Tetralogy of Fallot
Anatomic hallmark is anterior malalignment of infundibular (outlet) septum, which results in
- A large and unrestrictive ventricular septal defect (VSD)
- Various degrees of right ventricular outflow tract obstruction (RVOTO)
- Overriding aorta
- Right ventricular hypertrophy (RVH) secondary to exposure to systemic pressure
- The most common cyanotic congenital heart disease
- 3.5–10% of all congenital heart disease
About 20% of cases of tetralogy of Fallot are associated with a chromosome 22q11 microdeletion or duplication.
- Severity of clinical signs and symptoms depends on the degree of RVOTO and related right-to-left shunt at the VSD.
- Physiology is a spectrum ranging from too little pulmonary blood flow and right-to-left shunt at the VSD due to severe RVOT obstruction (“blue tet”) to moderate pulmonary overcirculation and left-to-right shunt at the VSD with minimal RVOTO (“pink tet”).
Commonly Associated Conditions
- May be associated with other syndromes including trisomy 21, Alagille syndrome, fetal alcohol syndrome, and those involving a variety of limb abnormalities
- Tetralogy of Fallot may also be associated with midline abdominal defects (e.g., omphalocele) as in the pentalogy of Cantrell.
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Cabana, Michael D., editor. "Tetralogy of Fallot." 5-Minute Pediatric Consult, 8th ed., Wolters Kluwer, 2019. Pediatrics Central, peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617579/all/Tetralogy_of_Fallot.
Tetralogy of Fallot. In: Cabana MDM, ed. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617579/all/Tetralogy_of_Fallot. Accessed June 9, 2023.
Tetralogy of Fallot. (2019). In Cabana, M. D. (Ed.), 5-Minute Pediatric Consult (8th ed.). Wolters Kluwer. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617579/all/Tetralogy_of_Fallot
Tetralogy of Fallot [Internet]. In: Cabana MDM, editors. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. [cited 2023 June 09]. Available from: https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617579/all/Tetralogy_of_Fallot.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Tetralogy of Fallot ID - 617579 ED - Cabana,Michael D, BT - 5-Minute Pediatric Consult UR - https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617579/all/Tetralogy_of_Fallot PB - Wolters Kluwer ET - 8 DB - Pediatrics Central DP - Unbound Medicine ER -