{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T20:16:54Z","timestamp":1792268214659,"version":"4.4.0"},"reference-count":24,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circ: Arrhythmia and Electrophysiology"],"published-print":{"date-parts":[[2013,10]]},"abstract":"<jats:sec>\n                    <jats:title>Background\u2014<\/jats:title>\n                    <jats:p>Fetal arrhythmias characteristic of long QT syndrome (LQTS) include torsades de pointes (TdP) and\/or 2\u00b0 atrioventricular block, but sinus bradycardia, defined as fetal heart rate &lt;3% for gestational age, is most common. We hypothesized that prenatal rhythm phenotype might predict LQTS genotype and facilitate improved risk stratification and management.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Method and Results\u2014<\/jats:title>\n                    <jats:p>\n                      Records of subjects exhibiting fetal LQTS arrhythmias were reviewed. Fetal echocardiograms, neonatal ECG, and genetic testing were evaluated. We studied 43 subjects exhibiting fetal LQTS arrhythmias: TdP\u00b12\u00b0 atrioventricular block (group 1, n=7), isolated 2\u00b0 atrioventricular block (group 2, n=4), and sinus bradycardia (group 3, n=32). Mutations in known LQTS genes were found in 95% of subjects tested. SCN5A mutations occurred in 71% of group 1, whereas 91% of subjects with KCNQ1 mutations were in group 3. Small numbers of subjects with KCNH2 mutations (n=4) were scattered in all 3 groups. Age at presentation did not differ among groups, and most subjects (n=42) were live-born with gestational ages of 37.5\u00b12.8 weeks (mean\u00b1SD). However, those with TdP were typically delivered earlier. Prenatal treatment in group 1 terminated (n=2) or improved (n=4) TdP. The neonatal heart rate\u2013corrected QT interval (mean\u00b1SE) of group 1 (664.7\u00b124.9) was longer than neonatal heart rate\u2013corrected QT interval in both group 2 (491.2\u00b127.6;\n                      <jats:italic>P<\/jats:italic>\n                      =0.004) and group 3 (483.1\u00b113.7;\n                      <jats:italic>P<\/jats:italic>\n                      &lt;0.001). Despite medical and pacemaker therapy, postnatal cardiac arrest (n=4) or sudden death (n=1) was common among subjects with fetal\/neonatal TdP.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions\u2014<\/jats:title>\n                    <jats:p>Rhythm phenotypes of fetal LQTS have genotype-suggestive features that, along with heart rate\u2013corrected QT interval duration, may risk stratify perinatal management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/circep.113.000618","type":"journal-article","created":{"date-parts":[[2013,8,31]],"date-time":"2013-08-31T00:23:27Z","timestamp":1377908607000},"page":"946-951","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":71,"title":["Arrhythmia Phenotype During Fetal Life Suggests Long-QT Syndrome Genotype"],"prefix":"10.1161","volume":"6","author":[{"given":"Bettina F.","family":"Cuneo","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan P.","family":"Etheridge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hitoshi","family":"Horigome","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Denver","family":"Sallee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anita","family":"Moon-Grady","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hsin-Yi","family":"Weng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael J.","family":"Ackerman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. Woodrow","family":"Benson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1161\/circulationaha.112.114132"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9149(03)00343-6"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.hrthm.2011.11.006"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1161\/circep.108.788349"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1161\/circep.109.882159"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2003.09.049"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1136\/heart.90.1.13"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1002\/uog.6433"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1161\/circulationaha.112.001216"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0223(199907)19:7<677::AID-PD597>3.0.CO;2-Z"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.hrthm.2005.01.020"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.97.7.640"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1161\/hc0702.105124"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.RES.83.6.668"},{"key":"e_1_3_3_16_2","doi-asserted-by":"publisher","DOI":"10.1161\/circulationaha.105.570200"},{"key":"e_1_3_3_17_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.84.3.1136"},{"key":"e_1_3_3_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2009.05.029"},{"key":"e_1_3_3_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-8703(95)90222-8"},{"key":"e_1_3_3_20_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.286.18.2264"},{"key":"e_1_3_3_21_2","doi-asserted-by":"publisher","DOI":"10.1161\/circulationaha.106.658021"},{"key":"e_1_3_3_22_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.hrthm.2012.02.023"},{"key":"e_1_3_3_23_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.92.12.3381"},{"key":"e_1_3_3_24_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(99)00582-3"},{"key":"e_1_3_3_25_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.cir.0000125523.14403.1e"}],"container-title":["Circulation: Arrhythmia and Electrophysiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/CIRCEP.113.000618","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,12]],"date-time":"2024-05-12T20:05:38Z","timestamp":1715544338000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/CIRCEP.113.000618"}},"subtitle":["Risk Stratification of Perinatal Long-QT Syndrome"],"short-title":[],"issued":{"date-parts":[[2013,10]]},"references-count":24,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2013,10]]}},"alternative-id":["10.1161\/CIRCEP.113.000618"],"URL":"https:\/\/doi.org\/10.1161\/circep.113.000618","relation":{},"ISSN":["1941-3149","1941-3084"],"issn-type":[{"value":"1941-3149","type":"print"},{"value":"1941-3084","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,10]]},"assertion":[{"value":"2012-11-20","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2013-08-01","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2013-08-30","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}