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Original research article
Arrhythmia risk and β-blocker therapy in pregnant women with long QT syndrome
  1. Kohei Ishibashi1,2,
  2. Takeshi Aiba1,
  3. Chizuko Kamiya3,
  4. Aya Miyazaki3,
  5. Heima Sakaguchi3,
  6. Mitsuru Wada1,
  7. Ikutaro Nakajima1,
  8. Koji Miyamoto1,
  9. Hideo Okamura1,
  10. Takashi Noda1,
  11. Toshifumi Yamauchi4,
  12. Hideki Itoh5,
  13. Seiko Ohno5,
  14. Hideki Motomura6,
  15. Yoshiharu Ogawa7,
  16. Hiroko Goto8,
  17. Takaomi Minami9,
  18. Nobue Yagihara10,
  19. Hiroshi Watanabe10,
  20. Kanae Hasegawa11,
  21. Akihiro Terasawa12,
  22. Hitoshi Mikami13,
  23. Kayo Ogino14,
  24. Yukiko Nakano15,
  25. Sato Imashiro16,
  26. Yosuke Fukushima16,
  27. Yoshimitsu Tsuzuki17,
  28. Koko Asakura18,
  29. Jun Yoshimatsu3,
  30. Isao Shiraishi3,
  31. Shiro Kamakura1,
  32. Yoshihiro Miyamoto19,
  33. Satoshi Yasuda1,
  34. Takashi Akasaka2,
  35. Minoru Horie5,
  36. Wataru Shimizu1,20,
  37. Kengo Kusano1
  1. 1 Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Japan
  2. 2 Department of Cardiovascular Medicine, Wakayama Medical University, Wakayama, Japan
  3. 3 Department of Perinatal and Pediatric Cardiology, National Cerebral and Cardiovascular Center, Suita, Japan
  4. 4 Department of Pediatrics, Ehime University Hospital, Ehime, Japan
  5. 5 Department of Cardiovascular and Respiratory Medicine, Shiga University of Medical Science, Otsu, Japan
  6. 6 Department of Pediatrics, Nagasaki University Hospital, Nagasaki, Japan
  7. 7 Department of Cardiovascular Medicine, Hyogo Children’s Hospital, Kobe, Japan
  8. 8 Department of Pediatric Cardiology, Gifu Prefectural General Medical Center, Gifu, Japan
  9. 9 Department of Pediatrics, Jichi Medical University, Shimotsuke, Japan
  10. 10 Department of Cardiovascular Biology and Medicine, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan
  11. 11 Department of Cardiology, University of Fukui Faculty of Medical Science, Fukui, Japan
  12. 12 Division of Clinical Training, Kasugai Municipal Hospital, Aichi, Japan
  13. 13 Department of Pediatrics, Iwate Prefectural Central Hospital, Iwate, Japan
  14. 14 Department of Pediatrics, Kurashiki Central Hospital, Kurashiki, Japan
  15. 15 Department of Cardiovascular Medicine, Hiroshima University, Hiroshima, Japan
  16. 16 Department of Pediatrics, Kochi Health Science Center, Kochi, Japan
  17. 17 Department of Pediatrics, St. Marianna University School of Medicine, Kawasaki, Japan
  18. 18 Department of Data Science, National Cerebral and Cardiovascular Center, Suita, Japan
  19. 19 Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Suita, Japan
  20. 20 Department of Cardiovascular Medicine, Nippon Medical School, Tokyo, Japan
  1. Correspondence to Dr Takeshi Aiba, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka 565-8565, Japan; aiba{at}


Background Pregnancy is one of the biggest concerns for women with long QT syndrome (LQTS).

Objectives This study investigated pregnancy-related arrhythmic risk and the efficacy and safety of β-blocker therapy for lethal ventricular arrhythmias in pregnant women with LQTS (LQT-P) and their babies.

Methods 136 pregnancies in 76 LQT-P (29±5 years old; 22 LQT1, 36 LQT2, one LQT3, and 17 genotype-unknown) were enrolled. We retrospectively analysed their clinical and electrophysiological characteristics and pregnancy outcomes in the presence (BB group: n=42) or absence of β-blocker therapy (non-BB group: n=94).

Results All of the BB group had been diagnosed with LQTS with previous events, whereas 65% of the non-BB group had not been diagnosed at pregnancy. Pregnancy increased heart rate in the non-BB group; however, no significant difference was observed in QT and Tpeak–Tend intervals between the two groups. In the BB group, only two events occurred at postpartum, whereas 12 events occurred in the non-BB group during pregnancy (n=6) or postpartum period (n=6). The frequency of spontaneous abortion did not differ between the two groups. Fetal growth rate and proportion of infants with congenital malformation were similar between the two groups, but premature delivery and low birthweight infants were more common in those taking BB (OR 4.79, 95% CI 1.51 to 15.21 and OR 3.25, 95% CI 1.17 to 9.09, respectively).

Conclusions Early diagnosis and β-blocker therapy for high-risk patients with LQTS are important for prevention of cardiac events during pregnancy and the postpartum period, and β-blocker therapy may be tolerated for babies in LQT-P cases.

  • Pregnancy
  • Clinical genetics
  • Pharmacology
  • Cardiac arrhythmias and resuscitation science
  • ECG/electrocardiogram

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  • Contributors Study conception and design: KI, TA. Acquisition of data: CK, AM, HS, MW, IN, KM, HO, TN, TY, HI, SO, HM, YO, HG, TM, NY, HW, KH, AT, HM, KO, YN, SI, YF, YT. Analysis and interpretation of data: KI, TA, KA. Drafting of manuscript: KI, TA. Critical revision: JY, IS, SK, YM, SY, TA, MH, WS, KK.

  • Funding This work was supported by a Grant-in-Aid for Scientific Research on Innovative Areas (22136011A02 to TA), a Grant-in-Aid for Scientific Research (C) (24591086 to TA) from MEXT of Japan, a Research Grant for Cardiovascular Diseases (H24-033, H26-040 to TA, SK, MH, WS) from the Ministry of Health, Labour, and Welfare, Japan, and a Grant for Translation Medicine by Japan Circulation Society to MH, WS, TA.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.