Hostname: page-component-cd9895bd7-jn8rn Total loading time: 0 Render date: 2024-12-25T08:15:22.523Z Has data issue: false hasContentIssue false
  • English
  • Français

Echo-morphological correlates concerning the functionally univentricular heart in the setting of isomeric atrial appendages

Published online by Cambridge University Press:  10 January 2006

William T. Mahle ,
Norman H. Silverman ,
Gerald R. Marx  and
Robert H. Anderson
William T. Mahle
Affiliation:
Children's Healthcare of Atlanta and the Emory University School of Medicine, Atlanta, Georgia, United States of America
Norman H. Silverman
Affiliation:
Lucille Packard Children's Hospital, Stanford University School of Medicine, Palo Alto, California, United States of America
Gerald R. Marx
Affiliation:
Children's Hospital, Harvard University School of Medicine, Boston, Massachusetts, United States of America
Robert H. Anderson
Affiliation:
Cardiac Unit, Institute of Child Health, University College, London, United Kingdom
Get access Rights & Permissions [Opens in a new window]

Extract

It has long been known that the most complex combinations of cardiac malformations are those found in the setting of the so-called “splenic syndromes”.1 Many aspects of these syndromes have been controversial over recent years, not least the presence or absence of features of isomerism within the heart.2,3 Recent experience with genetic manipulation of mice, nonetheless, has now shown that it is possible to generate unequivocal evidence of cardiac isomerism, particularly in those animals which show features of right isomerism when the genes responsible for morphologically leftness are knocked out.4 Furthermore, when the crucial philosophical principle known as the “morphological method”5 is applied to the hearts of patients known to have visceral heterotaxy, it is equally clear that patients falling within these groups, when judged on the extent of the pectinate muscles relative to the atrioventricular junctions, exhibit isomerism of either the morphologically right or left atrial appendages.3

Keywords

Aspleniapolyspleniavisceral heterotaxydouble inlet ventricle
Type
Research Article
Information
Cardiology in the Young , Volume 16 , Supplement S1: Controversies and Challenges in the Management of the Functionally Univentricular Heart , February 2006 , pp. 35 - 42
Copyright
© 2006 Cambridge University Press

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

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 1955; 44: 7–110.Google Scholar
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
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
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
Van Praagh R, Leidenfrost RD, Lee SK, Marx G, Wright GB, Van Praagh S. The morphologic method applied to the problem of “single” right ventricle. Am J Cardiol 1982; 50: 929–932.Google Scholar
Moller JH, Nakib A, Anderson RC, Edwards JE. Congenital cardiac disease associated with polysplenia. A developmental complex of bilateral “left-sidedness”. Circulation 1967; 36: 789–799.Google Scholar
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
Freedom RM. The asplenia syndrome: a review of significant extracardiac structural abnormalities in 29 necropsied patients. J Pediatr 1972; 81: 1130–1133.Google Scholar
Chin AJ, Fogel MA. Noninvasive Imaging of Congenital Heart Disease: Before and After Surgical Reconstruction. Futura, Armonk, NY, 1994.
Huhta JC, Smallhorn JF, Macartney FJ. Two dimensional echocardiographic diagnosis of situs. Br Heart J 1982; 48: 97–108.Google Scholar
Cohen MS, Jacobs ML, Weinberg PM, Rychik J. Morphometric analysis of unbalanced common atrioventricular canal using two-dimensional echocardiography. J Am Coll Cardiol 1996; 28: 1017–1023.Google Scholar
van Son JA, Phoon CK, Silverman NH, Haas GS. Predicting feasibility of biventricular repair of right-dominant unbalanced atrioventricular canal. Ann Thorac Surg 1997; 63: 1657–1663.Google Scholar
De Oliveira NC, Sittiwangkul R, McCrindle BW, et al. Biventricular repair in children with atrioventricular septal defects and a small right ventricle: anatomic and surgical considerations. J Thorac Cardiovasc Surg 2005; 130: 250–257.Google Scholar