PEDIATRICS Vol. 108 No. 3 September 2001, pp. 765-766

COMMENTARY:
Breath-Holding Spells and Pacemaker Implantation

Severe breath-holding spells (BHS), both cyanotic and pallid, are a well-known clinical entity to primary care providers.^1-3 This misnomer describes a nonvolitional event triggered by emotional upset resulting in crying to the point of unconsciousness whereby the child's color becomes pale or cyanotic. Indeed, descriptions can be found in 15th century medical textbooks.⁴ The prevalence occurs between 1.7% to 4.6% of well-patient surveys.^5,6 Clinical manifestations, natural history, and a previously enigmatic pathophysiology have been more clearly defined and articulated.⁷ This symptom complex needs to be differentiated from specific etiologies mimicking BHS.⁷ It remains amazing how such dramatic physiologic consequences can result from what most children do wellcry. This sudden, emotion-laden, human response sets into motion a cascade of intricate and interrelated reflex phenomena resulting in expiratory apnea, color change, and loss of consciousness. In the case of pallid events, profound bradycardia serves as the secondary mediator and physiologic hallmark of this primary parasympathetically mediated cascade. Although cyanotic spells are associated with greater sympathetically mediated autonomic activity, a more complex interplay among autonomic responses undoubtedly occurs during each breath-holding event. This is supported by the fact that upwards of 20% of children with severe BHS experience both types of spells with one type predominating.³

In this issue of Pediatrics, Kelly et al⁶ review their experience at the Mayo Clinic with pacemaker implantation as a treatment for BHS associated with significant bradycardia. It is important to note that even at a tertiary referral center such as the Mayo Clinic, only 10 patients have merited pacemaker implantation over the 10-year interval under study. Because patients were identified by retrospective chart review, precise clinical detail may not be available indicating the exact clinical parameters associated with each child's spells. Implantation was instituted after failure of medical therapies in 8 of 10 children, (anticonvulsants in 5, anticholinergics in 4, and theophyline in 2). No patient had prolonged QTc interval or other arrhythmia identified. Interventions were prompted by significant clinical accompaniments to the spells (ie, seizures, resuscitation, prolonged apnea). This cohort at the outset was somewhat atypical in that all 7 patients with any color change noted developed cyanosis (1 also had pallor) in the face of bradycardia, a clinical sign associated with pallid events. This suggests in hindsight that if these children had BHS they were experiencing mixed spells and therefore treatment aimed at one physiologic parameter may not have been sufficient. Indeed, half of the cohort continued to experience BHS albeit milder and half experienced resolution despite pacemaker insertion. The reason to implant permanent pacemakers was an attempt to alleviate or eliminate symptoms. A beneficial effect on outcome could be extracted from at least 2 patients who had recurrence of spells after the pacemaker was removed in 1, and failed in another. The natural course of gradual resolution of BHS is a likely intercurrent variable in the outcome of the group. If these children continue to require a pacemaker, particularly those who are older than age 7, then one must question whether a primary sinus node dysfunction was the specific underlying problem in this group. There were 3 children who required repair/replacement of the pacemaker (30%). Newer technologies may reduce this rate.

The children with previous seizure activity associated with their spells no longer required anticonvulsant therapy after implantation. These have not been found to be effective in ameliorating BHS and have had a variable effect on BHS-associated terminal seizures.⁹ All 10 children are healthy, active, normally developed, and engaged in age-appropriate grade levels in school at follow-up. These outcomes are reassuring for parents as well as those of us who continue to be challenged by children who experience these events. Pacemaker implantation for children with severe BHS and significant bradycardia, pallid or cyanotic, should be predicated on both the safety and efficacy of this intervention in the appropiate individual clinical setting. Although individual children may benefit from such an intervention, the vast majority can be managed without it.

Francis J. DiMario Jr, MD
Connecticut Children's Medical Center
Division of Pediatric Neurology
Hartford, CT 06106
From the Department of Pediatrics, University of Connecticut, Connecticut Children's Medical Center, Hartford, Connecticut

FOOTNOTES

Received for publication Jun 25, 2001; accepted Jun 25, 2001.

Reprint requests to (F.J.D.) Division of Pediatric Neurology, 282 Washington St, Hartford, CT 06106. E-mail: fdimari{at}ccmckids.org

ABBREVIATIONS

BHS, breath-holding spells.

REFERENCES

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2. DiMario FJ Jr Breath-holding spells in childhood. Am J Dis Child 1992; 146:125-131 [Abstract/Free Full Text]
3. DiMario FJ Jr Prospective study of children with cyanotic and pallid breath-holding spells. Pediatrics 2001; 107:265-269 [Abstract/Free Full Text]
4. Culpepper N. A Directory for Midwives: or a Guide for Women in Their Conception, Rearing and Suckling Their Children. London, United Kingdom: Hitch and Hawes; 1762:358
5. Bridge EM, Livingston S, Tietze C Breath-holding spells: their relationship to syncope, convulsions, and other phenomena. J Pediatr 1943; 23:539-661 [CrossRef]
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7. DiMario FJ Jr. Breath-holding spells in childhood. Curr Prob Pediatr 1999(Nov/Dec);281-299
8. Kelly AM, Porter CJ, McGoon MD, Espinosa RE, Osborn MJ, Hayes DL. Breath-holding spells associated with significant bradycardia: successful treatment with permanent pacemaker implantation. Pediatrics. 2001;108
9. Moorjani BI, Rothner AD, Kotgal P Breath-holding and prolonged seizures [abstract] . Ann Neurol 1995; 38:512