Hostname: page-component-cd9895bd7-dk4vv Total loading time: 0 Render date: 2024-12-26T20:25:21.382Z Has data issue: false hasContentIssue false
  • English
  • Français

Sudden cardiac death associated with cardiac catheterization in Williams syndrome: a case report and review of literature

Published online by Cambridge University Press:  05 April 2019

Samer Abu-Sultaneh Open the ORCID record for Samer Abu-Sultaneh [Opens in a new window] ,
Mercia J. Gondim ,
Ryan D. Alexy  and
Christopher W. Mastropietro
Samer Abu-Sultaneh*
Affiliation:
Department of Pediatrics, Division of Pediatric Critical Care Medicine, Indiana University School of Medicine, Riley Hospital for Children at Indiana University Health, Indianapolis, IN, USA
Mercia J. Gondim
Affiliation:
Department of Pathology & Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA
Ryan D. Alexy
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Indiana University School of Medicine and Riley Hospital for Children at Indiana University Health, Indianapolis, IN, USA
Christopher W. Mastropietro
Affiliation:
Department of Pediatrics, Division of Pediatric Critical Care Medicine, Indiana University School of Medicine, Riley Hospital for Children at Indiana University Health, Indianapolis, IN, USA
*
Author for correspondence: Samer Abu-Sultaneh, MD, FAAP, Pediatric Critical Care, Riley Hospital for Children at Indiana University Health, 705 Riley Hospital Drive, Riley Phase 2 Room 4900, Indianapolis, IN 46202-5225, USA. Tel: +1 317 948 7185; Fax: +1 317 944 7267; E-mail: sultaneh@iu.edu
Get access Rights & Permissions [Opens in a new window]

Abstract

Williams syndrome is a rare genetic disease that affects elastin production, leading to medium and large vessel stenoses and other abnormalities. Cardiac manifestations of Williams syndrome are the most life-threatening, occurring in 80% of children. Children with Williams syndrome are known to be at risk for sudden cardiac death. These tragic events are often precipitated by diagnostic or therapeutic procedures requiring anaesthesia or sedation, such as cardiac catheterisation. We present the case of a 3-month-old infant with Williams syndrome who suffered sudden cardiac arrest during cardiac catheterisation and subsequent arrest approximately 48 hours after the procedure. We also review the current literature focused on children with Williams syndrome who have suffered sudden cardiac arrest during or after cardiac catheterisation procedures.

Keywords

Williams syndromepaediatricscardiac catheterisationdeathsuddencardiaccoronary stenosisanaesthesiageneral
Type
Review Article
Information
Cardiology in the Young , Volume 29 , Issue 4 , April 2019 , pp. 457 - 461
Copyright
© Cambridge University Press 2019 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Pober, BR. Williams–Beuren syndrome. N Engl J Med 2010; 362: 239–252.CrossRefGoogle ScholarPubMed
Pober, BR, Johnson, M, Urban, Z. Mechanisms and treatment of cardiovascular disease in Williams–Beuren syndrome. J Clin Invest 2008; 118: 1606–1615.CrossRefGoogle ScholarPubMed
McCarty, HM, Tang, X, Swearingen, CJ, Collins, RT II. Comparison of electrocardiographic QTc duration in patients with supravalvar aortic stenosis with versus without Williams syndrome. Am J Cardiol 2013; 111: 1501–1504.CrossRefGoogle ScholarPubMed
Pham, PP, Moller, JH, Hills, C, Larson, V, Pyles, L. Cardiac catheterization and operative outcomes from a multicenter consortium for children with Williams syndrome. Pediatr Cardiol 2009; 30: 9–14.CrossRefGoogle ScholarPubMed
Collins, RT II. Cardiovascular disease in Williams syndrome. Circulation 2013; 127: 2125–2134.CrossRefGoogle ScholarPubMed
Wessel, A, Gravenhorst, V, Buchhorn, R, Gosch, A, Partsch, CJ, Pankau, R. Risk of sudden death in the Williams–Beuren syndrome. Am J Med Genet A 2004; 127A: 234–237.CrossRefGoogle ScholarPubMed
Bird, LM, Billman, GF, Lacro, RV, et al. Sudden death in Williams syndrome: report of ten cases. J Pediatr 1996; 129: 926–931.CrossRefGoogle ScholarPubMed
Horowitz, PE, Akhtar, S, Wulff, JA, Al Fadley, F, Al Halees, Z. Coronary artery disease and anesthesia-related death in children with Williams syndrome. J Cardiothorac Vasc Anesth 2002; 16: 739–741.CrossRefGoogle ScholarPubMed
Bragg, K, Fedel, GM, DiProsperis, A. Cardiac arrest under anesthesia in a pediatric patient with Williams syndrome: a case report. AANA J 2005; 73: 287–293.Google Scholar
Monfared, A, Messner, A. Death following tonsillectomy in a child with Williams syndrome. Int J Pediatr Otorhinolaryngol 2006; 70: 1133–1135.CrossRefGoogle Scholar
Gupta, P, Tobias, JD, Goyal, S, et al. Sudden cardiac death under anesthesia in pediatric patient with Williams syndrome: a case report and review of literature. Ann Card Anaesth 2010; 13: 44–48.CrossRefGoogle ScholarPubMed
Collins, RT II, Kaplan, P, Somes, GW, Rome, JJ. Long-term outcomes of patients with cardiovascular abnormalities and Williams syndrome. Am J Cardiol 2010; 105: 874–878.CrossRefGoogle ScholarPubMed
Olsen, M, Fahy, CJ, Costi, DA, Kelly, AJ, Burgoyne, LL. Anaesthesia-related haemodynamic complications in Williams syndrome patients: a review of one institution’s experience. Anaesth Intensive Care 2014; 42: 619–624.CrossRefGoogle ScholarPubMed
Hornik, CP, Collins, RT II, Jaquiss, RD, et al. Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. J Thorac Cardiovasc Surg 2015; 149: 1516–1522 e1.CrossRefGoogle ScholarPubMed
Conway, EE Jr., Noonan, J, Marion, RW, Steeg, CN. Myocardial infarction leading to sudden death in the Williams syndrome: report of three cases. J Pediatr 1990; 117: 593–595.CrossRefGoogle ScholarPubMed
Latham, GJ, Ross, FJ, Eisses, MJ, Richards, MJ, Geiduschek, JM, Joffe, DC. Perioperative morbidity in children with elastin arteriopathy. Paediatr Anaesth 2016; 26: 926–35.CrossRefGoogle ScholarPubMed
Krous, HF, Wahl, C, Chadwick, AE. Sudden unexpected death in a toddler with Williams syndrome. Forensic Sci Med Pathol 2008; 4: 240–245.CrossRefGoogle Scholar
Jakob, A, Unger, S, Arnold, R, et al. A family with a new elastin gene mutation: broad clinical spectrum, including sudden cardiac death. Cardiol Young 2011; 21: 62–65.CrossRefGoogle ScholarPubMed
Upadhyay, S, Seiden, H, Epstein, S. Left main coronary artery stenting after cardiac arrest in an infant with William’s syndrome. Catheter Cardiovasc Interv 2011; 78: 940–944.CrossRefGoogle Scholar
Burch, TM, McGowan, FX Jr, Kussman, BD, Powell, AJ, DiNardo, JA. Congenital supravalvular aortic stenosis and sudden death associated with anesthesia: what’s the mystery? Anesth Analg 2008; 107: 1848–1854.CrossRefGoogle ScholarPubMed
Fricke, TA, d’Udekem, Y, Brizard, CP, et al. Surgical repair of supravalvular aortic stenosis in children With Williams syndrome: a 30-year experience. Ann Thorac Surg 2015; 99: 1335–1341.CrossRefGoogle ScholarPubMed
van Pelt, NC, Wilson, NJ, Lear, G. Severe coronary artery disease in the absence of supravalvular stenosis in a patient with Williams syndrome. Pediatr Cardiol 2005; 26: 665–667.CrossRefGoogle Scholar