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Pediatric Complete Atrioventricular Septal Defects Workup

  • Author: Michael D Pettersen, MD; Chief Editor: P Syamasundar Rao, MD  more...
 
Updated: Mar 02, 2016
 

Laboratory Studies

Although basic chemistry panels and the CBC count may aid in overall care, complete atrioventricular septal defect (AVSD) requires no specific laboratory tests.

If Down syndrome or another chromosomal abnormality is suspected, chromosome studies are indicated.

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Electrocardiography

Electrocardiography reveals many typical findings and may provide clues to the presence of complete atrioventricular septal defect. The underlying rhythm most is often sinus. The PR interval may be prolonged secondary to atrial enlargement and increased atrial conduction time. The p-wave may be indicative of right atrial, left atrial, or biatrial enlargement.

The QRS complex reveals the most characteristic findings of atrioventricular septal defect. Posterior displacement of the atrioventricular node and His bundle results in left axis deviation with a superiorly oriented QRS frontal plane axis and counterclockwise depolarization pattern. The QRS frontal axis is usually between -30º and -90º. Right ventricular volume and pressure overload leads to evidence of right ventricular hypertrophy and the presence of an rsR’ or RSR’ pattern in the right precordial chest leads. Left ventricular hypertrophy may be present in the setting of significant mitral or common atrioventricular valve regurgitation.

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Imaging Studies

Chest radiography shows enlargement of the cardiac silhouette. Enlargement of the right atrium and right ventricle is most apparent. Evaluation of the left ventricle may be difficult because it is often displaced by the enlarged right ventricle. The main pulmonary artery segments are prominent, as well as the overall pulmonary vascular markings. In the setting of pulmonary vascular disease, the distal pulmonary vessels may have a lucent, pruned appearance.

Echocardiography reveals defects of the atrial and ventricular septae.[28, 29, 30]

The subcostal 4-chamber and long axial oblique (modified left oblique) views reveal many important aspects of complete atrioventricular septal defect, including the size of the atrial and ventricular defects, the nature of the atrioventricular valve attachments, the distribution of atrioventricular valve tissue, and the left ventricular (LV) outflow tract (LVOT).

The videos below demonstrate echocardiographic findings:

Apical 4-chamber echocardiographic image demonstrating a complete atrioventricular septal defect. A large primum atrial septal defect, a large inlet ventricular septal defect, and a single common orifice atrioventricular valve are noted.
Apical 4-chamber echocardiographic image with color Doppler demonstrating moderately-severe insufficiency of the common atrioventricular valve.
Parasternal long axis echocardiographic image of a complete atrioventricular septal defect. A large inlet ventricular septal defect is seen. Accessory atrioventricular valve tissue is visualized within the left ventricular outflow tract.
Subcostal sagittal echocardiographic image demonstrating the common atrioventricular valve. The anterior bridging leaflet inserts onto the interventricular septum consistent with a Rastelli type A valve.

Other anatomic features, such as ventricular size, atrioventricular valve insufficiency, aortic arch anatomy, and a patent ductus arteriosus (PDA), may be accurately assessed with echocardiography, especially in the infant.

Echocardiography also can reveal a single LV papillary muscle, which may influence the success of mitral reconstruction.

In some centers, 3-dimensional (3D) reconstructions of echocardiographic images are used to evaluate atrioventricular valve morphology, and proponents claim increased diagnostic accuracy with this technique compared with transthoracic echocardiography.[31, 32, 33]

Abnormal atrioventricular valve leaflets may be classified into the following 3 types:

  • Rastelli type A involves minimal bridging of the superior cushion-derived leaflet and attachment of the leftward component of the anterior bridging leaflet to the crest of the interventricular septum.
  • Rastelli type B is rare and involves chordal support of the anterior bridging leaflet attaching to the body of the right ventricle (RV).
  • Rastelli type C valve has a free-floating anterior bridging leaflet that is attached at its rightmost extent to the anterior papillary muscle of the RV.

Doppler echocardiography can reveal common atrioventricular valve regurgitation as well as the flow through the atrial and ventricular septal defects (VSDs).

Hemodynamic information, such estimated RV and pulmonary artery pressure, may be obtained.

Many clinicians believe that a preoperative echocardiogram with Doppler and color flow mapping provides sufficient anatomic and functional information for young infants undergoing repair and that cardiac catheterization may yield little additional information. Other diagnostic tools are occasionally used to diagnose atrioventricular canal defects.

The complete form of atrioventricular canal can be prenatally diagnosed by performing fetal echocardiography. Because two thirds of neonates with complete atrioventricular septal defect also have trisomy 21, this finding by fetal echocardiography should prompt a search for associated chromosomal abnormalities, especially Down syndrome. Fetuses with complete atrioventricular septal defect may develop hydrops fetalis if insufficiency of the common atrioventricular valve is severe.

Transesophageal echocardiography (TEE) is extremely valuable in the large child or adult patient in whom transthoracic echocardiographic windows are limited. It is also ideal for intraoperative evaluation at the time of repair in infancy. TEE provides detailed anatomic information regarding the atrioventricular valves, ventricular function, residual shunts, LVOT obstruction, and atrioventricular valve insufficiency or stenosis.

MRI has been used to identify complete atrioventricular septal defect.

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Procedures

Catheterization is no longer routine for anatomic delineation in many centers. However, when used, it is performed to verify whether the VSD component is nonrestrictive, to determine if additional VSDs are present, to calculate the pulmonary vascular resistance (PVR), and to determine if the pulmonary vascular bed is responsive to pulmonary vasodilators.

The most frequent use of catheterization in common atrioventricular canal is to accurately measure the PVR and, if it is elevated, to evaluate its response to vasodilators, such as oxygen, sodium nitroprusside, calcium-channel blockers, or inhaled nitric oxide.

PVR is calculated as the mean pulmonary artery pressure minus the mean left atrial pressure, divided by the pulmonary blood flow.

Response in the PVR (with oxygen, nitric oxide, or other pulmonary vasodilators) may suggest that a child with high PVR may still benefit from surgery to close atrial and ventricular communications, as outlined above.

Patients with a calculated PVR of 10 Wood units/m2 or greater that does not fall below 5-7 Wood units/m2 in response to vasodilators are at increased risk for death after surgical repair.

In patients younger than 1 year, irreversible pulmonary vascular obstructive disease (PVOD) is rare; hence, PVR data are often ignored.

The second most frequent use for cardiac catheterization is LV angiography to rule out coexisting muscular VSDs.

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Histologic Findings

Complete atrioventricular septal defect is associated with high flow at systemic pressure, which leads to severe hypertrophy of the media of the small arteries of the lung. Intimal fibrosis may also be seen.

Acute fibrous proliferation and atrophy of the peripheral pulmonary arterial media are associated with aging and Down syndrome, which, in addition, reduces the total cross sectional area of the pulmonary vascular bed.

Chronic hypoxemia, upper airway obstruction, and Down syndrome may hasten these vascular changes.

Except in rare cases, surgery within 6 months prevents irreversible PVOD.

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Contributor Information and Disclosures
Author

Michael D Pettersen, MD Consulting Staff, Rocky Mountain Pediatric Cardiology, Pediatrix Medical Group

Michael D Pettersen, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Received income in an amount equal to or greater than $250 from: Fuji Medical Imaging.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Alvin J Chin, MD Emeritus Professor of Pediatrics, University of Pennsylvania School of Medicine

Alvin J Chin, MD is a member of the following medical societies: American Association for the Advancement of Science, Society for Developmental Biology, American Heart Association

Disclosure: Nothing to disclose.

Chief Editor

P Syamasundar Rao, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children's Memorial Hermann Hospital

P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Paul M Seib, MD Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital

Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Drugs & Diseases gratefully acknowledge the contributions of previous authors Michael McConnell, MD, and John Scheitler, MD, to the original writing and development of this article.

References
  1. Van Praagh S, Vangi V, Sul JH, et al. Tricuspid atresia or severe stenosis with partial common atrioventricular canal: anatomic data, clinical profile and surgical considerations. J Am Coll Cardiol. 1991 Mar 15. 17(4):932-43. [Medline].

  2. Wenink AC, Zevallos JC. Developmental aspects of atrioventricular septal defects. Int J Cardiol. 1988 Jan. 18(1):65-78. [Medline].

  3. Moore KL, Persaud TVN. Before We are Born. 5th ed. Philidelphia, PA: WB Saunders; 1998.

  4. Wenink AC, Zevallos JC. Developmental aspects of atrioventricular septal defects. Int J Cardiol. 1988 Jan. 18(1):65-78. [Medline].

  5. Rastelli GC, Kirklin JW, Titus JL. Anatomic observations on complete form of persistent common atrioventricular canal with special reference to atrioventricular valves. Mayo Clin Proc. 1966. 41:296-308.

  6. Bharati S, Lev M. Common Atrioventricular Orifice - Complete Type. The Pathology of Congenital Heart Disease: A Personal Experience with More Than 6,300 Congenitally Malformed Hearts. Armonk, NY: Futura Publinshing Company, Inc; 1996. 553-586.

  7. Karl TR. Atrioventricular septal defect with tetralogy of Fallot or double-outlet right ventricle: surgical considerations. Semin Thorac Cardiovasc Surg. 1997 Jan. 9(1):26-34. [Medline].

  8. Hoffman JIE. Incidence of congenital heart disease: I. Postnatal incidence. Pediatr Cardiol. 1995. 16:103-113.

  9. Rosenthal GL, Wilson PD, Permutt T, Boughman JA, Ferencz C. Birth weight and cardiovascular malformations: a population-based study. The Baltimore-Washington Infant Study. Am J Epidemiol. 1991 Jun 15. 133(12):1273-81. [Medline].

  10. Hoffman JIE. Incidence of congenital heart disease: II. Prenatal incidence. Pediatr Cardiol. 1995. 155-165.

  11. Freeman SB, Taft LF, Dooley KJ, et al. Population-based study of congenital heart defects in Down syndrome. Am J Med Genet. 1998 Nov 16. 80(3):213-7. [Medline].

  12. Digilio MC, Marino B, Cicini MP, et al. Risk of congenital heart defects in relatives of patients with atrioventricular canal. Am J Dis Child. 1993 Dec. 147(12):1295-7. [Medline].

  13. Newfeld EA, Sher M, Paul MH, Nikaidoh H. Pulmonary vascular disease in complete atrioventricular canal defect. Am J Cardiol. 1977 May 4. 39(5):721-6. [Medline].

  14. Kozak MF, Kozak AC, Marchi CH, et al. Factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of repair of complete atrioventricular septal defect. Rev Bras Cir Cardiovasc. 2015 Jul-Sep. 30 (3):304-10. [Medline].

  15. Ginde S, Lam J, Hill GD, et al. Long-term outcomes after surgical repair of complete atrioventricular septal defect. J Thorac Cardiovasc Surg. 2015 Aug. 150 (2):369-74. [Medline].

  16. Harmandar B, Aydemir NA, Karaci AR, Sasmazel A, Saritas T, Bilal MS, et al. Results for surgical correction of complete atrioventricular septal defect: associations with age, surgical era, and technique. J Card Surg. 2012 Nov. 27(6):745-53. [Medline].

  17. Atz AM, Hawkins JA, Lu M, et al. Surgical management of complete atrioventricular septal defect: Associations with surgical technique, age, and trisomy 21. J Thorac Cardiovasc Surg. 2011 Jun. 141(6):1371-9. [Medline]. [Full Text].

  18. Tweddell JS, Litwin SB, Berger S, et al. Twenty-year experience with repair of complete atrioventricular septal defects. Ann Thorac Surg. 1996 Aug. 62(2):419-24. [Medline].

  19. Miller A, Siffel C, Lu C, Riehle-Colarusso T, Frias JL, Correa A. Long-term survival of infants with atrioventricular septal defects. J Pediatr. 2010 Jun. 156(6):994-1000. [Medline].

  20. Bando K, Turrentine MW, Sun K, et al. Surgical management of complete atrioventricular septal defects. A twenty-year experience. J Thorac Cardiovasc Surg. 1995 Nov. 110(5):1543-52; discussion 1552-4. [Medline].

  21. McElhinney DB, Reddy VM, Silverman NH, Hanley FL. Accessory and anomalous atrioventricular valvar tissue causing outflow tract obstruction: surgical implications of a heterogeneous and complex problem. J Am Coll Cardiol. 1998 Nov 15. 32(6):1741-8. [Medline].

  22. Alsoufi B, Al-Halees Z, Khouqeer F, Canver CC, et al. Results of Left Atrioventricular Valve Reoperations Following Previous Repair of Atrioventricular Septal Defects. J Card Surg. 2009 Jun 15. [Medline].

  23. Stulak JM, Burkhart HM, Dearani JA, et al. Reoperations after initial repair of complete atrioventricular septal defect. Ann Thorac Surg. 2009 Jun. 87(6):1872-7; discussion 1877-8. [Medline].

  24. Reeder GS, Danielson GK, Seward JB, et al. Fixed subaortic stenosis in atrioventricular canal defect: a Doppler echocardiographic study. J Am Coll Cardiol. 1992 Aug. 20(2):386-94. [Medline].

  25. Perloff JK. The Clinical Recognition of Congenital Heart Disease. Philadelphia, PA: WB Saunders; 1987. 322-38.

  26. Sheffield VC, et al. Identification of a complex congenital heart defect susceptibility locus by using DNA pooling and shared segment analysis. Hum Molec Genet. 1997. 6(1):117-121.

  27. Vannay A, Vasarhelyi B, Kornyei M, et al. Single-nucleotide polymorphisms of VEGF gene are associated with risk of congenital valvuloseptal heart defects. Am Heart J. 2006 Apr. 151(4):878-81. [Medline]. [Full Text].

  28. Cabrera A, Pastor E, Galdeano JM, et al. Cross-sectional echocardiography in the diagnosis of atrioventricular septal defect. Int J Cardiol. 1990 Jul. 28(1):19-23. [Medline].

  29. Chin AJ, Bierman FZ, Sanders SP, et al. Subxyphoid 2-dimensional echocardiographic identification of left ventricular papillary muscle anomalies in complete common atrioventricular canal. Am J Cardiol. 1983 Jun. 51(10):1695-9. [Medline].

  30. Silverman NH, Zuberbuhler JR, Anderson RH. Atrioventricular septal defects: cross-sectional echocardiographic and morphologic comparisons. Int J Cardiol. 1986 Dec. 13(3):309-31. [Medline].

  31. Del Pasqua A, Sanders SP, de Zorzi A, et al. Impact of three-dimensional echocardiography in complex congenital heart defect cases: the surgical view. Pediatr Cardiol. 2009 Apr. 30(3):293-300. [Medline].

  32. Takahashi K, Guerra V, Roman KS, Nii M, Redington A, Smallhorn JF. Three-dimensional echocardiography improves the understanding of the mechanisms and site of left atrioventricular valve regurgitation in atrioventricular septal defect. J Am Soc Echocardiogr. 2006 Dec. 19(12):1502-10. [Medline].

  33. Takahashi K, Mackie AS, Thompson R, Al-Naami G, Inage A, Rebeyka IM, et al. Quantitative real-time three-dimensional echocardiography provides new insight into the mechanisms of mitral valve regurgitation post-repair of atrioventricular septal defect. J Am Soc Echocardiogr. 2012 Nov. 25(11):1231-44. [Medline].

  34. Najm HK, Coles JG, Endo M, et al. Complete atrioventricular septal defects: results of repair, risk factors, and freedom from reoperation. Circulation. 1997 Nov 4. 96(9 Suppl):II-311-5. [Medline].

  35. Kaza AK, Colan SD, Jaggers J, et al. Surgical interventions for atrioventricular septal defect subtypes: the pediatric heart network experience. Ann Thorac Surg. 2011 Oct. 92(4):1468-75. [Medline].

  36. Ten Harkel AD, Cromme-Dijkhuis AH, Heinerman BC, et al. Development of left atrioventricular valve regurgitation after correction of atrioventricular septal defect. Ann Thorac Surg. 2005 Feb. 79(2):607-12. [Medline].

  37. Suzuki T, Bove EL, Devaney EJ, Ishizaka T, Goldberg CS, Hirsch JC. Results of definitive repair of complete atrioventricular septal defect in neonates and infants. Ann Thorac Surg. 2008 Aug. 86(2):596-602. [Medline].

  38. Myers PO, del Nido PJ, Marx GR, Emani S, Mayer JE Jr, Pigula FA. Improving left ventricular outflow tract obstruction repair in common atrioventricular canal defects. Ann Thorac Surg. 2012 Aug. 94(2):599-605; discussion 605. [Medline].

  39. Buchhorn R, Hulpke-Wette M, Ruschewski W, et al. Effects of therapeutic beta blockade on myocardial function and cardiac remodelling in congenital cardiac disease. Cardiol Young. 2003 Feb. 13(1):36-43. [Medline].

  40. [Guideline] Wilson W, Taubert KA, Gewitz M, et al. Prevention of Infective Endocarditis. Guidelines From the American Heart Association. A Guideline From the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007 Apr 19. [Medline].

  41. Alexi-Meskishvili V, Ishino K, Dahnert I, et al. Correction of complete atrioventricular septal defects with the double-patch technique and cleft closure. Ann Thorac Surg. 1996 Aug. 62(2):519-24; discussion 524-5. [Medline].

  42. Atz AM, Hawkins JA, Lu M, Cohen MS, Colan SD, Jaggers J. Surgical management of complete atrioventricular septal defect: associations with surgical technique, age, and trisomy 21. J Thorac Cardiovasc Surg. 2011 Jun. 141(6):1371-9. [Medline].

  43. Backer CL, Mavroudis C, Alboliras ET, Zales VR. Repair of complete atrioventricular canal defects: results with the two- patch technique. Ann Thorac Surg. 1995 Sep. 60(3):530-7. [Medline].

  44. Capouya ER, Laks H, Drinkwater DC Jr, et al. Management of the left atrioventricular valve in the repair of complete atrioventricular septal defects. J Thorac Cardiovasc Surg. 1992 Jul. 104(1):196-201; discussion 201-3. [Medline].

  45. Cousineua AJ, Lauer RM, Pierpont ME, et al. Linkage analysis of autosomal dominant atrioventricular canal defects: exclusion of chromosome 21. Human Genetics. 1994. 93(2):103-8. [Medline].

  46. DeLeon SY, Ilbawi MN, Wilson WR Jr, et al. Surgical options in subaortic stenosis associated with endocardial cushion defects. Ann Thorac Surg. 1991 Nov. 52(5):1076-82; discussion 1082-3. [Medline].

  47. Eisenberg LM, Markwald RR. Molecular regulation of atrioventricular valvuloseptal morphogenesis. Circ Res. 1995 Jul. 77(1):1-6. [Medline].

  48. Formigari R, Di Donato RM, Gargiulo G, et al. Better surgical prognosis for patients with complete atrioventricular septal defect and Down's syndrome. Ann Thorac Surg. 2004 Aug. 78(2):666-72; discussion 672. [Medline].

  49. Gembruch U, Knopfle G, Chatterjee et al. Prenatal diagnosis of atrioventricular canal malformations with up-to-date echocardiographic technology: report of 14 cases. Am Heart J. 1991 May. 121(5):1489-97. [Medline].

  50. Hanley FL, Fenton KN, Jonas RA, et al. Surgical repair of complete atrioventricular canal defects in infancy. Twenty-year trends. J Thorac Cardiovasc Surg. 1993 Sep. 106(3):387-94; discussion 394-7. [Medline].

  51. Jacobstein MD, Fletcher BD, Goldstein S, Riemenschneider TA. Evaluation of atrioventricular septal defect by magnetic resonance imaging. Am J Cardiol. 1985 Apr 15. 55(9):1158-61. [Medline].

  52. Kosaki K, Curry CJ, Roeder E, Jones KL. Ritscher-Schinzel (3C) syndrome: documentation of the phenotype. Am J Med Genet. 1997 Feb 11. 68(4):421-7. [Medline].

  53. Kurnit DM, Aldridge JF, Matsuoka R, Matthysse S. Increased adhesiveness of trisomy 21 cells and atrioventricular canal malformations in Down syndrome: a stochastic model. Am J Med Genet. 1985 Feb. 20(2):385-99. [Medline].

  54. Lange A, Mankad P, Walayat M, et al. Transthoracic three-dimensional echocardiography in the preoperative assessment of atrioventricular septal defect morphology. Am J Cardiol. 2000 Mar 1. 85(5):630-5. [Medline].

  55. LeBlanc JG, Williams WG, Freedom RM, Trusler GA. Results of total correction in complete atrioventricular septal defects with congenital or surgically induced right ventricular outflow tract obstruction. Ann Thorac Surg. 1986 Apr. 41(4):387-91. [Medline].

  56. Leversha AM, Wilson NJ, Clarkson PM, et al. Efficacy and dosage of enalapril in congenital and acquired heart disease. Arch Dis Child. 1994 Jan. 70(1):35-9. [Medline].

  57. Maslen CL. Molecular genetics of atrioventricular septal defects. Curr Opin Cardiol. 2004 May. 19(3):205-10. [Medline].

  58. Miyamura H, Eguchi S, Watanabe H, et al. Total circular annuloplasty with absorbable suture for the repair of left atrioventricular valve regurgitation in atrioventricular septal defect. J Thorac Cardiovasc Surg. 1994 Jun. 107(6):1428-31. [Medline].

  59. Rizzoli G, Mazzucco A, Maizza F, et al. Does Down syndrome affect prognosis of surgically managed atrioventricular canal defects?. J Thorac Cardiovasc Surg. 1992 Oct. 104(4):945-53. [Medline].

  60. Roach RM, Tandon R, Moller JH, Edwards JE. Ebstein's anomaly of the tricuspid valve in persistent common atrioventricular canal. Am J Cardiol. 1984 Feb 1. 53(4):640-2. [Medline].

  61. Rosenthal GL, Wilson PD, Permutt T, et al. Birth weight and cardiovascular malformations: a population-based study. The Baltimore-Washington Infant Study. Am J Epidemiol. 1991 Jun 15. 133(12):1273-81. [Medline].

  62. Ross DA, Nanton M, Gillis DA, Murphy DA. Atrioventricular canal defects: results of repair in the current era. J Card Surg. 1991 Sep. 6(3):367-72. [Medline].

  63. Sakamoto K, Galletti L, Touchot A, et al. Two-stage correction of transposition of great arteries with complete atrioventricular canal. Ann Thorac Surg. 1998 Jan. 65(1):250-2. [Medline].

  64. Sigfusson G, Ettedgui JA, Silverman NH, Anderson RH. Is a cleft in the anterior leaflet of an otherwise normal mitral valve an atrioventricular canal malformation?. J Am Coll Cardiol. 1995 Aug. 26(2):508-15. [Medline].

  65. Sim EK, Black MD, Smallhorn J, et al. Surgical repair of complete atrioventricular septal canal defects with absent posterior leaflet. Ann Thorac Surg. 1995 Nov. 60(5):1399-400. [Medline].

  66. Tedziagolska M. Two-patch repair of atrioventricular canal. Ann Thorac Surg. 1996 May. 61(5):1589-90. [Medline].

  67. Tennant SN, Hammon JW Jr, Bender HW Jr, et al. Familial clustering of atrioventricular canal defects. Am Heart J. 1984 Jul. 108(1):175-7. [Medline].

  68. Torfs CP, Christianson RE. Anomalies in Down syndrome individuals in a large population-based registry. Am J Med Genet. 1998 Jun 5. 77(5):431-8. [Medline].

  69. van Son JA, Kinzel P, Mohr FW. Repair of Ebstein's anomaly associated with partial atrioventricular canal. J Card Surg. 1997 Nov-Dec. 12(6):434-6. [Medline].

  70. van Son JA, Van Praagh R, Falk V, Mohr FW. Pericardial patch augmentation of the tissue-deficient mitral valve in common atrioventricular canal. J Thorac Cardiovasc Surg. 1996 Oct. 112(4):1117-9. [Medline].

  71. Yamaki S, Yasui H, Kado H, et al. Pulmonary vascular disease and operative indications in complete atrioventricular canal defect in early infancy. J Thorac Cardiovasc Surg. 1993 Sep. 106(3):398-405. [Medline].

  72. Zellers TM, Zehr R, Weinstein E, et al. Two-dimensional and Doppler echocardiography alone can adequately define preoperative anatomy and hemodynamic status before repair of complete atrioventricular septal defect in infants 111J Am Coll Cardiol</i>. 1994 Nov 15. 24(6):1565-70. [Medline].

  73. St Louis JD, Jodhka U, Jacobs JP, et al. Contemporary outcomes of complete atrioventricular septal defect repair: analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. J Thorac Cardiovasc Surg. 2014 Dec. 148(6):2526-31. [Medline].

  74. Zhong PP, Gu YQ, Wang AC, et al. [Complete atrioventricular septal defect: a clinicopathologic study of 35 cases] [Chinese]. Zhonghua Bing Li Xue Za Zhi. 2016 Feb 8. 45 (2):107-10. [Medline].

 
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Atrioventricular (A-V) valve leaflets viewed from the cardiac apex in normal valves (A) and in the Rastelli type A complete form of common A-V canal (B). In A, the normal tricuspid valve (TV) has anterior (AL), septal (SL), and posterior (PL) leaflets. A normal mitral valve (MV) has ALs and PLs.In B, the superior cushion–derived leaflet bridges the ventricular septum and attaches to the papillary muscle of the conus at its rightmost extent. A right superior leaflet (RSL) typically attaches to the papillary muscle of the conus and to the anterior papillary muscle of the right ventricle (RV), and a right lateral leaflet (RLL) attaches to the anterior papillary muscle of the RV and to the posterior papillary muscle of the RV. The inferior cushion–derived bridging leaflet is usually cleft, giving the appearance of a right inferior leaflet (RIL) and a left inferior leaflet (LIL).
Apical 4-chamber echocardiographic image demonstrating a complete atrioventricular septal defect. A large primum atrial septal defect, a large inlet ventricular septal defect, and a single common orifice atrioventricular valve are noted.
Apical 4-chamber echocardiographic image with color Doppler demonstrating moderately-severe insufficiency of the common atrioventricular valve.
Parasternal long axis echocardiographic image of a complete atrioventricular septal defect. A large inlet ventricular septal defect is seen. Accessory atrioventricular valve tissue is visualized within the left ventricular outflow tract.
Subcostal sagittal echocardiographic image demonstrating the common atrioventricular valve. The anterior bridging leaflet inserts onto the interventricular septum consistent with a Rastelli type A valve.
 
 
 
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