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24-hour BP in children with congenital central hypoventilation syndrome
Ha Trang1, Souham Boureghda, Isabelle Denjoy
1Service de Physiologie, Hôpital Robert Debré, Université Paris VII, INSERM E9935, Paris, France. ha.trang@rdb.ap-hop-paris.fr
Insights
Congenital central hypoventilation syndrome (CCHS) patients exhibit abnormal circadian blood pressure patterns, with lower daytime and higher nighttime pressures. This suggests autonomic dysfunction and necessitates lifelong cardiovascular monitoring for CCHS individuals.
Area of Science:
- Cardiology
- Pediatric Pulmonology
- Autonomic Neuroscience
Background:
- Congenital central hypoventilation syndrome (CCHS) is a rare disorder affecting the autonomic nervous system.
- Circadian blood pressure (BP) patterns are crucial for cardiovascular health.
- Previous studies have not fully elucidated the specific BP patterns in CCHS patients.
Purpose of the Study:
- To investigate the 24-hour ambulatory blood pressure patterns in pediatric patients with CCHS.
- To compare circadian BP profiles between CCHS patients and healthy controls.
- To explore potential links between abnormal BP patterns and autonomic dysfunction in CCHS.
Main Methods:
- A case-control study involving 11 CCHS patients and 11 matched controls.
- 24-hour ambulatory blood pressure monitoring was conducted for all participants.
- Polysomnography and noninvasive monitoring of oxygen saturation and end-tidal PCO(2) were performed.
Main Results:
- CCHS patients displayed lower blood pressure during wakefulness and higher blood pressure during sleep compared to controls.
- Nocturnal blood pressure dipping was significantly reduced in CCHS patients.
- Ten out of eleven CCHS patients were classified as non-dippers, indicating an abnormal circadian BP rhythm.
Conclusions:
- The study identified an abnormal circadian blood pressure pattern in children and adolescents with CCHS.
- This abnormal pattern may be associated with underlying autonomic nervous system dysfunction.
- Lifelong cardiovascular follow-up is recommended for individuals diagnosed with CCHS.
Objective:
To study circadian BP patterns in patients with congenital central hypoventilation syndrome (CCHS).
Design:
Case-control study.
Setting:
Teaching hospital in Paris, France.
Patients:
Eleven patients with CCHS (median age, 13 years; range, 6 to 18 years) and 11 sex- and height-matched control subjects.
Intervention:
None.
Methods:
Each subject underwent 24-h ambulatory BP monitoring. Oxygen saturation and end-tidal PCO(2) were monitored noninvasively. Polysomnography was performed to determine sleep times. All patients with CCHS received mechanical ventilation during sleep. Mean values for systolic BP (SBP) and diastolic BP (DBP) during wakefulness and sleep were analyzed. Nocturnal BP "dipping" was defined as the difference in mean SBP (and/or DBP) between wakefulness and sleep, divided by individual waking mean values. BP "dippers" were defined as subjects showing at least 10% nocturnal dipping.
Results:
Patients with CCHS had BPs in the low normal range of normative data. As compared to control subjects, patients with CCHS had lower BP during wakefulness (p = 0.003 and p = 0.016 for SBP and DBP, respectively), and higher BP during sleep (p = 0.016 and p = 0.002). Nocturnal BP dipping was abnormally reduced in patients with CCHS (p = 0.000). Ten of the 11 patients with CCHS were BP nondippers, compared to none of the control subjects.
Conclusion:
The abnormal circadian BP pattern observed in children and adolescents with CCHS may be related to autonomic nervous dysfunction. Lifelong cardiovascular follow-up is recommended for patients with CCHS.
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