Abstract
The atrial morphology and venous connections were assessed "blind" in 51 necropsy specimens from patients with visceral heterotaxy. This was compared with bronchial morphology as established by dissection. Six specimens were found to have both atria and bronchi in situs solitus or inversus, and were rejected. In the remainder, atrial isomerism was diagnosed, though this required minor revision of the atrial assessment in two patients. Thirty-four patients had isomeric right atria and bronchi, while 11 had isomeric left atria and bronchi. In seven cases, splenic status was unknown, but in seven of the remaining 38 (18.4%) atrial isomerism was not associated with either asplenia or polysplenia. Nevertheless, right isomerism was strongly associated with total anomalous pulmonary venous drainage (as is asplenia) and left isomerism was likewise associated with interruption of the inferior vena cava (as is polysplenia). Bilateral superior venae cavae and hepatic veins, and absence of the coronary sinus, were frequent in both forms of isomerism (as they are in asplenia and polysplenia). These findings suggest that atrial situs can be defined as solitus inversus, right isomerism, and left isomerism. This determination of atrial situs is quite independent of any other abnormalities of visceral situs. The high incidence of anomalies of both venous return and common atrium resulted in presumed complete mixing of blood at atrial level in all but one patient (97.8%), making the haemodynamic connection between atria and ventricles almost always ambiguous. To describe this anatomical connection as ambiguous when there are two ventricles present is therefore no more than recognition of anatomical and haemodynamic reality.
Full text
PDF










Images in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Anselmi G., Muñoz S., Blanco P., Machado I., De la Cruz M. V. Systematization and clinical study of dextroversion, mirror-image dextrocardia, and laevoversion. Br Heart J. 1972 Nov;34(11):1085–1098. doi: 10.1136/hrt.34.11.1085. [DOI] [PMC free article] [PubMed] [Google Scholar]
- BRANDT H. M., LIEBOW A. A. Right pulmonary isomerism associated with venous, splenic, and other anomalies. Lab Invest. 1958 Sep-Oct;7(5):469–504. [PubMed] [Google Scholar]
- Bharati S., Lev M. The course of the conduction system in dextrocardia. Circulation. 1978 Jan;57(1):163–171. doi: 10.1161/01.cir.57.1.163. [DOI] [PubMed] [Google Scholar]
- Brandt P. W., Calder A. L. Cardiac connections: the segmental approach to radiologic diagnosis in congenital heart disease. Curr Probl Diagn Radiol. 1977 May-Jun;7(3):1–35. doi: 10.1016/s0363-0188(77)80006-6. [DOI] [PubMed] [Google Scholar]
- Caruso G., Becker A. E. How to determine atrial situs? Considerations initiated by 3 cases of absent spleen with a discordant anatomy between bronchi and atria. Br Heart J. 1979 May;41(5):559–567. doi: 10.1136/hrt.41.5.559. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Deanfield J. E., Leanage R., Stroobant J., Chrispin A. R., Taylor J. F., Macartney F. J. Use of high kilovoltage filtered beam radiographs for detection of bronchial situs in infants and young children. Br Heart J. 1980 Nov;44(5):577–583. doi: 10.1136/hrt.44.5.577. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Dickinson D. F., Wilkinson J. L., Anderson K. R., Smith A., Ho S. Y., Anderson R. H. The cardiac conduction system in situs ambiguus. Circulation. 1979 May;59(5):879–885. doi: 10.1161/01.cir.59.5.879. [DOI] [PubMed] [Google Scholar]
- Freedom R. M., Treves S. Splenic scintigraphy and radionuclide venography in the heterotaxy syndrome. Radiology. 1973 May;107(2):381–386. doi: 10.1148/107.2.381. [DOI] [PubMed] [Google Scholar]
- IVEMARK B. I. 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 Nov;44(Suppl 104):7–110. [PubMed] [Google Scholar]
- Kirklin J. W., Pacifico A. D., Bargeron L. M., Jr, Soto B. Cardiac repair in anatomically corrected malposition of the great arteries. Circulation. 1973 Jul;48(1):153–159. doi: 10.1161/01.cir.48.1.153. [DOI] [PubMed] [Google Scholar]
- Landing B. H., Lawrence T. Y., Payne V. C., Jr, Wells T. R. Bronchial anatomy in syndromes with abnormal visceral situs, abnormal spleen and congenital heart disease. Am J Cardiol. 1971 Oct;28(4):456–462. doi: 10.1016/0002-9149(71)90010-5. [DOI] [PubMed] [Google Scholar]
- Layman T. E., Levine M. A., Amplatz K., Edwards J. E. "Asplenic syndrome" in association with rudimentary spleen. Am J Cardiol. 1967 Jul;20(1):136–140. doi: 10.1016/0002-9149(67)90120-8. [DOI] [PubMed] [Google Scholar]
- Leachman R. D., Angelini P., Cokkinos D. V. Hepato-atrial dissociation. Report of two cases and clinical implications. Chest. 1973 Jun;63(6):926–932. doi: 10.1378/chest.63.6.926. [DOI] [PubMed] [Google Scholar]
- Lev M., Liberthson R. R., Eckner F. A., Arcilla R. A. Pathologic anatomy of dextrocardia and its clinical implications. Circulation. 1968 Jun;37(6):979–999. doi: 10.1161/01.cir.37.6.979. [DOI] [PubMed] [Google Scholar]
- Lev M., Liberthson R. R., Golden J. G., Eckner F. A., Arcilla R. A. The pathologic anatomy of mesocardia. Am J Cardiol. 1971 Oct;28(4):428–435. doi: 10.1016/0002-9149(71)90006-3. [DOI] [PubMed] [Google Scholar]
- Liberthson R. R., Hastreiter A. R., Sinha S. N., Bharati S., Novak G. M., Lev M. Levocardia with visceral heterotaxy--isolated levocardia: pathologic anatomy and its clinical implications. Am Heart J. 1973 Jan;85(1):40–54. doi: 10.1016/0002-8703(73)90524-3. [DOI] [PubMed] [Google Scholar]
- Moller J. H., Nakib A., Anderson R. C., Edwards J. E. Congenital cardiac disease associated with polysplenia. A developmental complex of bilateral "left-sidedness". Circulation. 1967 Nov;36(5):789–799. doi: 10.1161/01.cir.36.5.789. [DOI] [PubMed] [Google Scholar]
- Partridge J. B., Scott O., Deverall P. B., Macartney F. J. Visualization and measurement of the main bronchi by tomography as an objective indicator of thoracic situs in congenital heart disease. Circulation. 1975 Jan;51(1):188–196. doi: 10.1161/01.cir.51.1.188. [DOI] [PubMed] [Google Scholar]
- Randall P. A., Moller J. H., Amplatz K. The spleen and congenital heart disease. Am J Roentgenol Radium Ther Nucl Med. 1973 Nov;119(3):551–559. doi: 10.2214/ajr.119.3.551. [DOI] [PubMed] [Google Scholar]
- Rose V., Izukawa T., Moës C. A. Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis. Br Heart J. 1975 Aug;37(8):840–852. doi: 10.1136/hrt.37.8.840. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Stanger P., Rudolph A. M., Edwards J. E. Cardiac malpositions. An overview based on study of sixty-five necropsy specimens. Circulation. 1977 Aug;56(2):159–172. doi: 10.1161/01.cir.56.2.159. [DOI] [PubMed] [Google Scholar]
- Tynan M. J., Becker A. E., Macartney F. J., Jiménez M. Q., Shinebourne E. A., Anderson R. H. Nomenclature and classification of congenital heart disease. Br Heart J. 1979 May;41(5):544–553. doi: 10.1136/hrt.41.5.544. [DOI] [PMC free article] [PubMed] [Google Scholar]
- VAN MIEROP L. H., WIGLESWORTH F. W. Isomerism of the cardiac atria in the asplenia syndrome. Lab Invest. 1962 Dec;11:1303–1315. [PubMed] [Google Scholar]
- VANPRAAGH R., VANPRAAGH S., VLAD P., KEITH J. D. ANATOMIC TYPES OF CONGENITAL DEXTROCARDIA: DIAGNOSTIC AND EMBRYOLOGIC IMPLICATIONS. Am J Cardiol. 1964 Apr;13:510–531. doi: 10.1016/0002-9149(64)90159-6. [DOI] [PubMed] [Google Scholar]
- Van Mierop L. H., Eisen S., Schiebler G. L. The radiographic appearance of the tracheobronchial tree as an indicator of visceral situs. Am J Cardiol. 1970 Oct;26(4):432–435. doi: 10.1016/0002-9149(70)90743-5. [DOI] [PubMed] [Google Scholar]
- Van Praagh R. Terminology of congenital heart disease. Glossary and commentary. Circulation. 1977 Aug;56(2):139–143. doi: 10.1161/01.cir.56.2.139. [DOI] [PubMed] [Google Scholar]
- Vaughan T. J., Hawkins I. F., Jr, Elliott L. P. Diagnosis of polysplenia syndrome. Radiology. 1971 Dec;101(3):511–518. doi: 10.1148/101.3.511. [DOI] [PubMed] [Google Scholar]
- Waldman J. D., Rosenthal A., Smith A. L., Shurin S., Nadas A. S. Sepsis and congenital asplenia. J Pediatr. 1977 Apr;90(4):555–559. doi: 10.1016/s0022-3476(77)80365-x. [DOI] [PubMed] [Google Scholar]