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Sökning: id:"swepub:oai:DiVA.org:umu-217540" > Effects of cohort, ...

Effects of cohort, genotype, variant, and maternal β-blocker treatment on foetal heart rate predictors of inherited long QT syndrome

Kaizer, Alexander M. (författare)
Biostatistics and Informatics, Colorado School of Public Health, University of Colorado-Anschutz Medical Campus, CO, Aurora, United States
Winbo, Annika (författare)
Umeå universitet,Pediatrik,Department of Physiology, University of Auckland, Auckland, New Zealand
Clur, Sally-Ann B (författare)
Department of Pediatric Cardiology, Emma Children's Hospital, Amsterdam University Medical Centers, Amsterdam, Netherlands; Department of Cardiology, University Medical Center, Amsterdam, Netherlands
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Etheridge, Susan P. (författare)
Department of Pediatrics, Division of Cardiology, University of Utah School of Medicine, UT, Salt Lake City, United States
Ackerman, Michael J. (författare)
Department of Cardiovascular Medicine, Division of Heart Rhythm Services, Mayo Clinic, MN, Rochester, United States; Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, MN, Rochester, United States; Department of Molecular Pharmacology & Experimental Therapeutics, Mayo Clinic, MN, Rochester, United States; Mayo Clinic, Windland Smith Rice Genetic Heart Rhythm Clinic and Windland Smith Rice Sudden Death Genomics Laboratory, MN, Rochester, United States
Horigome, Hitoshi (författare)
Department of Pediatrics, Section of Cardiology, Tsukuba University, Tsukuba, Japan
Herberg, Ulrike (författare)
Department of Pediatric Cardiology, RWTH University Hospital Aachen, Aachen, Germany; Department of Pediatric Cardiology, University Hospital Bonn, Bonn, Germany
Dagradi, Federica (författare)
Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Via Pier Lombardo 22, Milan, Italy
Spazzolini, Carla (författare)
Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Via Pier Lombardo 22, Milan, Italy
Killen, Stacy A S (författare)
Department of Pediatrics, Division of Cardiology, Vanderbilt University Medical Center, TN, Nashville, United States
Wacker-Gussmann, Annette (författare)
Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Center, Munich, Germany
Wilde, Arthur A M (författare)
Department of Cardiology, University Medical Center, Amsterdam, Netherlands; Department of Cardiology, Amsterdam UMC Location University of Amsterdam, Amsterdam, Netherlands; Department of Cardiology, Amseterdam University Medical Center, Amsterdam, Netherlands
Sinkovskaya, Elena (författare)
Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Eastern Virginia Medical School, VA, Norfolk, United States
Abuhamad, Alfred (författare)
Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Eastern Virginia Medical School, VA, Norfolk, United States
Torchio, Margherita (författare)
Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Via Pier Lombardo 22, Milan, Italy
Ng, Chai-Ann (författare)
Mark Cowley Lidwill Research Program in Cardiac Electrophysiology, Victor Chang Cardiac Research Institute, Darlinghurst, NSW, Australia; School of Clinical Medicine, UNSW Sydney, Darlinghurst, NSW, Australia
Rydberg, Annika (författare)
Umeå universitet,Pediatrik,Department of Cardiology, University Medical Center, Amsterdam, Netherlands
Schwartz, Peter J. (författare)
Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Via Pier Lombardo 22, Milan, Italy
Cuneo, Bettina F. (författare)
Department of Pediatrics, Section of Cardiology, University of Denver School of Medicine, 13123 16th Ave, Box 100, Aurora, CO 80045, USA
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 (creator_code:org_t)
Oxford University Press, 2023
2023
Engelska.
Ingår i: Europace. - : Oxford University Press. - 1099-5129 .- 1532-2092. ; 25:11
  • Tidskriftsartikel (refereegranskat)
Abstract Ämnesord
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  • AIMS: In long QT syndrome (LQTS), primary prevention improves outcome; thus, early identification is key. The most common LQTS phenotype is a foetal heart rate (FHR) < 3rd percentile for gestational age (GA) but the effects of cohort, genotype, variant, and maternal β-blocker therapy on FHR are unknown. We assessed the influence of these factors on FHR in pregnancies with familial LQTS and developed a FHR/GA threshold for LQTS.METHODS AND RESULTS: In an international cohort of pregnancies in which one parent had LQTS, LQTS genotype, familial variant, and maternal β-blocker effects on FHR were assessed. We developed a testing algorithm for LQTS using FHR and GA as continuous predictors. Data included 1966 FHRs at 7-42 weeks' GA from 267 pregnancies/164 LQTS families [220 LQTS type 1 (LQT1), 35 LQTS type 2 (LQT2), and 12 LQTS type 3 (LQT3)]. The FHRs were significantly lower in LQT1 and LQT2 but not LQT3 or LQTS negative. The LQT1 variants with non-nonsense and severe function loss (current density or β-adrenergic response) had lower FHR. Maternal β-blockers potentiated bradycardia in LQT1 and LQT2 but did not affect FHR in LQTS negative. A FHR/GA threshold predicted LQT1 and LQT2 with 74.9% accuracy, 71% sensitivity, and 81% specificity.CONCLUSION: Genotype, LQT1 variant, and maternal β-blocker therapy affect FHR. A predictive threshold of FHR/GA significantly improves the accuracy, sensitivity, and specificity for LQT1 and LQT2, above the infant's a priori 50% probability. We speculate this model may be useful in screening for LQTS in perinatal subjects without a known LQTS family history.

Ämnesord

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Reproduktionsmedicin och gynekologi (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Obstetrics, Gynaecology and Reproductive Medicine (hsv//eng)
MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Kardiologi (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Cardiac and Cardiovascular Systems (hsv//eng)

Nyckelord

Bradycardia
Channelopathy
Foetal arrhythmia
Foetus
Inherited arrhythmias
Long QT syndrome
Potassium currents
Stillbirth
Sudden death

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