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KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
- Atrioventricular septal defect (AVSD)
Synonyms
- Atrioventricular (AV) canal
- Endocardial cushion defect
Definitions
- Central cardiac defect involving
- Atrial septum [atrial septal defect (ASD)]
- Ventricular septum [ventricular septal defect (VSD)]
- AV valves
- Abnormal course of conducting system
- Defect can be balanced or unbalanced
- Balanced: Right (RV) and left (LV) ventricles are equal in size with equal commitment of AV valves
- Unbalanced: 1 ventricle gets majority of inflow and is equivalent to single ventricle
- Defect can be complete or partial
- Partial: Primum ASD, distinct mitral and tricuspid valve anuli, cleft in mitral valve
- Transitional: Partial AVSD that also has small inlet VSD, which is partially occluded by dense chordal attachments
- Complete: Primum ASD, contiguous inlet VSD, and common AV valve has single anulus
- Intermediate: Complete AVSD that has distinct right and left AV valve orifice despite having only 1 common anulus
- Partial: Primum ASD, distinct mitral and tricuspid valve anuli, cleft in mitral valve
- Central cardiac defect involving
IMAGING
General Features
Echocardiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Belfrage K et al: Initial fetal to initial postnatal echocardiogram in uncomplicated atrioventricular septal defects: do significant changes occur? Echocardiography. 37(12):2102-6, 2020
- Słodki M et al: Prenatal atrioventricular septal defect (AVSD) as a planned congenital heart disease with different outcome depending on the presence of the coexisting extracardiac abnormalities (ECA) and/or malformations (ECM). J Matern Fetal Neonatal Med. 33(15):2635-41, 2020
- Airaksinen R et al: Complete atrioventricular septal defect: evolution of results in a single center during 50 years. Ann Thorac Surg. 107(6):1824-30, 2019
- Mery CM et al: Contemporary outcomes after repair of isolated and complex complete atrioventricular septal defect. Ann Thorac Surg. 106(5):1429-37, 2018
- Calkoen EE et al: Atrioventricular septal defect: from embryonic development to long-term follow-up. Int J Cardiol. 202:784-95, 2016
- Ginde S et al: Long-term outcomes after surgical repair of complete atrioventricular septal defect. J Thorac Cardiovasc Surg. 150(2):369-74, 2015
- Gittenberger-de Groot AC et al: Morphogenesis and molecular considerations on congenital cardiac septal defects. Ann Med. 46(8):640-52, 2014
- Foker JE et al: Two-ventricle repairs in the unbalanced atrioventricular canal defect spectrum with midterm follow-up. J Thorac Cardiovasc Surg. 146(4):854-60.e3, 2013
- Beppu H et al: BMP type II receptor regulates positioning of outflow tract and remodeling of atrioventricular cushion during cardiogenesis. Dev Biol. 331(2):167-75, 2009
- Shuhaiber JH et al: Current options and outcomes for the management of atrioventricular septal defect. Eur J Cardiothorac Surg. 35(5):891-900, 2009
- Goddeeris MM et al: Intracardiac septation requires hedgehog-dependent cellular contributions from outside the heart. Development. 135(10):1887-95, 2008
- Suzuki T et al: Results of definitive repair of complete atrioventricular septal defect in neonates and infants. Ann Thorac Surg. 86(2):596-602, 2008
- Fesslova V et al: Spectrum and outcome of atrioventricular septal defect in fetal life. Cardiol Young. 12(1):18-26, 2002
- Hoffman JI et al: The incidence of congenital heart disease. J Am Coll Cardiol. 39(12):1890-900, 2002
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
- Atrioventricular septal defect (AVSD)
Synonyms
- Atrioventricular (AV) canal
- Endocardial cushion defect
Definitions
- Central cardiac defect involving
- Atrial septum [atrial septal defect (ASD)]
- Ventricular septum [ventricular septal defect (VSD)]
- AV valves
- Abnormal course of conducting system
- Defect can be balanced or unbalanced
- Balanced: Right (RV) and left (LV) ventricles are equal in size with equal commitment of AV valves
- Unbalanced: 1 ventricle gets majority of inflow and is equivalent to single ventricle
- Defect can be complete or partial
- Partial: Primum ASD, distinct mitral and tricuspid valve anuli, cleft in mitral valve
- Transitional: Partial AVSD that also has small inlet VSD, which is partially occluded by dense chordal attachments
- Complete: Primum ASD, contiguous inlet VSD, and common AV valve has single anulus
- Intermediate: Complete AVSD that has distinct right and left AV valve orifice despite having only 1 common anulus
- Partial: Primum ASD, distinct mitral and tricuspid valve anuli, cleft in mitral valve
- Central cardiac defect involving
IMAGING
General Features
Echocardiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Belfrage K et al: Initial fetal to initial postnatal echocardiogram in uncomplicated atrioventricular septal defects: do significant changes occur? Echocardiography. 37(12):2102-6, 2020
- Słodki M et al: Prenatal atrioventricular septal defect (AVSD) as a planned congenital heart disease with different outcome depending on the presence of the coexisting extracardiac abnormalities (ECA) and/or malformations (ECM). J Matern Fetal Neonatal Med. 33(15):2635-41, 2020
- Airaksinen R et al: Complete atrioventricular septal defect: evolution of results in a single center during 50 years. Ann Thorac Surg. 107(6):1824-30, 2019
- Mery CM et al: Contemporary outcomes after repair of isolated and complex complete atrioventricular septal defect. Ann Thorac Surg. 106(5):1429-37, 2018
- Calkoen EE et al: Atrioventricular septal defect: from embryonic development to long-term follow-up. Int J Cardiol. 202:784-95, 2016
- Ginde S et al: Long-term outcomes after surgical repair of complete atrioventricular septal defect. J Thorac Cardiovasc Surg. 150(2):369-74, 2015
- Gittenberger-de Groot AC et al: Morphogenesis and molecular considerations on congenital cardiac septal defects. Ann Med. 46(8):640-52, 2014
- Foker JE et al: Two-ventricle repairs in the unbalanced atrioventricular canal defect spectrum with midterm follow-up. J Thorac Cardiovasc Surg. 146(4):854-60.e3, 2013
- Beppu H et al: BMP type II receptor regulates positioning of outflow tract and remodeling of atrioventricular cushion during cardiogenesis. Dev Biol. 331(2):167-75, 2009
- Shuhaiber JH et al: Current options and outcomes for the management of atrioventricular septal defect. Eur J Cardiothorac Surg. 35(5):891-900, 2009
- Goddeeris MM et al: Intracardiac septation requires hedgehog-dependent cellular contributions from outside the heart. Development. 135(10):1887-95, 2008
- Suzuki T et al: Results of definitive repair of complete atrioventricular septal defect in neonates and infants. Ann Thorac Surg. 86(2):596-602, 2008
- Fesslova V et al: Spectrum and outcome of atrioventricular septal defect in fetal life. Cardiol Young. 12(1):18-26, 2002
- Hoffman JI et al: The incidence of congenital heart disease. J Am Coll Cardiol. 39(12):1890-900, 2002
