More about obstructive lesions of the right aortic arch

Abstract

I read with interest the recent articles about obstructive lesions of the right aortic arch [1Ad N. Vidne B.A. Coarctation of the aorta with right aortic arch surgical technique and new classification.Ann Thorac Surg. 1999; 67: 1125-1129Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar, 2McElhinney D.B. Tworetzky W. Hanley F.L. Rudolph A.M. Congenital obstructive lesions of the right aortic arch.Ann Thorac Surg. 1999; 67: 1194-1202Abstract Full Text Full Text PDF PubMed Scopus (48) Google Scholar]. Both articles did not review a rare and unique combination of anomalies—namely tetralogy of Fallot with coarctation of the aorta [3Elami 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: 203-206Abstract Full Text PDF PubMed Scopus (21) Google Scholar]. 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 [1Ad N. Vidne B.A. Coarctation of the aorta with right aortic arch surgical technique and new classification.Ann Thorac Surg. 1999; 67: 1125-1129Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar]. 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 [3Elami 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: 203-206Abstract Full Text PDF PubMed Scopus (21) Google Scholar], 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 [2McElhinney D.B. Tworetzky W. Hanley F.L. Rudolph A.M. Congenital obstructive lesions of the right aortic arch.Ann Thorac Surg. 1999; 67: 1194-1202Abstract Full Text Full Text PDF PubMed Scopus (48) Google Scholar, 4Momma K. Tetralogy of Fallot, absent pulmonary valve and coarctation of the aorta.Int J Cardiol. 1996; 54 ([Letter]): 287Abstract Full Text PDF PubMed Scopus (1) Google Scholar]. The high aortic arch in our case is a frequent finding in cases with tetralogy associated with 22q11 deletions [4Momma K. Tetralogy of Fallot, absent pulmonary valve and coarctation of the aorta.Int J Cardiol. 1996; 54 ([Letter]): 287Abstract Full Text PDF PubMed Scopus (1) Google Scholar]. Coarctation repair followed immediately or later by tetralogy repair was successful in 4 of the 5 operative survivors. ReplyThe Annals of Thoracic SurgeryVol. 69Issue 4Preview Full-Text PDF