Mothers with long QT syndrome are at increased risk for fetal death: findings from a multicenter international study

Cuneo, Bettina F.; Kaizer, Alexander M.; Clur, S. A.; Swan, Heikki; Herberg, Ulrike; Winbo, Annika; Rydberg, Annika; Haugaa, Kristina H.; Etheridge, Susan P.; Ackerman, Michael J.; Dagradi, Federica; Killen, Stacy A.S.; Wacker-Gußmann, Annette; Benson, D. Woodrow; Wilde, Arthur A.M.; Pan, Zhaoxing; Lam, Aimee; Spazzolini, Carla; Horigome, Hitoshi; Schwartz, Peter J.

doi:10.1016/j.ajog.2019.09.004

Cited by 37 publications

(30 citation statements)

References 35 publications

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“…Mothers with LQTS have an eight-fold increased risk for stillbirth and a two-fold higher risk for miscarriages as compared to the general population [ 4 ]. More stringent follow-up of LQTS women during pregnancy might therefore be necessary, particularly when severe and sustained sinus bradycardia (defined as fetal heart rate ≤ 3rd percentile for gestational age) is observed [ 18 ].…”

Section: Recommendations For Lqts Patients During Pregnancy Delivery and Postpartummentioning

confidence: 99%

Arrhythmierisiko während der Schwangerschaft und nach der Geburt bei Patientinnen mit Long-QT-Syndrom

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%

Arrhythmierisiko während der Schwangerschaft und nach der Geburt bei Patientinnen mit Long-QT-Syndrom

Asatryan

Rieder

Castiglione

et al. 2021

Herzschr Elektrophys

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“…Preestablished diabetes and hypertension are one of the most common comorbidities within pregnant women which might impact not only on the conditions for pregnant woman but also on the offspring. Indeed, previous studies have also observed how hypertension might increase risk of miscarriage [ 39 ], as well as diabetes [ 40 ] and cardiovascular diseases [ 41 , 42 ]. As a mechanism on this increased risk, it has been postulated a placental vascular pathology which might be enhanced with increasing maternal age [ 43 ].…”

Section: Discussionmentioning

confidence: 99%

A Validation Study on the Frequency and Natural History of Miscarriages Using the Spanish Primary Care Database BIFAP

et al. 2021

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(1) Background: There is a major gap of knowledge towards the natural history of miscarriages in electronic medical records. We aimed to calculate the frequency of miscarriages using data from BIFAP database. (2) Methods: We identified all pregnancy losses and carried out a multistep validation exercise. Potential cases with positive predictive values (PPV) of miscarriage confirmation <85% or those confirming other pregnancy loss were excluded. Kaplan–Meier figures and incidence rates (IRs) of miscarriage with 95% confidence intervals (CIs) expressed by 1000 person-weeks were calculated. Stratifying analysis by age, specific high-risk groups, and drug exposure within the pre-pregnancy period were performed restricted to women with recording last menstrual period (LMP). (3) Results: Women with confirmed miscarriage (N = 18,070), tended to be older, with higher frequency of comorbidities and drug utilization. Restricting to women with LPM recorded, IR of miscarriage was 10.89 (CI 95% 10.68–11.10) per 1000 women-weeks, with a median follow-up of 10 weeks (IQR: 8–12). The IR according to age was: 2.71 (CI 95% 2.59–2.84) in those aged <30 years compared to 9.11 (CI 95% 8.55–9.70) in women aged ≥40 years. Advanced maternal age (Hazard Ratio (HR, 95% confidence interval) CI 95%: 3.34 (3.08–3.62)), use of antihypertensives (1.49 (1.21–1.84), and use of drugs classified as D or X during pregnancy (1.17 (1.07–1.29)) showed to be positive predictors associated with increased risk of miscarriages. (4) Conclusion: BIFAP database can be used to identify women suffering from miscarriages, which will serve to further study risk factors associated with miscarriages with special attention to drug utilization.

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“…Pregnant iLQTS women seem to be at an increased risk of placental or myometrial dysfunction, which might result in stillbirth, miscarriage, preterm delivery, and intrauterine growth retardation (IUGR). Stillbirths are 8 times more frequently reported in iLQTS compared to the normal pregnant population (4 vs. approximately 0.5%) and miscarriages are twice more frequent (16 vs. 8%) [6]. One reason for intrauterine fetal death might be the risk of iLQTS and bradycardia in the fetus [7], as in 2/3 of newborns from iLQTS mothers/fathers, an iLQTS genotype can be detected [6].…”

Section: Introductionmentioning

confidence: 99%

“…Stillbirths are 8 times more frequently reported in iLQTS compared to the normal pregnant population (4 vs. approximately 0.5%) and miscarriages are twice more frequent (16 vs. 8%) [6]. One reason for intrauterine fetal death might be the risk of iLQTS and bradycardia in the fetus [7], as in 2/3 of newborns from iLQTS mothers/fathers, an iLQTS genotype can be detected [6]. Additionally, the birth weight for newborns of iLQTS mothers seems to be lower if adjusted for gestational age compared to those of iLQTS fathers [6].…”

Section: Introductionmentioning

confidence: 99%

“…One reason for intrauterine fetal death might be the risk of iLQTS and bradycardia in the fetus [7], as in 2/3 of newborns from iLQTS mothers/fathers, an iLQTS genotype can be detected [6]. Additionally, the birth weight for newborns of iLQTS mothers seems to be lower if adjusted for gestational age compared to those of iLQTS fathers [6]. It is important to highlight that pregnant iLQTS women themselves are at an increased risk for cardiac events (CE) postpartum, particularly in case of a LQTS type 2 (LQTS2) [8-11].…”

Section: Introductionmentioning

confidence: 99%

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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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Mothers with long QT syndrome are at increased risk for fetal death: findings from a multicenter international study

Cited by 37 publications

References 35 publications

Arrhythmierisiko während der Schwangerschaft und nach der Geburt bei Patientinnen mit Long-QT-Syndrom

Arrhythmierisiko während der Schwangerschaft und nach der Geburt bei Patientinnen mit Long-QT-Syndrom

A Validation Study on the Frequency and Natural History of Miscarriages Using the Spanish Primary Care Database BIFAP

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

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