Arrhythmias in Pediatric Syndromes

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US Cardiology, 2006;3(2):1-3

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Rhythm disorders, independent of structural congenital heart disease, have been described with myriad pediatric syndromes.Reviewing all pediatric syndromes associated with rhythm disorders is beyond the scope of this text.However, arrhythmia as a common or defining clinical feature of a pediatric syndrome is relatively rare. A representative sample of pediatric syndromes that prominently feature arrhythmias is presented here.

Bradyarrhythmia in Pediatric Syndromes
Kearns-Sayre Syndrome

Progressive conduction defects are a feature of Kearns-Sayre syndrome, a neuromuscular disorder. It is characterized by the triad of progressive external ophthalmoplegia, pigmentary degeneration of the retina, and cardiac conduction delay.1-2 It is associated with other neuropathies (sensorineural hearing loss, intention tremor, ataxia) and endocrinopathies.3 Symptoms manifest in childhood, usually late in the first decade of life.3 Neurologic deterioration precedes cardiac conduction deficits.1-6 Kearns-Sayre syndrome is caused by a large deletion of 45-75% of the mitochondria DNA.5,7 This compromises the function of the mitochondrial respiratory chain. In cardiac myocytes, there is a maladaptive increase in the number of dysfunctional mitochondria displacing and replacing myofibils.5,7 Parallel to other mitochondrial myopathies, these changes can also be seen as 'ragged red fibersÔÇÖ found on skeletal muscle biopsy.2-5

Serial standard 12 lead electrocardiograms (ECGs) show the progression from first degree heart block to complete heart block. ECG findings included all degrees of heart block, fascicular blocks and ST-T-wave changes. Progression from fascicular block or first and second degree atrioventricular (AV) block to complete heart block is inevitable.2-4,6-8 The rate of progression is unpredictable but accelerated. Notably, PR interval prolongation is often seen before development of complete heart block though it is not a prerequisite.6 Intracardiac electrophysiology studies show that most conduction block occurs infranodally (a disease of the His-Purkinje system).4,6,8

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