Abstract
Objective
There is a paucity of information regarding appropriate management of children with Down’s syndrome and a functional single ventricle. We report the results of bidirectional superior cavopulmonary shunts in six patients with Down’s syndrome with a functional single ventricle.
Methods
Between January 1991 and December 2004, we identified six patients with Down’s syndrome among 263 who had undergone bidirectional superior cavopulmonary shunts (BCPSs). There were four males and two females. The age at BCPS ranged from 1 to 12 years (mean 4.3 ± 3.9 years), and body weight varied between 8.2 and 29.4 kg (mean 13.8 ± 7.8 kg). All six patients had an unbalanced complete atrioventricular septal defect, with right ventricular hypoplasia present in five and left ventricular hypoplasia in one.
Results
There were no operative deaths, but one case required takedown of the BCPS. Except for this case, postoperative courses were generally uneventful. The median duration of follow-up was 46 months (range 12–80 months). Only two of five survivors after BCPS underwent a subsequent Fontan procedure, and one of these patients died of pulmonary hypertension post-operatively. The remaining three patients appeared to have significant risk factors for the Fontan procedure due to severe common atrioventricular valve regurgitation or persistent pulmonary vascular obstructive disease, including one who has completely dropped out from the Fontan track.
Conclusion
Down’s syndrome is a risk factor in patients with functionally single ventricle due to persistent pulmonary hypertension and airway obstruction. These results show that single ventricle repair in patients with Down’s syndrome is accompanied with difficulties, and patient selection for the Fontan procedure should be done carefully.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Knott-Craig CJ, Danielson GK, Schaff HV, Puga FJ, Weaver AL, Driscoll DD. The modified Fontan operation. J Thorac Cardiovasc Surg 1995;109:1237–1243.
Mayer JE, Bridges ND, Lock JE, Hanley FL, Jonas RA, Castañeda AR. Factors associated with reduction in mortality for Fontan operations in patients with single ventricle. J Thorac Cardiovasc Surg 1992;103:444–452.
Bartmus DA, Driscoll DJ, Offord KP, Humes RA, Mair DD, Schaff HV, et al. The modified Fontan operation for children less than 4 years old. J Am Coll Cardiol 1990;15:429–435.
Rowe RD, Uchida IA. Cardiac malformations in mongolism: a prospective study of 184 mongoloid children. Am J Med 1961;31:726–735.
Tandon R, Edwards JE. Cardiac malformations associated with Down’s syndrome. Circulation 1973;47:1349–1355.
Laursen HB. Congenital heart disease in Down’s syndrome. Br Heart J 1976;38:32–38.
Yamaki S, Yasui H, Kado H, Yonenaga K, Nakamura Y, Kikuchi T, et al. Pulmonary vascular disease and operative indication in complete atrioventricular canal defect in early infancy. J Thorac Cardiovasc Surg 1993;106:398–405.
Bertrand P, Navarro H, Caussade S, Holmgren N, Sanchez I. Airway anomalies in children with Down syndrome: endoscopic findings. Pediatr Pulmonol 2003;36:137–141.
Nakata S, Imai Y, Takanashi Y, Kurosawa H, Tezuka K, Nakazawa M, et al. A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow. J Thorac Cardiovasc Surg 1984;88:610–619.
Jacobs IN, Teague WG, Bland JW Jr. Pulmonary vascular complications of chronic airway obstruction in children. Arch Otolaryngol Head Neck Surg 1997;123:700–704.
Spicer R, Uzark K, Cocalis M, Moore J, Mainwaring R, Lamberti J. Down syndrome and functional single ventricle: the Fontan approach (abstract). Cardiol Young 1997;7:138.
Campbell RM, Adatia I, Gow RM, Webb GD, Williams WG, Freedom RM. Total cavopulmonary anastomosis in children with down’s syndrome. Ann Thorac Surg 1998;66:523–526.
Kim HK, Kim WH, Kim SC, Lim C, Lee CH, Kim SJ. Surgical strategy for pulmonary coarctation in the univentricular heart. Eur J Cardiothorac Surg 2006;29:100–104.
Ishibashi N, Koide M, Uchita S, Seguchi M. When should pulmonary artery angioplasty be performed for Fontan candidates with pulmonary coarctation? Two cases of pulmonary artery angioplasty with the Blalock-Taussig shunt on pump in neonates. Jpn J Thorac Cardiovasc Surg 2004;52:185–188.
Driscoll DJ, Oxfford KP, Feldt RH, Schaff HV, Puga FJ, Danielson GK. 5–15 Year follow-up after Fontan operation. Circulation 1992;85:469–496.
Yamaki S, Ajiki H, Haneda K, Takanashi Y, Ban T, Takahashi T. Pulmonary arterial changes in patients dying after a modified Fontan procedure following pulmonary artery banding. Heart Vessels 1994;9:263–268.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Wada, N., Takahashi, Y., Ando, M. et al. Single ventricle repair in children with Down’s syndrome. Gen Thorac Cardiovasc Surg 56, 104–108 (2008). https://doi.org/10.1007/s11748-007-0201-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11748-007-0201-z