Tetralogy of Fallot
&
Surgical Repair Techniques
Ted Hodges, MS3
UNC Pediatrics Clerkship
Newborn Nursery Presentation
November 21, 2014
Tetralogy of Fallot
Classical Characteristics
• Pulmonic stenosis
• Right ventricular
hypertrophy
• Overriding aorta
• Ventricular septal defect
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TOF Epidemiology
• Prevalence in the United States is 3.9 per
10,000 live births
• Accounts for 7-10% of congenital heart
defects
• Most common lesion requiring intervention in
first year of life
• Males and females equally affected
• Genetics remain unclear
» 85% sporadic
» 15% present as a portion of syndrome
• Down, Alagille, DiGeorge/Velocardiofacial
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Clinical Presentation
• Tetralogy of Fallot occurs on a spectrum
» Pink Tets → MAPCAs
• Pink Tets have minimal RVOT obstruction
and may present later with pulmonary
overcirculation and heart failure
• Children with the most severe RVOT
obstruction are cyanotic in the immediate
neonatal period
• Most children are somewhere between the
extremes
» Tet Spells
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Physical Exam
• Patients usually comfortable and in no acute
distress
• During Tet Spell
» Hyperpnea
» Cyanosis of lips and nail beds
• Murmur: “crescendo-decrescendo type with
harsh systolic ejection quality”
» Best heard at left upper sternal border
• Single S2 heart sound
» Minimal contribution from pulmonic valve
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ECG Findings
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TOF Repair Algorithm
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Blalock-Taussig Shunt
• Typically used for
palliation of
severely cyanotic
infants until
definitive procedure
• Functions like a
PDA
• Developed by Alfred
Blalock and Helen
Taussig at Johns
Hopkins in 1943
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TOF Corrective Repair
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TOF Prognosis
• Overall, prognosis is excellent
• STS data on over 3000 repairs in North
America from 2002 to 2007
» BT Shunt mortality: 6.2%
» TOF correction mortality: 1.9%
» 5 year survival with correction: 98.5%
• Today, more adults than children living with
congenital cardiac malformations
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References
•
Al Habib HF, et al. Contemporary patterns of management of tetralogy of Fallot: data from
the Society of Thoracic Surgeons Database. Ann Thoracic Surg 2010 Sep;90(3):813-9
•
Barron DJ. Tetralogy of Fallot: controversies in early management. World J Pediatric
Congent Heart Surg 2013 Apr;4(2):186-91.
•
http://www.uptodate.com.libproxy.lib.unc.edu/contents/pathophysiology-clinical-featuresand-diagnosis-of-tetralogy-of-fallot
•
http://www.surgery.ucsf.edu/conditions--procedures/tetralogy-of-fallot.aspx
•
http://www.uptodate.com.libproxy.lib.unc.edu/contents/management-and-outcome-oftetralogy-of-fallot
Images
•
http://www.stanfordchildrens.org/content-public/topic/images/83/125883.gif
•
http://www.uptodate.com.libproxy.lib.unc.edu/contents/image?imageKey=CARD%2F81339
&topicKey=PEDS%2F5769&rank=1~111&source=see_link&search=tetralogy+of+fallot&utd
Popup=true
•
http://babyheart.in/wp-content/uploads/2010/09/B-T-Shunt-Blalock-Taussig-shunt.jpg
•
http://www.sts.org/sites/default/files/documents/Figure%205%20.jpg
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