Skip to main content

Successful Staged Neonatal Repair of Tetralogy of Fallot with Long-Segment Hypoplasia of the Aorta


We describe an extremely rare combination of tetralogy of Fallot (TOF), right-sided cervical aortic arch with long-segment hypoplasia, and other vascular anomalies. A two-stage surgical approach included aortic arch reconstruction followed by right ventricular muscle bundle division and ventricular septal defect closure a few weeks later. The initial clinical presentation, perioperative course, and imaging studies are presented along with a review of the relevant literature. This is the first report of successful neonatal repair of TOF with long-segment hypoplasia of the aorta.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2
Fig. 3


  1. Bullaboy CA, Derkac WM, Johnson DH, Jennings RB Jr (1984) Tetralogy of Fallot and coarctation of the aorta: Successful repair in an infant. Ann Thorac Surg 38:400–401

    Article  CAS  PubMed  Google Scholar 

  2. de Santana RC, Guerra VC, Ikari NM, Ebaiad M (1999) Tetralogy of Fallot and aortic coarctation. A rare association. Arq Bras Cardiol 72:79–84

    Article  PubMed  Google Scholar 

  3. Elami AA, Rein AJJT, Preminger TJ, Milgalter E (1995) Tetralogy of Fallot, absent pulmonary valve, partial anomalous pulmonary venous return and coarctation of the aorta. Int J Cardiol 52:203–206

    Article  CAS  PubMed  Google Scholar 

  4. Freedom RM, Benson LN, Mikailian H (2005) Aortic coarctation in an infant with tetralogy and pulmonary atresia. Cardiol Young 15:667–668

    Article  PubMed  Google Scholar 

  5. Gunthard J, Murdison KA, Wagner HR, Norwood WI Jr (1992) Tetralogy of Fallot and coarctation of the aorta: a rare combination and its clinical implications. Pediatr Cardiol 13:37–40

    CAS  PubMed  Google Scholar 

  6. Ho SY, Anderson RH (1979) Coarctation, tubular hypoplasia, and the ductus arteriosus. Histological study of 35 specimens. Br Heart J 41:268–274

    Article  CAS  PubMed  Google Scholar 

  7. Korula RJ, Bais A, Lal N, Jairaj PS (1991) Interrupted aortic arch with tetralogy of Fallot. A report of an unsuccessful surgically treated case. J Cardiovasc Surg (Torino) 32:541–543

    CAS  Google Scholar 

  8. Purkayastha S, Gupta AK, Varma DR, Bodhey NK, Vattoth S (2006) Absence of the left common carotid artery with cervical origin of the right subclavian artery. Am J Neuroradiol 27:708–711

    CAS  PubMed  Google Scholar 

  9. Rudolph AM, Heymann MA, Spitznas U (1972) Hemodynamic considerations in the development of narrowing of the aorta. Am J Cardiol 30:514–525

    Article  CAS  PubMed  Google Scholar 

  10. Tasar M, Yetiser S, Tasar A, Ugurel S, Gonul E, Saglam M (2004) Congenital absence or hypoplasia of the carotid artery: radioclinical issues. Am J Otolaryngol 25:339–349

    Article  PubMed  Google Scholar 

  11. Tateishi M, Koide M, Mizukami A (2007) Tetralogy of Fallot with atypical coarctation of the aorta and carotid arterial stenosis due to fibromuscular dysplasia. Cardiol Young 17:689–690

    Article  PubMed  Google Scholar 

  12. Yip RC, Deekollu D, Arnold R (2001) Coarctation co-existing with tetralogy of Fallot and pulmonary atresia. Cardiol Young 11:88–90

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations


Corresponding author

Correspondence to Jimmy C. Lu.

Additional information

Jimmy C. Lu and Sanket S. Shah contributed equally to this work.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Lu, J.C., Shah, S.S., Owens, S.T. et al. Successful Staged Neonatal Repair of Tetralogy of Fallot with Long-Segment Hypoplasia of the Aorta. Pediatr Cardiol 31, 124–127 (2010).

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI:


  • Aortic arch hypoplasia
  • Coarctation
  • Interrupted aortic arch
  • Neonatal repair
  • Tetralogy of Fallot