DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20175600

Tetralogy of Fallot with Scimitar syndrome in VACTERL: a very rare association

Puneet Anand

Abstract


The most common congenital cardiac malformation that occurs in VACTERL association is ventricular septal defect. Scimitar syndrome is a very rare congenital anomaly of the heart occurring due to maldevelopment of the lung bud or the pulmonary vascularization during early embryogenesis. The occurrence of Scimitar syndrome in VACTERL and its association with another cardiac congenital anomaly such as Tetralogy of Fallot (TOF) is extremely rare. The association of Tetralogy of Fallot with Scimitar syndrome alters the management strategy. Hereby, a very rare combination of Scimitar syndrome and tetralogy of Fallot in a four-year-old boy with VACTERL is presented, who underwent a successful single-stage surgical correction.


Keywords


Anomalous drainage, Dextroposition, Embolization, Scimitar syndrome, Tetralogy of Fallot, VACTERL

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References


Quan L, Smith DW. The VATER association. Vertebral defects, anal atresia, T-E fistula with oesophageal atresia, radial and renal dysplasia: a spectrum of associated defects. J Pediatr. 1973;82:104-7.

Rittler M, Paz JE, Castilla EE. VACTERL association, epidemiologic definition and delineation. Am J Med Genet. 1996;63:529-36.

Solomon BD, Pineda-Alvarez DE, Raam MS, Bous SM, Keaton AA, Vélez JI, et al. Analysis of component findings in 79 patients diagnosed with VACTERL association. Am J Med Genet Part A. 2010;152(A):2236-44.

Cunningham BK, Hadley DW, Hannoush H, Meltzer AC, Niforatos N, Pineda‐Alvarez D, et al. Analysis of cardiac anomalies in VACTERL association. Birth Defects Research Part A: Clinical Molecular Teratol. 2013;97(12):792-7.

Alva C, Valero G, Martínez A, Riera C, David F, Jiménez S, et al. Scimitar syndrome associated to pulmonary atresia with interventricular communication. First reported case. Arch Cardiol Mex. 2004;74:301-5.

Ismail, Sameh R, Al-Muhaya MA,. Kabbani MS. Scimitar syndrome with tetralogy of Fallot and pulmonary atresia. Egyptian Heart J. 2014;74(4):301-5.

Takawira FF, Omar F. Unusual variant of scimitar syndrome associated with an absent right pulmonary artery, stenosis of the inferior vena cava, hemi-azygous continuation and the VACTERL association. Cardiovasc J Afr. 2013;24:e3-e6.

Fritz, Christian J, Reutter HM, Herberg U. Scimitar syndrome in a case with VACTERL association. Cardiology in the Young. 2014:1-4.