The Terrible T’s: T-wave Alternans, Timothy Syndrome, and a Two-year-oldRebecca S. Isserman MD, Matthew F. Pearsall MD, Allan F. Simpao MD

The Children’s Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA

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SKRIPKO, JAYSE ID:055715868 04-MAR-2016 09:15:57 CHOP-DSU ROUTINE RECORD

** ** ** ** * PEDIATRIC ECG ANALYSIS * ** ** ** **NORMAL SINUS RHYTHMST ABNORMALITY AND T-WAVE INVERSION IN INFEROLATERAL LEADSPROLONGED QT

25mm/s 10mm/mV 150Hz 8.0 SP2 12SL 241 HD CID: 18

Referred by: BENJAMIN CHANG Confirmed By: MAULLY SHAH M.D.

BPM130Vent. ratems96PR intervalms64QRS durationmsQT/QTc 398/585

2476346P-R-T axes

25-FEB-2014 (2 yr)Male Caucasian

Room:PACULoc:18

Technician: EPERRYTest ind:EKG CHANGES

DX: ACCT#:2039137964

Page 1 of 1 EID:105 EDT: 16:42 06-MAR-2016 ORDER: ACCOUNT: 2039495680

Background  •  T-­‐wave  alternans  is  an  uncommon  

electrocardiographic  (ECG)  finding  of  beat-­‐to-­‐beat  alterna;on  in  T-­‐wave  shape  or  amplitude.  

•  T-­‐wave  alternans  is  associated  with  a  prolonged  QT  interval  and  long  QT  syndrome  (LQTS).  

•  T-­‐wave  alternans  may  degenerate  to  torsades  de  pointes.1  

Case  Descrip8on  •  A  2-­‐year-­‐old,  15.7-­‐kg  male  with  bilateral  hand  and  

foot  syndactyly  presented  for  skin  graK  revision  of  prior  syndactyly  repair.      

•  Medical  history  included  developmental  delay.  •  The  pa;ent  had  two  unevenMul  anesthe;cs  at  6  

and  9  months  of  age.  •  Induc;on  was  via  mask  with  sevoflurane  8%  in  

N2O  and  O2,  followed  by  a  propofol  2mg/kg  bolus  prior  to  a  smooth  tracheal  intuba;on.  

•  Midway  through  the  case,  ephedrine  (10mg  total)  was  dosed  intermiXently  for  hypotension.  

•  The  BP  improved  and  vital  signs  stabilized,  yet  the  ECG  demonstrated  T-­‐wave  alternans  (Figure  1).    

Intraopera8ve  Management  

B

•  Immediate  cardiology  consult  to  assist  with  management.  •  Intravenous  magnesium  (20mg/kg)  was  given.  •  T-­‐waves  normalized;  pa;ent  remained  stable  and  was  

extubated  without  incident.  •  A  basic  metabolic  panel  was  sent  and  was  normal.  •  A  12-­‐lead  ECG  (Figure  2)  showed  a  QTc  interval  of  585  ms.  Figure  2:  12-­‐lead  EKG  rhythm  strip  (QTc  =  585  ms;  normal  QTcis  <450  ms)  

Outcome  

Discussion  

References:  1.  Zareba  W,  et  al.  T  wave  alternans  in  idiopathic  long  QT  syndrome.  J  Am  Coll  Cardiol;  1994;23:1541–6.  2.  Kies  SJ,  et  al.  Anesthesia  for  Pa;ents  with  Congenital  Long  QT  Syndrome.  Anesthesiology.  2005;102:204–10.  3.  Nathan  AT,  et  al.  Case  Scenario  Anesthesia-­‐related  Cardiac  Arrest  in  a  Child  with  Timothy  Syndrome.  Anesthesiology;  2012;117:1117–26.  

•  The  pa;ent  was  admiXed  to  the  cardiology  service  aKer  the  procedure.  

•  He  was  started  on  a  beta-­‐blocker  and  had  an  AICD  implanted.  

•  Gene;c  tes;ng  revealed  a  heterozygous  variant  in  the  CACNA1C  gene,  consistent  with  Timothy  Syndrome  (LQTS  type  8).  

•  There  are  13  known  genotypes  of  LQTS  (overall  incidence  1  :  2,500).  

•  Timothy  syndrome  is  associated  with  arrhythmias  during  anesthesia,  syndactyly,  and  craniofacial  and  cogni;ve  abnormali;es.      

•  Anesthe;c  management  includes  avoidance  of  QT-­‐prolonging  medica;ons  (sevoflurane,  ondansetron,  ephedrine,  phenylephrine,  and  albuterol)  and  premedica;on  and  pain  control  to  minimize  sympathe;c  s;mula;on.2,3  

•  Prompt  recogni;on  and  treatment  of  T-­‐wave  alternans  is  crucial  to  prevent  progression  to  a  lethal  arrhythmia.  

Figure  1:  T-­‐wave  alternans  on  (A)  OR  monitor  and  (B)  intraopera;ve  rhythm  strip  A