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

  • William T. Mahle (a1), Norman H. Silverman (a2), Gerald R. Marx (a3) and Robert H. Anderson (a4)


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


Corresponding author

Correspondence to: William T. Mahle MD, Children's Healthcare of Atlanta, Emory University School of Medicine, 1405 Clifton Road, NE, Atlanta, GA 30322-1062, USA. Tel: +1 404 315 2672; Fax: +1 404 325 6021; E-mail:


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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: 7110.
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: 15041506.
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: 561569.
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: 11891196.
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: 929932.
Moller JH, Nakib A, Anderson RC, Edwards JE. Congenital cardiac disease associated with polysplenia. A developmental complex of bilateral “left-sidedness”. Circulation 1967; 36: 789799.
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: 846849.
Freedom RM. The asplenia syndrome: a review of significant extracardiac structural abnormalities in 29 necropsied patients. J Pediatr 1972; 81: 11301133.
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: 97108.
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: 10171023.
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: 16571663.
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: 250257.


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

  • William T. Mahle (a1), Norman H. Silverman (a2), Gerald R. Marx (a3) and Robert H. Anderson (a4)


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