Laboratory Studies

Routine preoperative studies (eg, complete blood cell [CBC] count, platelet counts, electrolyte levels, blood urea nitrogen [BUN] levels, creatinine levels) are indicated. Typing and crossmatching blood are necessary for cardiopulmonary bypass preparation.

Arterial blood gas (ABG) determinations are not routinely warranted; however, cyanosis alerts the physician to the possibility of pulmonary vascular obstructive disease in older patients or concurrent right-sided obstructive lesions.

Chest radiography

In incomplete atrioventricular (AV) septal defects (AVSDs), chest radiographs usually reveal mild cardiomegaly and increased pulmonary vascular markings. In complete AVSDs, Significant cardiomegaly and pulmonary overcirculation are depicted on chest radiographs.

Doppler echocardiography

In incomplete AVSDs, Doppler echocardiography findings are diagnostic of the atrial defect, the absence of ventricular level shunting, and the presence of any AV valve abnormalities. In complete AVSDs, Doppler echocardiography findings are diagnostic, defining the atrial and ventricular level shunting, valvular anatomy, and any associated anomalies.

Electrocardiography

In incomplete atrioventricular (AV) septal defects (AVSDs), electrocardiography (ECG) reveals left axis deviation, prominent P waves associated with atrial enlargement, and a prolonged PR interval. In complete AVSDs, ECG reveals biventricular hypertrophy, atrial enlargement, prolonged PR interval, leftward axis, and counterclockwise frontal plane loop.

Cardiac catheterization

In incomplete atrioventricular (AV) septal defects (AVSDs), cardiac catheterization is indicated only in adults with a diagnosis of incomplete AVSDs or in patients manifesting physical or radiologic signs of decreased pulmonary blood flow. Decreased pulmonary artery blood flow may be a result of pulmonary vascular disease or concurrent right-sided obstructive lesions. High fraction of inspired oxygen (FiO₂) and nitric oxide may be needed to assess the reversibility of increased pulmonary vascular resistance.

In complete AVSD, perform cardiac catheterization for patients older than 1 year, patients with signs or symptoms of increased pulmonary vascular resistance, or in some individuals to further evaluate other associated major cardiac anomalies. High FiO₂ and nitric oxide may be needed to assess the reversibility of increased pulmonary vascular resistance.

Treatment & Management

Christensen N, Andersen H, Garne E, et al. Atrioventricular septal defects among infants in Europe: a population-based study of prevalence, associated anomalies, and survival. Cardiol Young. 2013 Aug. 23 (4):560-7. [QxMD MEDLINE Link].
Muresan D, Marginean C, Zaharie G, Stamatian F, Rotar IC. Complete atrioventricular septal defect in the era of prenatal diagnosis. Med Ultrason. 2016 Dec 5. 18 (4):500-7. [QxMD MEDLINE Link].
Spicer RL. Cardiovascular disease in Down syndrome. Pediatr Clin North Am. 1984 Dec. 31(6):1331-43. [QxMD MEDLINE Link].
Lillehei CW, Cohen M, Warden HE. The direct-vision intracardiac correction of congenital anomalies by controlled cross-circulation: results in thirty-two patients with ventricular septal defects, tetralogy of Fallot, and atrioventicularis communis defects. Surgery. 1955. 38:11.
Lev M. The architecture of the conduction system in congenital heart disease: I. Common atrioventricular orifice. Arch Pathol. 1958. 65:174.
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. [QxMD MEDLINE Link].
Rastelli G, Kirklin JW, Titus JL. Anatomic observations on complete form of persistent common atrioventricular canal with special reference to atrioventricular valves. Mayo Clin Proc. 1966 May. 41(5):296-308. [QxMD MEDLINE Link].
Studer M, Blackstone EH, Kirklin JW, et al. Determinants of early and late results of repair of atrioventricular septal (canal) defects. J Thorac Cardiovasc Surg. 1982 Oct. 84(4):523-42. [QxMD MEDLINE Link].
Piccoli GP, Wilkinson JL, Macartney FJ, et al. Morphology and classification of complete atrioventricular defects. Br Heart J. 1979 Dec. 42(6):633-9. [QxMD MEDLINE Link].
Bharati S, Kirklin JW, McAllister HA Jr, Lev M. The surgical anatomy of common atrioventricular orifice associated with tetralogy of Fallot, double outlet right ventricle and complete regular transposition. Circulation. 1980 Jun. 61(6):1142-9. [QxMD MEDLINE Link].
Draulans-Noe HA, Wenink AC, Quaegebeur J. Single papillary muscle ("parachute valve") and double-orifice left ventricle in atrioventricular septal defect convergence of chordal attachment: surgical anatomy and results of surgery. Pediatr Cardiol. 1990 Jan. 11(1):29-35. [QxMD MEDLINE Link].
Harmandar B, Aydemir NA, Karaci AR, 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. [QxMD MEDLINE Link].
Puga FJ. Reoperation after repair of atrioventricular canal defects. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 1998. 1:123-8. [QxMD MEDLINE Link].
Stewart S, Alexson C, Manning J. Partial atrioventricular canal defect: the early and late results of operation. Ann Thorac Surg. 1987 May. 43(5):527-9. [QxMD MEDLINE Link].
Nunn GR. Atrioventricular canal: modified single patch technique. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2007. 28-31. [QxMD MEDLINE Link].
McElhinney DB, Reddy VM, Silverman NH, et al. Atrioventricular septal defect with common valvar orifice and tetralogy of Fallot revisited: making a case for primary repair in infancy. Cardiol Young. 1998 Oct. 8(4):455-61. [QxMD MEDLINE Link].
Najm HK, Van Arsdell GS, Watzka S, et al. Primary repair is superior to initial palliation in children with atrioventricular septal defect and tetralogy of Fallot. J Thorac Cardiovasc Surg. 1998 Dec. 116(6):905-13. [QxMD MEDLINE Link].
Ong J, Brizard CP, d'Udekem Y, et al. Repair of atrioventricular septal defect associated with tetralogy of Fallot or double-outlet right ventricle: 30 years of experience. Ann Thorac Surg. 2012 Jul. 94 (1):172-8. [QxMD MEDLINE Link].
Vitanova K, Cleuziou J, Schreiber C, et al. Long-term outcome of patients with complete atrioventricular septal defect combined with the tetralogy of Fallot: staged repair is not inferior to primary repair. Ann Thorac Surg. 2017 Mar. 103 (3):876-80. [QxMD MEDLINE Link].
Buratto E, Ye XT, King G, et al. Long-term outcomes of single-ventricle palliation for unbalanced atrioventricular septal defects: Fontan survivors do better than previously thought. J Thorac Cardiovasc Surg. 2017 Feb. 153 (2):430-8. [QxMD MEDLINE Link].
Arunamata A, Balasubramanian S, Mainwaring R, Maeda K, Selamet Tierney ES. Right-dominant unbalanced atrioventricular septal defect: echocardiography in surgical decision making. J Am Soc Echocardiogr. 2017 Mar. 30 (3):216-26. [QxMD MEDLINE Link].
Minich LL, Tani LY, Pagotto LT, et al. Size of ventricular structures influences surgical outcome in Down syndrome infants with atrioventricular septal defect. Am J Cardiol. 1998 Apr 15. 81(8):1062-5. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Prifti E, Bonacchi M, Baboci A, Giunti G, Krakulli K, Vanini V. Surgical outcome of reoperation due to left atrioventricular valve regurgitation after previous correction of complete atrioventricular septal defect. J Card Surg. 2013 Nov. 28 (6):756-63. [QxMD MEDLINE Link].
Vida VL, Tessari C, Castaldi B, et al. Early correction of common atrioventricular septal defects: a single-center 20-year experience. Ann Thorac Surg. 2016 Dec. 102 (6):2044-51. [QxMD MEDLINE Link].
Buratto E, Ye XT, Bullock A, et al. Long-term outcomes of reoperations following repair of partial atrioventricular septal defect. Eur J Cardiothorac Surg. 2016 Aug. 50 (2):293-7. [QxMD MEDLINE Link].
Kadoba K, Jonas RA, Mayer JE, Castaneda AR. Mitral valve replacement in the first year of life. J Thorac Cardiovasc Surg. 1990 Nov. 100(5):762-8. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Berger TJ, Kirklin JW, Blackstone EH, et al. Primary repair of complete atrioventricular canal in patients less than 2 years old. Am J Cardiol. 1978 May 1. 41(5):906-13. [QxMD MEDLINE Link].
Reddy VM, McElhinney DB, Brook MM, et al. Atrioventricular valve function after single patch repair of complete atrioventricular septal defect in infancy: how early should repair be attempted?. J Thorac Cardiovasc Surg. 1998 May. 115(5):1032-40. [QxMD MEDLINE Link].
Michielon G, Stellin G, Rizzoli G, Casarotto DC. Repair of complete common atrioventricular canal defects in patients younger than four months of age. Circulation. 1997 Nov 4. 96(9 Suppl):II-316-22. [QxMD MEDLINE Link].
Vet TW, Ottenkamp J. Correction of atrioventricular septal defect. Results influenced by Down syndrome?. Am J Dis Child. 1989 Nov. 143(11):1361-5. [QxMD MEDLINE Link].
Suzuki T, Bove EL, Devaney EJ, et al. Results of definitive repair of complete atrioventricular septal defect in neonates and infants. Ann Thorac Surg. 2008 Aug. 86(2):596-602. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link]. [Full Text].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Karatza AA, Bush A, Magee AG. Safety and efficacy of Sildenafil therapy in children with pulmonary hypertension. International Journal of Cardiology. 2005. 100:267-273. [QxMD MEDLINE Link].
Nicholson IA, Nunn GR, Sholler GF, et al. Simplified single patch technique for the repair of atrioventricular septal defect. J Thorac Cardiovasc Surg. 1999 Oct. 118(4):642-6. [QxMD MEDLINE Link].
Thies WR, Breymann T, Matthies W, et al. Primary repair of complete atrioventricular septal defect in infancy. Eur J Cardiothorac Surg. 1991. 5(11):571-4. [QxMD MEDLINE Link].
El-Rassi I, Charafedine F, Majdalani M, Arabi M, Khater D, Bitar F. Surgical repair of partial atrioventricular defect. Multimed Man Cardiothorac Surg. 2015. 2015:[QxMD MEDLINE Link].
Gao C, Yang M, Xiao C, Zhang H. Totally robotic repair of atrioventricular septal defect in the adult. J Cardiothorac Surg. 2015 Nov 6. 10:156. [QxMD MEDLINE Link].
Ghosh P, Bhaumik P, Ghosh S, Ozbek U, Feingold E, Maslen C, et al. Polymorphic haplotypes of CRELD1 differentially predispose Down syndrome and euploids individuals to atrioventricular septal defect. Am J Med Genet A. 2012 Nov. 158A (11):2843-8. [QxMD MEDLINE Link].
Cohen MS, Jegatheeswaran A, Baffa JM, et al. Echocardiographic features defining right dominant unbalanced atrioventricular septal defect: a multi-institutional Congenital Heart Surgeons' Society study. Circ Cardiovasc Imaging. 2013 Jul. 6 (4):508-13. [QxMD MEDLINE Link].
Backer CL, Eltayeb O, Monge MC, Mazwi ML, Costello JM. Shunt lesions part I: patent ductus arteriosus, atrial septal defect, ventricular septal defect, and atrioventricular septal defect. Pediatr Crit Care Med. 2016 Aug. 17 (8 suppl 1):S302-9. [QxMD MEDLINE Link].
Devlin PJ, Backer CL, Eltayeb O, Monge MC, Hauck AL, Costello JM. Repair of partial atrioventricular septal defect: age and outcomes. Ann Thorac Surg. 2016 Jul. 102 (1):170-7. [QxMD MEDLINE Link].

Media Gallery

(a) An incomplete atrioventricular septal defect (AVSD) with right superior leaflet (RSL), right lateral leaflet (RLL), right inferior leaflet (RIL), left superior leaflet (LSL), left lateral leaflet (LLL), and left inferior leaflet (LIL). (b) A complete ASD with superior bridging leaflet (SBL), inferior bridging leaflet (IBL), LLL, RSL, and RIL. The locations of the atrioventricular (AV) node and bundle of His are indicated. All images are surgeon's-eye views with cranial leftward, caudad rightward, superior upward, and posterior downward.
Rastelli classification. (a) Rastelli type A. (b) Rastelli type B. (c) Rastelli type C.
The common atrioventricular (AV) valve is floated to a closed position using isotonic sodium chloride solution. The central apposition points of the superior and inferior bridging leaflets are identified and marked with fine polypropylene sutures.
Two-patch technique. A patch of polytetrafluoroethylene (Gore-Tex) is fashioned and secured along the crest of the ventricular septal defect.
Two-patch technique. Interrupted horizontal mattress sutures are placed through the crest of the ventricular septal defect (VSD) patch and the inferior and superior bridging leaflets, dividing the common atrioventricular (AV) valve into right and left components.
Two-patch technique. The pericardial patch is secured to the crest of the prosthetic ventricular septum with the superior and inferior bridging leaflet sandwiched between the 2 patches.
One-patch technique. The superior and inferior bridging leaflets are divided into right and left components.
One-patch technique. The leaflets are resuspended to the patch by passing sutures through the cut edge of the left atrioventricular (AV) valve leaflet, the patch, and the cut edge of the right AV valve and tying the sutures.
The cleft of the mitral valve between the superior and inferior bridging leaflets is closed.
The atrial septal defect (ASD) is closed with an autologous pericardial patch. The coronary sinus is placed in the left atrium to avoid injury to the conduction system. The rim of the ASD, the atrioventricular (AV) node, and the bundle of His are indicated. The dashes represent the proposed suture line.