Cardiac rhythm disturbances among children with idiopathic congenital central hypoventilation syndrome

Abstract

The objective of this study was to determine whether subjects with congenital central hypoventilation syndrome (CCHS) had an increased frequency of cardiac arrhythmias and decreased heart rate variability when compared to subjects without a known deficit in control of breathing, and that these abnormalities would be exaggerated by anesthesia. Continuous ambulatory Holter recordings were obtained in patients with CCHS and compared to two otherwise healthy control groups without a deficit in control of breathing: one with an intact airway (n = 11) and a second group with a tracheostomy (n = 6). Holter recordings were obtained before, during (under general anesthesia), and after bronchoscopy. Fourteen children with CCHS (age: 9.3 ± 4.4 years mean ± S.D.) were studied, and 7 underwent bronchoscopy. Seventeen control children were studied (age 6.6 ± 3.6 years): 11 without a tracheostomy, and 6 with a tracheostomy who also underwent bronchoscopy. Maximum heart rate during baseline recording was significantly lower in the CCHS subjects as compared to controls (P = 0.0001). At baseline the difference in the number of arrhythmias/24 hr/subject in all CCHS vs. all control subjects was significant (P = 0.0002); for the subjects who had bronchoscopy, CCHS vs. control, the difference was also significant (P = 0.03). In addition, there was a significant decrease in the number of events/24 hr/subject among the CCHS subjects between baseline and post‐bronchoscopy (P = 0.0288). The predominant arrhythmias were sinus bradycardia and transient asystole. The longest asystole in a CCHS subject was 6.50 sec, and in a control subject, 1.42 sec (at baseline the means of the longest asystole were 2.69 ± 1.4 vs. 1.24 ± 0.13; P = 0.003 in the CCHS vs. control groups). Other indices of heart rate variability were significantly reduced in the CCHS subjects (P < 0.05). These results substantiate our hypothesis that subjects with CCHS have more arrhythmias than controls, an increased frequency of bradyarrhythmias, and decreased cyclical sinus arrhythmia. Pediatr Pulmonol. 2000; 29:351–358. © 2000 Wiley‐Liss, Inc.

Congenital central hypoventilation syndrome: PHOX2B genotype determines risk for sudden death

“…15 Furthermore, heart rate variability is decreased. 12,16 Children with CCHS exhibit an increased frequency of arrhythmia, primarily sinus bradycardia and transient asystole, with documented pauses as long as 6.5 sec in CCHS versus 1.4 sec in controls. 16 Children with CCHS exhibit lower blood pressure values during wakefulness and higher blood pressure values during sleep (vs. controls), indicating attenuation of the normal sleep-related blood pressure decrement.…”

“…12,16 Children with CCHS exhibit an increased frequency of arrhythmia, primarily sinus bradycardia and transient asystole, with documented pauses as long as 6.5 sec in CCHS versus 1.4 sec in controls. 16 Children with CCHS exhibit lower blood pressure values during wakefulness and higher blood pressure values during sleep (vs. controls), indicating attenuation of the normal sleep-related blood pressure decrement. 14 They also demonstrate a limited capacity to elevate their blood pressure on standing and head-up tilt positions, and do not demonstrate the normal standing-related blood pressure overshoot.…”

Presentation and Treatment of Monozygotic Twins with Congenital Central Hypoventilation Syndrome

“…It is most pronounced during nonrapid eye movement (NREM) sleep during which breathing is primarily controlled by the autonomic nervous system (ANS) (2). ANS dysfunction is a hallmark of CCHS, and clinical manifestations of the disease may include diminished heart rate variability and transient abrupt asystoles, decreased pupillary light response, esophageal dysmotility, breath-holding spells, reduced basal body temperature, sporadic profuse sweating, lack of perception of dyspnea, altered perception of anxiety, and a lack of physiological responsiveness to exercise and environmental stressors (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16). Additionally, CCHS may also be associated with neural crest tumours and Hirschsprung's disease.…”

FMRI Responses to Hyperoxia in Congenital Central Hypoventilation Syndrome

“…CCHS patients show substantial deficits in both sympathetic and parasympathetic components of autonomic nervous system control, which are manifested by such signs as extreme reduction in respiratoryrelated heart rate variability during sleep (7), diminished blood pressure "dipping" during the night (8), syncope to relatively mild provocation (9), a high incidence of cardiac arrhythmia (10), intolerance to heat and cold, and poor control of sweating (11). The autonomic deficits have the potential to modify compensatory cardiovascular changes and organ perfusion to alterations in O 2 levels.…”