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Ann Thorac Surg 2004;78:1886-1887
© 2004 The Society of Thoracic Surgeons

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Correspondence

Reply

La Timone Children's Hospital, 264 Rue St. Pierre, Marseille 13385, France

olivier.ghez{at}ap-hm.fr

To the Editor:

We thank Drs Wong, Sett, and Human for their very interesting letter. The case reported by these authors adds to the knowledge of the rare association of right-sided defects with aortic stenosis. However, their case is very different from ours. In our case [1], the aortic valve and pulmonary valve were bicuspid. The intracardiac anatomy was typical of tetralogy of Fallot with hypoplastic conal septum. In their letter, they mentionned a perimembranous defect, which had to be enlarged before VSD closure; this is an unusual finding in tetralogy of Fallot. Furthermore, the association of Alagille syndrome could account for the pulmonary atresia and multiple stenoses of pulmonary arteries needing angioplasties [2] in a patient presenting with aortic stenosis. The possibility of an associated genetic problem should be considered. The association of hypoparathyroidism without 22q11 deletion is also rare and interesting [3]. Concerning left ventricular hypertrophy in our case, the aortic stenosis was only minimal in the neonatal period, which is why the commissurotomy was done at 15 months, at the time of repair.

We would like to take this opportunity to provide follow-up information about our patient. Three years after repair, our patient is doing well. Right ventricular pressure as estimated by echo-Doppler is 50 mm Hg for 104 mm Hg arterial pressure. A 17 mm Hg peak gradient is measured across the aortic valve.

A previous letter suggested by our case report of tetralogy of Fallot with aortic stenosis was published in The Annals [4, 5]. This association would be interesting to consider for further studies.

References

1. Ghez OY, Chetaille PM, Campbell BJ, Van Praagh R, Metras D. Tetralogy of Fallot with aortic valvular stenosis: surgical correction in one case. Ann Thorac Surg. 2002;73:967–969[Abstract/Free Full Text]
2. McElhinney DB, Krantz ID, Bason L, et al. Analysis of cardiovascular phenotype and genotype-phenotype correlation in individuals with a JAG1 mutation and/or Alagille syndrome. Circulation. 2002;106:2567–2574[Abstract/Free Full Text]
3. Koch A, Hofbeck M, Buheitel G, et al. Hypoparathyroidism in conotruncal heart defects. Eur J Pediatr. 2002;161:208–211[Medline]
4. Marino B, Mileto F. Tetralogy of fallot with aortic valvular stenosis and deletion 22q11: reply. Ann Thorac Surg. 2003;75:2010–2011[Free Full Text]
5. Ghez O, Chetaille P, Metras D, Campbell BJ, Van Praagh R. Tetralogy of fallot with aortic valvular stenosis and deletion 22q11: reply. Ann Thorac Surg. 2003;75:2011[Free Full Text]

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): Olivier Ghez Bernard Kreitmann Dominique Metras Permission Requests Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Ghez, O. Articles by Metras, D. Search for Related Content PubMed Articles by Ghez, O. Articles by Metras, D.