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Ann Thorac Surg 2000;69:1296-1297
© 2000 The Society of Thoracic Surgeons

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CORRESPONDENCE

More about obstructive lesions of the right aortic arch

^a Department of Cardiothoracic Surgery, Hadassah University Hospital, PO Box 12000, Jerusalem 91120, Israel

e-mail: eamir{at}md2.huji.ac.il

To the Editor

I read with interest the recent articles about obstructive lesions of the right aortic arch [1, 2]. Both articles did not review a rare and unique combination of anomalies—namely tetralogy of Fallot with coarctation of the aorta [3]. One article even explained, according to the flow-related development of the great arteries, why hypoplasia of the aortic isthmus is unlikely to develop in the presence of decreased or absent right-sided outflow tract [1]. However, seven such cases were reported by 1995, of which six had a right aortic arch. The two recent articles reviewed together 22 cases of right aortic arch with coarctation and situs solitus. Failure to include six published cases (21% of the total published experience) resulted in the lack of discussion of the possible pathogenetic mechanisms and the surgical implications of this particular combination. As demonstrated in our report [3], neither fetal hydrodynamic conditions nor ductal closure could explain the formation of coarctation in this rare association of anomalies. We proposed that external compression of the right-sided aorta during fetal life, by a retroaortic innominate vein, may have been responsible for aortic buckling and segmental narrowing. Microdeletions in chromosome 22q11 may result in insufficiency of the neural crest cells and developmental abnormalities in the conotruncus and the embryonic pharyngeal arches [2, 4]. The high aortic arch in our case is a frequent finding in cases with tetralogy associated with 22q11 deletions [4]. Coarctation repair followed immediately or later by tetralogy repair was successful in 4 of the 5 operative survivors.

References

1. Ad N., Vidne B.A. Coarctation of the aorta with right aortic arch. Ann Thorac Surg 1999;67:1125-1129.[Abstract/Free Full Text]
2. McElhinney D.B., Tworetzky W., Hanley F.L., Rudolph A.M. Congenital obstructive lesions of the right aortic arch. Ann Thorac Surg 1999;67:1194-1202.[Abstract/Free Full Text]
3. Elami A., Rein A.J.J.T., Preminger T., Milgalter E. Tetralogy of Fallot, absent pulmonary valve, partial anomalous pulmonary venous return and coarctation of the aorta. Int J Cardiol 1995;52:203-206.[Medline]
4. Momma K. Tetralogy of Fallot, absent pulmonary valve and coarctation of the aorta. Int J Cardiol 1996;54:287.[Medline]

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Amir Elami Permission Requests Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Elami, A. Search for Related Content PubMed Articles by Elami, A.