{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T08:00:55Z","timestamp":1783065655970,"version":"3.54.6"},"reference-count":21,"publisher":"S. Karger AG","issue":"6","license":[{"start":{"date-parts":[[2015,1,1]],"date-time":"2015-01-01T00:00:00Z","timestamp":1420070400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2015,1,1]],"date-time":"2015-01-01T00:00:00Z","timestamp":1420070400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Respiration"],"published-print":{"date-parts":[[2015]]},"abstract":"<jats:p>&lt;b&gt;&lt;i&gt;Background:&lt;\/i&gt;&lt;\/b&gt; Congenital central hypoventilation syndrome (CCHS) is a rare disorder affecting central control of breathing. Thus, patients require lifelong assisted ventilation. Diaphragm pacing (DP) may permit decannulation in those who are ventilator dependent only during sleep. &lt;b&gt;&lt;i&gt;Objective:&lt;\/i&gt;&lt;\/b&gt; The purpose of this study is to determine if patients with CCHS can be successfully ventilated by DP without tracheostomy. &lt;b&gt;&lt;i&gt;Methods:&lt;\/i&gt;&lt;\/b&gt; We reviewed the records of 18 CCHS patients (mean age 19.5 \u00b1 10.1 years; 44% female) who were ventilated by DP only during sleep. &lt;b&gt;&lt;i&gt;Results:&lt;\/i&gt;&lt;\/b&gt; Prior to diaphragm pacer implantation surgery, 14 CCHS patients had been using home portable positive pressure ventilation (PPV) via tracheostomy, 1 had been on PPV via endotracheal tube, and 3 had been using noninvasive PPV (NPPV). Of the patients with tracheostomy prior to DP (n = 15), 11 (73%) were decannulated and ventilated successfully by DP without tracheostomy. Of all the patients reviewed (n = 18), 13 (72%) were successfully ventilated by DP without tracheostomy. Obesity prevented successful DP without tracheostomy in 1 patient, and upper airway obstruction prevented success in another patient. Snoring and\/or obstructive apneas were present in some patients, but they were improved by diaphragm pacer changes, adenotonsillectomy, and\/or use of nasal steroids. &lt;b&gt;&lt;i&gt;Conclusions:&lt;\/i&gt;&lt;\/b&gt; DP without tracheostomy can be successfully achieved in patients with CCHS. Snoring and obstructive apneas, when present, can be managed by diaphragm pacer changes and medical therapies. Obesity can pose a challenge to successful DP.<\/jats:p>","DOI":"10.1159\/000381401","type":"journal-article","created":{"date-parts":[[2015,4,27]],"date-time":"2015-04-27T21:01:20Z","timestamp":1430168480000},"page":"534-538","source":"Crossref","is-referenced-by-count":40,"title":["Diaphragm Pacing without Tracheostomy in Congenital Central Hypoventilation Syndrome Patients"],"prefix":"10.1159","volume":"89","author":[{"given":"Bonnie","family":"Diep","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annie","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheila","family":"Kun","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. Gordon","family":"McComb","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donald B.","family":"Shaul","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cathy E.","family":"Shin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas G.","family":"Keens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iris A.","family":"Perez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2015,4,25]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.160.1.16010"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200807-1069ST"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1038\/ng1130"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2003.015412"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1016\/j.prrv.2004.04.009"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1002\/0471751723.ch66"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1016\/j.jsmc.2010.05.013"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.10438"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1007\/BF02282890"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.10392"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1099-0496(199707)24:1<66::AID-PPUL12>3.0.CO;2-8"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1097\/00000658-197010000-00020"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1159\/000076685"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1586\/17434440.2.5.577"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1097\/00006123-198512000-00021"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1111\/j.1540-8159.1988.tb06362.x"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)80588-8"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1136\/adc.70.5.418"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.1053\/jpsu.2002.33821"},{"key":"ref20","doi-asserted-by":"publisher","DOI":"10.5301\/ijao.5000197"},{"key":"ref21","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2013-200911"}],"container-title":["Respiration"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/381401","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/381401","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T17:53:42Z","timestamp":1745430822000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000381401"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015]]},"references-count":21,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2015,6,1]]}},"URL":"https:\/\/doi.org\/10.1159\/000381401","archive":["Portico"],"relation":{},"ISSN":["0025-7931","1423-0356"],"issn-type":[{"value":"0025-7931","type":"print"},{"value":"1423-0356","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015]]}}}