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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 13  |  Issue : 1  |  Page : 44-48
Sudden cardiac death under anesthesia in pediatric patient with williams syndrome: A case report and review of literature

1 Division of Pediatric Critical Care, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
2 Division of Pediatric Anesthesiology, University of Missouri, Columbia, MO 65212, USA
3 Department of Medical Education, Metrowest Medical Center, Framingham, MA 01702, USA
4 Division of Pediatric Cardiac Anesthesiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
5 Division of Pediatric Cardiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA

Click here for correspondence address and email

Date of Submission18-Nov-2008
Date of Acceptance16-Feb-2009
Date of Web Publication11-Jan-2010


Williams syndrome is a complex syndrome characterized by developmental abnormalities, craniofacial dysmorphic features, and cardiac anomalies. Sudden death has been described as a very common complication associated with anesthesia, surgery, and procedures in this population. Anatomical abnormalities associated with the heart pre-dispose these individuals to sudden death. In addition to a sudden and rapid downhill course, lack of response to resuscitation is another significant feature seen in these patients. The authors report a five-year-old male with Williams syndrome, hypothyroidism, and attention deficit hyperactivity disorder. He suffered an anaphylactic reaction during CT imaging with contrast. Resuscitation was unsuccessful. Previous reports regarding the anesthetic management of patients with Williams are reviewed and the potential for sudden death or peri-procedure related cardiac arrest discussed in this report. The authors also review reasons for refractoriness to defined resuscitation guidelines in this patient population.

Keywords: Anaphylactic reaction, cardiac catheterization, dysmorphism, Williams syndrome, resuscitation

How to cite this article:
Gupta P, Tobias JD, Goyal S, Miller MD, Melendez E, Noviski N, De Moor MM, Mehta V. Sudden cardiac death under anesthesia in pediatric patient with williams syndrome: A case report and review of literature. Ann Card Anaesth 2010;13:44-8

How to cite this URL:
Gupta P, Tobias JD, Goyal S, Miller MD, Melendez E, Noviski N, De Moor MM, Mehta V. Sudden cardiac death under anesthesia in pediatric patient with williams syndrome: A case report and review of literature. Ann Card Anaesth [serial online] 2010 [cited 2022 Aug 12];13:44-8. Available from:

   Introduction Top

Williams syndrome (WS), initially described in 1961 by Williams, Barratt-Boyes, and Lowe, [1] indicates dysmorphic features, congenital heart disease (CHD), and distinctive behavioural and emotional traits. [2],[3] Its annual incidence is estimated at one in 20,000-50,000 live births. [4] These patients present major peri-operative challenges as sudden death has been described during surgery, invasive procedures (cardiac catheterization), and anesthetic care [Table 1]. [4],[5],[6],[7],[8],[9],[10],[11],[12] Additionally, when cardiac arrest occurs, resuscitative efforts have generally been unsuccessful. Coronary lesions in these patients may be responsible for the occurrence of sudden death, cardiac arrest during anesthesia, and difficulties in resuscitation of patients with WS. [4] The authors report a five-year-old boy with WS who suffered an anaphylactic reaction following the administration of contrast dye during computed tomography (CT) imaging. Resuscitative efforts were unsuccessful.

We also review previous reports regarding the anesthetic management of patients with WS and discuss the potential for sudden death or peri-procedure related cardiac arrest in this population.

   Case Report Top

As per the regulations of the Massachusetts General Hospital, IRB approval is not required for retrospective case reports. A five-year-old, African American boy with WS presented for cardiac CT imaging to evaluate the origins of the coronary arteries and great vessels in preparation for surgical repair of supravalvular aortic stenosis (SVAS). His last trans-thoracic echocardiogram performed approximately one month before the procedure, demonstrated worsening SVAS, mild LV hypertrophy, normal coronary arteries, and normal LV function. Electrocardiography (ECG) revealed no evidence of T wave or ST segment changes with a corrected QT interval of 420 msec. Physical examination revealed the characteristic faces of WS. His vital signs were within normal limits for age. Pertinent findings revealed a soft supra-sternal thrill and a III/VI systolic murmur which radiated to the neck. His review of systems was otherwise negative. The patient was kept nil per os for six hours. Anesthetic induction performed on the CT imaging table included increasing concentration of sevoflurane in oxygen. After the establishment of peripheral intravenous access, succinylcholine was administered and the trachea was intubated on the second attempt with a 5.0 cuffed endotracheal tube. Direct laryngoscopy and visualization of vocal cords was unremarkable with a grade I view. However, the patient's position on the CT table was sub-optimal and required repositioning with two intubation attempts. Intra-operative monitoring included standard ASA monitors (pulse oximetry, non-invasive blood pressure monitoring, continuous ECG, precordial stethoscope, and end tidal carbon dioxide). Given the short duration of procedure, maintenance anesthesia consisted only of sevoflurane in air/oxygen. The patient's vital signs were stable throughout the procedure with no ST or T wave changes. After the preliminary CT scan images were taken, a test dose of 6 mL of intravenous Isovue 370 contrast (370 mg iodine/ml) was administered with no apparent reaction. Four minutes later, an additional 34 mL of contrast was administered intravenously. Approximately one minute after the second dose of contrast, the pulse oximetry waveform disappeared, neither carotid nor femoral arterial pulses could be palpated, and a blood pressure could not be obtained. There was ST depression of approximately one mm followed by a wide complex bradycardia. Standard ACLS resuscitation was administered including ventilation with 100% oxygen, chest compressions, and pharmacologic support including atropine, epinephrine, bicarbonate, vasopressin, and isoproterenol. Transcutaneous pacing was attempted without capture and fluid boluses administered without response. Despite multiple rounds of medications, resuscitation was unsuccessful and the patient expired. Post-mortem examination revealed an elevated serum tryptase concentration of 51.5 ng/ml (normal values: median 10 ng/ml, range 2-23 ng/ml), and extremely edematous pharyngeal tissue consistent with an anaphylactic reaction. Cardiac examination revealed biventricular hypertrophy with SVAS with post-stenotic dilatation and elastic thickening in the main pulmonary artery

   Discussion Top

The multi-system involvement, especially the potential for coronary artery abnormalities and the risk of sudden death, raises many considerations for the peri-operative management of patients with WS. Various types of acquired and congenital cardiac involvement have been reported in association with WS including supra-valvular cardiac anomalies (SVAS), peripheral pulmonary stenosis, coronary artery lesions, mitral valve prolapse, coarctation of the aorta, patent ductus arteriosus, peripheral arterial abnormalities, and intracardiac lesions including ventricular septal defect and tetralogy of Fallot. [1],[3],[13],[14],[15],[16] Coronary artery lesions vary from severe stenosis to proximal dilation with tortuosity with rare reports of dissection. [4],[16] As noted in our patient, these patients not only have an increased risk of sudden death and cardiac arrest, but in many cases, the cardiac arrests have been refractory to defined resuscitation guidelines. Bird et al. noted that most of the fatalities in these patients occur either immediately after the initiation of procedure or after its successful completion. [5] Bird et al. also noted that the severity of SVAS is not a predictor of sudden death which was further confirmed by Wessel et al. [5],[9]

Isolated coronary anomalies are rare in Williams syndrome, but should be considered, especially in the presence of heart failure or ischemia as emphasized by van Pelt et al. in their report of a six-year-old patient who expired due to ischemic cardiomyopathy despite surgical revascularization. [11] As noted by many authors, the sudden and rapid downhill course appears to be a pathognomonic feature of the deaths reported in these patients. [4],[12] Although sudden death has been found on post-mortem examination to be the result of myocardial ischemia related to abnormalities of the coronary arteries; in many other cases, as in our patient, these changes are absent on pathological examination of the heart. [12] The presenting symptoms in these patients at the time of arrest are generally hypotension and bradycardia. [4],[5] The aetiology of cardiac arrest may relate either to coronary artery stenosis/obstruction or severe biventricular outflow tract obstruction. Decreased coronary blood flow with myocardial ischemia may result either from anatomical obstruction of the coronary ostium or stenosis of the coronary arteries. In patients with SVAS, the coronary orifice may be obstructed because of distortion of the aortic leaflets with adherence of the left or right coronary cusp to the wall of the aorta immediately above the ostia. Additionally, a high prestenotic pressure may be transmitted to the coronary arteries and result in intimal fibrosis and muscular hypertrophy leading to narrowing of the coronary arteries. In either case, the mechanisms for sudden death include myocardial ischemia, decreased cardiac output, and ventricular arrhythmias. Furthermore, aortic stenosis activates left ventricular baroreceptors, which causes reflex bradycardia and cardiac arrhythmis resulting in sudden death during periods of stress. [7] For the above mentioned reasons, the choice of medications during resuscitation is controversial. Although epinephrine may be life-saving, avoidance of multiple or large doses of epinephrine should be considered as this has the potential to increase systemic vascular resistance which in turn can decrease coronary blood flow in the setting of SVAS. Systemic blood flow should be maintained with effective chest compressions rather than the repeated administration of epinephrine. Investigation with other medications (isorproterenol, vasopressin) in this population appeared warranted given the invariable poor response to resuscitation that has been reported in literature.

Given the invariable association of CHD with WS, the pre-anesthetic evaluation of these patients should include an ECG and transthoracic echocardiogram. Holter monitoring may be indicated if the baseline screening ECG is suggestive of conduction issues or arrhythmias. [4] Given the high incidence of coronary artery lesions, a coronary angiography may be indicated in patients whose history, physical examination or preoperative work-up are suggestive of coronary ischemia. Stress ECG or echocardiography may be another modality to evaluate and follow the myocardial health of children with WS. Monitoring in these patients should include standard ASA monitors and consideration of invasive arterial blood pressure monitoring given that many of the arrests are preceded by hypotension and/or bradycardia. Intra-operative esophageal echocardiography has been suggested especially during the management of complex surgical procedures. [17]

The anesthetic management in these patients may be challenging as there are limited data regarding this issue. Given the characteristic cranio-facial features, mask ventilation and tracheal intubation may be difficult because of a flattened mid-face, wide mouth, dental malocclusion, and poor dentition. [18] Many of these concerns have been previously summarized by Medley et al. [2] Although our patient had an appropriate sized endo-tracheal tube with easy visualization of vocal cords and a grade I view, Medley et al. reported concerns regarding their patient - that her trachea was intubated with a smaller endo-tracheal tube than expected for her age and developed postoperative stridor likely because of laryngeal edema. [2] There may also be issues regarding the choice of neuromuscular blocking agent for endo-tracheal intubation and/or ongoing neuromuscular blockade during anesthetic care. In this population, there is generally some degree of associated musculo-skeletal involvement including joint laxity that may lead to contractures and muscular weakness. Lipid storage in muscles and increased variability in fiber size has also been reported. [18] Patients with other co-morbid conditions with similar biopsy findings such as Duchennes muscular dystrophy may have variable responses to neuromuscular blocking agents (NMBA) including prolonged neuromuscular blockade after non-depolarizing agents. [19],[20] Given the potential for such problems, titration of NMBA with train-of-four monitoring is suggested. Although succinylcholine was used in our patient, there is no data on which to make specific recommendations regarding its safety. Therefore, given the association of other myopathic conditions with an exaggerated hyperkalemic response to the administration of succinylcholine, we would caution against its use. [21] Although ketamine may provide hemodynamic stability through the release of endogenous catecholamines, it may induce tachycardia thereby altering the balance between myocardial oxygen supply and demand. Audrzejowski et al. reported the successful use of etomidate for anesthetic induction. [22] The use of inhalational anesthetic agents (sevoflurane or desflurane) as the sole agent is debatable as they may result in peripheral vasodilatation with a decrease of pre-load and after-load, thereby reducing blood pressure and myocardial perfusion. Sevoflurane offers the advantage of less vasodilatation and a decrease in heart rate which may preserve the myocardial oxygen supply-demand ratio better than desflurane which may result in tachycardia and vasodilatation.

Anecdotal evidence has reported the administration of a combination of opioids (fentanyl or remifentanil), inhalational anesthetic agents, and non-depolarizing neuromuscular blocking agents (cis-atacurium). [2],[17],[22],[23]

In conclusion, patients with WS present many peri-operative and peri-procedural challenges. A thorough pre-operative/pre-procedure screening is suggested to identify patients with anatomical abnormalities which may result in coronary artery involvement. Even with appropriate care, sudden cardiac arrest and death have been reported. Unfortunately, many of these patients are refractory to standard resuscitation protocols. Given these concerns, a thorough explanation of the risk-benefit ratio should be considered whenever diagnostic tests are considered, in patients with WS, along with a thorough discussion with parents regarding the potential risks involved.

   References Top

1.Williams JCP, Barratt-Boyes BG, Lowe JB. Supravalvular aortic stenosis. Circulation 1961:24:1311-8.  Back to cited text no. 1      
2.Medley J, Russo P, Tobias JD. Perioperative care of the patient with Williams syndrome. Pediatr Anesth 2005;15:243-7.  Back to cited text no. 2      
3.Greenberg F: Williams syndrome professional symposium. Am J Med Genet 1990;6:85-8.  Back to cited text no. 3      
4.Horowitz PE, Akhtar S, Wulff JA, Al Fadley F, Al Halees Z. Coronary artery disease and anaesthesia-related death in children with Williams syndrome. J Cardiothorac Vasc Anesth 2002;16:739-41.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  
5.Bird LM, Billman GF, Lacro RV, Spicer RL, Jariwala LK, Hoyme HE, et al. Sudden death in Williams syndrome: report of ten cases. J Pediatr 1996;129:926-31.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  
6.Bonnet D, Cormier V, Villain E, Bonhoeffer P, Kachaner J. Progressive left main coronary artery obstruction leading to myocardial infarction in a child with Williams syndrome. Eur J Pediatr 1997;156:751-3.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]  
7.Imashuku S, Hayashi S, Kuriyama K, Hibi S, Tabata Y, Todo S. Sudden death of a 21-year-old female with Williams syndrome showing rare complications. Pediatr Int 2000;42:322-4.  Back to cited text no. 7      
8.Suαrez-Mier MP, Morentin B. Supravalvular aortic stenosis, Williams syndrome and sudden death. A case report. Forensic Sci Int 22;106:45-53.  Back to cited text no. 8      
9.Wessel A, Gravenhorst V, Buchhorn R, Gosch A, Partsch CJ, Pankau thR. Risk of sudden death in the Williams-Beuren syndrome. Am J Med Genet A 2004;127:234-7.  Back to cited text no. 9      
10.Bragg K, Fedel GM, DiProsperis A. Cardiac arrest under anaesthesia in a pediatric patient with Williams syndrome: a case report. AANA 2005;73:287-93.  Back to cited text no. 10      
11.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;26:665-7.  Back to cited text no. 11      
12.Monfared A, Messner A. Death following tonsillectomy in a child with Williams syndrome. Int J Pediatr Otorhinolaryngol 2006;70:1133-5.  Back to cited text no. 12  [PUBMED]  [FULLTEXT]  
13.Beuren AJ, Schulze C, Eberle P, Harmjanz, Apitz J. Syndrome of supravalvular aortic stenosis, peripheral pulmonary stenosis, mental retardation, and similar facial appearance. Am J Cardiol 1964;13:471-83.  Back to cited text no. 13      
14.Becker AE, Becket M J, Edwards JE. Mitral valvular abnormalities associated with supravalvular aortic stenosis. Am J Cardiol 1972;29:90-4.  Back to cited text no. 14      
15.Ino T, Nishimoto K, Iwahava M, Akimoto K, Boku H, Kaneko K, et al. Progressive vascular lesions in Williams-Beuren syndrome. Pediatr Cardiol 1988;9:55-8.  Back to cited text no. 15      
16.Conway EE, Noonan J, Marion RW, Steeg CN. Myocardial infarction leading to sudden death in the Williams syndrome: report of three cases. J Pediatr 1990;117:593-5.  Back to cited text no. 16      
17.Audrzejowski J. Anaesthesia for MRI angiography in a patient with Williams syndrome. Anaesthesia 200;55:97-8.  Back to cited text no. 17      
18.Lashkari A, Smith A, Graham J. Williams-Beuren Syndrome. Clin Pediatr 1999;38:189-208.  Back to cited text no. 18      
19.Tobias JD, Atwood R. Mivacurium in children with Duchenne muscular dystrophy. Pediatr Anesth 1994;4:57-60.  Back to cited text no. 19      
20.Frankowski GA, Johnson JO, Tobias JD. Rapacuronium administration to two children with Duchenne's muscular dystrophy. Anesth Analg 2000;91:27-8.   Back to cited text no. 20  [PUBMED]  [FULLTEXT]  
21.Naguib M, Flood P, McArdle JJ, Brenner HR. Advances in neurobiology of the neuromuscular junction. Anesthesiology 2002;96:202-31.  Back to cited text no. 21  [PUBMED]  [FULLTEXT]  
22.Mammi I, Iles DE, Smeets D, Clementi M, Tenconi R. Anesthesiologic problems in Williams syndrome: the CACNL2A locus is not involved. Hum Genet 1996;98:317-20.  Back to cited text no. 22  [PUBMED]  [FULLTEXT]  
23.Kawahito S, Kitahata H, Kimura H, Tanaka K, Sakai Y, Hirose Y, et al. Anaesthetic management of a patient with Williams syndrome undergoing aortoplasty for supravalvular aortic stenosis. Can J Anaesth 1998;45:1203-6.  Back to cited text no. 23  [PUBMED]  [FULLTEXT]  

Correspondence Address:
Punkaj Gupta
175 Cambridge Street, 5th Floor, Boston MA 02114
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9784.58834

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