Pregnancy in catecholaminergic polymorphic ventricular tachycardia: therapeutic optimization and multidisciplinary care are key to success

Schwangerschaft bei katecholaminerger polymorpher ventrikulärer Tachykardie: Therapieoptimierung und multidisziplinäre Betreuung sind der Schlüssel zum Erfolg

Abstract

Women of child-bearing age comprise a large proportion of the patients followed by inherited arrhythmia clinics. Despite being a rare and dangerous diagnosis, cardiac and obstetric care providers should know that catecholaminergic polymorphic ventricular tachycardia (CPVT) is not a contraindication to pregnancy. In fact, pregnancy was not associated with an increased risk of CPVT-associated arrhythmias in a recent large cohort study, and most guideline-based anti-arrhythmic drug treatments are life-saving and carry a low risk of teratogenesis. In principle, the potential for CPVT destabilization may be more likely to occur after anti-arrhythmic drugs are decreased or stopped during pregnancy, when an implantable cardioverter defibrillator (ICD) shock exacerbates catecholamine release, or if adrenaline surges are triggered by labor and delivery. Therefore, all pregnant women should be followed by a cardio-obstetrics team with extensive knowledge of CPVT diagnosis, as well as arrhythmia risk stratification fand management. This multidisciplinary care should begin preconception and involve counseling on preimplantation genetic testing, choosing safe and effective anti-arrhythmic drugs, stopping contraindicated medications, optimal programming of ICDs, and planning for the brief hyper-adrenergic period of labor and delivery. The latest data on pregnancy in CPVT is reviewed here and the optimal care for this rare and complex patient population outlined.

Zusammenfassung

Frauen im gebärfähigen Alter machen einen großen Teil der Patienten aus, die in Ambulanzen für angeborene Arrhythmien behandelt werden. Obwohl es sich um eine seltene und potenziell gefährliche Erkrankung handelt, sollten Kardiologen und Geburtshelfer wissen, dass eine katecholaminerge polymorphe ventrikuläre Tachykardie (CPVT) keine Kontraindikation für eine Schwangerschaft darstellt. In einer neuen großen Kohortenstudie war eine Schwangerschaft nicht mit einem erhöhten Risiko für Arrhythmien bei CPVT assoziiert, die meisten in den Leitlinien empfohlenen Antiarrhythmika sind lebensrettend und nur mit einem geringen teratogenen Risiko assoziiert. Prinzipiell kann eine Destabilisierung bei CPVT mit höherer Wahrscheinlichkeit auftreten, wenn Antiarrhythmika während der Schwangerschaft in der Dosis reduziert oder abgesetzt werden, wenn die Schockabgabe durch einen implantierbaren Kardioverter-Defibrillator die Katecholaminfreisetzung steigert oder wenn bei Wehentätigkeit oder Geburt ein Adrenalinanstieg ausgelöst wird. Daher sollten alle Schwangeren mit CPVT von einem kardiologisch-geburtshilflichen Team versorgt werden, das über weitreichende Kenntnisse in Diagnostik, Risikostratifizierung und Management assoziierter Arrhythmien verfügt. Die multidisziplinäre Betreuung sollte bereits vor der Schwangerschaft beginnen und Folgendes beinhalten: Beratung zu genetischer Testung, Auswahl sicherer und effektiver Antiarrhythmika, Absetzen von Medikamenten, die während der Schwangerschaft kontraindiziert sind, optimale Programmierung implantierbarer Kardioverter-Defibrillatoren und Planung für die kurze hyperadrenerge Periode von Wehen und Geburt. In der vorliegenden Übersicht werden die aktuellen Daten zur Schwangerschaft bei CPVT zusammengefasst und ein Entwurf für die optimale Betreuung von Patientinnen mit dieser seltenen, aber komplexen Erkrankung vorgestellt.

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Funding

Dr. Krahn receives support from the Sauder Family and Heart and Stroke Foundation Chair in Cardiology (Vancouver, BC), the Paul Brunes Chair in Heart Rhythm Disorders (Vancouver, BC), and the Paul Albrechtson Foundation (Winnipeg, MB). The study was supported by the Heart in Rhythm Organization (Dr. Krahn, Principal Investigator), which receives support from the Canadian Institute of Health Research (RN380020 – 406814).

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Correspondence to Andrew D. Krahn MD, FRCPC, FHRS.

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T.M. Roston, J. Grewal, and A.D. Krahn declare that they have no competing interests.

For this article no studies with human participants or animals were performed by any of the authors.

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Roston, T.M., Grewal, J. & Krahn, A.D. Pregnancy in catecholaminergic polymorphic ventricular tachycardia: therapeutic optimization and multidisciplinary care are key to success. Herzschr Elektrophys (2021). https://doi.org/10.1007/s00399-021-00755-6

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Keywords

  • CPVT
  • Maternal/fetal risk
  • Sudden cardiac arrest
  • Inherited arrhythmia
  • Ryanodine receptor

Schlüsselwörter

  • CPVT
  • Mütterliches/fetales Risiko
  • Plötzlicher Herztod
  • Angeborene Arrhythmie
  • Ryanodinrezeptor