RSS-Feed abonnieren
DOI: 10.1055/a-2231-9348
Fetal Long QT Syndrome – Challenges in Perinatal Management: A Review and Case Report. Induction of Labor and Vaginal Birth Under Continuous Magnesium Therapy
Fetales Long QT Syndrom – Herausforderungen im geburtshilflichen Management. Geburtseinleitung und vaginale Entbindung unter kontinuierlicher Magnesiumtherapie![](https://www.thieme-connect.de/media/zgn/202404/lookinside/thumbnails/06-2023-0769_10-1055-a-2231-9348-1.jpg)
Abstract
Congenital LQTS is an often undetected inherited cardiac channel dysfunction and can be a reason for intrauterine fetal demise. It can present in utero as CTG and ultrasound abnormalities, i. e., bradycardia, ventricular tachycardia, or fetal hydrops. Diagnosis is made by CTG, echocardiography, or fMCG. Intrauterine therapy with a ß blocker and i. v. magnesium should be started. Our objective was to examine the current knowledge about diagnosis and treatment of LQTS and in particular to highlight the opportunity of vaginal birth under continuous intravenous magnesium therapy. Therefore, a thorough MEDLINE and Google Scholar search was conducted. Randomized controlled trials, meta-analyses, prospective and retrospective cohort trials, and case reports were considered. We showed the possibility of vaginal delivery under continuous magnesium therapy in a case of suspected fetal LQTS. A stepwise concept for diagnosis, monitoring, and peripartum management in low, intermediate, and high risk cases of fetal LQTS is presented. If risk is low or intermediate, a vaginal delivery under continuous monitoring is reasonable. Induction of labor at term should be evaluated.
Zusammenfassung
Das kongenitale LQTS ist eine häufig unerkannte genetisch erworbene kardiale Ionenkanalerkrankung, die Ursache für einen intrauterinen Fruchttod sein kann. Im CTG oder im pränatalen Ultraschall können sich Auffälligkeiten wie Bradykardien, ventrikuläre Tachykardien oder ein Hydrops fetalis zeigen. Die Diagnose wird regelhaft mittels CTG, Echokardiagraphie oder fMCG gestellt. Eine intrauterine Therapie mit Betablockern und intravenöser Magnesiumgabe sollte begonnen werden. Das Ziel der vorliegenden Arbeit ist die Aufarbeitung des aktuellen Forschungsstandes zur Diagnosestellung und Therapie des LQTS sowie darüber hinaus die Präsentation einer vaginalen Geburt bei vermutetem fetalen LQTS unter kontinuierlicher intravenöser Magnesiumtherapie. Es erfolgte eine detaillierte PUBMED und Google Scholar Recherche. Randomisiert kontrollierte Studien, Metaanalysen, prospektive und retrospektive Kohortenstudien sowie Fallberichte wurden eingeschlossen. Wir zeigen die Möglichkeit einer vaginalen Geburt unter intravenöser Magnesiumtherapie bei vermutetem fetalen LQTS. Außerdem präsentieren wir ein schrittweises Konzept für Diagnosestellung, Überwachung und peripartaler Entscheidungsfindung für Niedrig-, Intermediär- und Hochrisikofälle. Im Falle eines niedrigen oder intermediären Risikos ist der Versuch einer vaginale Geburt unter kontinuierlicher Magnesiumtherapie eine vertretbare Entscheidung. Die Geburtseinleitung am Termin sollte evaluiert werde.
Schlüsselwörter
Fetale Überwachung - Ultraschall - Geburtshilfe - Neonatologie - Management - fetale HerzfrequenzPublikationsverlauf
Eingereicht: 16. Juni 2023
Angenommen nach Revision: 06. Dezember 2023
Artikel online veröffentlicht:
22. Februar 2024
© 2024. Thieme. All rights reserved.
Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany
-
References
- 1 Schwartz PJ, Stramba-Badiale M, Crotti L. et al. Prevalence of the congenital long-QT syndrome. Circulation 2009; 120: 1761-1767 DOI: 10.1161/CIRCULATIONAHA.109.863209.
- 2 Beckmann BM, Wilde AAM, Kääb S. Clinical utility gene card for: Long-QT Syndrome (types 1-13). Eur J Hum Genet 2013; 21: 1185
- 3 Miller TE, Estrella E, Myerburg RJ. et al. Recurrent third-trimester fetal loss and maternal mosaicism for long-QT syndrome. Circulation 2004; 109: 3029-3034 DOI: 10.1161/01.CIR.0000130666.81539.9E.
- 4 Giudicessi JR, Wilde AAM, Ackerman MJ. The genetic architecture of long QT syndrome: A critical reappraisal. Trends Cardiovasc Med 2018; 28: 453-464 DOI: 10.1016/j.tcm.2018.03.003.
- 5 Moss AJ, Schwartz PJ, Crampton RS. et al. The long QT syndrome: a prospective international study. Circulation 1985; 71: 17-21 DOI: 10.1161/01.CIR.71.1.17.
- 6 Cuneo BF, Strasburger JF, Yu S. et al. In utero diagnosis of long QT syndrome by magnetocardiography. Circulation 2013; 128: 2183-2191 DOI: 10.1161/CIRCULATIONAHA.113.004840.
- 7 Bukowski R, Carpenter M, Conway D. et al. Causes of death among stillbirths. JAMA 2011; 306: 2459 DOI: 10.1001/jama.2011.1823.
- 8 Cuneo BF, Etheridge SP, Horigome H. et al. Arrhythmia phenotype during fetal life suggests long-QT syndrome genotype. Circ Arrhythm Electrophysiol 2013; 6: 946-951 DOI: 10.1161/CIRCEP.113.000618.
- 9 American College of Obstetricians and Gynecologists Practice Bulletin No. 106 Intrapartum fetal heart rate monitoring: nomenclature, interpretation, and general management principles. Obstet Gynecol 2009; 114: 192-202 DOI: 10.1097/AOG.0b013e3181aef106.
- 10 Mitchell JL, Cuneo BF, Etheridge SP. et al. Fetal heart rate predictors of long QT syndrome. Circulation 2012; DOI: 10.1161/CIRCULATIONAHA.112.114132.
- 11 Ishikawa S, Yamada T, Kuwata T. et al. Fetal presentation of long QT syndrome – evaluation of prenatal risk factors: a systematic review. Fetal Diagn Ther 2013; 33: 1-7 DOI: 10.1159/000339150.
- 12 Donofrio MT, Moon-Grady AJ, Hornberger LK. et al. Diagnosis and treatment of fetal cardiac disease. Circulation 2014; 129: 2183-2242 DOI: 10.1161/01.cir.0000437597.44550.5d.
- 13 Blais BA, Satou G, Sklansky MS. et al. The diagnosis and management of long QT syndrome based on fetal echocardiography. HeartRhythm Case Rep 2017; 3: 407-410 DOI: 10.1016/j.hrcr.2017.04.007.
- 14 Alvarez SGV, Khoo NS, Colen T. et al. The incremental benefit of color tissue Doppler in fetal arrhythmia assessment. J Am Soc Echocardiogr 2019; 32: 145-156 DOI: 10.1016/j.echo.2018.08.009.
- 15 Steinhard J, Heinig J, Schmitz R. et al. Tissue Doppler Imaging am fetalen Herzen - eine neue parametrische Ultraschalltechnik in der pränatalen Medizin. Ultraschall Med 2007; 28: 578-583 DOI: 10.1055/s-2007-963643.
- 16 Willruth A, Steinhard J, Enzensberger C. et al. Fetal colour tissue Doppler imaging (cTDI): biventricular reference ranges for the time segments of the cardiac cycle in second and third trimesters of gestation. Arch Gynecol Obstet 2016; 294: 917-924 DOI: 10.1007/s00404-016-4076-z.
- 17 Clur S-AB, Vink AS, Etheridge SP. et al. Left ventricular isovolumetric relaxation time is prolonged in fetal long-QT syndrome. Circ Arrhythm Electrophysiol 2018; 11: e005797 DOI: 10.1161/CIRCEP.117.005797.
- 18 Vink AS, Kuipers IM, De Bruin-Bon RHACM. et al. A potential diagnostic approach for foetal long-QT syndrome, developed and validated in children. Pediatr Cardiol 2018; 39: 1413-1422 DOI: 10.1007/s00246-018-1911-y.
- 19 Peters M, Crowe J, Piéri J-F. et al. Monitoring the fetal heart non-invasively: a review of methods. J Perinat Med 2001; 29: 408-416 DOI: 10.1515/JPM.2001.057.
- 20 Winbo A, Fosdal I, Lindh M. et al. Third trimester fetal heart rate predicts phenotype and mutation burden in the type 1 long QT syndrome. Circ Arrhythm Electrophysiol 2015; 8: 806-814 DOI: 10.1161/CIRCEP.114.002552.
- 21 Schwartz PJ. Idiopathic long QT syndrome: Progress and questions. Am Heart J 1985; 109: 399-411 DOI: 10.1016/0002-8703(85)90626-X.
- 22 Page RL. Treatment of arrhythmias during pregnancy. Am Heart J 1995; 130: 871-876 DOI: 10.1016/0002-8703(95)90090-X.
- 23 Crotti L, Celano G, Dagradi F. et al. Congenital long QT syndrome. Orphanet J Rare Dis 2008; 3: 18 DOI: 10.1186/1750-1172-3-18.
- 24 Vincent GM, Schwartz PJ, Denjoy I. et al. High efficacy of β-blockers in long-QT syndrome Type 1. Circulation 2009; 119: 215-221 DOI: 10.1161/CIRCULATIONAHA.108.772533.
- 25 Shimizu W, Antzelevitch C. Differential effects of beta-adrenergic agonists and antagonists in LQT1, LQT2 and LQT3 models of the long QT syndrome. J Am Coll Cardiol 2000; 35: 778-786 DOI: 10.1016/S0735-1097(99)00582-3.
- 26 Went TR, Sultan W, Sapkota A. et al. A systematic review on the role of βeta-blockers in reducing cardiac arrhythmias in long QT syndrome subtypes 1-3. Cureus 2021; 13: e17632 DOI: 10.7759/cureus.17632.
- 27 Tanaka H, Katsuragi S, Tanaka K. et al. Maternal and neonatal outcomes in labor and at delivery when long QT syndrome is present. J Matern Fetal Neonatal Med 2016; 29: 1117-1119 DOI: 10.3109/14767058.2015.1036023.
- 28 Duan L, Ng A, Chen W. et al. Beta-blocker subtypes and risk of low birth weight in newborns. J Clin Hypertens (Greenwich) 2018; 20: 1603-1609 DOI: 10.1111/jch.13397.
- 29 Kubiak K, Koch R, Klockenbusch W. et al. Update reference charts: fetal biometry between the 15th and 20th week of gestation. Fetal Diagn Ther 2016; 40: 195-204 DOI: 10.1159/000443880.
- 30 Ruan Y, Liu N, Bloise R. et al. Gating properties of SCN5A mutations and the response to mexiletine in long-QT syndrome Type 3 patients. Circulation 2007; 116: 1137-1144 DOI: 10.1161/CIRCULATIONAHA.107.707877.
- 31 Simpson JM, Maxwell D, Rosenthal E. et al. Fetal ventricular tachycardia secondary to long QT syndrome treated with maternal intravenous magnesium: case report and review of the literature. Ultrasound Obstet Gynecol 2009; 34: 475-480 DOI: 10.1002/uog.6433.
- 32 Tanel RE, Triedman JK, Walsh EP. et al. High-rate atrial pacing as an innovative bridging therapy in a neonate with congenital long QT syndrome. J Cardiovasc Electrophysiol 1997; 8: 812-817 DOI: 10.1111/j.1540-8167.1997.tb00840.x.
- 33 CredibleMeds. Available from: www.crediblemeds.org date accessed 23.03.2020
- 34 Deutsche Gesellschaft für pädiatrische Kardiologie. Leitlinie Pädiatrische Kardiologie: Tachykarde Herzrhythmusstörungen im Kindes-, Jugend- und jungen Erwachsenenalter (EMAH-Patienten). Leitlinien 2011; 1–10. Available from: https://register.awmf.org/assets/guidelines/023-022l_S2k_Tachykarde_Herzrhythmusstoerungen-Kinder-Jugendliche-junge-Erwachsene_2019-04.pdf
- 35 Hofbeck M, Ulmer H, Beinder E. et al. Prenatal findings in patients with prolonged QT interval in the neonatal period. Heart 1997; 77: 198-204 DOI: 10.1136/hrt.77.3.198.
- 36 Crotti L, Tester DJ, White WM. et al. Long QT syndrome–associated mutations in intrauterine fetal death. JAMA 2013; 309: 1473 DOI: 10.1001/jama.2013.3219.
- 37 Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J. et al. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J 2018; 39: 3165-3241
- 38 Po’ G, Oliver EA, Reddy UM. et al. The impact of induction of labor at 39 weeks in low-risk women on the incidence of stillbirth. Am J Obstet Gynecol 2020; 223: 139-140
- 39 Migliorelli F, De Oliveira SS, Martínez de Tejada B. The ARRIVE Trial: Towards a universal recommendation of induction of labour at 39 weeks?. Eur J Obstet Gynecol Reprod Biol 2020; 244: 192-195 DOI: 10.1016/j.ejogrb.2019.10.034.
- 40 Grobman WA, Rice MM, Reddy UM. et al. Labor induction versus expectant management in low-risk nulliparous women. N Engl J Med 2018; 379: 513-523 DOI: 10.1056/NEJMoa1800566.
- 41 Southall DP, Arrowsmith WA, Oakley JR. et al. Prolonged QT interval and cardiac arrhythmias in two neonates: sudden infant death syndrome in one case. Arch Dis Child 1979; 54: 776-779 DOI: 10.1136/adc.54.10.776.
- 42 Presbitero P, Mangiardi L, Antolini R. Congenital long QT syndrome inducing 2:1 atrioventricular block: early detection in fetal life. Int J Cardiol 1989; 24: 109-112 DOI: 10.1016/0167-5273(89)90049-1.
- 43 Green DW, Ackerman NB, Lund G. et al. Prolonged QT syndrome presenting as fetal bradycardia. J Matern-Fetal Neonatal Med 1992; 1: 202-205 DOI: 10.3109/14767059209161919.
- 44 Trippel DL, Parsons MK, Gillette PC. Infants with long-QT syndrome and 2:1 atrioventricular block. Am Heart J 1995; 130: 1130-1134 DOI: 10.1016/0002-8703(95)90222-8.
- 45 Vigliani M. Romano-Ward syndrome diagnosed as moderate fetal bradycardia: A case report. J Reprod Med 1995; 40: 725-728
- 46 Yamada M, Nakazawa M, Momma K. Fetal ventricular tachycardia in long QT syndrome. Cardiol Young 1998; 8: 119-122 DOI: 10.1017/s1047951100004753.
- 47 Ohkuchi A, Shiraishi H, Minakami H. et al. Fetus with long QT syndrome manifested by tachyarrhythmia: A case report. Prenat Diagn 1999; 19: 990-992 DOI: 10.1002/(sici)1097-0223(199910)19:10<990::aid-pd679>3.0.co;2-%23.
- 48 Hamada H, Horigome H, Asaka M. et al. Prenatal diagnosis of long QT syndrome using fetal magnetocardiography. Prenat Diagn 1999; 19: 677-680 DOI: 10.1002/(SICI)1097-0223(199907)19:7<677::aid-pd597>3.0.co;2-z.
- 49 Donofrio MT, Gullquist SD, O’Connell NG. et al. Fetal presentation of congenital long QT syndrome. Pediatr Cardiol 1999; 20: 441-444 DOI: 10.1007/s002469900510.
- 50 Manning N, Anthony JP, Ostman-Smith I. et al. Prenatal diagnosis and successful preterm delivery of a fetus with long QT syndrome. BJOG 2000; 107: 1049-1051 DOI: 10.1111/j.1471-0528.2000.tb10413.x.
- 51 Chang I-K, Shyu M-K, Lee C-N. et al. Prenatal diagnosis and treatment of fetal long QT syndrome: a case report. Prenat Diagn 2002; 22: 1209-1212 DOI: 10.1002/pd.475.
- 52 Cuneo BF, Ovadia M, Strasburger JF. et al. Prenatal diagnosis and in utero treatment of torsades de pointes associated with congenital long QT syndrome. Am J Cardiol 2003; 91: 1395-1398 DOI: 10.1016/S0002-9149(03)00343-6.
- 53 Hosono T, Kawamata K, Chiba Y. et al. Prenatal diagnosis of long QT syndrome using magnetocardiography: a case report and review of the literature. Prenat Diagn 2002; 22: 198-200 DOI: 10.1002/pd.283.
- 54 Schneider U, Haueisen J, Loeff M. et al. Prenatal diagnosis of a long QT syndrome by fetal magnetocardiography in an unshielded bedside environment. Prenat Diagn 2005; 25: 704-708 DOI: 10.1002/pd.1205.
- 55 Collazos JC, Acherman RJ, Law IH. et al. Sustained fetal bradycardia with 1:1 atrioventricular conduction and long QT syndrome. Prenat Diagn 2007; 27: 879-881 DOI: 10.1002/pd.1784.
- 56 Acherman RJ, Evans WN, Schwartz JK. et al. Right ventricular noncompaction associated with long QT in a fetus with right ventricular hypertrophy and cardiac arrhythmias. Prenat Diagn 2008; 28: 551-553 DOI: 10.1002/pd.2014.
- 57 Horigome H, Iwashita H, Yoshinaga M. et al. Magnetocardiographic demonstration of torsade de pointes in a fetus with congenital long QT syndrome. J Cardiovasc Electrophysiol 2008; 19: 334-335 DOI: 10.1111/j.1540-8167.2007.01026.x.
- 58 Takahashi K, Shiraishi H, Ohkuchi A. et al. Irregular peak-to-peak intervals between ascending aortic flows during fetal ventricular tachycardia in long QT syndrome. Ultrasound Obstet Gynecol 2009; 33: 118-120 DOI: 10.1002/uog.6263.
- 59 Fujimoto Y, Matsumoto T, Honda N. et al. Prenatal diagnosis of long QT syndrome by non-invasive fetal electrocardiography. J Obstet Gynaecol Res 2009; 35: 555-561 DOI: 10.1111/j.1447-0756.2008.00982.x.
- 60 Tomek V, Skovranek J, Gebauer RA. Prenatal diagnosis and management of fetal long QT syndrome. Pediatr Cardiol 2009; 30: 194-196 DOI: 10.1007/s00246-008-9294-0.
- 61 Furushima H, Chinushi M, Sato A. et al. Fetal atrioventricular block and postpartum augmentative qt prolongation in a patient with long-QT syndrome with KCNQ1 mutation. J Cardiovasc Electrophysiol 2010; 21: 1170-1173 DOI: 10.1111/j.1540-8167.2010.01758.x.
- 62 Fukushima A, Nakai K, Matsumoto A. et al. Prenatal diagnosis of polymorphic ventricular tachycardia using 64-channel magnetocardiography. Heart Vessels 2010; 25: 270-273 DOI: 10.1007/s00380-009-1195-5.
- 63 Komarlu R, Beerman L, Freeman D. et al. Fetal and neonatal presentation of long QT syndrome. Pacing Clin Electrophysiol 2012; 35: e87-e90 DOI: 10.1111/j.1540-8159.2011.03040.x.
- 64 Crimmins S, Vashit S, Doyle L. et al. A multidisciplinary approach to prenatal treatment of congenital long QT syndrome. J Clin Ultrasound 2017; 45: 168-170 DOI: 10.1002/jcu.22386.
- 65 Miyake A, Sakaguchi H, Miyazaki A. et al. Successful prenatal management of ventricular tachycardia and second-degree atrioventricular block in fetal long QT syndrome. HeartRhythm Case Rep 2017; 3: 53-57 DOI: 10.1016/j.hrcr.2016.09.001.
- 66 Desai L, Wakai R, Tsao S. et al. Fetal diagnosis of KCNQ1-variant long QT syndrome using fetal echocardiography and magnetocardiography. Pacing Clin Electrophysiol 2020; 43: 430-433 DOI: 10.1111/pace.13900.
- 67 Shima Y, Horigome H, Nozaki Y. et al. Successful trans-maternal nadolol pharmacotherapy in a fetus presenting with long QT syndrome type 2 complicated by torsade de pointes. J Cardiol Cases 2020; 22: 265-268 DOI: 10.1016/j.jccase.2020.07.006.
- 68 Cherian AG, Lankala P, Krupa J. et al. Ventricular noncompaction and long QT syndrome – A deadly double hit for the foetus. Indian Pacing Electrophysiol J 2021; 21: 186–190 DOI: 10.1016/j.ipej.2021.03.001.
- 69 Santi A, Restrepo M. Congenital long QT syndrome: A challenging diagnosis by fetal echocardiography. Ann Pediatr Cardiol 2022; 15: 64-66 DOI: 10.4103/apc.apc_34_21.
- 70 Zidere V, Vigneswaran TV, Dumitrascu-Biris I. et al. Presentation and genetic confirmation of long QT syndrome in the fetus. HeartRhythm Case Rep 2022; 8: 674-678 DOI: 10.1016/j.hrcr.2022.07.007.