screening, diagnosek en ‘advances in fetal monitoring' 2018/Foetale car… · Monique Haak...

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MoniqueHaak

Foetale cardiologie screening, diagnos0ek en ‘advancesinfetalmonitoring'

1 13-Sep-16

DetectievanCHD:-adequateopvang

-mindermorbiditeitenmortaliteit

-gelegenheidaanvullendediagnostiek

-keuzeopties

-mindertrauma

Waaromwillenwescreenen?

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2007….entoenkwamdeSEO

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nationaal protocol uniforme opleidingseisen hart: alleen het 4 kamerbeeld 2012: outflow tracts en 3VV

–Johnny Appleseed

“Type a quote here.”

2002-2006 2007-2011

Category Total Prenataldetection% Total Prenataldetection%

Septaldefects 272 37.1 230 50.4

Valvularanomalies,biventricular 65 20.0 65 32.3

Venousreturnanomalies 23 4.3 27 11.1

Aorticarchanomalies 117 12.0 91 29.7

Conotruncalanomalies 267 26.6 229 59.8

HRHS 22 50.0 18 66.7

HLHS 85 54.1 82 97.6

Otheruniventricular 83 57.8 78 94.9

Complexwithatrialisomerism 31 64.5 31 93.5

Miscellaneous 66 72.7 63 74.6

Total 1031 36.2 914 59.7(87)

IsolatedCHD 632 23.3 537 44.1(53)

2002-2006 2007-2011

Category Total Prenataldetection% Total Prenataldetection%

Septaldefects 272 37.1 50.4

Valvularanomalies,biventricular 65 20.0 65 32.3

Venousreturnanomalies 23 4.3 27 11.1

Aorticarchanomalies 117 12.0 91 29.7

Conotruncalanomalies 267 26.6 229 59.8

HRHS 22 50.0 18 66.7

HLHS 85 54.1 82 97.6

Otheruniventricular 83 57.8 78 94.9

Complexwithatrialisomerism 31 64.5 31 93.5

Miscellaneous 66 72.7 63 74.6

Total 1031 36.2 914 59.7(87)

IsolatedCHD 632 23.3 537 44.1(53)

2002-2006 2007-2011

Heartdefect Total Prenataldetection(%) Total Prenataldetection(%) Difference(95%CI) p

VSD 167 28.7 136 39.0 10.3(-0.4-21.0) 0.060

AVSD 105 50.5 94 67.0 16.5(3.0-30.0) 0.018

PVstenosis 25 8.0 26 19.2 11.2(-7.3-29.7) 0.244

Aovalvestenosis 23 8.7 26 26.9 18.2(-2.4-38.8) 0.100

AVdysplasia/Ebstein* 17 47.1 13 69.2 17.6(-12.4-56.6) 0.367

TAPVR 22 9.1 20 10.0 0.9(-16.9-18.7) 0.639

Coarctation 86 8.1 70 25.7 17.6(5.9-29.3) 0.003

TetralogyofFallot 63 22.2 48 41.7 19.5(2.2-36.8) 0.028

DORV(Fallot-type) 35 48.6 41 82.9 34.3(14.1-54.5) 0.001

S-TGA 63 14.3 43 44.2 29.9(12.5-47.3) 0.001

TGAcomplex 34 26.5 32 53.1 26.6(3.8-49.4) 0.027

Truncusarteriosus 19 31.6 27 85.2 53.6(28.5-78.7) 0.000

PA–VSD 33 24.2 21 61.9 37.7(12.3-63.1) 0.006

TA 20 55.0 17 88.2 33.2(6.0-60.4) 0.028

UnbalancedAVSD 18 77.8 28 92.9 15.1(-6.3-36.5) 0.138

recenteregetallen

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Toekomstinscreening

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-ongeveer110nwepatientenperjaar-groeivan10%/jaar-multidiciplinaireaanpak-zowelgrotealskleinezaken-vankaryogram->QF->array->WES

-vastprotocol-relatiefveelsecond/thirdopinions

2018……foetalehartpoliLUMC

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-geenklassieklinkshypoplastischhartsyndroom-zeercomplexehartafwijking;univentriculairmetrechtsgelegenLV

-echter:ernstigeAVkleplekkageinuniventriculairecirculatie

Counseling

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additioneleafwijkingen:retrognatiegeringeIUGR

-(nog)geenuitlegoverdetailsuniventriculairecirculatiechirurgie-aandachtvoorfoetusalsgeheel-eerstsyndromaleoorzakenuitsluiten

multidiciplinaireaanpak

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clinicaldecisionmaking

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waar is de collector?

hij moet er zijn!zie ‘m niet..

bloed moet toch ergens heen….

ik zie ‘m echt niet

kan het ook afwezig zijn?

hmmmm…

mijn settings zijn optimaal

-detectieisolatedTAPVRlaag-daaromweinigprenataleervaring….

-hypoplasiecollectorvein->neonataleemergency->oplftvan1-3dgnoud

-counselling:onzekerheden(mogelijkeacutelevensbedreigendesituatie?)

-inleiding;CATkamerklaar,ECMO,OK,kinderhart-chirurgstaanklaar

dilemma

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-ongecompliceerdevaginalepartus,APGAR9-5-?-navelstrengpH7.28

-NSklem->cyanose-intubatie,pH6.8na30minuten-ECMOviathoracotomie-catherisatie->ernstigehypoplasiepulmonaryvenousreturn

partus

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prenatale echo is beter dan we kunnen geloven……

vanwitteravennaardailyproblems

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Historisch

-‘eyeballing'-M-Mode-fractionalshortening-IntracardialeDoppler-Dopplerpre-cardinalveins

-MPI-Ejectionfraction/CO(3D-US)

Assessmentofcardiacfunction

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MyocardialPerformanceIndex

(ICT+IRT)/ET

29Cruz-Martinez,FetalDiagnosisTher2012

-combinesdiastolicandsystolicfunction

-dependentofpre-andafterload

-RVisdominantventricleinfetallife

-difficulttomeasure(MPI)• inter-andintra-observerbias

Flowrelatedcardiacfunctiontools

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-learningcurve(>60examinations)-widerange

MyocardialPerformanceIndex

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Cruz-Martinez,FetalDiagnosisTher2012

Wallmotionanalysistechniques

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tissuedopplerspeckletracking

2DWallMotionTracking

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Speckletracking

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Originallydevelopedforadultheartassessment

Differenceswithadultheart:sizeandmovement

BalancebetweenFrameRateandSpatialResolutionFRbetween0,7and0,9xBPMpreferred(thusFR>100/sec)

Althoughbasicallyangleindependent,needsgooddefinitionofspeckle-imagequality-apical4CV(longitudinalstrain)-vendordependentanalysis-comparisondifficult

SpeckleTracking

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cTDI

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cTDI

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shapeofthesignaldependentofposition

largeROI-globalheart

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angledependentchangewithmovement

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Howtodealwiththiscurve?

ContractionandRelaxationTime

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Startofcontraction->tostartofrelaxation≠ejectiontime!

Startofstretching->toendofstretching≠fillingtime!

TDIvsspeckletracking

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TDI Speckletracking

FR high moderate

detects velocity displacement

angledependancy +(-) -(+)

strength timingofevents strainandvolume

weakness spatialresolution temporalresolution

handling easyandfast easybutoffline

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C-TDICTDIandWMT

C-TDIC-TDIenWMT

WMT

kortetermijn:monitoringopresultaat(FNR-DR-revisies-FPR)loskomenvan4lossevlakken->naarsweepen3Ddenken!

middellangetermijn:geneticacardiacfunction

langetermijnautomaticimageanalysisMRI

Toekomst

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Dankvooruwaandachtwww.fetalheartacademy.nl

dankaan:KatinkaTeunissen,PhebeAdamavanScheltema,SheilaEverwijn,AmbervanNisselnooij,ChristinevanVelzen,JasmijnOosterhuis,FennaJansen&CAHALgroep

m.c.haak@lumc.nl