Abstract
Purpose
Congestive heart failure is one of the major causes of early death of patients with trisomy 18. Ventricular septal defect (VSD) is the most common heart defect in patients with trisomy 18, and closure of the VSD may elongate the lifespan of the patient. Morphological characteristics of these patients, such as thoracic deformity, prominent right ventricular hypertrophy, and dysplastic tricuspid valve may complicate closure of the VSD. We report our initial experience of VSD closure in patients with trisomy 18 and estimate the feasibility of the surgical procedure.
Methods
Between June 2005 and September 2007, five female patients with trisomy 18 and VSD underwent radical operations. Four of them had undergone previous palliative surgery entailing pulmonary artery banding.
Results
All patients survived surgery. The average cardiac arrest time during surgery was 74 ± 22 min. No early death occurred within 30 days of surgery. One patient with hydrocephalus treated by a previous ventriculoperitoneal shunt died in hospital owing to postoperative intracranial hypertension. Four of five patients were discharged from the hospital. Of the four discharged patients, a girl who underwent primary radical operation died of pneumonia. Another patient died of sudden cardiopulmonary arrest. The average survival of the patients in this study was 815 ± 389 days at data acquisition, with two patients still alive.
Conclusion
Closure of VSDs in patients with trisomy 18 was found feasible and was associated with extended survival.
Similar content being viewed by others
References
Novick WM, Sandoval N, Lazorhysynets VV, Castillo V, Baskevitch A, Mo X, et al. Flap valve double patch closure of ventricular septal defects in children with increased pulmonary vascular resistance. Ann Thorac Surg 2006;81:788.
Carter PE, Pearn JH, Bell J, Martin N, Anderson NG. Survival in trisomy 18: life tables for use in genetic counselling and clinical paediatrics. Clin Genet 1985;27:59–61.
Goldstein H, Nielsen KG. Rates and survival of individuals with trisomy 13 and 18: data from a 10-year period in Denmark. Clin Genet 1988;34:366–372.
Embleton ND, Wyllie JP, Wright MJ, Burn J, Hunter S. Natural history of trisomy 18. Arch Dis Child Fetal Neonatal Ed 1996;75:F38–F41.
Baty BJ, Blackburn BL, Carey JC. Natural history of trisomy 18 and trisomy 13. I. Growth, physical assessment, medical histories, survival, and recurrence risk. Am J Med Genet 1994;49:175–188.
Rasmussen SA, Wong LY, Yang Q, May KM, Friedman JM. Population-based analyses of mortality in trisomy 13 and trisomy 18. Pediatrics 2003;111(Pt 1):777–784.
Root S, Carey JC. Survival in trisomy 18. Am J Med Genet 1994;49:170–174.
Niedrist D, Riegel M, Achermann J, Schinzel A. Survival with trisomy 18: data from Switzerland. Am J Med Genet A 2006;140:952–959.
Van Praagh S, Truman T, Firpo A, Bano-Rodrigo A, Fried R, McManus B, et al. Cardiac malformations in trisomy-18: a study of 41 postmortem cases. J Am Coll Cardiol 1989;13:1586–1597.
Lizárraga MA, Mintegui S, Sánchez Echániz J, Galdeano JM, Pastor E, Cabrera A. Heart malformations in trisomy 13 and trisomy 18. Rev Esp Cardiol 1991;44:605–610.
Musewe NN, Alexander DJ, Teshima I, Smallhorn JF, Freedom RM. Echocardiographic evaluation of the spectrum of cardiac anomalies associated with trisomy 13 and trisomy 18. J Am Coll Cardiol 1990;15:673–677.
Kaneko Y, Kobayashi J, Yamamoto Y, Yoda H, Kanetaka Y, Nakajima Y, et al. Intensive cardiac management in patients with trisomy 13 or trisomy 18. Am J Med Genet A 2008;146A:1372–1380.
Graham EM, Bradley SM, Shirali GS, Hills CB, Atz AM. Effectiveness of cardiac surgery in trisomies 13 and 18 (from the Pediatric Cardiac Care Consortium). Am J Cardiol 2004;93:801–803.
Balderston SM, Shaffer EM, Washington RL, Sondheimer HM. Congenital polyvalvular disease in trisomy 18: echocardiographic diagnosis. Pediatr Cardiol 1990;11:138–142.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kobayashi, J., Kaneko, Y., Yamamoto, Y. et al. Radical surgery for a ventricular septal defect associated with trisomy 18. Gen Thorac Cardiovasc Surg 58, 223–227 (2010). https://doi.org/10.1007/s11748-009-0431-3
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11748-009-0431-3