Right-Dominant Unbalanced Atrioventricular Canal and Genetic Syndromes




We read the interesting report by Szwast et al in a recent issue of The American Journal of Cardiology . The investigators analyzed the predictors of survival after biventricular repair in patients with right-dominant unbalanced atrioventricular canal. Regarding the presence of genetic syndromes among 45 patients analyzed in the study, only 6 (13.3%) had trisomy 21, and all underwent successful biventricular repair. These data confirm previous observations on the prevalence of left ventricular hypoplasia in children with non–Down syndrome atrioventricular canal and the general good surgical prognosis in children with atrioventricular canal and Down syndrome. Moreover, Szwast et al showed that the more severe left ventricular hypoplasia was associated with other types of genetic syndrome different from trisomy 21, such as Ellis-van Creveld syndrome and CHARGE syndrome. This aspect was also previously noted. The knowledge and recognition of specific cardiac and extracardiac phenotypes associated with various genetic syndromes may guide the perioperative treatment of congenital heart disease, particularly in patients with atrioventricular canal.


References



  1. 1. Szwast A.L., Marino B.S., Rychik J., Gaynor J.W., Spray T.L., and Cohen M.S.: Usefulness of left ventricular inflow index to predict successful biventricular repair in right-dominant unbalanced atrioventricular canal. Am J Cardiol 2011; 107: pp. 103-109

  2. 2. De Biase L., Di Ciommo V., Ballerini L., Bevilacqua M., Marcelletti C., and Marino B.: Prevalence of left-sided obstructive lesions in patients with atrioventricular canal without Down’s syndrome. J Thorac Cardiovasc Surg 1986; 91: pp. 467-469

  3. 3. Formigari R., Di Donato R.M., Gargiulo G., Di Carlo D., Feltri C., Picchio F.M., and Marino B.: Better surgical prognosis for patients with complete atrioventricular septal defect and Down’s syndrome. Ann Thorac Surg 2004; 78: pp. 666-672

  4. 4. Digilio M.C., Marino B., Toscano A., Giannotti A., and Dallapiccola B.: Atrioventricular canal defect without Down syndrome: a heterogeneous malformation. Am J Med Genet 1999; 85: pp. 140-146

  5. 5. Formigari R., Michielon G., Digilio M.C., Piacentini G., Carotti A., Giardini A., Di Donato R.M., and Marino B.: Genetic syndromes and congenital heart defects: how is surgical management affected? Eur J Cardiothorac Surg 2009; 35: pp. 606-614

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Dec 16, 2016 | Posted by in CARDIOLOGY | Comments Off on Right-Dominant Unbalanced Atrioventricular Canal and Genetic Syndromes

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