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The diverse cardiac morphology seen in hearts with isomerism of the atrial appendages with reference to the disposition of the specialised conduction system

Published online by Cambridge University Press:  20 September 2006

Audrey Smith ,
Siew Yen Ho ,
Robert H. Anderson ,
M. Gwen Connell ,
Robert Arnold ,
James L. Wilkinson  and
Andrew C. Cook
Audrey Smith
Affiliation:
Cardiac Unit, Institute of Child Health, University College, London
Siew Yen Ho
Affiliation:
Department of Paediatrics, National Heart and Lung Institute, Imperial College, London
Robert H. Anderson
Affiliation:
Cardiac Unit, Institute of Child Health, University College, London
M. Gwen Connell
Affiliation:
Institute of Child Health, Royal Liverpool Children's Hospital, University of Liverpool, United Kingdom
Robert Arnold
Affiliation:
Institute of Child Health, Royal Liverpool Children's Hospital, University of Liverpool, United Kingdom
James L. Wilkinson
Affiliation:
The Cardiology Department, The Royal Children's Hospital, Victoria, Australia
Andrew C. Cook
Affiliation:
Cardiac Unit, Institute of Child Health, University College, London
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Abstract

Congenital cardiac malformations which include isomerism of the atrial appendages are amongst the most challenging of problems for diagnosis and also for medical and surgical management. The nomenclature for pathological description is controversial, but difficulties can be overcome by the use of a segmental approach. Such an approach sets out the morphology and the topology of the chambers of the heart, together with the types and modes of the atrioventricular, ventriculo-arterial, and venous connections. We have applied this method to a study of 35 hearts known to have isomerism of the atrial appendages. We have already published accounts of 27 of these cases, but these were reviewed for this study in the light of our increased awareness of the implications of isomerism, and 8 new cases were added. After examining, or re-examining, the morphology of every heart in detail, we grouped them together according to their ventricular topology and modes of atrioventricular connection. Then we studied the course of the specialised conduction system, by the use of the light microscope, first in each individual case, and then together in their groups. We conclude that the pathways for atrioventricular conduction in hearts with isomerism of the atrial appendages are conditioned both by ventricular topology, and by the atrioventricular connections. Based on our experience, we have been able to establish guidelines that direct the clinician to the likely location of the conduction tissues.

Keywords

Atrioventricular nodesinus nodevisceral heterotaxysequential segmental analysisventricular topology
Type
Original Article
Information
Cardiology in the Young , Volume 16 , Issue 5 , October 2006 , pp. 437 - 454
Copyright
© 2006 Cambridge University Press

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References

Van Praagh R, Van Praagh S. Atrial isomerism in the heterotaxy syndromes with asplenia, or polysplenia or normally formed spleen: an erroneous concept. Am J Cardiol 1990; 66: 1504–1506.Google Scholar
Uemera H, Ho SY, Devine WA, Kilpatrick LL, Anderson RH. Atrial appendages and levoatrial connections in hearts from patients with visceral heterotaxy. Ann Thorac Surg 1995; 60: 561–569.Google Scholar
Humes RA, Feldt RH, Porter CJ, Julsrud PR, Puga FJ, Danielson GK. The modified Fontan operation for asplenia and polysplenia syndrome. J Thorac Cardiovasc Surg 1988; 96: 212–218.Google Scholar
Van Mierop LHS, Gessner IH, Schlieber GL. Asplenia and polysplenia syndromes. In: Bergsma (ed.). Birth Defects, original article series, 8. Williams and Wilkins, Baltimore, 1972, pp 74–82.
Ho SY, Fagg NLK, Anderson RH, Cook AC, Allan L. Disposition of the atrioventricular conduction tissue in the heart with isomerism of the atrial appendages: its relationship to congenital heart block. JACC 1992; 20: 904–910.Google Scholar
Sinzobahamvya N, Arenz C, Brecher AM, Urban AE. Atrial isomerism: a surgical experience. Cardiovasc Surg 1999; 7: 436–442.Google Scholar
Dickinson DF, Wilkinson JL, Anderson KR, Smith A, Ho SY, Anderson RH. The cardiac conducting system in situs ambiguous. Circulation 1979; 59: 879–885.Google Scholar
Smith A, Ho SY, Anderson RH. Histologic study of the cardiac conducting system as a routine procedure. Medical Laboratory Sciences 1977; 34: 223–229.Google Scholar
Moorman AFM, Christoffels VM, Anderson RH. Anatomic substrates for cardiac conduction. Heart Rhythm 2005; 2: 875–886.Google Scholar
Landing BH, Lawrence TK, Paynes VC, Wells TR. Bronchial anatomy in syndromes with abnormal visceral situs, abnormal spleen and congenital heart disease. Am J Cardiol 1971; 12: 456–462.Google Scholar
Liu C, Liu W, Lu M-F, Brown NA, Martin JF. Regulation of left-right asymmetry by thresholds of Pitx2 activity. Development 2001; 128: 2039–2048.Google Scholar
Bamforth SD, Braganca J, Farthing CR, et al. Cited2 controls left-right patterning and heart development through a Nodal-Pitx2c pathway. Nat Genet 2004; 36: 1189–1196.Google Scholar
Van Mierop LHS, Wigglesworth FW. Isomerism of the cardiac atria in the asplenia syndrome. Lab Invest 1962; 11: 1303–1315.Google Scholar
Van Praagh S, Kreutzer A, Alday L, Van Praagh R. Systemic and pulmonary venous connections in visceral heterotaxy, with emphasis on the diagnosis of the atrial situs: a study of 109 postmortem cases. In: Clark E, Takeo A (eds.). Developmental Cardiology, morphogenesis and function. Futura, Mt.Kisco, NY, 1990, pp 671–721.
Sanchez-Quintana D, Cabrera JA, Farre J, Climent V, Anderson RH, Ho SY. Sinus node revisited in the era of electroanatomical mapping and catheter ablation. Heart 2005; 91: 189–194.Google Scholar
Nagakawa M, Thompson RP, Terracio L, Borg TK. Developmental anatomy of HNK-1 immunoreactivity in the embryonic rat heart: co-distribution with early conduction tissue. Anat Embryol 1993; 187: 445–460.Google Scholar
Pohanka I, Vitek B. The conducting system of the heart in the syndrome of visceral symmetry. Folia Morphol (Praha) 1978; 26: 379–388.Google Scholar
Ho SY, Seo JW, Brown NA, Cook AC, Fagg NLK, Anderson RH. Morphology of the sinus node in human and mouse hearts with isomerism of the atrial appendages. Br Heart J 1995; 74: 437–441.Google Scholar
Lamers WH, Wessels A, Verbeek FJ, et al. New findings concerning ventricular septation in the human heart. Implications for maldevelopment. Circulation 1992; 86: 1194–1205.Google Scholar
Rinne K, Smith A, Ho SY. A unique case of ventricular isomerism? Cardiol Young 2000; 10: 42–45.Google Scholar
Brown NA, Anderson RH. Symmetry and laterality in the human heart: developmental implications. In: Heart Development. Harvey RP, Rosenthal N (eds.). Academic Press, San Diego, London, 1999, pp 447–462.
Franco D, Kelly R, Moorman AFM, Lamers WH, Buckingham M, Brown NA. MLC3F transgene expression in iv mutant mice reveals the importance of left-right signaling pathways for the acquisition of left and right atrial but not ventricular compartment identity. Developmental Dynamics 2001; 221: 206–215.Google Scholar
Anderson RH, Becker AE, Arnold R, Wilkinson JL. The cardiac conduction tissues in congenitally corrected transposition. Circulation 1974; 50: 911–923.Google Scholar
Dick M 2nd, Van Praagh R, Rudd M, Folkerth T, Castaneda AR. Electrophysiological delineation of the specialized conduction system in two patients with corrected transposition of the great arteries in situs inversus (IDD). Circulation 1977; 55: 896–900.Google Scholar
Hosseinpour A-R, McCarthy KP, Griselli M, Sethia B, Ho SY. Congenitally corrected transposition: size of the pulmonary trunk and septal malalignment. Ann Thorac Surg 2004; 77: 2163–2166.Google Scholar
Anderson RH. The conduction tissues in congenitally corrected transposition. Ann Thorac Surg 2004; 77: 1881–1882.Google Scholar
Wilkinson JL, Anderson RH, Arnold R, Hamilton DI, Smith A. The conducting tissues in primitive ventricular hearts without an outlet chamber. Circulation 1976; 53: 930–938.Google Scholar