Brugada in Children

Brugada

Syncope is a rather common presentation in the Pediatric ED.  While the etiology is most often a benign one, our vigilance assists us in detecting some of the more concerning entities.  Yes, the episode may have been caused by Hair-Grooming, but we are on guard against Prolonged QTc, Catecholaminergic Polymorphic VTach, Seizures, and even Eating Disorders.  While juggling these ominous entities, let us not overlook the potential for Brugada Syndrome.

 

Brugada Syndrome: Basics

  • Potentially lethal channelopathy that is associated with VTach or VFib
  • Inheritance: Autosomal Dominant
    • Second leading cause of death in young Thai men (#1 is trauma).
    • Likely expressed with incomplete penetrance.
  • Typically presents in young adulthood, but it does occur in children.
  • Regarded as a rare entity, but…
    • It has only been recognized for past 30 years, so we are still learning about it.
    • True incidence is not known as it is difficult to diagnose.
      • The ECG findings may not always be present
      • The specific genetic tests are abnormal in only ~1/3rd of cases
    • May eventually be found to be “subtle and difficult to diagnose,” rather than rare. [Crosson, 2015]

 

Brugada Syndrome: Diagnosis

  • Diagnostic Criteria are the same for adults and children. [Crosson, 2015]
  • 3 types of ECG changes:
    • Type 1
      • “Coved” ST Elevation with at least 2 mm of elevation in at least 1 right precordial lead
      • Only pattern considered diagnostic!
      • Can be spontaneous or induced during provocative testing
    • Type 2
      • Saddle-back ST elevation
      • Should consider diagnosis in the right clinical setting
    • Type 3
      • Saddle-back ST pattern without significant elevation
      • Should consider diagnosis in the right clinical setting
Brugada Types

Brugada Sign Types from http://circep.ahajournals.org

  • May be asymptomatic upon initial evaluation.
  • Consider with:
    • Syncope
      • Often occurs while at rest or with fever!
    • Nocturnal agonal respirations
    • Family history of Sudden Cardiac Death
    • Family history of SIDS
    • Family history of Type 1 ECG findings
    • Self-terminated polymorphic VTach
    • VFib

 

Brugada Syndrome in Children

  • True incidence suspected to be less than in young adults, but not fully known.
  • Conduction delays are common in pediatric cases. [Chockalingam, 2012]
  • Children are less likely to have Brugada ECG Pattern.
  • Children are less likely to have symptoms.
  • Children are more likely Monomorphic VTach (compared to VFib in older patients).

 

Brugada Syndrome: Treatment

  • Management is tailored for each individual case.
  • Determining risk factors for who with Brugada is at risk for developing fatal arrhythmia is still debated.
  • Currently the best known therapy is AICD.
    • Children have greater rate of AICD complications, so often reserved for severe cases. [Chockalingam, 2012]
    • Asymptomatic patients or those with positive genetic testing or drug-induced Type 1 may be monitored closely, particularly during febrile illnesses.
  • Medications
    • Quinidine – adjunct to AICD
    • Fever Control
      • Febrile illnesses have been shown to trigger events in children [Kim, 2014; Chockalingam, 2012; Zaidi, 2010; Probst, 2007]
      • That child who had a syncopal event and is noted to be febrile deserves attention!

 

 

References

Crosson JE1, Nies M. Brugada syndrome in children. Expert Rev Cardiovasc Ther. 2015 Feb;13(2):173-81. PMID: 25583159. [PubMed] [Read by QxMD]

Swayampakula AK1, Fong J, Kulkarni A. Arrhythmogenic causes of syncope. Pediatr Emerg Care. 2014 Dec;30(12):894-5. PMID: 25469601. [PubMed] [Read by QxMD]

Kim G1, Kyung YC2, Kang IS2, Song J2, Huh J2, On YK3. A pediatric case of Brugada syndrome diagnosed by fever-provoked ventricular tachycardia. Korean J Pediatr. 2014 Aug;57(8):374-8. PMID: 25210526. [PubMed] [Read by QxMD]

Chockalingam P1, Clur SA, Breur JM, Kriebel T, Paul T, Rammeloo LA, Wilde AA, Blom NA. The diagnostic and therapeutic aspects of loss-of-function cardiac sodium channelopathies in children. Heart Rhythm. 2012 Dec;9(12):1986-92. PMID: 22885917. [PubMed] [Read by QxMD]

Zaidi AN1. An unusual case of Brugada syndrome in a 10-year-old child with fevers. Congenit Heart Dis. 2010 Nov-Dec;5(6):594-8. PMID: 21106020. [PubMed] [Read by QxMD]

Lee YS1, Baek JS, Kim SY, Seo SW, Kwon BS, Kim GB, Bae EJ, Park SS, Noh CI. Childhood brugada syndrome in two korean families. Korean Circ J. 2010 Mar;40(3):143-7. PMID: 20339501. [PubMed] [Read by QxMD]

Probst V1, Denjoy I, Meregalli PG, Amirault JC, Sacher F, Mansourati J, Babuty D, Villain E, Victor J, Schott JJ, Lupoglazoff JM, Mabo P, Veltmann C, Jesel L, Chevalier P, Clur SA, Haissaguerre M, Wolpert C, Le Marec H, Wilde AA. Clinical aspects and prognosis of Brugada syndrome in children. Circulation. 2007 Apr 17;115(15):2042-8. PMID: 17404158. [PubMed] [Read by QxMD]

Brugada P1, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report. J Am Coll Cardiol. 1992 Nov 15;20(6):1391-6. PMID: 1309182. [PubMed] [Read by QxMD]

Sean Fox

I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same. I trained at the Combined Emergency Medicine and Pediatrics residency program at University of Maryland, where I had the tremendous fortune of learning from world renown educators and clinicians. Now I have the unbelievable honor of working with an unbelievably gifted group of practitioners at Carolinas Medical Center. I strive every day to inspire my residents as much as they inspire me.

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  1. January 6, 2016

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