{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T08:23:20Z","timestamp":1781079800890,"version":"3.54.1"},"reference-count":23,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2021,5,13]],"date-time":"2021-05-13T00:00:00Z","timestamp":1620864000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Anesthesia"],"published-print":{"date-parts":[[2021,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Marfan syndrome and Loeys\u2010Dietz syndrome are connective tissue disorders associated with cardiac and vascular disease. Patients often require surgical repair, but limited data exist to describe their perioperative management.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aims<\/jats:title><jats:p>Our goals were to review the perioperative features of patients with Marfan and Loeys\u2010Dietz syndrome that may affect anesthesia care and to describe the differences in preoperative clinical characteristics and intra\u2010operative anesthetic management.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We conducted a retrospective cohort study of patients with Marfan and Loeys\u2010Dietz syndrome who underwent cardiac surgery at a single institution. We collected demographic and perioperative data from the electronic medical record and performed descriptive statistics to characterize the patient populations and describe their anesthetic management.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In 71 patients (40 Marfan, 31 Loeys\u2010Dietz), we found significant differences between the Marfan and Loeys\u2010Dietz patients in airway difficulty, preoperative weight, blood utilization, valvular disease, and age at first cardiac surgery. Patients with Loeys\u2010Dietz syndrome had higher preoperative rates of severe noncardiac comorbidities, including gastroesophageal reflux and asthma that required chronic medical therapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Despite undergoing similar surgical procedures, patients with Marfan and Loeys\u2010Dietz syndrome have different intrinsic patient characteristics and comorbidities that may affect their perioperative care. This retrospective cohort study identified some factors, but additional collection and reporting of patient data based on multicenter experience are essential for the ongoing optimization of perioperative care in these patient populations.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pan.14196","type":"journal-article","created":{"date-parts":[[2021,4,22]],"date-time":"2021-04-22T07:41:08Z","timestamp":1619077268000},"page":"820-826","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Perioperative management and considerations in pediatric patients with connective tissue disorders undergoing cardiac surgery"],"prefix":"10.1111","volume":"31","author":[{"given":"Jessica A.","family":"Berger","sequence":"first","affiliation":[{"name":"Department of Anesthesiology and Critical Care Medicine Children's Hospital of Philadelphia Philadelphia PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dana D.","family":"Huh","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teresa","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology and Critical Care Medicine Johns Hopkins University School of Medicine Baltimore MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rajeev S.","family":"Wadia","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology and Critical Care Medicine Johns Hopkins University School of Medicine Baltimore MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melania M.","family":"Bembea","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology and Critical Care Medicine Johns Hopkins University School of Medicine Baltimore MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7538-4630","authenticated-orcid":false,"given":"Dheeraj K.","family":"Goswami","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology and Critical Care Medicine Johns Hopkins University School of Medicine Baltimore MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,5,13]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1177\/1089253213513842"},{"key":"e_1_2_7_3_1","volume-title":"GeneReviews(\u00ae)","author":"Loeys 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