 2016; Greely, Cripe, & Nathan, 2015)
 Pre-op Assessment
● Patient age:
○ If not treated, polycythemia can
develop.
● Frequency/severity of Tet Spells
● Review Home Medications
○ Phosphodiesterase-3 inhibitors?
○ Beta Blockers?
○ Diuretics?
● Auscultation:
○ Likely, systolic murmur(Bhimji
at &left sternal
Mancini, 2017b; Steppan & Maxwell, 2018)
Pre-op Planning
● Decrease chance of hypercyanotic spell
○ Consider premedication with anxiolytics or sedatives to avoid/dampen
sympathetic stimulation
■ PO midazolam: 0.5mg/kg PO
■ Morphine 0.2 mg/kg IM
■ Ketamine intranasally 10 mg/kg or 1-2 mg/kg IV
● Performed better to preserve SVR
○ Prevent/Correct acidosis and hypoxemia
○ Plan for hypercyanotic spell
■ IVF to raise SVR
■ Phenylephrine (5 mcg/kg)
■ Beta Blocker, especially Propranolol, to decrease infandibulur spasm
Anesthesia Induction & Management
● Goal: Maintain intravascular volume and SVR
● Induction
○ Ketamine often preserves SVR (Butterworth, Mackey, &
Wasnick, 2013)

○ Dexmedetomidine prior to incision decreases

less post-op junctional ectopic tachycardia (Gautum,
Turiya,, & Srinivasan, 2017)

○ Avoid neuromuscular blockades with histamine

release (Butterworth et al., 2013)
● Maintenance
○ Avoid N2O; increases PVR (Butterworth et al., 2013)
○ In general, volatile gases decrease SVR, which
is undesirable (Butterworth et al., 2013; Haque et al., 2016)
Keep in Mind...
● Drugs may have slowed onset due to R-to-L
shunting
● Keep SVR up to prevent worsening acidosis and
hypoxemia
● Glucose control is important
● How to “break” a hypercyanotic spell
○ May need phenylephrine gtt
○ NO Norepinephrine or DOPAmine; may ↓
SVR or stimulate infundibular spasm
○ IVF to increase SVR
○ Esmolol gtt may decrease infundibular
(Butterworth et al., 2013; Haque et al., 2016)
Complications of Repairs
● Right Ventricular Hypertrophy→ Failure
● Right Ventricular obstruction
○ Foramen ovale often left open for this reason
● Hypoxemia
● Pulmonary valve regurgitation or repeat replacement (most common)
● Cardiac Arrhythmias
○ RBBB commonly seen after surgery. Usually remains asymptomatic
● Infection
● Dizziness, fainting, seizures
● Delayed growth and development

(Bhimji & Mancini, 2017a; Greeley et al, 2013; Steppan & Maxwell, 2018)
Post-Op Assessment and Management
● Monitor closely and prepare for hypercyanotic spells
● Inotropic and vasoactive requirements
● Hemodynamic parameters to maintain global perfusion
● Ventilatory support and prolonged intubation
● AV pacing to maintain CO and perfusion
● Elevated RV:LV pressure ratio
● 5% of individuals will require revision/reoperation
○ Residual VSD shunt with RV obstruction pressure > 60 mmHg is
EMERGENT
● Pulmonary insufficiency will remain until valve replaced/repaired
○ Transpulmonary and transarterial approaches utilized to preserve
QUESTION #1
Which of the following are interventions one may try in an attempt to stop
a Hypercyanotic Attack (Tet Spell)? Select all that apply

a. Downward Dog position

b. Apply oxygen
c. Knee-Chest squat position
d. Phenylephrine administration
e. Norepinephrine administration

Click here for answer: B, C, D

QUESTION #2
Which induction drugs might you consider as possibilities for a patient
who has Tetralogy of Fallot? (Select two)

a. Versed
b. Ketamine
c. Propofol
d. Etomidate
e. Dexmedetomidine

Click here for answer: B and E

QUESTION #3
Which of the following are the “defects” of Tetralogy of Fallot? (Select
four)

a. Left ventricular hypertrophy

b. Aortic coarctation
c. Pulmonary stenosis
d. Aortic Stenosis
e. Ventricular-Septal Defect
f. Right ventricular hypertrophy

Click here for answer: B, C, E, F

QUESTION #4
Which drug is the best choice to use to raise blood pressure for a patient
who has Tetralogy of Fallot?

a. Norepinephrine
b. DOPAmine
c. Phenylephrine
d. Vasopressin

Click here for answer: C

QUESTION #5
Which of the following is the most common post-operative complication
seen after surgery to repair Tetralogy of Fallot defects?

a. Arrhythmias
b. Pulmonary regurgitation
c. Bleeding
d. Infection

Click here for answer: B

References
Bhimji, S., & Mancini, M. C. (2017a). Tetralogy of Fallot treatment & management: Postoperative monitoring and results.

Retrieved Feburary 14, 2018, from https://emedicine.medscape.com/article/2035949-treatment#d16

Bhimji, S., & Mancini, M. C. (2017b). Tetralogy of Fallot treatment & management: Surgical considerations. Retrieved

February 14, 2018, from https://emedicine.medscape.com/article/2035949-treatment#d13

Butterworth, J. F., Mackey, D. C., & Wasnick, J. D. (2013). Anesthesia for patients with cardiovascular disease. In Morgan &

Mikhail's clinical anesthesiology (5th ed., pp. 375-486). New York City, NY: McGraw-Hill.

Gautum, N. K., Turiya, Y., & Srinivasan, C. (2017, December). Preincision initiation of dexmedetomidine maximally reduces

the risk of junctional ectopic tachycardia in children undergoing ventricular septal defect repairs. Journal of

Cardiothoracic and Vascular Anesthesia, 31(6), 1960-1965. http://dx.doi.org/10.1053/j.jvca.2017.04.010

References
Greeley, W. J., Cripe, C. C., & Nathan, A. T. (2015). Anesthesia for pediatric cardiac surgery. In R. D. Miller, N. H. Cohen,
L.

I. Erikkson, L. A. Fleisher, J. P. Weiner-Kronish, & W. L. Young (Eds.), Miller's anesthesia (Vol 2.) (8th ed., pp.

2799-2853). Philadelphia, PA: Elsevier.

Haque, M., Smith, J., Onal, O., & Animasahun, A. (2016). Anaesthesia recommendations for patients suffering from

Tetralogy of Fallot. Retrieved February 14, 2018, from

http://www.orphananesthesia.eu/de/erkrankungen/zu-erledigen/doc_view/268-tetralogy-of-fallot.html

Mayo Clinic Staff. (2017). Tetralogy of Fallot. Retrieved Feburary 16, 2018, from

https://www.mayoclinic.org/diseases-conditions/tetralogy-of-fallot/symptoms-causes/syc-20353477

Steppan, J., & Maxwell, B. G. (2018). Congenital heart disease. In R. L. Hines & K. E. Marschall (Eds.), Stoelting's

anesthesia and co-existing disease (7th ed., pp. 129-149). Philadelphia, PA: Elsevier.