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2015 | 5 | 2 | 6-10
Tytuł artykułu

Relative Certainty as Opposed to Uncertainty in the Diagnosis of Isomerism

Treść / Zawartość
Warianty tytułu
Języki publikacji
EN
Abstrakty
EN
The situation in which the bodily organs are “jumbled up”, frequently described in terms of visceral heterotaxy, was first brought to prominence by Ivemark, who emphasised the situation in terms of anatomy and development of the spleen. Putschar and Mannion then indicated that “between the normal situs, which is asymmetrical, and the situs inversus, which is the asymmetrical mirror-image of normality, a symmetrical situs sometimes exists, and this is the essence of bodily isomerism”. In the setting of the congenitally malformed heart, however, the isomeric features are found uniformly only in the atrial appendages. To date, these such subtle features have largely been recognised at autopsy, but if specifically sought for, they should be identified by the echocardiographer, even when working in the prenatal setting. The positive diagnosis of cardiac isomerism, therefore, depends on the recognition of isomeric atrial appendages. There is no evidence of isomerism at ventricular or arterial level. The relationship of the great vessels as they traverse the diaphragm, nonetheless, has been shown to be helpful in pointing to the need for more specific examination of the atrial appendages. When analysed on this basis, there can only be left or right isomerism, although the isomeric features are not always found uniformly throughout the bodily organs. Should there be disharmony between the systems, the specific findings should be described for each system, thus removing any suggestion of ambiguity. The distinction between left and right isomerism is crucial for counselling, not only for immediate decisions regarding the progress of the pregnancy in question, but for future potential pregnancies. Distinguishing between pregnancies developing with right and left isomerism could also provide the key for determining the genes responsible for the production of laterality.
Wydawca

Czasopismo
Rocznik
Tom
5
Numer
2
Strony
6-10
Opis fizyczny
Daty
wydano
2015-06-01
otrzymano
2015-06-03
zaakceptowano
2015-06-29
online
2015-12-30
Twórcy
  • Division of Pediatric Cardiology, University of Florida College of Medicine, Gainesville, and Congenital Heart Institute of Florida, St. Petersburg, Florida, United States of America, sejjran@ucl.ac.uk
  • Department of Prenatal Cardiology, Polish Mother's Memorial Hospital Research Institute, Lodz, Poland
  • Department of Diagnoses and Prevention Fetal Malformations Medical University of Lodz, Poland
  • Institute of Genetic Medicine, University of Newcastle, Newcastle-upon-Tyne, United Kingdom
Bibliografia
  • 1. Ivemark B.: Implications of agenesis of the spleen in the pathogenesis of conotruncus anomalies in childhood. An analysis of the heart malformations in the splenic agenesis syndrome, with 14 new cases. Acta Paed Scand 1955, 44 (Suppl 104): 1-110
  • 2. Putschar WGJ, Mannion WC.: Congenital absence of the spleen and associated anomalies. Am J Clin Path 1956, 26: 429-470
  • 3. Van Praagh R.: The segmental approach to diagnosis in congenital heart disease. In: Bergsma D (ed.): Birth defects original article series, 1972, vol. VIII, No. 5. The National Foundation - March of Dimes. Williams and Wilkins, Baltimore: 4-234
  • 4. 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[Crossref]
  • 5. Uemura H, Ho SY, Devine WA, Kilpatrick LL, Anderson RH.: Atrial appendages and venoatrial connections in hearts from patients with visceral hetertotaxy. Ann Thor Surg 1995, 60: 561-569
  • 6. Jacobs JP, Anderson RH, Weinberg P et al.: The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young 2007, 17 (Suppl. 2): 1-28[WoS]
  • 7. 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[Crossref]
  • 8. Van Praagh R, David I, Wright GB, Van Praagh S.: Large RV plus small LV is not single RV. Circulation 1980, 61:1057-1058
  • 9. Keeton BR, Macartney FJ, Hunter S, Mortera C, Rees P, Shinebourne EA, Tynan MJ, Wilkinson JL, Anderson RH.: Univentricular heart of right ventricular type with double or common inlet. Circulation 1979, 59: 403‑411[Crossref]
  • 10. Bamforth SD, Bragança J, Farthing CR, Schneider JE, Broadbent C, Michell AC, Clarke K, Neubauer S, Norris D, Brown NA, Anderson RH, Bhattacharya S.: Controls left-right patterning and heart development through a Nodal-Pitx2c pathway. Nat Genet 2004, 36: 1189-1196[Crossref]
  • 11. Meno C, Shimono A, Saijoh Y, Yashiro K, Mochida K, Ohishi S, Noji S, Kondoh S, Hamada H.: Lefty-1 is required for left-right determination as a regulator of lefty-2 and nodal. Cell 1998, 94: 287-229[Crossref]
  • 12. Sharland G.: Fetal cardiology simplified - a practical manual. Tfm publishing, Castle Hill Barns 2013: 60-66
  • 13. 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 [Crossref]
Typ dokumentu
Bibliografia
Identyfikatory
Identyfikator YADDA
bwmeta1.element.-psjd-doi-10_12847_06151
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