Hostname: page-component-8448b6f56d-sxzjt Total loading time: 0 Render date: 2024-04-17T09:35:33.363Z Has data issue: false hasContentIssue false
  • English
  • Français

Determining bronchial morphology for the purposes of segregating so-called heterotaxy

Published online by Cambridge University Press:  10 July 2015

Rohit S. Loomba ,
Andrew N. Pelech ,
Parinda H. Shah  and
Robert H. Anderson
Rohit S. Loomba*
Affiliation:
Children’s Hospital of Wisconsin, Division of Cardiology, Milwaukee, Wisconsin, United States of America
Andrew N. Pelech
Affiliation:
Children’s Hospital of Wisconsin, Division of Cardiology, Milwaukee, Wisconsin, United States of America
Parinda H. Shah
Affiliation:
Advocate Illinois Masonic Medical Center, Division of Radiology, Chicago, Illinois, United States of America
Robert H. Anderson
Affiliation:
Institute of Genetic Medicine, Newcastle University, Newcastle Upon Tyne, United Kingdom
*
Correspondence to: R. S. Loomba, Children’s Hospital of Wisconsin, Division of Cardiology, 9000 Wisconsin Avenue, Milwaukee, WI 53226, United States of America. Loomba.rohit@gmail.com
Get access Rights & Permissions [Opens in a new window]

Abstract

Introduction

Heterotaxy is a unique clinical entity in which lateralisation of the thoraco-abdominal organs is abnormal, typically with isomerism of the bronchial tree and atrial appendages. This study was carried out to determine whether routine clinical imaging such as chest radiographs, angiographic images, and CT/MRI can determine bronchial isomerism, and how sidedness of bronchial isomerism correlates with overall features anticipated in hearts with isomeric atrial appendages.

Methods and results

We identified 73 patients with heterotaxy, in whom imaging clearly demonstrated the bronchial tree, seen at our institution since 1998. We calculated bronchial angles and lengths using all the available imaging modalities to determine the presence and sidedness of bronchial isomerism. This was then compared with the anticipated presence of isomeric atrial appendages based on the overall clinical findings, as the appendages themselves had not specifically been imaged.

The ratio of bronchial lengths revealed bronchial isomerism in all patients, with bronchial angles permitting distinction of right as opposed to left isomerism. We noted discordances between the identified bronchial isomerism and the presumed arrangement of the atrial appendages in nearly 20% of the patients in our cohort.

Conclusion

Routine clinical imaging with chest radiographs, angiographic imaging, and CT/MRI can determine the presence of bronchial isomerism in patients with so-called heterotaxy. Right as opposed to left isomerism can be distinguished based on bronchial angles. The finding of bronchial isomerism correlates well, but not totally, with the presumed isomerism of the atrial appendages as predicted from the identified intra-cardiac morphology.

Keywords

Bronchial morphologyisomerismheterotaxyX-rayCT
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Issue 4 , April 2016 , pp. 725 - 737
Check for updates
Copyright
© Cambridge University Press 2015 

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

1. Jacobs, JP, Anderson, RH, Weinberg, PM, et al. The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young 2007; 17 (Suppl 2): 1–28.Google Scholar
2. 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
3. Van Mierop, LH, Eisen, S, Schiebler, GL. The radiographic appearance of the tracheobronchial tree as an indicator of visceral situs. Am J Cardiol 1970; 26: 432–435.CrossRefGoogle ScholarPubMed
4. Partridge, J. The radiological evaluation of atrial situs. Clin Radiol 1979; 30: 95–103.Google Scholar
5. Landing, BH, Lawrence, TY, Payne, VC Jr, Wells, TR. Bronchial anatomy in syndromes with abnormal visceral situs, abnormal spleen and congenital heart disease. Am J Cardiol 1971; 28: 456–462.Google Scholar
6. Partridge, JB, Scott, O, Deverall, PB, Macartney, FJ. Visualization and measurement of the main bronchi by tomography as an objective indicator of thoracic situs in congenital heart disease. Circulation 1975; 51: 188–196.CrossRefGoogle ScholarPubMed
7. Ivemark, BI. Implications of agenesis of the spleen on the pathogenesis of conotruncus anomalies in childhood; an analysis of the heart malformations in the splenic agenesis syndrome, with fourteen new cases. Acta Paediatr Suppl 1955; 44: 7–110.Google Scholar
8. 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
9. Meno, C, Ito, Y, Saijoh, Y, et al. Two closely-related left-right asymmetrically expressed genes, lefty-1 and lefty-2: their distinct expression domains, chromosomal linkage and direct neuralizing activity in Xenopus embryos. Genes Cells 1997; 2: 513–524.Google Scholar
10. Meno, C, Shimono, A, Saijoh, Y, et al. Lefty-1 is required for left-right determination as a regulator of lefty-2 and nodal. Cell 1998; 94: 287–297.Google Scholar
11. Hildreth, V, Webb, S, Chaudhry, B, et al. Left cardiac isomerism in the Sonic hedgehog null mouse. J Anat 2009; 214: 894–904.Google Scholar
12. Uemura, H, Ho, SY, Devine, WA, Kilpatrick, LL, Anderson, RH. Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy. Ann Thorac Surg 1995; 60: 561–569.Google Scholar
13. Wolla, CD, Hlavacek, AM, Schoepf, UJ, Bucher, AM, Chowdhury, S. Cardiovascular manifestations of heterotaxy and related situs abnormalities assessed with CT angiography. J Cardiovasc Comput Tomogr 2013; 7: 408–416.Google Scholar
14. Calder, LA. 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 2011; 32: 875–884.Google Scholar
15. Uemura, H, Ho, SY, Devine, WA, Anderson, RH. Analysis of visceral heterotaxy according to splenic status, appendage morphology, or both. Am J Cardiol 1995; 76: 846–849.Google Scholar
16. Van Mierop, LH, Gessner, IH. The morphologic development of the sinoatrial node in the mouse. Am J Cardiol 1970; 25: 204–212.Google Scholar
17. Van Mierop, LH GI, Gessner, IH, Schiebler, GL. Asplenia and polysplenia syndromes. Birth Defects 1972; 8: 26–44.Google Scholar
18. Macartney, FJ, Zuberbuhler, JR, Anderson, RH. Morphological considerations pertaining to recognition of atrial isomerism. Consequences for sequential chamber localisation. Br Heart J 1980; 44: 657–667.CrossRefGoogle ScholarPubMed
19. Ho, SY, Seo, JW, Brown, NA, Cook, AC, Fagg, NL, Anderson, RH. Morphology of the sinus node in human and mouse hearts with isomerism of the atrial appendages. Br Heart J 1995; 74: 437–442.Google Scholar
20. Seo, JW, Brown, NA, Ho, SY, Anderson, RH. Abnormal laterality and congenital cardiac anomalies. Relations of visceral and cardiac morphologies in the iv/iv mouse. Circulation 1992; 86: 642–650.Google Scholar
21. Nakhleh, N, Francis, R, Giese, RA, et al. High prevalence of respiratory ciliary dysfunction in congenital heart disease patients with heterotaxy. Circulation 2012; 125: 2232–2242.Google Scholar
22. Bartz, PJ, Driscoll, DJ, Dearani, JA, et al. Early and late results of the modified fontan operation for heterotaxy syndrome 30 years of experience in 142 patients. J Am Coll Cardiol 2006; 48: 2301–2305.Google Scholar
23. Peoples, WM, Moller, JH, Edwards, JE. Polysplenia: a review of 146 cases. Pediatr Cardiol 1983; 4: 129–137.CrossRefGoogle ScholarPubMed
24. Harden, B, Tian, X, Giese, R, et al. Increased postoperative respiratory complications in heterotaxy congenital heart disease patients with respiratory ciliary dysfunction. J Thorac Cardiovasc Surg 2014; 147: e1292.Google Scholar
25. Kurosawa, H, Kawada, N. The conduction system in heterotaxy. World J Pediatr Congenit Heart Surg 2011; 2: 275–277.Google Scholar
26. Smith, A, Ho, SY, Anderson, RH, et al. The diverse cardiac morphology seen in hearts with isomerism of the atrial appendages with reference to the disposition of the specialised conduction system. Cardiol Young 2006; 16: 437–454.Google Scholar
27. Ho, SY, Fagg, N, Anderson, RH, Cook, A, Allan, L. Disposition of the atrioventricular conduction tissues in the heart with isomerism of the atrial appendages: its relation to congenital complete heart block. J Am Coll Cardiol 1992; 20: 904–910.Google Scholar
28. Ghawi, H, Zghouzi, MM, Emahbes, TM, Awad, SM. Prenatal diagnosis of isolated levocardia and a structurally normal heart: two case reports and a review of the literature. Pediatr Cardiol 2013; 34: 1034–1037.CrossRefGoogle Scholar
29. Jo, DS, Jung, SS, Joo, CU. A case of unusual visceral heterotaxy syndrome with isolated levocardia. Korean Circ J 2013; 43: 705–709.CrossRefGoogle ScholarPubMed
30. Liberthson, RR, Hastreiter, AR, Sinha, SN, Bharati, S, Novak, GM, Lev, M. Levocardia with visceral heterotaxy-isolated levocardia: pathologic anatomy and its clinical implications. Am Heart J 1973; 85: 40–54.Google Scholar
31. Katsuya, S, Yamada, S, Ukita, M, et al. Isolated levocardia: prenatal diagnosis and management. Congenit Anom (Kyoto) 2009; 49: 56–60.CrossRefGoogle ScholarPubMed
32. Salomon, LJ, Baumann, C, Delezoide, AL, et al. Abnormal abdominal situs: what and how should we look for? Prenat Diagn 2006; 26: 282–285.Google Scholar
33. Imamura, T, Momoi, N, Go, H, Ogasawara, K, Sato, M, Hosoya, M. Rare case of isolated levocardia with polysplenia including normally structured lung without cardiac anomaly. Congenit Anom (Kyoto) 2011; 51: 187–190.Google Scholar
34. Fukuzawa, J, Haneda, T, Ishii, Y, et al. A case report of isolated levocardia without intracardiac anomalies associated with sick sinus syndrome. Jpn Circ J 1993; 57: 245–250.Google Scholar
35. Kakura, H, Miyahara, K, Sohara, H, et al. Isolated levocardia associated with absence of inferior vena cava, lobulated spleen and sick sinus syndrome. A case report. Jpn Heart J 1998; 39: 235–241.Google Scholar