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Thoracic Situs As an Indicator of Atrial Appendage Morphology: A Postmortem Study of 306 Specimens With Situs Solitus in 250 and Heterotaxy in 56 Cases

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Abstract

Symmetry of bronchi, lung lobation, and atrial appendages is frequently found in patients with heterotaxy syndromes. To evaluate the reliability of thoracic situs as an indicator of atrial appendage morphology, thoracic situs was assessed in 306 postmortem cases: 250 with atrial situs solitus and 56 with heterotaxy syndromes. Five features that indicate thoracic situs were assessed: (1) lung lobation, (2) lengths of main bronchi, (3) ratio of left to right (L/R) bronchial lengths, (4) relationship of bronchi to ipsilateral pulmonary artery, and (5) number of cartilage rings in each main bronchus. In the group with heterotaxy, the expected symmetrical lung lobation, lengths of bronchi, number of cartilage rings, and relations to pulmonary arteries were found in 77, 77, 77, and 95% of cases, respectively. The ratios of L/R bronchial lengths were ≤1.5 in 90% of cases. The relations of the bronchi to the pulmonary arteries were the best predictors of symmetrical atrial appendages or splenic syndromes. Bronchial–atrial discordance occurred in ten cases: in situs solitus in one case and in heterotaxy in nine cases. Detection of heterotaxy syndromes is important because of the associated severe congenital cardiac defects. No single feature of thoracic situs is completely reliable. All available data should be used to make the diagnosis.

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References

  1. Anderson C, Devine WA, Anderson RH, Debich DE, Zuberbuhler JR (1990) Abnormalities of the spleen in relation to congenital malformations of the heart: a survey of necropsy findings in children. Br Heart J 63:122–128

    Article  PubMed  CAS  Google Scholar 

  2. Brandt PWT, Calder AL (1977) Cardiac connections: the segmental approach to radiologic diagnosis in congenital heart disease. Curr Probl Diagn Radiol 7:1–35

    Article  PubMed  CAS  Google Scholar 

  3. Brandt HM, Liebow AA (1958) Right pulmonary isomerism associated with venous, splenic, and other anomalies. Lab Invest 7:469–504

    PubMed  CAS  Google Scholar 

  4. Calder AL (1990) Morphological features in situs ambiguus. In: Crupi G, Parenzan L, Anderson RH (eds) Perspectives in pediatric cardiac surgery. Futura Publishing Company, Mount Kisco, NY, pp 273–277

    Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  6. Clarkson PM, Brandt PW, Barratt-Boyes BG, Neutze JM (1972) “Isolated atrial inversion”. Visceral situs solitus, viscero atrial discordance, discordant ventricular d loop without transposition, dextrocardia: diagnosis and surgical correction. Am J Cardiol 29:877–881

    Article  PubMed  CAS  Google Scholar 

  7. Cohen SR, Landing BH (1976) Tracheostenosis and bronchial abnormalities associated with pulmonary artery sling. Ann Otol Rhinol Laryngol 85:582–590

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  9. Debich DE, Devine WA, Anderson RH (1990) Polysplenia with normally structured hearts. Am J Cardiol 65:1274–1275

    Article  PubMed  CAS  Google Scholar 

  10. Devine WA, Debich DE, Taylor SR (1988) Symmetrical bronchial pattern with normal atrial morphology. Int J Cardiol 20:395–398

    Article  PubMed  CAS  Google Scholar 

  11. Epstein ML, Formanek AG, Bessinger FB, Edwards JE (1980) Situs inversus with complete transposition. Am Heart J 99:510–516

    Article  PubMed  CAS  Google Scholar 

  12. Evans WN (2010) Thoracoabdominal situs: a practical approach accompanied by a short history of descriptive terms. Pediatr Cardiol 31:1049–1051

    Article  PubMed  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  14. Freedom RM, Harrington DP (1974) Anatomically corrected malposition of the great arteries. Report of 2 cases, one with congenital asplenia; frequent association with juxtaposition of atrial appendages. Br Heart J 36:207–215

    Article  PubMed  CAS  Google Scholar 

  15. Freedom RM, Culham G, Rowe RD (1978) The criss-cross and superoinferior ventricular heart: an angiocardiographic study. Am J Cardiol 42:620–628

    Article  PubMed  CAS  Google Scholar 

  16. Gerlis LM, Dura-Vila G, Ho SY (2000) Isomeric arrangement of the left atrial appendages and visceral heterotaxy: two atypical cases. Cardiol Young 10:140–144

    Article  PubMed  CAS  Google Scholar 

  17. Jewell F, Joffe H, Wilde P (1993) Isomerism of the left atrial appendages associated with bronchoatrial discordance and unusual systemic venous drainage. Cardiol Young 3:225–228

    Article  Google Scholar 

  18. Landay MJ, Chaw C, Bordlee RP (1982) Bilateral left lungs: unusual variation of hilar anatomy. AJR Am J Roentgenol 138:1162–1164

    PubMed  CAS  Google Scholar 

  19. Landing BH, Lawrence TY, 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 

  20. 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 

  21. Moller JH, Nakib A, Anderson RC, Edwards JE (1967) Congenital cardiac disease associated with polysplenia. A developmental complex of bilateral “left-sidedness”. Circulation 36:789–799

    PubMed  CAS  Google Scholar 

  22. 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 

  23. Peoples WM, Moller JH, Edwards JE (1983) Polysplenia: a review of 146 cases. Pediatr Cardiol 4:129–137

    Article  PubMed  CAS  Google Scholar 

  24. Phoon CK, Neill CA (1994) Asplenia syndrome: Insight into embryology through an analysis of cardiac and extracardiac anomalies. Am J Cardiol 73:581–587

    Article  PubMed  CAS  Google Scholar 

  25. 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 

  26. Prendiville TW, Barton LL, Thompson WR, Fink DL, Holmes KW (2010) Heterotaxy syndrome: defining contemporary disease trends. Pediatr Cardiol 31:1052–1058

    Article  PubMed  Google Scholar 

  27. Raman R, Al Ali SY, Poole CA, Dawson BV, Carman JB, Calder L (2003) Isomerism of the right atrial appendages: clinical, anatomical, and microscopic study of a long-surviving case with asplenia and ciliary abnormalities. Clin Anat 16:269–276

    Article  PubMed  CAS  Google Scholar 

  28. Rose V, Izukawa T, Moes CA (1975) Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart J 37:840–852

    Article  PubMed  CAS  Google Scholar 

  29. Sapire DW, Ho SY, Anderson RH, Rigby ML (1986) Diagnosis and significance of atrial isomerism. Am J Cardiol 58:342–346

    Article  PubMed  CAS  Google Scholar 

  30. Stanger P, Rudolph AM, Edwards JE (1977) Cardiac malpositions: an overview based on study of sixty-five necropsy specimens. Circulation 56:159–172

    PubMed  CAS  Google Scholar 

  31. Tunaoglu FS, Olgunturk R, Gucuyener K, Demirsoy S, Uzamis C (1991) Unusual variation of asplenia syndrome. Jpn Heart J 32:859–865

    Article  PubMed  CAS  Google Scholar 

  32. Uemura H, Ho SY, Devine WA, Anderson RH (1995) Analysis of visceral heterotaxy according to splenic status, appendage morphology, or both. Am J Cardiol 76:846–849

    Article  PubMed  CAS  Google Scholar 

  33. Van Mierop LH, Wiglesworth FW (1962) Isomerism of the cardiac atria in the asplenia syndrome. Lab Invest 11:1303–1315

    Google Scholar 

  34. Van Mierop LH, 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 

  35. Van Praagh R (1972) The segmental approach to diagnosis in congenital heart disease. Birth Defects (Original Article Series) 8:4–23

    Google Scholar 

  36. Van Praagh S, Kreuzer J, Alday L, Van Praagh R (1990) Systemic and pulmonary venous connections in visceral heterotaxy, with emphasis on the diagnosis of the atrial situs: A study of 109 post mortem cases. In: Clark E, Takao A (eds) Developmental cardiology: morphogenesis and function. Futura Publishing Company, Mount Kisco, NY, pp 671–721

    Google Scholar 

  37. Winer-Muram HT, Tonkin IL (1989) The spectrum of heterotaxic syndromes. Radiol Clin North Am 27:1147–1170

    PubMed  CAS  Google Scholar 

  38. Winer-Muram H, Ellis JV, Scott RL, Pinstein ML (1985) Isolated left thoracic isomerism. Radiology 155:10

    PubMed  CAS  Google Scholar 

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Correspondence to A. Louise Calder.

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Louise Calder, A. Thoracic Situs As an Indicator of Atrial Appendage Morphology: A Postmortem Study of 306 Specimens With Situs Solitus in 250 and Heterotaxy in 56 Cases. Pediatr Cardiol 32, 875–884 (2011). https://doi.org/10.1007/s00246-011-0003-z

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