Management and outcome of atrioventricular (AV) canal defects
- Craig E Fleishman, MD
Craig E Fleishman, MD
- Associate Professor of Pediatrics
- University of Central Florida College of Medicine
- Aykut Tugertimur, MD, FACC
Aykut Tugertimur, MD, FACC
- Attending Pediatric Cardiologist
- Assistant Professor of Pediatrics
- Hofstra Northwell School of Medicine
- Section Editors
- John K Triedman, MD
John K Triedman, MD
- Section Editor — Pediatric Cardiology
- Professor of Pediatrics
- Harvard Medical School
- Heidi M Connolly, MD, FASE
Heidi M Connolly, MD, FASE
- Section Editor — Congenital Heart Disease
- Professor of Medicine
- Mayo Medical School
Atrioventricular (AV) canal defects are a group of congenital cardiac defects involving, to varying extent, the AV septum and AV valves (ie, mitral and tricuspid valves). They are also referred to as AV septal defects, endocardial cushion defects, or persistent AV ostium.
The management and outcome of patients with AV canal defects will be reviewed here. The anatomy, pathophysiology, clinical features, and diagnosis of AV canal defects are discussed separately. (See "Clinical manifestations, pathophysiology, and diagnosis of atrioventricular (AV) canal defects".)
VARIOUS FORMS OF AV CANAL DEFECTS
●Complete AV canal defect is a result of complete failure of fusion between endocardial cushions. It is characterized by a primum atrial septal defect (ASD) that is contiguous with a ventricular septal defect (VSD) and a common AV valve.
●Partial AV canal defect is due to incomplete fusion of the endocardial cushions and consists of a primum ASD and a common valvular annulus with two separate valve orifices. Due to abnormal fusion of the left tubercle of the superior and inferior cushions, the anterior leaflet of the mitral valve is cleft, which is often associated with mitral valve regurgitation.
Subscribers log in hereLiterature review current through: Nov 2017. | This topic last updated: Feb 09, 2016.References
- 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; 106:387.
- Crawford FA Jr, Stroud MR. Surgical repair of complete atrioventricular septal defect. Ann Thorac Surg 2001; 72:1621.
- Backer CL, Stewart RD, Bailliard F, et al. Complete atrioventricular canal: comparison of modified single-patch technique with two-patch technique. Ann Thorac Surg 2007; 84:2038.
- Singh RR, Warren PS, Reece TB, et al. Early repair of complete atrioventricular septal defect is safe and effective. Ann Thorac Surg 2006; 82:1598.
- Masuda M, Kado H, Tanoue Y, et al. Does Down syndrome affect the long-term results of complete atrioventricular septal defect when the defect is repaired during the first year of life? Eur J Cardiothorac Surg 2005; 27:405.
- LILLEHEI CW, COHEN M, WARDEN HE, VARCO RL. 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 atrioventricularis communis defects. Surgery 1955; 38:11.
- Backer CL, Stewart RD, Mavroudis C. What is the best technique for repair of complete atrioventricular canal? Semin Thorac Cardiovasc Surg 2007; 19:249.
- Wilcox BR, Jones DR, Frantz EG, et al. Anatomically sound, simplified approach to repair of "complete" atrioventricular septal defect. Ann Thorac Surg 1997; 64:487.
- 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; 141:1371.
- Complete atrioventricular canal. In: Comprehensive Surgical Management of Congenital Heart Disease, Jonas RA, DiNardo J, Laussen PC, et al. (Eds), 2004. p.397.
- Nunn GR. Atrioventricular canal: modified single patch technique. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2007; :28.
- Carpentier A. Surgical anatomy and management of the mitral component of atrioventricular canal defects. In: Paediatric Cardiology, Anderson RH, Shinebourne EA (Eds), Churchill Livingstone, Edinburgh 1978. p.477.
- Wetter J, Sinzobahamvya N, Blaschczok C, et al. Closure of the zone of apposition at correction of complete atrioventricular septal defect improves outcome. Eur J Cardiothorac Surg 2000; 17:146.
- Sharma V, Burkhart HM, Schaff HV, et al. Management of zone of apposition in parachute left atrioventricular valve in atrioventricular septal defect. Ann Thorac Surg 2013; 95:1665.
- Cohen MS, Jacobs ML, Weinberg PM, Rychik J. Morphometric analysis of unbalanced common atrioventricular canal using two-dimensional echocardiography. J Am Coll Cardiol 1996; 28:1017.
- Jegatheeswaran A, Pizarro C, Caldarone CA, et al. Echocardiographic definition and surgical decision-making in unbalanced atrioventricular septal defect: a Congenital Heart Surgeons' Society multiinstitutional study. Circulation 2010; 122:S209.
- Szwast AL, Marino BS, Rychik J, et al. Usefulness of left ventricular inflow index to predict successful biventricular repair in right-dominant unbalanced atrioventricular canal. Am J Cardiol 2011; 107:103.
- 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; 6:508.
- SOMERVILLE J. OSTIUM PRIMUM DEFECT: FACTORS CAUSING DETERIORATION IN THE NATURAL HISTORY. Br Heart J 1965; 27:413.
- Minich LL, Atz AM, Colan SD, et al. Partial and transitional atrioventricular septal defect outcomes. Ann Thorac Surg 2010; 89:530.
- Morris CD, Magilke D, Reller M. Down's syndrome affects results of surgical correction of complete atrioventricular canal. Pediatr Cardiol 1992; 13:80.
- Hill NS, Roberts KR, Preston IR. Postoperative pulmonary hypertension: etiology and treatment of a dangerous complication. Respir Care 2009; 54:958.
- Wessel DL, Adatia I, Giglia TM, et al. Use of inhaled nitric oxide and acetylcholine in the evaluation of pulmonary hypertension and endothelial function after cardiopulmonary bypass. Circulation 1993; 88:2128.
- Backer CL, Mavroudis C, Alboliras ET, Zales VR. Repair of complete atrioventricular canal defects: results with the two-patch technique. Ann Thorac Surg 1995; 60:530.
- Ashraf MH, Amin Z, Sharma R, Subramanian S. Atrioventricular canal defect: two-patch repair and tricuspidization of the mitral valve. Ann Thorac Surg 1993; 55:347.
- Baslaim G, Basioni A. Repair of complete atrioventricular septal defects: results with maintenance of the coronary sinus on the right atrial side. J Card Surg 2006; 21:545.
- Murashita T, Kubota T, Oba J, et al. Left atrioventricular valve regurgitation after repair of incomplete atrioventricular septal defect. Ann Thorac Surg 2004; 77:2157.
- 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; 79:607.
- Michielon G, Stellin G, Rizzoli G, et al. Left atrioventricular valve incompetence after repair of common atrioventricular canal defects. Ann Thorac Surg 1995; 60:S604.
- Birim O, van Gameren M, de Jong PL, et al. Outcome after reoperation for atrioventricular septal defect repair. Interact Cardiovasc Thorac Surg 2009; 9:83.
- Stulak JM, Burkhart HM, Dearani JA, et al. Reoperations after initial repair of complete atrioventricular septal defect. Ann Thorac Surg 2009; 87:1872.
- Permut LC, Mehta V. Late results and reoperation after repair of complete and partial atrioventricular canal defect. Semin Thorac Cardiovasc Surg 1997; 9:44.
- Lee HR, Montenegro LM, Nicolson SC, et al. Usefulness of intraoperative transesophageal echocardiography in predicting the degree of mitral regurgitation secondary to atrioventricular defect in children. Am J Cardiol 1999; 83:750.
- Kim HK, Kim WH, Hwang SW, et al. Predictive value of intraoperative transesophageal echocardiography in complete atrioventricular septal defect. Ann Thorac Surg 2005; 80:56.
- 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; 148:2526.
- Stulak JM, Burkhart HM, Dearani JA, et al. Reoperations after repair of partial atrioventricular septal defect: a 45-year single-center experience. Ann Thorac Surg 2010; 89:1352.
- Pontailler M, Kalfa D, Garcia E, et al. Reoperations for left atrioventricular valve dysfunction after repair of atrioventricular septal defect. Eur J Cardiothorac Surg 2014; 45:557.
- Ginde S, Lam J, Hill GD, et al. Long-term outcomes after surgical repair of complete atrioventricular septal defect. J Thorac Cardiovasc Surg 2015; 150:369.
- Alsoufi B, Al-Halees Z, Khouqeer F, et al. Results of left atrioventricular valve reoperations following previous repair of atrioventricular septal defects. J Card Surg 2010; 25:74.
- Rhodes J, Warner KG, Fulton DR, et al. Fate of mitral regurgitation following repair of atrioventricular septal defect. Am J Cardiol 1997; 80:1194.
- Van Hare GF, Ackerman MJ, Evangelista JA, et al. Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 4: Congenital Heart Disease: A Scientific Statement From the American Heart Association and American College of Cardiology. Circulation 2015; 132:e281.
- Marino BS, Lipkin PH, Newburger JW, et al. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation 2012; 126:1143.
- VARIOUS FORMS OF AV CANAL DEFECTS
- OVERVIEW OF MANAGEMENT
- COMPLETE AV CANAL DEFECT
- Initial medical management
- Surgical interventions
- - Primary complete repair
- Surgical timing
- Surgical procedures
- Cleft AV valve
- - Single ventricle palliation
- PARTIAL AND TRANSITIONAL AV CANAL DEFECT
- Postoperative complications
- - Pulmonary hypertension
- - Arrhythmias
- - AV valve complications
- AV valve regurgitation
- AV valve stenosis
- Transesophageal echocardiography
- Long-term outcome
- FOLLOW-UP CARE
- Physical examination
- Endocarditis prophylaxis
- Neurodevelopmental outcome
- SUMMARY AND RECOMMENDATIONS