Tetralogy of Fallot
- Tetralogy of Fallot is a ventricular septal defect associated with right ventricular (RV) outflow obstruction (infundibular and/or valvular pulmonic stenosis), concentric RV hypertrophy, and overriding aorta (1).
- Pathophysiology depends primarily on severity of RV outflow tract obstruction.
- RV and left ventricular (LV) pressures generally are equal owing to the defect.
- RV pressures can be elevated with ventricular septal defect proximal to the level of RV obstruction.
- Right-to-left shunting predominates, resulting in varying degrees of arterial oxygen desaturation.
- System(s) affected: cardiovascular, pulmonary
Pregnancy is usually well tolerated following successful surgical repair.
- 5–10% of all congenital heart disease (1)
- Most common cardiac anomaly within the 1st year of life requiring intervention
- Predominant age: newborn; case reports of adult presentation
- Predominant sex: male = female
In the United States: 3.9/10,000 live births
Etiology and Pathophysiology
- Minority of cases are familial, autosomal dominant mode of inheritance.
- 20% association with velocardiofacial syndrome (22q11 deletion) by FISH analysis
- Predominately sporadic
- Increased incidence with advanced maternal age
Commonly Associated Conditions
- Stenotic pulmonary artery
- Patent ductus arteriosus
- Atrial septal defect
- Right-sided aortic arch: 25%
- Atrial septal defect: 15%
- Anomalous coronary arteries: 9%
- 20–25% associated with a syndrome, including Down, Alagille, DiGeorge, and velocardiofacial
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Domino, Frank J., et al., editors. "Tetralogy of Fallot." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2020. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688834/all/Tetralogy_of_Fallot.
Tetralogy of Fallot. In: Domino FJF, Baldor RAR, Golding JJ, et al, eds. 5-Minute Clinical Consult. Wolters Kluwer; 2020. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688834/all/Tetralogy_of_Fallot. Accessed May 29, 2023.
Tetralogy of Fallot. (2020). In Domino, F. J., Baldor, R. A., Golding, J., & Stephens, M. B. (Eds.), 5-Minute Clinical Consult (27th ed.). Wolters Kluwer. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688834/all/Tetralogy_of_Fallot
Tetralogy of Fallot [Internet]. In: Domino FJF, Baldor RAR, Golding JJ, Stephens MBM, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2020. [cited 2023 May 29]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688834/all/Tetralogy_of_Fallot.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Tetralogy of Fallot ID - 1688834 ED - Domino,Frank J, ED - Baldor,Robert A, ED - Golding,Jeremy, ED - Stephens,Mark B, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688834/all/Tetralogy_of_Fallot PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -