Congenital Heart Disease, Cyanotic
Basics
Description
Description
- Aberrant embryonic development results in mixing of deoxygenated and oxygenated blood returning to systemic circulation by 2 mechanisms:
- Right-to-left intracardiac shunt
- Anatomic defects of the aortic root
- Subtypes: 5 T's, 2 E's, single ventricle:
- Tetralogy of Fallot (TOF):
- Ventricular septal defect (VSD)
- Right ventricular (RV) outflow obstruction
- Overriding aorta
- RV hypertrophy (RVH)
- Transposition of the great arteries (TGA):
- Aorta arises from RV and pulmonary artery from left ventricle (LV)
- Tricuspid atresia:
- No outlet from right atrium to RV
- Obligatory atrial level connection
- Truncus arteriosus:
- Single arterial trunk for systemic, pulmonic, and carotid circulations
- Total anomalous pulmonary venous return (TAPVR):
- Pulmonary veins drain into systemic venous circulation
- Supracardiac, cardiac, infracardiac, or mixed
- Ebstein anomaly of tricuspid valve:
- Abnormal and displaced tricuspid valve divides RV resulting in poor RV function
- Eisenmenger syndrome:
- Complication in longstanding acyanotic heart disease with L→R shunts
- Pulmonary vascular resistance reaches suprasystemic levels; R→L shunt
- Single ventricle physiology:
- Total mixing of systemic and venous return
- Tetralogy of Fallot (TOF):
- Subdivide by amount of pulmonary blood flow:
- Decreased in TOF and tricuspid atresia
- Increased in TGA, truncus, and TAPVR
Etiology
For most forms, cause is unknown:Etiology
- Genetic cause: Chromosome 22 deletion in DiGeorge syndrome (conotruncal defects; TOF, truncus; also coarctation of aorta)
- Environmental/teratogenic: TGA seen more often in infants of diabetic mothers
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Citation
Schaider, Jeffrey J., et al., editors. "Congenital Heart Disease, Cyanotic." 5-Minute Emergency Consult, 6th ed., Lippincott Williams & Wilkins, 2020. Emergency Central, emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307228/2.0/Congenital_Heart_Disease_Cyanotic.
Congenital Heart Disease, Cyanotic. In: Schaider JJJ, Barkin RMR, Hayden SRS, et al, eds. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2020. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307228/2.0/Congenital_Heart_Disease_Cyanotic. Accessed October 15, 2024.
Congenital Heart Disease, Cyanotic. (2020). In Schaider, J. J., Barkin, R. M., Hayden, S. R., Wolfe, R. E., Barkin, A. Z., Shayne, P., & Rosen, P. (Eds.), 5-Minute Emergency Consult (6th ed.). Lippincott Williams & Wilkins. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307228/2.0/Congenital_Heart_Disease_Cyanotic
Congenital Heart Disease, Cyanotic [Internet]. In: Schaider JJJ, Barkin RMR, Hayden SRS, Wolfe RER, Barkin AZA, Shayne PP, Rosen PP, editors. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2020. [cited 2024 October 15]. Available from: https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307228/2.0/Congenital_Heart_Disease_Cyanotic.
* Article titles in AMA citation format should be in sentence-case
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T1 - Congenital Heart Disease, Cyanotic
ID - 307228
ED - Barkin,Adam Z,
ED - Shayne,Philip,
ED - Rosen,Peter,
ED - Schaider,Jeffrey J,
ED - Barkin,Roger M,
ED - Hayden,Stephen R,
ED - Wolfe,Richard E,
BT - 5-Minute Emergency Consult
UR - https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307228/2.0/Congenital_Heart_Disease_Cyanotic
PB - Lippincott Williams & Wilkins
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DB - Emergency Central
DP - Unbound Medicine
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