{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T13:02:43Z","timestamp":1791118963166,"version":"4.1.0"},"reference-count":24,"publisher":"Portland Press Ltd.","issue":"3","content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2005,3,1]]},"abstract":"<jats:p>The effect of CCHS (congenital central hypoventilation syndrome, or Ondine's curse) on short-term BP (blood pressure) and HR (heart rate) variability was evaluated in 16-year-old subjects presenting a form of CCHS requiring night ventilatory assistance. The 12 patients were compared with 12 age- and gender-matched healthy volunteers. Recordings were obtained during daytime while the subjects were breathing spontaneously. Continuous BP was measured with a Finapres\u00ae device in the supine, head-up tilt and standing positions. The manoeuvre of actively standing was also analysed. HR levels were elevated in CCHS subjects at supine rest (+23%) with a reduced HR overall variability (\u221288%). The low- and high-frequency components of HR variability were affected. BP levels were preserved at rest, but the manoeuvres demonstrated a limited capacity to elevate BP. There was no overshoot in BP during the manoeuvre of actively standing, and steady standing BP levels in patients were not higher than supine BP levels as usually observed in healthy controls. The spontaneous baroreflex sensitivity estimated using the sequence technique or the cross-spectral analysis fell in the patients to approx. one-third of the sensitivity estimated in the healthy controls whatever the position. This cardiovascular profile suggests a predominant vagal dysfunction with signs of vagal withdrawal and baroreflex failure, and relative preservation of the cardiac and vascular sympathetic function. It is likely that the impaired ontogeny of the visceral reflexes, considered now to cause CCHS syndrome, includes the baroreceptive pathway and mainly its vagal component.<\/jats:p>","DOI":"10.1042\/cs20040282","type":"journal-article","created":{"date-parts":[[2005,2,18]],"date-time":"2005-02-18T10:26:10Z","timestamp":1108722370000},"page":"225-230","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":73,"title":["Short-term blood pressure and heart rate variability in congenital central hypoventilation syndrome (Ondine's curse)"],"prefix":"10.1042","volume":"108","author":[{"given":"Ha","family":"TRANG","sequence":"first","affiliation":[{"name":"D\u00e9partement de Physiologie, INSERM E9935, H\u00f4pital Robert Debr\u00e9, 48 Boulevard S\u00e9rurier, 75019 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arlette","family":"GIRARD","sequence":"additional","affiliation":[{"name":"Unit\u00e9 de Pharmacologie Clinique, INSERM E0107, Service de N\u00e9phrologie, H\u00f4pital Necker-Enfants Malades, 75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique","family":"LAUDE","sequence":"additional","affiliation":[{"name":"Unit\u00e9 de Pharmacologie Clinique, INSERM E0107, Service de N\u00e9phrologie, H\u00f4pital Necker-Enfants Malades, 75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Luc","family":"ELGHOZI","sequence":"additional","affiliation":[{"name":"Unit\u00e9 de Pharmacologie Clinique, INSERM E0107, Service de N\u00e9phrologie, H\u00f4pital Necker-Enfants Malades, 75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2005,2,18]]},"reference":[{"key":"2021120120370666000_B1","doi-asserted-by":"crossref","DOI":"10.1378\/chest.127.1.72","article-title":"The French congenital central hypoventilation syndrome registry: general data, phenotype, and genotype","author":"Trang","year":"2005","journal-title":"Chest"},{"key":"2021120120370666000_B2","doi-asserted-by":"crossref","first-page":"953","DOI":"10.1203\/01.PDR.0000125262.82405.3C","article-title":"Temporal trends of cardiac and respiratory responses to ventilatory challenges in congenital central hypoventilation syndrome","volume":"55","author":"Macey","year":"2004","journal-title":"Pediatr. 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