Embed Size (px)
Citation preview
Pregnancy & Neonatal Management Guide for Long QT Syndrome (LQTS) TYPES 1 & 2
SEPTEMBER 2018
BC Inherited Arrhythmia Program (BCIAP) and Cardiac Obstetrics (COB) Clinic
BC Inherited Arrhythmia Program (BCIAP) and Cardiac Obstetrics (COB) Clinic
2 Definition of ‘High Risk’
2 Potential Triggers of LQTS Cardiac Events
3 Initial Visit
4 Ongoing Pregnancy
5 Labour & Delivery
6 The Neonate
7 Postpartum
8 Breast Feeding
9 Contact Information
10 References
11 Contributors
Pregnancy & Neonatal Management Guide for Long QT Syndrome (LQTS) TypeS 1 & 2
SepTember 2018
Pregnancy & Neonatal Guide for Long QT Syndrome (LQTS) Types 1 & 2 • September 2018 2
BC Inherited Arrhythmia Program (BCIAP) and Cardiac Obstetrics (COB) Clinic
‘High risk’ LQTS patients*
Any LQTS patient meeting one or more of the following criteria is designated ‘high risk’ throughout this document:
• RestingQTc>500ms(intheabsenceofreversibleQTprolongingfactors)
• Previouscardiacarrest• ImplantableCardioverterDefibrillator(ICD)
in place• Previously documentedhighburdenof
non-sustained ventricular tachycardia or ventricular ectopy
potential Triggers of LQTS Cardiac events**† QT-Prolonging DrugsItisimportantforLQTSpatientstoavoid taking QT-prolonging drugs. Some of these medications are commonly used during pregnancy, delivery, and postpartum1,2 such as certain anesthetics, certain antibiotics,oxytocin,domperidoneforlactation.
• Visitwww.crediblemeds.orgordownloadtheCredibleMeds Mobile App for the full list of drugs to avoid.
Sudden Auditory ArousalNote that sudden loud noises may trigger cardiac events, particularly in LQTS type 2 associated with mutations in the KCNH2 gene.
† Only triggers relevant to this document are listed. For expanded information on LQTS triggers, see Schwartz PJ and Ackerman MJ.3
Pregnancy & Neonatal Guide for Long QT Syndrome (LQTS) Types 1 & 2 • September 2018 3
BC Inherited Arrhythmia Program (BCIAP) and Cardiac Obstetrics (COB) Clinic
Genetic Counselling Given hereditary LQTS is usually autosomal dominant,whenoneparentisaffectedthereisa50%chanceofrecurrenceineachpregnancy.InrarecaseswherebothparentsareaffectedwithLQTS,therisktoeachchildis75%.Ifnotalreadydone,affectedindividualscanbereferredtotheBC Inherited Arrhythmia Program (BCIAP) for genetic counselling and consideration of genetic testing.
pregnancy management plan ForwomenaffectedbyLQTS,pre-conception/earlypregnancycounsellingshouldbeprovidedandamanagement plan for the pregnancy and postpartum periodestablished.• Assessriskstatusandeffectivenessofcurrent
management(includingbeta-blockermedication—seebelow).
Beta-blockadeThe most common pharmacologic treatment for LQTSisbeta-blockermedicationtoreducetheriskof cardiac events and sudden cardiac death.3,4. • Dosageandchoiceofbeta-blockershouldbe
individualizedbasedonclinicalstatus(heartrate,evidenceofsymptoms,tolerance,etc).Thegoalis to minimize risk of life-threatening arrhythmia andmaximizemedicationadherence,witharestingheartrate<100bpm.
• Exercisestresstestingmaybehelpfulinassessingadequacyofbeta-blockade.
• ThecardiologistsandobstetriciansassociatedwiththeBCIAPandCardiacObstetrics(COB)clinicmost often use nadolol5,6or bisoprolol7,8 to treat pregnantwomenwithLQTS.Nadololshouldbeused preferentially in high risk* women.
– Metoprolol is not advised as it is not as effective for LQTS.9
*See definition, page 2
• Beta-blockersareunlikelytoincreasetheriskofcongenitalanomaliesinthefetusabovethebackgroundrate.4,10-12 However, it is recommended thatallwomentake0.4mg(400micrograms)offolicacideveryday,beginningatleastthreemonthsbeforeconceptionandthroughoutpregnancy to reduce the chance of congenital anomalies in the fetus.13
refer to Specialty ClinicsRefer all pregnant women to the BCIAP, who will optimize management and provide genetic counselling. High risk* women should also be referred to the Cardiac Obstetrics (COB) Clinic at St.Paul’sHospitalinVancouver.• Initiatereferral as early as possible to facilitate
bookingandcoordinationofappointment.Pleasesenddatingscanalongwithreferral,ifavailable.
• BCIAPreferralform:www.cardiacbc.ca/Documents/BCIAP%20Vancouver%20referral%20form.pdf
• COBClinicreferralform: www.heartcentre.ca/professionals/referrals
• Themulti-disciplinaryCOBteamwilldevelopanindividualizedcardiac-obstetricalcaredeliveryplan for the high risk patient, including advice on beta-blockermanagement/titration,managementofsymptoms,andlabouranddeliveryplanning.Thecareplanwillbecommunicatedtotheprimary health care provider and delivery team.
Initial Visit (preconception or first prenatal visit)
Pregnancy & Neonatal Guide for Long QT Syndrome (LQTS) Types 1 & 2 • September 2018 4
BC Inherited Arrhythmia Program (BCIAP) and Cardiac Obstetrics (COB) Clinic
maternal Assessment• Recordmaternal heart rate, blood pressure, and
beta-blocker dosage and type on the prenatal recordateveryprenatalvisit,toaidwithdosage/titration decisions and communication with specialists when needed.
Beta-blocker Titration• Beta-blockerdosagemayneedtobeincreased
through the latter stages of pregnancy due to increasingbloodvolumeanddrugexcretion;however,cautionshouldbeexercisedbecauseside effects with higher doses may lead to non-adherence.
• Beta-blockersideeffectsincludeposturalhypotension, fatigue, depression and worsening of reactive airways disease. There is clear overlap of these side effects with symptoms reported during pregnancy,soconsultationwithbothcardiacandobstetricexpertsmaybewarrantedtointerpretsymptoms and adjust medications.
Fetal evaluationThefollowingissuggested,andcanbeperformedlocally: • Routinedetailedultrasoundscanat18to20weeks • Repeatultrasoundsat28 to 32 weeks and
36weeks gestation. – Ifconcernwithfetalgrowthisidentifiedat
any stage, a follow-up scan is recommended in two weeks’ time, as well as referral to local obstetrician(ordiscussionwithCOBclinicfor those high risk* patientsbeingfollowedbyCOBclinic).
• InsomecasesofhereditaryLQTS,thefetusmaypresentwithseverebradycardiaorotherarrhythmias.18Ifpersistentfetalbradycardiaor other fetal arrhythmia is noted, referral to Obstetrics/MaternalFetalMedicineisindicated.
Pre-labour Anesthesia Consultation• Initiatereferraltoanesthesiologist/anesthetistto
planforsafeanalgesiaandanesthesiainlabouranddelivery(alsoseeDrakeEetal1).
Ongoing pregnancy
Although the majority of women with LQTS do notexperiencecardiaceventsduringpregnancy,14,15 beta-blockers should be continued throughout pregnancy for prevention of cardiac events.
Increased risk for fetal intrauterine growth restriction (IUGR)hasbeenreportedwithmaternalbeta-blockeruse,16butitisunclearwhetherthisisaresultofunderlyingmaternalhealthconditionspredisposingtoIUGR(forexample,hypertensionormaternalheartdisease).Althoughfewstudieshaveassessedwomenwithprimaryarrhythmiaonbeta-blockertherapy,onestudysupportsanassociatedriskforIUGR,17andamorerecentstudyalsosuggestslowerbirthweightandearlierdeliveryofinfantsofwomenwithLQTSbeingtreatedwithbeta-blockers.4
*See definition, page 2
Pregnancy & Neonatal Guide for Long QT Syndrome (LQTS) Types 1 & 2 • September 2018 5
BC Inherited Arrhythmia Program (BCIAP) and Cardiac Obstetrics (COB) Clinic
• Home deliveries are not advised.• Ensure resuscitation equipment including an
externaldefibrillatorisavailableinornearbythedelivery suite.
• Pain managementshouldbereadilyavailable,andplannedearlyepiduralshouldbeconsidered.
• Nurse in a calm, quiet environment.**• Althoughsecond stage pushing could increase the
heartrate,itisgenerallythoughttobesafewithadequatebeta-blockade.
• Decisionsregardingepidural and assisted deliveryshouldbemadebasedonobstetricalindication;theseinterventionsarerarelyindicatedfor LQTS status.1
Avoid circumstances that could increase the risk forventriculararrhythmias,suchas:• Electrolyte imbalance
– Checkserum K+ and Mg2+ levels on all patients at the time of admission, and manage accordingly.
• Excessive bleeding, whichisconcerningbecauseof the associated tachycardia, while hypotension limitstheabilitytogivebeta-blockers.– Rapidrestorationofbloodvolumeshouldbe
considered in the setting of a hemorrhage in bothhighandlowriskLQTScases.
Labour & Delivery
(Circumstances to Avoid – Continued)• QT prolonging drugs and certain anesthetics**1,2
– OxytocinisonthelistofdrugstoavoidinLQTS. There is no known increased risk with routineIMinjection;however,prolongationoftheQTchasbeenreportedwithIVinfusion.Inthecaseofanoxytocininfusion,thepatient’sstatusshouldbemonitoredcarefully(preferablyone-to-onenursing),sincethecombinationofhemorrhageandanoxytocininfusion could increase the risk for cardiac events.Inapatientthatrequireshigherdosesofoxytocin,intermittentECGsmaybeconsidered to monitor the QTc. AQTc>500msshouldbediscussedwithanElectrophysiologist.Ifconsultisneeded,pleasecontacttheon-callcardiologist/ElectrophysiologistavailablelocallyorcontactSt.Paul’sHospital(604-682-2344)andasktospeaktotheon-callElectrophysiologist. IVbeta-blockersmaybeconsidered,howeverit is important to note that intrapartum IVbeta-blockersmayincreasetheriskofhypoglycemiaintheneonate(seepage6).
Careprovidersshouldbeawarethatmost deliveries occur without eventandcanusuallybecarriedoutatlocalhospitals.Withadequatebetablockade,themaximumheartrateshouldbeblunted,andsafedeliverywithoutinstrumentation(barringothermaternalorfetalindications)shouldbeexpected.
Lower fetal heart ratesarenormalinbabiesofmothersonbeta-blockers,withnormalheartratefluctuationat a lower heart rate range, which does not normally indicate fetal distress.
High risk*womenwillhavebeenassessedearlierinpregnancyattheBCIAPandCOBclinictodetermineoptimal delivery management and location.19,20
**See definition, page 2
Pregnancy & Neonatal Guide for Long QT Syndrome (LQTS) Types 1 & 2 • September 2018 6
BC Inherited Arrhythmia Program (BCIAP) and Cardiac Obstetrics (COB) Clinic
For those infants exposed to maternal beta-blockers in‑utero:• Observefordrowsiness,hypotensionand
hypoglycemia.• Screen neonates for hypoglycemia as per the
CanadianPaediatricSocietyguidelinesfornewbornsatriskforhypoglycaemia.22
• Motherandbabyshouldstayinhospital(preferablytogetherinthemother-babysuite) 24–36hoursafterdelivery.
For all infants with a mother or father with LQTS, ECGs should be performed at day one and three weeks of life andfaxed(high-resolution)tothepediatricheartrhythmspecialiston-callatBCChildren’sHospitalforreview:• Complete‘Request for Neonatal ECG Review’fax
sheetavailableat www.cardiacbc.ca/our-services/programs/bc-inherited-arrhythmia-program
and fax to 604-875-3463.• Along with fax, send e-mail tobothConnieEns
([email protected]),andKaylaYard([email protected])withsubjectline‘Incoming Neonatal ECG Review’toalerttoincomingfax.
The Neonate
Genetic testing for those infants in families with a knownmutationshouldbeofferedthroughareferraltotheBCIAPgeneticcounsellors.
Neonateswhowereexposedtobeta-blockersinuteromaybeat-riskforpostnatalsymptomsofbeta-blockadeand hypoglycemia.12,21
Pregnancy & Neonatal Guide for Long QT Syndrome (LQTS) Types 1 & 2 • September 2018 7
BC Inherited Arrhythmia Program (BCIAP) and Cardiac Obstetrics (COB) Clinic
maternal Assessment• Toensureadherencetobeta-blockertherapy
postpartum, maternal assessment with review of symptoms and heart rate is recommended at 1 week postpartum and monthly thereafter until 9 months postpartum.
• Arrange exercise stress testing for all women at 3–6monthspostpartum to assess adequacy ofbeta-blockadeandassistwithbeta-blockertitration(ifneeded).Beta-blockersaretypicallytitratedbasedonchangeinpeakexerciseheartrate(target15–20%reductiononexercisetest).– Stresstestcanbearrangedlocallyinmost
cases. Please contact the BCIAP if further information or guidance is needed.
– For high risk* womenfollowedbytheCOBclinic,theCOBclinicwilladviseonexercisetestingandbeta-blockerdosage.
postpartum
Beta-blocker TitrationForthosepatientswhosebeta-blockerdosewasincreasedduringpregnancy,adecreasemaybeneeded in the postpartum period. • Optimaltimingofthedownwardtitrationwill
varybetweenwomen,butgenerallystartsaround3–6months postpartum.
• Womenshouldultimatelybereturnedtotheirpre-pregnancy optimal therapy and dosing.
• Forhigh risk* womenfollowedbytheCOBclinic,theCOBclinicwilladviseonoptimalbeta-blockertitration.
WomenwithLQTShaveanincreasedriskforcardiacevents,includingsuddencardiacdeath,inthefirstnine months following delivery.14,15Itisespeciallyimportantforwomentocontinuetakingbeta-blockersthroughout the postpartum period.
*See definition, page 2
Pregnancy & Neonatal Guide for Long QT Syndrome (LQTS) Types 1 & 2 • September 2018 8
BC Inherited Arrhythmia Program (BCIAP) and Cardiac Obstetrics (COB) Clinic
• Noneofthebeta-blockerscommonlyusedforLQTSbytheBCIAParecontraindicatedinbreastfeeding;however,exposedinfantsshouldbemonitoredforfeaturesofbeta-blockade.Insomecases,extrapublichealthnursevisitsmightbeconsidered.
• Choiceofmaternalbeta-blockermusttakeintoaccountboththestabilityofthemotherandtheriskofbeta-blockadeintheinfant.Althoughtheremaybeexcretionofthebeta-blockerintobreastmilk,changingthetypeofmaternalbeta-blockerafterdeliverybecauseoftheoreticalrisk is generally not advocated due to the risk of destabilizingthemother.
breastfeeding
• Domperidone, commonly used to stimulate lactation, is on the list of QT-prolonging drugs† and is contraindicated in LQTS patients.
Beta-blockersasagroupareexcretedinbreastmilkandthereispotentialforbeta-blockadeinnursinginfants.However,fewadverseeffectshaveactuallybeenreportedinnursinginfants.
†See potential triggers, page 2
Pregnancy & Neonatal Guide for Long QT Syndrome (LQTS) Types 1 & 2 • September 2018 9
BC Inherited Arrhythmia Program (BCIAP) and Cardiac Obstetrics (COB) Clinic
CardiacObstetricsClinic:St.Paul’sHospitalRoom5051,1081BurrardStreetVancouver,BCV6Z1Y6PHONE:604-806-8520FAX:604-806-8800EMAIL:[email protected]/services/cardiac-obstetrics
BCInheritedArrhythmiaProgram:www.cardiacbc.ca/our-services/programs/bc-inherited-arrhythmia-program
Adultpatients–Vancouversite:St.Paul’sHospital211-1033DavieSt.VancouverBCV5N1E1PHONE:(604)682-2344ext.66765FAX:(604)806-9474
Adult Patients – Victoria site:DepartmentofMedicalGeneticsVictoriaGeneralHospital1 Hospital WayVictoria,BCV8Z6R5PHONE:(250)727-4461FAX:(250)727-4295
PediatricBCIAPsite:HeartCentre–BCChildren’sHospital1F41,4480OakStVancouver,BCV6H3V4PHONE: (604)875-2295FAX:(604)875-3463
Contact Information Motherisk Helpline (informationaboutbeta-blockerexposureduringpregnancy&breastfeeding)PHONE:1-877-439-2744;416-813-6780http://www.motherisk.org/
The Canadian SADS Foundation (patient-friendlyinformation&supportgroup)PHONE:(905)[email protected]://sads.ca
Pregnancy & Neonatal Guide for Long QT Syndrome (LQTS) Types 1 & 2 • September 2018 10
BC Inherited Arrhythmia Program (BCIAP) and Cardiac Obstetrics (COB) Clinic
1. DrakeEetal.Briefreview:anestheticimplicationsoflongQTsyndromeinpregnancy.CanJAnesthesia2007;54(7):561–572.
2. MartillottiGetal.LongQTsyndromeinpregnancy:Arevaginaldeliveryanduseofoxytocinpermitted?:A case report. J Obstet Gynaecol Can2012;34(11):1073–1076.
3. SchwartzPJandAckermanMJ.ThelongQTsyndrome: a transatlantic clinical approach to diagnosis and therapy. Eur Heart J2013;34:3109-16.
4. IhibashiKetal.Arrhythmiariskandβ-blockertherapy in pregnant women with long QT syndrome. Heart2017;103:1374-1379.
5. Abu-ZeitoneAetal.Efficacyofdifferentbeta-blockersin the treatment of long QT syndrome. J Am Coll Cardiol 2014;64(13):1352-1358.
6. AckermanMJetal.Beta-blockertherapyforlongQT syndrome and catecholaminergic polymorphic ventriculartachycardia:Areallbeta-blockersequivalent?Heart Rhythm2017;14(1):e41-e44.
7. SteinbergCetal.Experiencewithbisoprololinlong-QT1 and long-QT2 syndrome. J Interv Card Electrophysiol 2016;47:163-170.
8. FazioGetal.RoleofbisoprololinpatientswithlongQT syndrome. Ann Noninvasive Electrocardiol 2013;18(5):467-470.
9. ChockalingamPetal.Notallbeta-blockersareequalin the management of long QT syndrome types 1 and 2: Higher recurrence of events under metoprolol. J Am Coll Cardiol2012;60:2092-2099.
10. DuanLetal.β-Blockerexposureinpregnancyandrisk of fetal cardiac anomalies. JAMA Int Med 2017;177(6):885-887.
11. YakoobMYetal.Theriskofcongenitalmalformationsassociatedwithexposuretoβ-Blockersearly in pregnancy: A meta-analysis. Hypertension 2013;62(2):375-381.
12. DavisRLetal.Risksofcongenitalmalformationsandperinataleventsamonginfantsexposedtocalciumchannelandbeta-blockersduringpregnancy.Pharmacoepidemiol Drug Saf 2011;20(2):138-145.
references 13. WilsonDetal.Pre-conceptionfolicacidand
multivitamin supplementation for the primary and secondarypreventionofneuraltubedefectsandotherfolic acid-sensitive congenital anomalies. J Obstet Gynaecol Can2015;37(6):534–549.
14. SethRetal.LongQTsyndromeandpregnancy.J Am Coll Cardiol2007;49(10):1092–1098.
15. RashbaEJetal.Influenceofpregnancyontheriskfor cardiac events in patients with hereditary long QT syndrome. Circulation1998;97(5):451-456.
16. TanakaKetal.Beta-blockersandfetalgrowthrestriction in pregnant women with cardiovascular disease. Circ J2016;80:2221-2226.
17. ErsbøllASetal.Treatmentwithoralbeta-blockersduringpregnancycomplicatedbymaternalheartdisease increases the risk of fetal growth restriction. BJOG2014;121(5):618–626.
18. IshikawaSetal.FetalpresentationoflongQTsyndrome – evaluation of prenatal risk factors: A systematic review. Fetal Diagn Ther2013;33:1-7.
19. Pregnancyoutcomeandmanagementofwomenwithanimplantablecardioverterdefibrillator:asinglecentreexperience.Europace2012;14:1740-1745.
20. Bouleetal.Pregnancyinwomenwithanimplantablecardioverter-defibrillator:isitsafe?Europace2014;16:1587-1594.
21. BatemanBTetal.Latepregnancyβblockerexposureandrisksofneonatalhypoglycemiaandbradycardia.Pediatrics 2016;138(3):1-8.
22. AzizKandDanceyP(CanadianPaediatricSocietyFetusandNewbornCommittee).Screeningguidelinesfornewbornsatriskforlowbloodglucose.Paediatric Child Health2004;9(10):723-729.
Additionalreading:JacksonHet al. Long QT syndrome. CMAJ2011;183(11):
1272-1275.
Pregnancy & Neonatal Guide for Long QT Syndrome (LQTS) Types 1 & 2 • September 2018 11
BC Inherited Arrhythmia Program (BCIAP) and Cardiac Obstetrics (COB) Clinic
• LauraArbourMDMScMScFRCPCFCCMG,DepartmentofMedicalGenetics,UniversityofBritishColumbiaandIslandHealthAuthority.
• KirstenBartels,MSc,CCGC,ProgramCoordinator,BCInheritedArrhythmiaProgram,St.Paul’sHospital.
• HayleyBosMDMPHFRCSC,DivisionofFetalandMaternalMedicine,DepartmentofObstetricsandGynecology,UniversityofBritishColumbia,andIslandHealth Authority.
• ConnieEnsRN,Children’sHeartCentre,BCChildren’sHospital.
• JasmineGrewalMDFRCPC,DivisionofCardiology,DepartmentofMedicine,UniversityofBritishColumbiaandSt.Paul’sHospital.
• JulieHathawayMScCGCCCGC,St.Paul’sHospital(CurrentlyBlueprintGenetics).
• MarlaKeissMDFRCPC,DivisionofCardiology,DepartmentofMedicine,UniversityofBritishColumbiaandSt.Paul’sHospital.
Contributors (Listed alphabetically)
• AndrewDKrahnMDFRCPCFHRS,DivisionofCardiology,DepartmentofMedicine,UniversityofBritishColumbia,andSt.Paul’sHospital.
• SarahMcIntosh‡MScCCGC,DepartmentofMedicalGenetics,UniversityofBritishColumbia.
• ValerieRychelMDFRCSC,DivisionofGeneralObstetricsandGynecology,DepartmentofObstetricsandGynecology,UniversityofBritishColumbiaandSt.Paul’sHospital.
• ShubhayanSanatani,MDFRCPCCCDSFHRS,DivisionofCardiology,DepartmentofPediatricsUniversityofBritishColumbia,andtheBCChildren’sHospital.
• ElizabethSherwinMDFRCPC,DivisionofCardiology,DepartmentofPediatrics,UniversityofBritishColumbia(CurrentlyatChildren’sNationalHealthCentre,Washington,DC).
• DannaSpears,MDFRCPCD.ABIM,DivisionofCardiology–Electrophysiology,UniversityHealthNetwork, Toronto General Hospital.
Forquestions,commentspleasecontactDr.LauraArbour([email protected])orBCIAPCoordinatorat604-682-2344ext66765
‡Lead writer
![National Standards for Bereavement Care Following Pregnancy Loss and Perinatal Death' (Presentation at Maternity and Neonatal Network, April 2015) [MNN 13]](https://img.pdfslide.us/doc/110x75/58e4df6f1a28abf5048b68c7/national-standards-for-bereavement-care-following-pregnancy-loss-and.jpg)
