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Congenital Heart Disease, Cyanotic

Congenital Heart Disease, Cyanotic is a topic covered in the 5-Minute Emergency Consult.

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  • 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


For most forms, cause is unknown

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Rosen, Peter, et al., editors. "Congenital Heart Disease, Cyanotic." 5-Minute Emergency Consult, 5th ed., Lippincott Williams & Wilkins, 2016. Emergency Central, emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307228/all/Congenital_Heart_Disease_Cyanotic.
Congenital Heart Disease, Cyanotic. In: Rosen P, Shayne P, Barkin AZ, et al, eds. 5-Minute Emergency Consult. 5th ed. Lippincott Williams & Wilkins; 2016. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307228/all/Congenital_Heart_Disease_Cyanotic. Accessed June 27, 2019.
Congenital Heart Disease, Cyanotic. (2016). In Rosen, P., Shayne, P., Barkin, A. Z., Wolfe, R. E., Hayden, S. R., Barkin, R. M., & Schaider, J. J. (Eds.), 5-Minute Emergency Consult. Available from https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307228/all/Congenital_Heart_Disease_Cyanotic
Congenital Heart Disease, Cyanotic [Internet]. In: Rosen P, Shayne P, Barkin AZ, Wolfe RE, Hayden SR, Barkin RM, Schaider JJ, editors. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2016. [cited 2019 June 27]. Available from: https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307228/all/Congenital_Heart_Disease_Cyanotic.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Congenital Heart Disease, Cyanotic ID - 307228 ED - Rosen,Peter, ED - Shayne,Philip, ED - Barkin,Adam Z, ED - Wolfe,Richard E, ED - Hayden,Stephen R, ED - Barkin,Roger M, ED - Schaider,Jeffrey J, BT - 5-Minute Emergency Consult UR - https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307228/all/Congenital_Heart_Disease_Cyanotic PB - Lippincott Williams & Wilkins ET - 5 DB - Emergency Central DP - Unbound Medicine ER -