Arrhythmia risk and β-blocker therapy in pregnant women with long QT syndrome

Ishibashi, Keiichiro; Aiba, Takeshi; Kamiya, Chizuko; Miyazaki, Aya; Sakaguchi, Heima; Wada, Mitsuru; Nakajima, Ikutaro; Miyamoto, Koji; Okamura, Hideo; Noda, Takashi; Yamauchi, Toshifumi; Itoh, Hideki; Ohno, Seiko; Motomura, Hideki; Ogawa, Yoshiharu; Goto, Hiroko; Minami, Takaomi; Yagihara, Nobue; Watanabe, Hiroshi; Hasegawa, Kanae; Terasawa, Akihiro; Mikami, Hitoshi; Ogino, Kayo; Nakano, Yukiko; Imashiro, Sato; Fukushima, Yukio; Yoshimitsu, Tetsuo; Asakura, Koko; Yoshimatsu, Jun; Shiraishi, Isao; Kamakura, Shiro; Miyamoto, Yoshihiro; Yasuda, Satoshi; Akasaka, Takashi; Horie, Minoru; Shimizu, Wataru; Kusano, Kengo

doi:10.1136/heartjnl-2016-310617

Cited by 50 publications

(25 citation statements)

References 21 publications

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“…The choice of β‑blockers is less established due to the limited evidence available today; while more data exists for fetal safety of metoprolol and propranolol [ 29 ], propranolol or nadolol seem to be the most effective in reducing the arrhythmic risk [ 8 ]. For these reasons, propranolol is the most widely used for LQTS and often the preferred β‑blocker in pregnancy and the postpartum period at 2–3 mg/kg/day [ 33 ].…”

Section: Recommendations For Lqts Patients During Pregnancy Delivery and Postpartummentioning

confidence: 99%

Arrhythmic risk during pregnancy and postpartum in patients with long QT syndrome

Asatryan

Rieder

Castiglione

et al. 2021

Herzschr Elektrophys

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Congenital long QT syndrome (LQTS) is a genetic disorder characterized by a prolonged QT interval in the surface electrocardiogram (ECG) that predisposes affected individuals to arrhythmic syncope, ventricular torsades-de-pointes, and sudden cardiac death at a young age. Investigations of large patient cohorts revealed sex-related differences in the LQTS phenotype. Adult women with LQTS are at higher risk for cardiac arrhythmias than are adult men with LQTS. Sex hormones are thought to play the primary role for these gender differences. Clinical experience and translational studies indicated that females with LQTS have a lower risk for cardiac arrhythmias during pregnancy and elevated risk in the postpartum period due to contrasting effects of estradiol and progesterone, as well as postpartum hormones on the action potential and arrhythmia substrate. However, this pro- or anti-arrhythmic potential of hormones varies depending on the underlying genotype, partly since sex hormones have distinct effects on different (affected) cardiac ion channels. Thus, a comprehensive evaluation of women with LQTS prior to and during pregnancy, during labor, and in the postpartum period with consideration of the patient’s disease- and gene-specific risk factors is essential to providing precision management in this patient group. This review discusses the current understanding of hormonal influences in LQTS and provides practical guidance for the optimal management of LQTS patients during pregnancy, delivery, and the postpartum period.

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Section: Recommendations For Lqts Patients During Pregnancy Delivery and Postpartummentioning

confidence: 99%

Arrhythmic risk during pregnancy and postpartum in patients with long QT syndrome

Asatryan

Rieder

Castiglione

et al. 2021

Herzschr Elektrophys

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“…β-receptor blockers are effective in reducing life-threatening CE in iLQTS and therefore should be continued during pregnancy and postpartum as pregnant iLQTS women are at a risk to develop CE [8-10, 17, 45]. β-receptor blockers are not teratogenic, but they are categorized by former FDA category as “C,” whereas atenolol is categorized as “D.” They can cause IUGR commonly and additionally bradycardia, apnea, hypoglycemia, and hyperbilirubinemia have been reported in the newborn infant [17-20].…”

Section: Discussionmentioning

confidence: 99%

Intrauterine Growth Retardation in Pregnant Women with Long QT Syndrome Treated with Beta-Receptor Blockers

2021

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Pregnant women with inherited long QT syndrome (iLQTS) are at an increased risk for preterm delivery and intrauterine growth retardation (IUGR) due to their underlying disease. Additionally, they are at a risk of arrhythmogenic events, particularly during the postpartum period because of physiological changes and increased emotional/physical stress. β-receptor blockers can effectively prevent life-threatening Torsades de Pointes ventricular tachycardia and they are the treatment of choice in iLQTS. Use of β-receptor blockers in pregnancy is recommended, although IUGR is commonly reported for prenatally exposed infants. IUGR, particularly in preterm infants, can result in adverse neonatal outcomes. This review was performed to support clinicians in their selection of β-receptor blocker treatment for their pregnant iLQTS women by (i) summarizing the available literature addressing the impact of different β-receptor blockers on IUGR and (ii) reporting additional aspects which might influence the β-receptor blocker selection. In general, experts recommend to use nonselective β-receptor blockers, such as nadolol and propranolol, for iLQTS management as these drugs seem to be superior in effectiveness. However, β-1-selective receptor blockers, such as bisoprolol or metoprolol, seem to affect less likely uterine contraction, peripheral vasodilation, and are associated with lower IUGR rates and fetal hypoglycemia. They are therefore recommended, except atenolol, as first-line therapy for pregnant women. Additionally, maternal factors such as iLQTS genotype, other underlying comorbidities (e.g., diabetes mellitus type 1, asthma bronchiale), and uteroplacental dysfunction or fetal factors have to be taken into account. Therefore, each woman with iLQTS who wants to become pregnant should be well-advised for a personalized β-receptor blocker therapy according to the individual risk-benefit evaluation by a multidisciplinary team of cardiologists, gynecologists, pediatric cardiologists, neonatologists, and clinical pharmacologists. During pregnancy, a close monitoring of IUGR and, after birth, monitoring of bradycardia, hypoglycemia, and respiratory depression in the neonate is mandatory. This review summarizes available data on β-receptor blocker-related risk for IUGR in prenatally exposed infants and illustrates which factors might influence β-receptor blocker selection with the aim to support clinicians in their pharmacological management of their pregnant iLQTS patients.

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“…Oxytocin may be involved in increased aortic dissection in the puerperium. However, since some diseases, such as perinatal cardiomyopathy and QT prolongation syndrome, are exacerbated during the postpartum period, 17, 18 attention should be paid to the onset of aortic dissection during this period. If symptoms suggestive of aortic dissection such as back pain are observed even after the end of pregnancy, aggressive diagnostic examinations should be performed.…”

Section: Discussionmentioning

confidence: 99%

Aortic dissection during pregnancy and puerperium: A Japanese nationwide survey

Tanaka

Kamiya

Horiuchi

et al. 2021

J of Obstet and Gynaecol

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Aim This study aimed to characterize the incidence and clinical significance of pregnancy‐related aortic dissection using a large‐scale survey in Japan. Methods A questionnaire requesting the detailed information included in the clinical charts of pregnancy‐related aortic dissection cases (without any personally identifying information) was designed between 2013 and 2017 and administered to 407 perinatal centers in Japan. The response rate was 70.5%. Seventeen cases of pregnancy‐related aortic dissection were identified. Results Maternal death due to aortic dissection was observed in nine patients (56.2%) while seven survived (43.8%). Dissection occurred during the postpartum period in 10 cases (62.5%), the third trimester in 4 (25.0%), labor in 1 (6.2%), and the second trimester in 1 (6.2%). The most common underlying diseases were: Marfan syndrome (25.0%), Loeys‐Dietz syndrome (6.2%), hypertension (6.2%), and Takayasu aortitis (6.2%). Stanford type A aortic dissection was associated with maternal death during both pregnancy and the postpartum period. However, deceased patients showed lower rates of pre‐diagnosed connective tissue disease than did survivors. Conclusions The mortality rate of aortic dissection that occurred during pregnancy or postnatal periods was more than 50%. Aortic dissection occurred more frequently in the postnatal period than during pregnancy, and less frequently in women previously diagnosed with connective tissue disease than in women without any medical history of aortic disorders. If symptoms suggestive of aortic dissection, such as severe back pain, are observed, even after the end of pregnancy, exhaustive diagnostic examinations should be carried out.

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Arrhythmia risk and β-blocker therapy in pregnant women with long QT syndrome

Abstract: 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.

Cited by 50 publications

References 21 publications

Arrhythmic risk during pregnancy and postpartum in patients with long QT syndrome

Arrhythmic risk during pregnancy and postpartum in patients with long QT syndrome

Intrauterine Growth Retardation in Pregnant Women with Long QT Syndrome Treated with Beta-Receptor Blockers

Aortic dissection during pregnancy and puerperium: A Japanese nationwide survey

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