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The Segmental Approach to Congenital Heart Disease

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Neonatal Heart Disease

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Abstract

With the potential and indeed reality of an infinite variety of complex cardiac anomalies, how does one bring an internal and external consistency to the nomenclature applied to these anomalies? The answer lies in the development of a system of nomenclature that is based on cardiac morphology-anatomy and cardiac connections (Fig. 10.1 A and B); a system that should appeal to a wide constituency because it promotes communication and understanding; and a system that facilitates in its nomenclature the appreciation of a heart’s construction and potentials for “heartache” [2, 17, 26, 39, 40, 41, 52, 64, 65, 68, 69, 74, 77]. The Rubik’s cube solution for congenital heart disease was the “benchmark” development by Richard Van Praagh of a segmental approach to congenital heart disease.

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References

  1. Anderson RH, Macartney FJ, Tynan M et al. (1983). Univentricular atrioventricular connection: the single ventricle trap unsprung. Pediatr Cardiol 4: 273–280

    Article  PubMed  CAS  Google Scholar 

  2. Anderson RH, Becker AE, Freedom RM et al. (1984) Sequential segmental analysis of congenital heart disease. Pediatr Cardiol 5: 281–288

    Article  PubMed  CAS  Google Scholar 

  3. Anderson RH, Macartney FJ, Shinebourne EA, Tynan M (1987) Paediatric cardiology, vol. 1. Churchill Livingstone, Edinburgh, pp 65–82

    Google Scholar 

  4. Arciniegas JG, Soto B, Coghan HC, Bargeron Jr LM (1981) Congenital heart malformations: sequential angiographic analysis. AJR 137: 673–681

    PubMed  CAS  Google Scholar 

  5. Bersch W (1971) On the importance of the bulboauricular flange for the formal genesis of congenital heart defects with special regard to the ventricular septum defects. Arch Abt A Pathol Anat 354: 252–267

    Article  CAS  Google Scholar 

  6. Brandt PWT, Calder AL (1977) Cardiac connections: the segmental approach to radiologic diagnosis in congenital heart disease. 7 (3): 3–35

    Google Scholar 

  7. Anderson RH, Becker AE, Freedom RM et al. (1984) Sequential segmental analysis of congenital heart disease. Pediatr Cardiol 5: 281–288

    Article  PubMed  CAS  Google Scholar 

  8. Anderson RH, Macartney FJ, Shinebourne EA, Tynan M (1987) Paediatric cardiology, vol. 1. Churchill Livingstone, Edinburgh, pp 65–82

    Google Scholar 

  9. Arciniegas JG, Soto B, Coghan HC, Bargeron Jr LM (1981) Congenital heart malformations: sequential angiographic analysis. AJR 137: 673–681

    PubMed  CAS  Google Scholar 

  10. Bersch W (1971) On the importance of the bulboauricular flange for the formal genesis of congenital heart defects with special regard to the ventricular septum defects. Arch Abt A Pathol Anat 354: 252–267

    Article  CAS  Google Scholar 

  11. Brandt PWT, Calder AL (1977) Cardiac connections: the segmental approach to radiologic diagnosis in congenital heart disease. 7 (3): 3–35

    Google Scholar 

  12. Calcaterra G, Anderson RH, Lau KC, Shinebourne EA (1979) Dextrocardia-value of segmental analysis in its categorisation. Br Heart J 42: 497–507

    Article  PubMed  CAS  Google Scholar 

  13. Carr I, Tynan MJ, Aberdeen E, Bonham Carter RE, Graham G, Waterston DG (1968) Predictive accuracy of the loop rule in 109 children with classical complete transposition of the great arteries. Circulation 38 (Suppl 5): 52

    Google Scholar 

  14. Caruso G, Becker AE (1979). How to determine atrial situs? Consideration initiated by 3 cases of absent spleen with discordant anatomy between bronchi and atria. Br Heart J 41: 559–567

    Article  PubMed  CAS  Google Scholar 

  15. Ceballos R, Soto B, Bargeron Jr LM (1981) Angiographic anatomy of the normal heart through axial angiography. Circulation 64: 351–358

    Article  PubMed  CAS  Google Scholar 

  16. Clarkson PM, Brandt PWT, Barratt-Boyes BG, Neutze JM (1972) “Isolated Atrial Inversion”. Visceral situs solitus, visceroatrial discordance, discordant ventricular d loop without transposition, dextrocardia: diagnosis and surgical correction. Am J Cardiol 29: 877–882

    Article  PubMed  CAS  Google Scholar 

  17. de la Cruz MV, Anselmi G, Munoz-Castellanos L, Nadal-Ginard B, Munoz-Armas S (1971) Systematiza- tion and embryological and anatomical study or mirror- image dextrocardias, dextroversions, and laevover- sions. Br Heart J 33: 841–853

    Article  PubMed  Google Scholar 

  18. Deanfield JE, Leanage R, Stroobant J, Chrispin AR, Taylor JFN, Macartney FJ (1980) Use of high kilovoltage filtered beam radiographs for detection of bronchial situs in infants and young children. Br Heart J 44:577- 583

    Google Scholar 

  19. Dickinson DF, Wilkinson JL, Anderson KR, Smith A, Ho SY, Anderson RH (1979) The cardiac conduction system in situs ambiguus. Circulation 59: 879–885

    PubMed  CAS  Google Scholar 

  20. Elliott LP (1978) An angiocardiographic and plain film approach to complex congenital heart disease: classification and simplified nomenclature. Curr Prob Cardiol 3 (3): 5–64

    Article  Google Scholar 

  21. Elliott LP, Cramer GG, Amplatz K (1966) The anomalous relationship of the inferior vena cava and abdominal aorta as a specific angiocardiographic sign in asplenia. Radiology 87: 859–863

    PubMed  CAS  Google Scholar 

  22. Escarous A (1989) Left pulmonary isomerism with normal arrangement of the heart and abdominal organs. Computed tomography of two adults. Int J Cardiol 24: 118–120

    Article  PubMed  CAS  Google Scholar 

  23. Fitzer PM (1976) An approach to cardiac malposition and the heterotaxy syndrome using Tc sulfur colloid imaging. AJR 127:1021 –1025

    Google Scholar 

  24. Forde WJ, Finby N (1961) Roentgenographic features of congenital asplenia: a teratologic syndrome of visceral symmetry. AJR 86: 523–526

    CAS  Google Scholar 

  25. Freedom RM (1983) Supero-inferior ventricle and criss-cross atrioventricular connections: an analysis of the myth and mystery. In: Belloli GP, Squarcia U (eds) Pediatric cardiology and cardiosurgery. Karger, Basel, pp 48–62 (Modern problems in paediatrics, vol 22 )

    Google Scholar 

  26. Freedom RM (1984) The “anthropology” of the segmental approach to the diagnosis of complex congenital heart disease. Cardiovasc Intervent Radiol 7: 121–123

    Article  PubMed  CAS  Google Scholar 

  27. Freedom RM, Ellison RC (1973) Coronary sinus rhythm in the polysplenia syndrome. Chest 63: 952–958

    Article  PubMed  CAS  Google Scholar 

  28. Freedom RM, Fellows KE (1973) Radiographic visceral patterns in the asplenia syndrome. Radiology 107:387- 391

    Google Scholar 

  29. Freedom RM, Harrington DP, White RI Jr (1974) The differential diagnosis of levo-transposed or malposed aorta. An angiocardiographic study. Circulation 50: 1040–1046

    PubMed  CAS  Google Scholar 

  30. Freedom RM, Culham G, Rowe RD (1978) The crisscross and superoinferior ventricular heart. An angiocardiographic study. Am J Cardiol 42: 620–627

    Google Scholar 

  31. Freedom RM, Culham JAG, Moes CAF (1984) Angiocardiography of congenital heart disease. Macmillan, New York, pp 17–45

    Google Scholar 

  32. Hastreiter AR, Rodriguez-Coronel A (1968) Discordant situs of thoracic and abdominal viscera. Am J Cardiol 22: 111–118

    Article  PubMed  CAS  Google Scholar 

  33. Huhta JC, Smallhorn JF, Macartney FJ (1982) Two dimensional echocardiographic diagnosis of situs. Br Heart J 48: 97–108

    Article  PubMed  CAS  Google Scholar 

  34. Huhta JC, Smallhorn JF, Macartney FJ, Anderson RH, De Leval M (1982) Cross-sectional echocardiographic diagnosis of systemic venous return, Br Heart J 48:388- 403

    Google Scholar 

  35. Huhta JC, Smallhorn JF, Macartney FJ (1984) Cross-sectional echocardiographic diagnosis of azygos continuation of the inferior vena cava. Cathet Cardiovasc Diagn 10: 221–232

    Article  PubMed  CAS  Google Scholar 

  36. Landing BH, Lawrence T-Y K, Payne VC Jr, Wells TR (1971) Bronchial anatomy in syndromes with abnormal visceral situs, abnormal spleen and congenital heart disease. Am J Cardiol 28: 456–462

    Article  PubMed  CAS  Google Scholar 

  37. Leachman RD, Angelini P, Cokkinos DV (1973) Hepatoatrial dissociation. Report of two cases and clinical implications. Chest 63: 926–932

    Google Scholar 

  38. Lev M (1954) Pathologic diagnosis of positional variations in cardiac chambers in congenital heart disease. Lab Invest 3: 71–82

    PubMed  CAS  Google Scholar 

  39. Lev M, Liberthson RR, Golden JG, Eckner FAO, Arcilla RA (1971) The pathologic anatomy of mesocardia. Am J Cardiol 28: 428–435

    Article  PubMed  CAS  Google Scholar 

  40. Liberthson RR, Hastreiter AR, Sinha SN, Bharati S, Novak GM, Lev M (1973) Levocardia with visceral heterotaxy-isolated levocardia: pathologic anatomy and its clinical implications. Am Heart J 85: 40–54

    Article  PubMed  CAS  Google Scholar 

  41. Macartney FJ, Shinebourne EA, Anderson RH (1976) Connexions, relations, discordance, and distortions. Br Heart J 38: 323–326

    Article  PubMed  CAS  Google Scholar 

  42. Macartney FJ, Zuberbuhler JR, Anderson RH (1980) Morphologic considerations pertaining to recognition of atrial isomerism. Consequences for sequential chamber localisation. Br Heart J 44: 657–667

    Article  PubMed  CAS  Google Scholar 

  43. Meredith MA, Hutchins GM, Moore GW (1979) Role of the left interventricular sulcus in formation of the interventricular septum and crista supraventricularis in normal human cardiogenesis. Anat Rec 194:417–428

    Google Scholar 

  44. Momma K, Linde L (1969) Abnormal P wave axis in congenital heart disease associated with asplenia and polysplenia. J Electrocardiol 2: 395–402

    Article  PubMed  CAS  Google Scholar 

  45. Otero Coto E, Jiminez MQ, Cabrera A, Deverall PB, Caffarena JM (1978) Aortic levopositions without ventricular inversion. Eur J Cardiol 8: 523–541

    Google Scholar 

  46. Partridge J (1979) The radiological evaluation of atrial situs. Clin Radiol 30: 95–103

    Article  PubMed  CAS  Google Scholar 

  47. Partridge JB, Scott O, Deverall PB, Macartney FJ (1975) Visualization and measurement of the main bronchi by tomography as an objective indicator of thoracic situs in congenital heart disease. Circulation 51: 188–196

    PubMed  CAS  Google Scholar 

  48. Pipitone S, Calcaterra G, Grillo R, Thiene G, Sperandeo V (1985) Broncho-atrial discordance: a clinically diagnosed case. Int J Cardiol 9: 374–378

    Article  PubMed  CAS  Google Scholar 

  49. Roberts WC, Anderson RC, Edwards JE (1961) The significance of asplenia in the recognition of inoperable congenital heart disease. Circulation 26: 851–857

    Google Scholar 

  50. Ruttenberg HD, Neufeld HN, Lucas Jr RV et al. (1964) Syndrome of congenital cardiac disease with asplenia. Distinction from other forms of congenital cyanotic cardiac disease. Am J Cardiol 13: 387–406

    Google Scholar 

  51. Seo JW, Choe GY, Chi JG (1989) An unusual ventricular loop associated with right juxtaposition of the atrial appendages. Int J Cardiol 25: 219–228

    Article  PubMed  CAS  Google Scholar 

  52. Shinebourne EA, Macartney FJ, Anderson RH (1976) Sequential chamber localisation - logical approach to diagnosis in congenital heart disease. Br Heart J 38:327- 340

    Google Scholar 

  53. Soto B, Pacifico AD, Souza Jr AS, Bargeron Jr LM, Ermocilla R, Tonkin IL (1978) Identification of thoracic isomerism from the plain chest radiograph. AJR 131: 995–1002

    PubMed  CAS  Google Scholar 

  54. Tonkin ILD, Tonkin AK (1982) Visceroatrial situs abnormalities: sonographic and computed tomographic appearance. Am J Radiol 138: 509–515

    CAS  Google Scholar 

  55. Tynan M, Anderson RH (1979) Terminology of transposition of the great arteries. In: Godman MJ, Marquis RM (eds) Heart disease in the newborn. Churchill Livingstone, Edinburgh, pp 341–349 (Paediatric cardiology, vol 2 )

    Google Scholar 

  56. Tynan MJ, Becker AE, Macartney FJ, Quero Jiminez M, Shinebourne EA, Anderson RH (1979) Nomenclature and classification of congenital heart disease. Br Heart J 41: 544–553

    Article  PubMed  CAS  Google Scholar 

  57. Van Der Horst RL, Gotsman MS (1972) Abnormalities of atrial depolarization in infradiaphragmatic interruption of inferior vena cava. Br Heart J, 34: 295–300

    Article  PubMed  Google Scholar 

  58. Van Mierop LHS, Wiglesworth FW (1962) Isomerism of the cardiac atria in the asplenia syndrome. Lab Invest 11: 1303–1307

    Google Scholar 

  59. Van Mierop LHS, Patterson PR, Reynold RW (1964) Two cases of congenital asplenia with isomerism of the cardiac atria and the sinoatrial nodes. Am J Cardiol 13: 407–412

    Article  Google Scholar 

  60. Van Mierop LHS, Eisen S, Schiebler GL (1970) The radiographic appearance of the tracheobronchial tree as an indicator of visceral situs. Am J Cardiol 26: 432–435

    Article  PubMed  Google Scholar 

  61. Van Mierop LHS, Gessner IH, Schiebler GL (1972) Asplenia and polysplenia syndromes. Birth Defects: Orig Article Series VIII: 36–44

    Google Scholar 

  62. Van Praagh R (1972) The segmental approach to diagnosis in congenital heart disease. Birth Defects: Orig Article Series VIII: 4–23

    Google Scholar 

  63. Van Praagh R (1976) The story of anatomically corrected malposition of the great arteries. Chest 69: 2–4

    Article  PubMed  Google Scholar 

  64. Van Praagh R (1977) Terminology of congenital heart disease. Glossary and commentary. Circulation 56:139- 143

    Google Scholar 

  65. Van Praagh R (1984) Diagnosis of complex congenital heart disease: morphologic-anatomic method and ter-minology. Cardiovasc Intervent Radiol 7: 115–120

    Article  PubMed  Google Scholar 

  66. Van Praagh R, Van Praagh S (1966) Isolated ventricular inversion. A consideration of the morphogenesis, definition and diagnosis of nontransposed and transposed great arteries. Am J Cardiol 17: 395–406

    Google Scholar 

  67. Van Praagh R, Van Praagh S (1967) Anatomically corrected transposition of the great arteries. Br Heart J 29: 112–119

    Article  PubMed  Google Scholar 

  68. Van Praagh R, Van Praagh S (1990) Atrial isomerism in the heterotaxy syndromes with asplenia, or polysplenia, or normally formed spleen: an erroneous concept. Am J Cardiol 66: 1504–1506

    Article  PubMed  Google Scholar 

  69. Van Praagh R, Ongley PA, Swan HJC (1964) Anatomic types of single or common ventricle in man. Morphologic and geometric aspects of 60 autopsied cases. Am J Cardiol 13: 367–386

    Article  Google Scholar 

  70. Van Praagh R, Van Praagh S, Vlad P, Keith JD (1964) Anatomic types of congenital dextrocardia. Diagnostic and embryologic implications. Am J Cardiol 13: 510–531

    Google Scholar 

  71. Van Praagh R, Van Praagh S, Vlad P, Keith JD (1965) Diagnosis of the anatomic types of congenital dextrocardia. Am J Cardiol 15: 234–247

    Article  Google Scholar 

  72. Van Praagh R, Durnin R, Jockin H et al. (1975) Anatomically corrected malposition of the great arteries ( S,D,L ). Circulation 51: 20–31

    Google Scholar 

  73. Van Praagh S, LaCorte M, Fellows KE (1980) Superoinferior ventricles: anatomic and angiocardiographic findings in 10 postmortem cases. In: Van Praagh R, Takao A (eds) Etiology and morphogenesis of congenital heart disease. Futura, New York, pp 317–378

    Google Scholar 

  74. Van Praagh R, Layton WM, Van Praagh S (1980) The morphogenesis of normal and abnormal relationships between the great arteries and ventricles: pathologic and experimental data. In: Van Praagh R, Takao A (eds) Etiology and morphogenesis of congenital heart disease. Futura, New York, pp 271–316

    Google Scholar 

  75. Van Praagh R, David I, Van Praagh S (1982) What is a ventricle? The single ventricle trap. Pediatr Cardiol 2: 79–84

    Article  PubMed  Google Scholar 

  76. Van Praagh R, Weinberg PM, Smith SD, Foran RB, Van Praagh S (1989) Malpositions of the heart. In: Adams FH, Emmanouilides GC, Riemenschneider TA (eds) Moss’ heart disease in infants, children, and adolescents, 3rd edn. Williams and Wilkins, Baltimore, pp 530–580

    Google Scholar 

  77. Weinberg PM, Van Praagh R, Wagner HR, Cuaso CC (1980) New from of criss-cross atrioventricular relation: an expanded view of the meaning of D and L-loops. World congress of paediatric cardiology, no. 139

    Google Scholar 

  78. Wilkinson JL, Acerete F (1973) Terminological pitfalls in congenital heart disease. Reappraisal of some confusing terms, with an account of a simplified system of basic nomenclature. Br Heart J 35: 1166–1177

    Google Scholar 

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  1. R. M. Freedom
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  2. J. F. Smallhorn
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© 1992 Springer-Verlag London Limited

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Freedom, R.M., Smallhorn, J.F. (1992). The Segmental Approach to Congenital Heart Disease. In: Neonatal Heart Disease. Springer, London. https://doi.org/10.1007/978-1-4471-1814-5_10

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  • DOI: https://doi.org/10.1007/978-1-4471-1814-5_10

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-1816-9

  • Online ISBN: 978-1-4471-1814-5

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