Broad spectrum of defects characterized by involvement of atrial septum, ventricular septum, & 1 or both AV valves
Complete AVSD indicates presence of both atrial & ventricular septal defects with common AV valve
Partial AVSD indicates atrial septal involvement with separate mitral & tricuspid valve orifices
Unbalanced AVSD indicates 1 ventricular chamber is hypoplastic as compared to other, depending on direction of AV valve flow
IMAGING
General Features
Echocardiographic Findings
CT Findings
MR Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Nayak S et al: Echocardiographic assessment of atrioventricular canal defects. Echocardiography. 37(12):2199-210, 2020
Elders B et al: Altered ascending aortic wall shear stress in patients with corrected atrioventricular septal defect: a comprehensive cardiovascular magnetic resonance and 4D flow MRI evaluation. Cardiol Young. 29(5):637-42, 2019
Ye XT et al: Partition of common atrioventricular valve in a patient with dextrocardia and univentricular circulation. Semin Thorac Cardiovasc Surg. 31(1):113-5, 2019
Han J et al: Goose neck appearance: endocardial cushion defect. J Med Imaging Radiat Oncol. 62 Suppl 1:31, 2018
Sarısoy Ö et al: Long-term outcomes in patients who underwent surgical correction for atrioventricular septal defect. Anatol J Cardiol. 20(4):229-34, 2018
Zhu Y et al: Preliminary study of the application of transthoracic echocardiography-guided three-dimensional printing for the assessment of structural heart disease. Echocardiography. 34(12):1903-8, 2017
Davey BT et al: The natural history of atrioventricular valve regurgitation throughout fetal life in patients with atrioventricular canal defects. Pediatr Cardiol. 37(1):50-4, 2016
Saremi F et al: Septal atrioventricular junction region: comprehensive imaging in adults. Radiographics. 36(7):1966-86, 2016
Calkoen EE et al: Characterization and quantification of dynamic eccentric regurgitation of the left atrioventricular valve after atrioventricular septal defect correction with 4D flow cardiovascular magnetic resonance and retrospective valve tracking. J Cardiovasc Magn Reson. 17:18, 2015
Rice K et al: Three-dimensional echocardiography of congenital abnormalities of the left atrioventricular valve. Echo Res Pract. 2(1):R13-24, 2015
Salizzoni S et al: An iatrogenic atrioventricular septal defect that developed following transfemoral TAVI. J Heart Valve Dis. 23(2):216-8, 2014
Richardson, RR: Atlas of Pediatric Cardiac CTA Congenital Heart Disease. New York, NY: Springer, 2013
Lapierre C et al: Segmental approach to imaging of congenital heart disease. Radiographics. 30(2):397-411, 2010
Bell A et al: Noninvasive assessment of pulmonary artery flow and resistance by cardiac magnetic resonance in congenital heart diseases with unrestricted left-to-right shunt. JACC Cardiovasc Imaging. 2(11):1285-91, 2009
Nicol ED et al: Assessment of adult congenital heart disease with multi-detector computed tomography: beyond coronary lumenography. Clin Radiol. 62(6):518-27, 2007
Ten Harkel AD et al: Development of left atrioventricular valve regurgitation after correction of atrioventricular septal defect. Ann Thorac Surg. 79(2):607-12, 2005
Formigari R et al: Better surgical prognosis for patients with complete atrioventricular septal defect and Down's syndrome. Ann Thorac Surg. 78(2):666-72; discussion 672, 2004
Freeman SB et al: Population-based study of congenital heart defects in Down syndrome. Am J Med Genet. 80(3):213-7, 1998
van Son JA et al: Predicting feasibility of biventricular repair of right-dominant unbalanced atrioventricular canal. Ann Thorac Surg. 63(6):1657-63, 1997
Tweddell JS et al: Twenty-year experience with repair of complete atrioventricular septal defects. Ann Thorac Surg. 62(2):419-24, 1996
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Clinical Issues
TERMINOLOGY
Abbreviations
Atrioventricular septal defect (AVSD)
Synonyms
AVSD
Atrioventricular canal (AVC) defect
Endocardial cushion defect
Complete AVC defect
Ostium primum defect
Partial AVC defect
Partial AVSD
Incomplete endocardial cushion defect
Definitions
Broad spectrum of defects characterized by involvement of atrial septum, ventricular septum, & 1 or both AV valves
Complete AVSD indicates presence of both atrial & ventricular septal defects with common AV valve
Partial AVSD indicates atrial septal involvement with separate mitral & tricuspid valve orifices
Unbalanced AVSD indicates 1 ventricular chamber is hypoplastic as compared to other, depending on direction of AV valve flow
IMAGING
General Features
Echocardiographic Findings
CT Findings
MR Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Nayak S et al: Echocardiographic assessment of atrioventricular canal defects. Echocardiography. 37(12):2199-210, 2020
Elders B et al: Altered ascending aortic wall shear stress in patients with corrected atrioventricular septal defect: a comprehensive cardiovascular magnetic resonance and 4D flow MRI evaluation. Cardiol Young. 29(5):637-42, 2019
Ye XT et al: Partition of common atrioventricular valve in a patient with dextrocardia and univentricular circulation. Semin Thorac Cardiovasc Surg. 31(1):113-5, 2019
Han J et al: Goose neck appearance: endocardial cushion defect. J Med Imaging Radiat Oncol. 62 Suppl 1:31, 2018
Sarısoy Ö et al: Long-term outcomes in patients who underwent surgical correction for atrioventricular septal defect. Anatol J Cardiol. 20(4):229-34, 2018
Zhu Y et al: Preliminary study of the application of transthoracic echocardiography-guided three-dimensional printing for the assessment of structural heart disease. Echocardiography. 34(12):1903-8, 2017
Davey BT et al: The natural history of atrioventricular valve regurgitation throughout fetal life in patients with atrioventricular canal defects. Pediatr Cardiol. 37(1):50-4, 2016
Saremi F et al: Septal atrioventricular junction region: comprehensive imaging in adults. Radiographics. 36(7):1966-86, 2016
Calkoen EE et al: Characterization and quantification of dynamic eccentric regurgitation of the left atrioventricular valve after atrioventricular septal defect correction with 4D flow cardiovascular magnetic resonance and retrospective valve tracking. J Cardiovasc Magn Reson. 17:18, 2015
Rice K et al: Three-dimensional echocardiography of congenital abnormalities of the left atrioventricular valve. Echo Res Pract. 2(1):R13-24, 2015
Salizzoni S et al: An iatrogenic atrioventricular septal defect that developed following transfemoral TAVI. J Heart Valve Dis. 23(2):216-8, 2014
Richardson, RR: Atlas of Pediatric Cardiac CTA Congenital Heart Disease. New York, NY: Springer, 2013
Lapierre C et al: Segmental approach to imaging of congenital heart disease. Radiographics. 30(2):397-411, 2010
Bell A et al: Noninvasive assessment of pulmonary artery flow and resistance by cardiac magnetic resonance in congenital heart diseases with unrestricted left-to-right shunt. JACC Cardiovasc Imaging. 2(11):1285-91, 2009
Nicol ED et al: Assessment of adult congenital heart disease with multi-detector computed tomography: beyond coronary lumenography. Clin Radiol. 62(6):518-27, 2007
Ten Harkel AD et al: Development of left atrioventricular valve regurgitation after correction of atrioventricular septal defect. Ann Thorac Surg. 79(2):607-12, 2005
Formigari R et al: Better surgical prognosis for patients with complete atrioventricular septal defect and Down's syndrome. Ann Thorac Surg. 78(2):666-72; discussion 672, 2004
Freeman SB et al: Population-based study of congenital heart defects in Down syndrome. Am J Med Genet. 80(3):213-7, 1998
van Son JA et al: Predicting feasibility of biventricular repair of right-dominant unbalanced atrioventricular canal. Ann Thorac Surg. 63(6):1657-63, 1997
Tweddell JS et al: Twenty-year experience with repair of complete atrioventricular septal defects. Ann Thorac Surg. 62(2):419-24, 1996
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