QualityoflifeinACHD M3CAcademy

November22nd,2018

PascalAMEDROMD,PhD

M3CRegionalCenter,MontpellierUniversityHospital,FrancePhyMedExp,INSERMU1046,CNRSUMR9214,UniversityofMontpellier,France

Qualityoflife(QoL)

• Generalwell-beingofindividualsandsocieties• Lifesatisfaction

Health-relatedqualityoflife(HR-QoL)

• It’spartofaperson’soverallQoLthatrepresentsthefunctionaleffectofanillnessanditsconsequenttherapyuponapatient,asperceived– bythepatient(self-reportedHR-QoL)– and/orbyhis/herfamily(proxy-reportedHR-QoL)

UsesofHR-QoLmeasurement

• Moreinclinicalresearchthaninmedicalpractice

• Recommendedindrugtrials(EMA)• Mayimprovepatient-doctorrelationship• Reliabletoassesstheeffectivenessandrelativesmeritsofdifferenttreatments

• Healthserviceevaluation• Policymaking

Health-relatedqualityoflife(HR-QoL)

• HR-QoL

• NonHR-QoL

• QoL

HR-QoLinstruments

• Shouldincludeatleast4dimensions:– Physicalfunctioning– Psychologicalfunctioning– Socialfunctioning– Generalhealthperception

• Genericquestionnaires– SF-36https://www.rand.org/health/surveys_tools/mos/36-item-

short-form/survey-instrument.html• Specificquestionnaires– CAMPHOR

RascatiK.EssentialsofPharmacoeconomics.WoltersKluwer/Lippincott,Williams,&Wilkins.PhiladelphiaPA;2013.

HR-QoLbelongsto«patient-relatedoutcomes»(PRO’s)

Chenetal.ProcAmThorSoc2008

ComponentsofQoLinACHD

QoL

NYHA

Drugs

Sexuality

SpiritualityReligion

WealthAgeGender

Family

CHDseverity

VO2max

ACHD=CHF

Amedroetal.CardiolYoung2017

ComponentsofQoLinACHD

QoL

NYHA

Drugs

Sexuality

SpiritualityReligion

WealthAgeGender

Family

CHDseverity

VO2max

CPETinACHDfollow-up

Baumgartneretal.EurHeartJ2010Amedroetal.IntJCardiol2017Marcadetetal.ACVD2018

ComponentsofQoLinACHD

QoL

NYHA

Drugs

Sexuality

SpiritualityReligion

WealthAgeGender

Family

CHDseverity

VO2maxUnclear

Amedroetal.PedCardiol2015Jacksonetal.IntJCardiol2016

ComponentsofQoLinACHD

QoL

NYHA

Drugs

Sexuality

SpiritualityReligion

WealthAgeGender

Family

CHDseverity

VO2max

≥1cardiacdrug

Jacksonetal.IntJCardiol2016

ComponentsofQoLinACHD

QoL

NYHA

Drugs

Sexuality

SpiritualityReligion

WealthAgeGender

Family

CHDseverity

VO2max

28%sexualdysfunctioninACHD

Huangetal.CurrCardiolRep.2018

ComponentsofQoLinACHD

QoL

NYHA

Drugs

Sexuality

SpiritualityReligion

WealthAgeGender

Family

CHDseverity

VO2max 4000ACHDpatients

15countries

Moonsetal.IntJCardiol2019

ComponentsofQoLinACHD

QoL

NYHA

Drugs

Sexuality

SpiritualityReligion

WealthAge

Gender

Family

CHDseverity

VO2max

Income≤$30,000

Jacksonetal.IntJCardiol2016

ComponentsofQoLinACHD

QoL

NYHA

Drugs

Sexuality

SpiritualityReligion

WealthGender

Family

CHDseverity

VO2max

YesinnonsevereCHD

(male<female)

Amedroetal.PedCardiol2015

ComponentsofQoLinACHD

QoL

NYHA

Drugs

Sexuality

SpiritualityReligion

WealthGender

Family

CHDseverity

VO2max

MuchlessinsevereCHD

(male=female)

Amedroetal.CardiolYoung2017

ComponentsofQoLinACHD

QoL

NYHA

Drugs

Sexuality

SpiritualityReligion

WealthAge

Family

CHDseverity

VO2max

Children>Teens>Youngadults>

Adults

Amedroetal.PedCardiol2015

ComponentsofQoLinACHD

QoL

NYHA

Drugs

Sexuality

SpiritualityReligion

WealthAgeGender

Family

CHDseverity

VO2maxSiblings,anxiety,depression,mourning

Amedroetal.CardiolYoung2017Eatonetal.CardiolYoung2017Yu-mietal.QualLifeOutcomes2018

IsHR-QoLapredictorofmortality?

• InPAH-CHDpatients:decreaseinSF36afterinitiationofPAH-specifictherapyisapredictorofmortality

Blocketal.NethHeartJ2015

Mostimportantdeterminantsofsurvival:-HRdecreaseSF-36(PCS)=3.4-HRNYHA=4.5

YES!

Survival

NYHA

QoL

DoesHR-QoLcorrelatewithNYHAinACHDusinggenericinstruments?

Amedroetal.CardiolYoung2016

YES!

DoesHR-QoLcorrelatewithNYHAinACHDusingspecificinstruments?

Amedroetal.CardiolYoung2016

YES!

QoLandphysicalcapacity

ConceptofWOOD(WHO1980)

AfunctionaltestduringexercisebetterreflectsQoLrelatedtoa

chronicdiseasethananyevaluationatrest

P.H. Wood, Appreciating the consequences of disease: the international classification ofimpairments,disabilities,andhandicaps,WHOChronicle.34(1980)

Survival

VO2max

QoL

DoesQoLcorrelatewithphysicalcapacityinCHD?

• CorrelationbetweenQoLandVO2max– Inchronicheartfailure1,2– InACHDpatients3,4– InpediatricCHDpatients5

• CPETrecommendedinACHDfollow-up6– VO2max– VE/VCO2slope

• AndnowinpediatricCHDfollow-up7,8

YES!

1Myersetal.AmHeartJ20082Mancinietal.Circulation19913Hageretal.Heart20054Giardinietal.AnnThoracSurg20085Amedroetal.IntJCardiol20166Baumgartneretal.EurHeartJ20107Amedroetal.Heart20178Marcadetetal.ArchCardiovascDis2018

QoLandphysicalactivity

• RegularphysicalactivityinACHDimprovesQoL1– Inthephysicaldimension– Andinthementalwell-being

• RegardlessACHDseverity,surgery,age,betablockers,pacemaker,SpO2

• CardiacrehabilitationinACHDpatientsshouldbepromoted2– safe– improvesVO2max

– probablyimprovesQoL31Hageretal.Heart20172Amedroetal.PressMed20173Amedroetal.IntJCardiol2019

QoLandCHDseverity

• DependsontheclassificationofCHDseverity• Mostlyyes–Whenusingsimpleclassification(Bethesda)– AndforthephysicaldimensionofHR-QoL

• Nodatawithexhaustiveclassifications– ACC-CHD

Remainsunclear

ACC-CHDClassification

Amedroetal.Heart2017

ICD-10classification

Kempnyetal.EurHeartJ2012

Survival

CHDseverity

QoL

DoesQoLcorrelatewithCHDseverity?

• PhysicaldimensionofHR-QoLimpairedinsevereCHD(Fontan,Mustard,Senning)1

• PhysicaldimensionofHR-QoLmoreimpairedinsimpleTGAthancomplexTGV2

1Kamphuisetal.Heart20022Culbertetal.Circulation2003

MostlyYESinthephysicaldimension!

DoesQoLcorrelatewithCHDseverity?

Amedroetal.CardiolYoung2017

Notalwaysinthementaldimension

InACHD

DoesQoLcorrelatewithCHDseverity?

Amedroetal.PedCardiol2015

Notalwaysinthementaldimension

InchildrenwithCHD

GoodQoLlevelsinsevereCHD• Medicaladvances– Newtechniques(catheterization)

– Betterpatient’sglobalhealthcare

– Patienttherapeuticeducationprograms(transition)1

• AustralianACHDpatients:meanscore902

• CopinginCHDpatients2-4– Disabilityparadox– Responseshift– Senseofcoherence

1Sableetal.Circulation20112Eatonetal.CardiolYoung20173Moonsetal.EurJCardiovascNurs.20134Amedroetal.PedCardiol2015

ImpairedQoLinnon-severeCHD

Amedroetal.PedCardiol2015

ImpairedQoLinnon-severeCHD

• Lackofautonomy1

• Patientsonthe«sideline»fromchildhood1

• Parentalbarrierstophysicalactivity2

• MusculardeconditioningandimpairedVO2maxeveninsimpleCHD(ASD,VSD)– InACHD3– Inteenagers4

1Amedroetal.PedCardiol20152Moolaetal.QualHealthRes20113Kempnyetal.EurHeartJ20124Amedroetal.Heart2018

QoLinter-countryvariationinACHD

Moonsetal.IntJCardiol2018

PredictorsofQoLinACHDworldwide

• Independentpatient-relatedfactors:– Functionalclass– Unemploymentstatus

• Otherfactors:– Standardofliving(grossdomesticproductspercapitaandhumandevelopmentindex)

– Healthcaresystemcharacteristics(totalhealthexpenditurepercapitaandoverallhealthsystemperformance)

Moonsetal.IntJCardiol2018

Remainingquestions&projects

• PredictorsofHR-QoLduringprenataldiagnosis• QoLandtype/severityofCHD– Anatomy– Physiology– CombinedCHDlesions– Invasivetreatment:surgery,cardiaccatheterization

• CompositescoresincludingQoLassessmentinclinicaltrials

• UndeterminedcomponantsofHR-QoLinCHD• RandomizedtrialswithQoLasprimaryoutcome– Qualirehab– Transition

Whatdoesmyqualityoflifecorrelatewith?