{"doi":"10.1093/europace/euad319","title":"Effects of cohort, genotype, variant, and maternal β-blocker treatment on foetal heart rate predictors of inherited long QT syndrome","abstract":"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.","journal":"EP Europace","year":2023,"id":347083,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":12,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9641,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2023-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1088515,"name":"Annika Winbo","orcid":"0000-0002-9323-3166","position":1,"is_corresponding":false},{"id":729576,"name":"Sally‐Ann B. Clur","orcid":"0000-0001-5276-1475","position":2,"is_corresponding":false},{"id":344973,"name":"Susan P. Etheridge","orcid":"0000-0002-2492-422X","position":3,"is_corresponding":false},{"id":32887,"name":"Michael J. Ackerman","orcid":"0000-0002-8011-3333","position":4,"is_corresponding":false},{"id":1088516,"name":"Hitoshi Horigome","orcid":"0000-0002-6090-7531","position":5,"is_corresponding":false},{"id":1088517,"name":"Ulrike Herberg","orcid":"0000-0002-9386-0258","position":6,"is_corresponding":false},{"id":658459,"name":"Federica Dagradi","orcid":"0000-0002-1090-3292","position":7,"is_corresponding":false},{"id":344950,"name":"Carla Spazzolini","orcid":"0000-0003-1209-0157","position":8,"is_corresponding":false},{"id":525461,"name":"Stacy A. S. Killen","orcid":"0000-0002-6458-3669","position":9,"is_corresponding":false},{"id":386204,"name":"Annette Wacker‐Gussmann","orcid":"0000-0001-5143-5029","position":10,"is_corresponding":false},{"id":230116,"name":"Arthur A.M. Wilde","orcid":"0000-0002-0528-0852","position":11,"is_corresponding":false},{"id":1040794,"name":"Elena Sinkovskaya","orcid":null,"position":12,"is_corresponding":false},{"id":260838,"name":"Alfred Abuhamad","orcid":null,"position":13,"is_corresponding":false},{"id":845738,"name":"Margherita Torchio","orcid":"0000-0003-3887-6772","position":14,"is_corresponding":false},{"id":314112,"name":"Chai‐Ann Ng","orcid":"0000-0002-3120-5450","position":15,"is_corresponding":false},{"id":1088518,"name":"Annika Rydberg","orcid":"0000-0001-9655-7783","position":16,"is_corresponding":false},{"id":30332,"name":"Peter J. Schwartz","orcid":"0000-0003-0367-1048","position":17,"is_corresponding":false},{"id":355543,"name":"Bettina F. Cuneo","orcid":"0000-0001-9085-0882","position":18,"is_corresponding":false},{"id":303666,"name":"Alexander Kaizer","orcid":"0000-0003-2334-5514","position":0,"is_corresponding":true}],"reference_count":30,"raw_metadata":null,"created_at":"2026-07-19T01:11:57.720122Z","pmid":"37975542","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}