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Lee MJ, Monteil DC, Spooner MT. Peripartum management of patient with long QT3 after successful implantable cardioverter defibrillator device discharge resulting in device failure: a case report. Eur Heart J Case Rep. 2021;5(12):ytab487doi: 10.1093/ehjcr/ytab487.
Lee, M. J., Monteil, D. C., & Spooner, M. T. (2021). Peripartum management of patient with long QT3 after successful implantable cardioverter defibrillator device discharge resulting in device failure: a case report. European heart journal. Case reports, 5(12), ytab487. https://doi.org/10.1093/ehjcr/ytab487
Lee, Melissa J, et al. "Peripartum management of patient with long QT3 after successful implantable cardioverter defibrillator device discharge resulting in device failure: a case report." European heart journal. Case reports vol. 5,12 (2021): ytab487. doi: https://doi.org/10.1093/ehjcr/ytab487
Lee MJ, Monteil DC, Spooner MT. Peripartum management of patient with long QT3 after successful implantable cardioverter defibrillator device discharge resulting in device failure: a case report. Eur Heart J Case Rep. 2021 Nov 30;5(12):ytab487. doi: 10.1093/ehjcr/ytab487. eCollection 2021 Dec. PMID: 34926986; PMCID: PMC8672658.
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Eur Heart J Case Rep. 2021 Nov 30;5(12):ytab487. doi: 10.1093/ehjcr/ytab487. eCollection 2021 Dec.

Peripartum management of patient with long QT3 after successful implantable cardioverter defibrillator device discharge resulting in device failure: a case report.

European heart journal. Case reports

Melissa J Lee, Danielle C Monteil, Michael T Spooner

Affiliations

  1. Department of Internal Medicine, Naval Medical Center Portsmouth, 620 John Paul Jones Cir, Portsmouth, VA 23708, USA.
  2. Department of Neurodevelopmental Pediatrics, Naval Medical Center Portsmouth, 620 John Paul Jones Cir, Portsmouth, VA 23708, USA.
  3. Department of Cardiology, Naval Medical Center Portsmouth, 620 John Paul Jones Cir, Portsmouth, VA 23708, USA.

PMID: 34926986 PMCID: PMC8672658 DOI: 10.1093/ehjcr/ytab487

Abstract

BACKGROUND: Long QT3 syndrome type 3 (LQT3) is a gain of function mutation of the SCN5A gene that is inherited in an autosomal dominant fashion. Long QT3 syndrome type 3 results in an increase in arrhythmic events during rest, sleep, and bradycardia by extending the QT interval and inducing Torsades de pointes and sudden cardiac death. Attempting to block the sodium channel with Class I anti-arrhythmics or blocking adrenergic tone with beta-blockers especially in women has shown to be beneficial. There have been few large-scale studies on treating patients with LQT3 due to its lethality and underreported number of cases. Specifically, the safety and efficacy of pharmacologic treatment in pregnant LQT3 patients are unknown.

CASE SUMMARY: This case demonstrates the safe use of Mexiletine and Propranolol in a 3rd-trimester pregnant LQT3 patient after a presumed ventricular arrhythmia and device-lead electrical short from therapy rendered her implantable cardioverter defibrillator inoperable in a VVI mode (venticular demand pacing). With appropriate medications, the patient was safely monitored through the remainder of her pregnancy and safely delivered at 36 weeks of pregnancy a healthy baby girl. The daughter, heterozygous for LQT3, showed no evidence of intrauterine growth restriction or other side effects from the medications.

DISCUSSION: There are many variants of the SCN5A gene mutations that can lead to different phenotypes and not all mutations are responsive to the same medications. In this case, Mexiletine and Propranolol, both of which have only recently shown to benefit certain variants or LQT3 respectively, were safely started during the 3rd trimester of pregnancy without harming the foetus.

© The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.

Keywords: ICD discharge; Life-Vest; Long QT3 syndrome; Mexiletine; Peripartum management; case report

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