Hostname: page-component-cd9895bd7-7cvxr Total loading time: 0 Render date: 2024-12-28T02:31:14.400Z Has data issue: false hasContentIssue false
  • English
  • Français

The morphology and diagnosis of atrioventricular septal defects

Published online by Cambridge University Press:  19 August 2008

Robert H. Anderson ,
Edward J. Baker ,
Siew Yen Ho ,
Michael L. Rigby  and
Tjark Ebels
Robert H. Anderson*
Affiliation:
From the Department ofPaediatrics, National Heart & Lung Institute, London
Edward J. Baker
Affiliation:
Department of Paediatric Cardiology, Guy's Hospital, London
Siew Yen Ho
Affiliation:
From the Department ofPaediatrics, National Heart & Lung Institute, London
Michael L. Rigby
Affiliation:
From the Department ofPaediatrics, National Heart & Lung Institute, London
Tjark Ebels
Affiliation:
Department of Cardiothoracic Surgery, University of Groningen
*
Robert H. Anderson, Department of Paediatrics, National Heart ' Lung Institute, Dovehouse Street. London SW3 6LY, United Kingdom
Get access Rights & Permissions [Opens in a new window]

Summary

In this review, we discuss and describe those features which distinguish hearts with abnormal atrioventricular septation from the normal heart. The hearts, best described as atrioventricular septal defects, are unified by having a common atrioventricular junction guarded by a valve having five leaflets. The left component has three leaflets and cannot be interpreted in terms of a cleft in a normal mitral valve. The papillary muscles supporting this valve are also markedly dissimilar from the arrangement seen in normal hearts. The subaortic outflow tract is displaced superiorly compared to the normal heart, and is no longer wedged between the left valve and the septum. There is marked discrepancy in the inlet and outlet lengths of the ventricular mass, these dimensions being equal in hearts with normal atrioventricular septation. Although having the above features in common, atrioventricular septal defects show anatomic variations related to the arrangement of the bridging leaflets and their relationship to the septal structures. There may be a common valvar orifice or separate right and left orifices. The anatomic potential for shunting may be at atrial or ventricular levels, or both. Rarely, the septal structures may be intact. Other important features include ventricular dominance, the left ventricular outflow tract, and the disposition of the atrioventricular conduction tissues.

Type
The World Forum for Pediatric Cardiology Symposium on Atrioventricular Septal Defect
Information
Cardiology in the Young , Volume 1 , Issue 4 , October 1991 , pp. 290 - 305
Copyright
Copyright © Cambridge University Press 1991

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Watkins, E, Gross, RE.Experiences with surgical repair of atrial septal defects. J Thorac Cardiovasc Surg 1955; 30: 469–491.Google ScholarPubMed
Van Mierop, LHS, Alley, RD, Kausel, HW, Stranahan, A.The anatomy and embryology of endocardial cushion defects. J Thorac Cardiovasc Surg 1962; 43: 71–82.CrossRefGoogle ScholarPubMed
Rokitansky, CF von.Die Defecte der Scheidewande des Herzens. Pathologisch-Anatomische. Wilhelm Braumuller, Vienna, 1875, pp 27–36.Google Scholar
Monckeberg, JG.Das Verhalten des Atrioventrikularsystems bei persistierendem Ostium atrioventriculare commune. Centralblatt fur allgemeine Pathologie und pathologische Anatomie 1923. 1924; 34: 139.Google Scholar
Abbott, M.Atlas of Congenital Heart Disease. The American Heart Association, New York, 1936.Google Scholar
Allwork, SP.Anatomical-embryological correlates in atrioven tricular septal defect. Br Heart J 1982; 47: 419–429.CrossRefGoogle Scholar
Penkoske, PA, Neches, WH, Anderson, RH, Zuberbuhler, JR.Further observations on the morphology of atrioventricular septal defects. J Thorac Cardiovasc Surg 1985; 90: 611–622.CrossRefGoogle ScholarPubMed
Ebels, TJ, Anderson, RH, Devine, WA, Debich, DE, Penkoske, PA, Zuberbuhler, JR.Anomalies of the left atrioventricular valve and related ventricular septal morphology in atrioven tricular septal defects. J Thorac Cardiovasc Surg 1990; 99: 299–307.Google Scholar
Brandt, PWT, Clarkson, PM, Neutze, JM, Barratt-Boyes, EG.Left ventricular cineangiography in endocardial cushion de fect (persistent common atrioventricular canal). Australas Radiol 1972; 16: 367–376.CrossRefGoogle Scholar
Gerbode, F, Hultgren, H, Melrose, D, Osborn, J.Syndrome of left ventricular-right atrial shunt, successful surgical repair of defect in five cases with observation of bradycardia on closure. Ann Surg 1958; 148: 433–446.CrossRefGoogle ScholarPubMed
McKay, R, Battistessa, SA, Wilkinson, JL, Wright, JP.A communication from the left ventricle to the right atrium: a defect in the central fibrous body. Int J Cardiol 1989; 23: 117–123.CrossRefGoogle Scholar
Becker, AE, Anderson, RH.Atrioventricular septal defects. What's in a name. J Thorac Cardiovasc Surg 1982; 83: 461–469.CrossRefGoogle Scholar
Wenink, ACG, Zevallos, JC.Developmental aspects of atrio ventricular septal defects. Int J Cardiol 1988; 18: 65–78.CrossRefGoogle Scholar
Silverman, NH, Ho, SY, Anderson, RH, Smith, A, Wilkinson, JL.Atrioventricular septal defect with intact atrial and ventricular septal structures. Int J Cardiol 1984; 5: 567–572.CrossRefGoogle ScholarPubMed
Ho, SY, Russell, G, Gerlis, LM.Atrioventricular septal defect with intact septal structures in a 74-year-old. Int J Cardiol 1990; 26: 371–373.CrossRefGoogle ScholarPubMed
Peacock, TB.Malformation of the heart consisting in an imperfection of the auricular and ventricular septa. Trans Pathol Soc (London) 1846; 1: 61–62.Google Scholar
Carpentier, A. Surgical anatomy aṅd management of the mitral component of atrioventricular canal defects. In: Paediatric Cardiology 1977. Anderson, RH and Shinebourne, EA (eds). Churchill Livingstone, Edinburgh, 1978, pp 477–486.Google Scholar
Ebels, TJ. New surgical aspects ofatrioventricular septal defect. Thesis. Rijksuniversiteit Groningen. Drukkerij Van Denderen B.V., Groningen, 1989.Google Scholar
Hyrtl, J.Onomatologica anatomica. Wilhelm Braumuller, Vienna, 1880, pp 328–331.Google Scholar
Keith, A.The Hunterian lectures on malformations of the heart. Lancet 1909; 2: 433–435.Google Scholar
Van Mierop, LHS. Pathology and pathogenesis of endocardial cushion defects. Surgical implications. In: Second Henry Ford Hospital International Symposium on Cardiac Surgery. Davila, JC (ed). Appleton-Century-Crofts, New York, 1977, pp 201–207.Google Scholar
McGoon, DC, Puga, FJ, Danielson, GK. Atrioventricular canal. In: Gibbon's Surgery of the Heart. Sabiston, DC, Spencer, FC (eds). WB Saunders Co, Philadelphia, 1983, pp 1051–1066.Google Scholar
Kurnit, DM, Aldridge, JF, Mattsuoka, R, Matthysse, S.Increased adhesiveness of trisomy 21 cells and atrioventricular canal malformations in Down syndrome: a stochastic model. Am J Med Genetics 1985; 20: 385–399.CrossRefGoogle ScholarPubMed
Wenink, ACG, Gittenberger-de Groot, AC.The role of atrio ventricular endocardial cushions in the septation of the heart. Int J Cardiol 1985; 8: 25–44.CrossRefGoogle Scholar
Barratt-Boyes, BG. Introduction. Atrioventricular septal defects. In: Crupi, G, Anderson, RH, Parenzan, L (eds). Perspectives in Pediatric Cardiology Volume 2. Pediatric Cardiac Surgery Part 1. Futura Publishing Company, Inc., Mount Kisco, New York, 1989, pp 73–80.Google Scholar
Wakai, CS, Edwards, JE.Pathologic study of persistent com mon atrioventricular canal. Am Heart J 1958; 56: 779–794.CrossRefGoogle Scholar
Lev, M, Bharati, S, McAllister, HA, Kirklin, JW.The surgical anatomy of the atrioventricular valve in the intermediate type of common atrioventricular orifice. Am J Cardiol 1979; 43: 364–370.Google Scholar
Draulens-Noe, HAY, Wenink, ACG, Quaegebeur, JM.Ventricular septal defect in atrioventricular (AV) septal defect - a correlation with AV valve morphology. Pediatr Cardiol 1984; 5: 227–228.Google Scholar
Bharati, S, Lev, M.The spectrum of common atrioventricular orifice (canal). Am Heart J 1973; 86: 553–561.CrossRefGoogle ScholarPubMed
Pillai, R, Ho, SY, Anderson, RH, Shinebourne, EA, Lincoln, C.Malalignment of the interventricular septum with atrioven tricular septal defect: its implications concerning conduction tissue disposition. Thorac Cardiovasc Surgeon 1984; 32: 1–3.CrossRefGoogle Scholar
Corwin, RD, Singh, AK, Karlson, KE.Double-outlet right atrium: A rare endocardial cushion defect. Am Heart J 1983; 105: 1156–1157.CrossRefGoogle Scholar
Otero Coto, E, Calabro, R, Marsico, F, López Arranz, JS.Right atrial outlet atresia with straddling left atrioventricular valve. A form of double outlet atrium. Br Heart J 1981; 45: 317–324.CrossRefGoogle Scholar
Piccoli, GP, HD, SY, Wilkinson, JL, Macartney, FJ, Gerlis, LM, Anderson, RH.Left sided obstructive lesions in atrioventricular septal defects. J Thorac Card Surg 1982; 83: 453–460.Google ScholarPubMed
Ebels, TJ, Ho, SY, Anderson, RH, Meijboom, EJ, Eijgelaar, A.The surgical anatomy of the left ventricular outflow tract in atrioventricular septal defect. Ann Thorac Surg 1986; 41: 483–488.CrossRefGoogle ScholarPubMed
Anderson, RH, Ho, SY, Becker, AE.The surgical anatomy of the conduction tissues. Thorax 1983; 38: 408–420.CrossRefGoogle ScholarPubMed
Pillai, R, Ho, SY, Anderson, RH, Lincoln, C.Ostium primum atrioventricular septal defect: an anatomical surgical review. Ann Thorac Surg 1986; 41: 458–461.CrossRefGoogle ScholarPubMed
Wilcox, BR, Anderson, RH, Henry, GW.Unusual opening of coronary sinus in atrioventricular septal defects. Ann Thorac Surg 1990; 50: 767–770.CrossRefGoogle ScholarPubMed
Piccoli, GP, Wilkinson, JL, Macartney, FJ, Gerlis, LM, Anderson, RH.Morphology and classification of complete atrioven tricular defects. Br Heart J 1979; 42: 633–639.CrossRefGoogle Scholar
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.Google ScholarPubMed
Silverman, NH, Zuberbuhler, JR, Anderson, RH.Atrioventricular septal defects: cross-sectional echocardiographic and morphologic comparisons. Int J Cardiol 1986; 13: 309–332.CrossRefGoogle ScholarPubMed
Jacobstein, MD, Fletcher, ED, Goldstein, S, Riemenschneider, T.Evaluation of atrioventricular septal defect by magnetic resonance imaging. Am J Cardiol 1985; 55: 1158–1161.CrossRefGoogle ScholarPubMed
Parsons, JM, Baker, EJ, Anderson, RH, Ladusans, EJ, Hayes, A, Qureshi, SA, Deverall, PB, Fagg, N, Cook, A, Maisey, MN, Tynan, M.Morphological evaluation of atrioventricular septal defects by magnetic resonance imaging. Br Heart J 1990; 64: 138–145.CrossRefGoogle ScholarPubMed
Ebels, TJ, Meijboom, EJ, Anderson, RH, Schasfoort-van Leeuwen, MJM, Lenstra, D, Eijgelaar, A, Bossina, KK, Homan van der Heide, JN.Anatomic and functional “obstruction” of the outflow tract in atrioventricular septal defects with separate valve orifices (“ostium primum atrial septal defect”): an echocardiographic study. Am J Cardiol 1984; 54: 843–847.CrossRefGoogle Scholar
Meijboom, EJ, Wyse, RKH, Ebels, TJ, Deanfield, JE, Quaegebeur, JM, Anderson, RH, Brenner, JI.Doppler mapping of postoperative left atrioventricular valve regurgitation. Circulation 1988; 77: 311–315.CrossRefGoogle ScholarPubMed