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Atrioventricular canal defects

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Opinion statement

Atrioventricular (AV) canal defects should be diagnosed by means of echocardiography. Infants with complete AV canal defects should undergo intracardiac surgical repair before pulmonary vascular obstructive disease develops, preferably before 6 months of age. Repair of partial AV canal defects or primum atrial septal defects can be postponed until 18 to 36 months of age if the patient does not have pulmonary hypertension or growth failure. The one- and two-patch techniques can both provide excellent results with a low mortality rate in repair of complete AV canal defects. Repair of the cleft mitral valve at the time of initial surgery is advised to preserve long-term mitral valve function. Tetralogy of Fallot with AV canal defect can also be repaired but at an older age and with a greater incidence of reoperation. Unbalanced AV canal defects associated with a hypoplastic ventricle may necessitate initial pulmonary artery banding and a subsequent decision about the suitability of two-ventricle repair. If ventricular septation is unsuitable, a single-ventricle path is chosen. Bidirectional cavopulmonary anastomosis and, eventually, the Fontan procedure are used.

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References and Recommended Reading

  1. Rastelli GC, Ongley PA, Kirklin JW, McGoon DC: Surgical repair of the complete form of persistent common atrioventricular canal. Thorac Cardiovasc Surg 1968, 55:299–308.

    CAS  Google Scholar 

  2. Warnes C, Somerville J: Double mitral valve orifice in atrioventricular defects. Br Heart J 1983, 49:59–64.

    PubMed  CAS  Google Scholar 

  3. Najm HK, Coles JG, Endo M, et al.: Complete atrioventricular septal defects: results of repair, risk factors, and freedom from reoperation. Circulation 1997, 96:II311-II315. This is a comprehensive review of surgical results in 363 patients from a single center. The results suggest that closure of the mitral cleft improves survival.

    Google Scholar 

  4. Tandon R, Moller JH, Edwards JE: Single papillary muscle of the left ventricle associated with persistent common atrioventricular canal: variant of parachute mitral valve. Pediatr Cardiol 1986, 7:111–114.

    Article  PubMed  CAS  Google Scholar 

  5. Reeder GS, Danielson GK, Seward JB, et al.: Fixed subaortic stenosis in atrioventricular canal defect: a Doppler echocardiographic study. J Am Coll Cardiol 1992, 20:386–394.

    Article  PubMed  CAS  Google Scholar 

  6. Bergin ML, Warnes CA, Tajik AJ, Danielson GK: Partial atrioventricular canal defect: long-term follow-up after initial repair in patients > or = 40 years old. J Am Coll Cardiol 1995, 25:1189–1194.

    Article  PubMed  CAS  Google Scholar 

  7. Burke RP, Horvath K, Landzberg M, et al.: Long-term follow-up after surgical repair of ostium primum atrial septal defect in adults. J Am Coll Cardiol 1996, 27:696–699.

    Article  PubMed  CAS  Google Scholar 

  8. Newfeld EA, Sher M, Paul MH, Nikaidoh H: Pulmonary vascular disease in complete atrioventricular canal defect. Am J Cardiol 1977, 39:721–726.

    Article  PubMed  CAS  Google Scholar 

  9. King RM, Puga FJ, Danielson GK, et al.: Prognostic factors and surgical treatment of partial atrioventricular canal. Circulation 1986, 74:I42-I46. This landmark study provides 20-year follow-up data for 199 patients; 40-year follow-up data should appear in the near future.

    PubMed  CAS  Google Scholar 

  10. Morris CD, Magilke D, Reller M: Down’s syndrome affects results of surgical correction of complete atrioventricular canal. Pediatr Cardiol 1992, 13:80–84.

    Article  PubMed  CAS  Google Scholar 

  11. Clapp S, Perry BL, Farooki ZQ, et al.: Down’s syndrome, complete atrioventricular canal, and pulmonary vascular obstructive disease. Thorac Cardiovasc Surg 1990, 100:115–121.

    CAS  Google Scholar 

  12. Kimball TR, Daniels SR, Meyer RA, et al.: Effect of digoxin on contractility and symptoms in infants with a large ventricular septal defect. Am J Cardiol 1991, 68:1377–1382.

    Article  PubMed  CAS  Google Scholar 

  13. Dajani AS, Taubert KA, Wilson W, et al.: Prevention of bacterial endocarditis: recommendations by the American Heart Association. JAMA 1997, 277:1794–1801.

    Article  PubMed  CAS  Google Scholar 

  14. Gembruch U, Knopfle G, Chatterjee M, et al.: Prenatal diagnosis of atrioventricular canal malformations with up-to-date echocardiographic technology: report of 14 cases. Am Heart J 1991, 121:1489–1497.

    Article  PubMed  CAS  Google Scholar 

  15. Gutgesell HP, Cheatham J, Latson LA, et al.: Atrioventricular valve abnormalities in infancy: two-dimensional echocardiographic and angiocardiographic comparison. J Am Coll Cardiol 1983, 2:531–537.

    Article  PubMed  CAS  Google Scholar 

  16. Lipshultz SE, Sanders SP, Mayer JE, et al.: Are routine preoperative cardiac catheterization and angiography necessary before repair of ostium primum atrial septal defect? J Am Coll Cardiol 1988, 11:373–378.

    Article  PubMed  CAS  Google Scholar 

  17. Minich LA, Snider R, Bove EL, et al.: Echocardiographic evaluation of atrioventricular orifice anatomy in children with atrioventricular septal defect. J Am Coll Cardiol 1992, 19:149–153.

    Article  PubMed  CAS  Google Scholar 

  18. Marek J, Škovrànek J, Hucin B, et al.: Seven-year experience of noninvasive preoperative diagnostics in children with congenital heart defects: comprehensive analysis of 2,788 consecutive patients. Cardiology 1995, 86:488–495.

    Article  PubMed  CAS  Google Scholar 

  19. Stevenson JG, Sorenson GK, Gartman DM, et al.: Transesophageal echocardiography during repair of congenital cardiac defects: identification of residual problems necessitating reoperation. J Am Soc Echocardiogr 1993, 6:356–365.

    PubMed  CAS  Google Scholar 

  20. Backer CL, Mavroudis C, Alboliras ET, Zales VR: Repair of complete atrioventricular canal defects: results with the two-patch technique. Ann Thorac Surg 1995, 60:530–537.

    Article  PubMed  CAS  Google Scholar 

  21. Mavroudis C, Backer CL: The two-patch technique for complete atrioventricular canal. Semin Thorac Cardiovasc Surg 1997, 9:35–43.

    PubMed  CAS  Google Scholar 

  22. Pearl JM, Laks H: Intermediate and complete forms of atrioventricular canal. Semin Thorac Cardiovasc Surg 1997, 9:8–20.

    PubMed  CAS  Google Scholar 

  23. Hanley FL, Fenton KN, Jonas RA, et al.: Surgical repair of complete atrioventricular canal defects in infancy. Twenty-year trends. Thorac Cardiovasc Surg 1993, 106:387–397.

    CAS  Google Scholar 

  24. Bando K, Turrentine MW, Sun K, et al.: Surgical management of complete atrioventricular septal defects. A twenty-year experience. Thorac Cardiovasc Surg 1995, 110:1543–1554.

    Article  CAS  Google Scholar 

  25. 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, 62:519–525.

    Article  PubMed  CAS  Google Scholar 

  26. Tweddell JS, Litwin SB, Berger S, et al.: Twenty-year experience with repair of complete atrioventricular septal defects. Ann Thorac Surg 1996, 62:419–424.

    Article  PubMed  CAS  Google Scholar 

  27. Sadeghi AM, Laks H, Pearl JM: Primum atrial septal defect. Semin Thorac Cardiovasc Surg 1997, 9:2–7.

    PubMed  CAS  Google Scholar 

  28. Giamberti A, Marino B, diCarlo D, et al.: Partial atrioventricular canal with congestive heart failure in the first year of life: surgical options. Ann Thorac Surg 1996, 62:151–154.

    Article  PubMed  CAS  Google Scholar 

  29. Drinkwater DC Jr, Laks H: Unbalanced atrioventricular septal defects. Semin Thorac Cardiovasc Surg 1997, 9:21–25.

    PubMed  Google Scholar 

  30. Karl TR: Atrioventricular septal defect with tetralogy of Fallot or double-outlet right ventricle: surgical considerations. Semin Thorac Cardiovasc Surg 1997, 9:26–34. This article appeared as part of a seminar on atrioventricular canal defects. All the papers from the seminar are worth reading. This article 1) gives an excellent technical description of repair of the atrioventricular canal associated with right ventricular outflow tract obstruction and 2) reports excellent results with this procedure in 26 patients.

    PubMed  CAS  Google Scholar 

  31. Malm T, Karl TR, Mee RBB: Transatrial-transpulmonary repair of atrioventricular septal defect with right ventricular outflow tract obstruction. J Card Surg 1993, 8:622–627.

    PubMed  CAS  Google Scholar 

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  1. The Cleveland Clinic Foundation, Center for Pediatric and Congenital Heart Disease, M-41, 9500 Euclid Avenue, 44195, Cleveland, OH, USA

    Daniel J. Murphy Jr. MD

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  1. Daniel J. Murphy Jr. MD
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Murphy, D.J. Atrioventricular canal defects. Curr Treat Options Cardio Med 1, 323–333 (1999). https://doi.org/10.1007/s11936-999-0027-3

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