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Chapter04:HeartFailure
RelatedQuestions
Previous:CoronaryArteryDisease

HeartFailure

PathophysiologyofHeartFailure
Heartfailureisacomplexclinicalsyndromeinwhichcardiacoutputisinsufficientformeetingthe
demandsofthebody,causingsymptomsofexertionaldyspneaandfatigue.Approximatelyonehalfof
patientswithheartfailurehaveleftventricularsystolicdysfunction,orheartfailurewithreduced
ejectionfraction(HFrEF)theotherhalfhavenormalsystolicfunction,orheartfailurewithpreserved
ejectionfraction(HFpEF).Regardlessofwhetherleftventricularejectionfractionisreducedor
preserved,symptomsofexertionaldyspneaandfatiguearethesame,andassessmentofejection
fractionisrequiredtodifferentiatebetweenthesetwoentities.
CommoncausesofHFrEFincludehypertension,coronaryarterydisease,myocarditis,certaindrugs
(forexample,doxorubicin,trastuzumab,cyclophosphamide),andtoxins,includingalcohol,cocaine,
amphetamines,cobalt,andlead.SystemicdiseasesthatmaycauseHFrEFincludehypoand
hyperthyroidism,HIVinfection,systemiclupuserythematosus,scleroderma,andneuromuscular
diseasessuchasDuchenneandBeckermusculardystrophy.AhighpercentageofHFrEFcasesare
idiopathic.ThemostcommoncausesofHFpEFarehypertensionandcoronaryarterydiseaseless
commoncausesincludeinfiltrativediseasessuchasamyloidosisandhemochromatosis.Ingeneral,
patientswithHFpEFtendtobeolder,heavierwomenwhohaveahistoryofhypertension,coronary
disease,anddiabetesmellitus.
Althoughthesymptomsofexertionaldyspneaandfatigueandhemodynamicabnormalitiesofreduced
strokevolumeandelevatedventricularfillingpressuresaresimilarbetweenHFpEFandHFrEF,there
aresignificantdifferencesinthepathophysiologybetweenthetwodiseaseprocesses.Inpatientswith
HFrEF,thecommondefectisanabnormalityofmyocardialcontraction.Reducedsystolicfunction
resultsinprogressiveventriculardilation.Incontrast,patientswithHFpEFhavesimilarsymptomsbut
normalsystoliccontractionandanabnormalityindiastolicrelaxation.Thisresultsinrestrictedfilling
andhighfillingpressures.Tomaintainanormalcardiacoutput(heartratestrokevolume),patients
withHFpEFtendtohaveahigherheartrate.Clinically,becausethesepatientshaveaverysmallleft
ventricularsize,theyareusuallymuchmoresensitivetovolumeloadingthanpatientswithHFrEF.

DiagnosisandEvaluationofHeartFailure
ClinicalEvaluation
ApproximatelyhalfofallheartfailurehospitaladmissionsresultfromHFpEF.Thereisnodifference
inmortalitybetweenpatientswithHFpEFandHFrEF.Forbothgroupsofpatients,thereisabouta
50%survivalrateat3yearsafterpresentingwithsymptomsofheartfailure.
Classicsymptomsofacuteheartfailureincludeexertionaldyspnea,paroxysmalnocturnaldyspnea,

orthopnea,andperipheraledema.Inpatientswithstablechronicheartfailure,symptomsaretypically
similartothoseofnewonsetheartfailurebutlessintense.Apatientmaynormallysleepontwoor
threepillowsowingtonocturnaldyspnea,butwithdecompensationwillsleeponmorepillowsor
movetosleepinginarecliner.Similarly,apatientatbaselinemaybecomeshortofbreathwalkingup
oneflightofstairs,butwithdecompensationmayhavedyspneaputtingonhisorherclothes.Patients
withpreviouslydiagnosedheartfailureoftendonotpresentwithclassicfindings.Thesepatientsmay
presentwithincreasedabdominalgirthratherthanperipheraledema,orwithnauseaandanorexia
causedbygutedemaratherthanwithexertionaldyspnea.Educatingpatientstoweighthemselves
dailyandreportchangesinweightorbaselinesymptomsmayreduceheartfailurehospitaladmissions
ifactedonquicklyenough.
Thefirststepintheevaluationofapatientwithsignsandsymptomsofheartfailureisathorough
historyandphysicalexamination,whichshouldbeperformedtoevaluateforpossiblecausesofheart
failureandassesscardiovascularriskfactors.Adetailedhistory,includingalcoholandillicitdruguse,
alternativetherapies,familyhistory,andanyhistoryofchemotherapy,shouldbeobtained.Features
thatincreasethelikelihoodofheartfailureincludethepresenceofparoxysmalnocturnaldyspnea(>2
foldlikelihood)andthepresenceofanS3(11timesgreaterlikelihood).Thelikelihoodofheartfailure
isdecreased50%bytheabsenceofdyspneaonexertionandbytheabsenceofcracklesonpulmonary
auscultation.ElevatedjugularvenouspressureandanS3areindependentlyassociatedwithadverse
outcomes,includingprogressionofheartfailure.

Diagnosis
RelatedQuestion
Question116
Inpatientswhopresentwithacutedyspneaofundeterminedetiology,Btypenatriureticpeptide
(BNP)levelscanbeusedtoquicklydifferentiatebetweendyspneasecondarytoheartfailure(elevated
BNP)anddyspnearelatedtopulmonarydisease(lowtonormalBNP).TheBreathingNotProperly
studyevaluatedpatientswhopresentedtotheemergencydepartmentwithdyspnea.Patientswhohad
heartfailurehadameanBNPlevelgreaterthan600pg/mL(600ng/L),whereasthosewith
noncardiaccausesofdyspneahadlevelsofapproximately50pg/mL(50ng/L).Patientswithahistory
ofleftventriculardysfunctionbutnotanacuteexacerbationhadaBNPlevelofapproximately200
pg/mL(200ng/L).BNPlevelsincreasewithageandworseningkidneyfunctionandarereducedin
patientswithanelevatedBMI.
BNPlevelsalonearenotdiagnosticforheartfailurebutshouldbeusedonlyasaninitialtesttoguide
thediagnosticevaluationinpatientswithdyspneaofuncertainetiology.BNPlevelsshouldnotbe
usedtofollowapatient'sclinicalcourse.ThereisnobenefittofollowingBNPlevelsduringa
hospitalizationtodetermineifdiuresishasbeenadequate.Additionally,routineuseofBNP
measurementinoutpatientswithheartfailureisnothelpfulfordeterminingifapatientisfluid
overloaded.
A12leadelectrocardiogram(ECG)shouldbeobtainedforallpatientswithheartfailure.AnECGcan
behelpfultoevaluateforpossiblemyocardialinfarction,tachyarrhythmia,orleftventricular
hypertrophy.Achestradiographshouldbeobtainedtoevaluateforconcomitantpulmonarydisease.
Chestradiographycanalsobehelpfulforidentifyingvascularcongestionindicatingvolumeoverload
orpleuraleffusions.
Theinitiallaboratoryassessmentofheartfailureshouldincludeelectrolytelevels,urinalysisand
kidneyfunction,glucoseandlipidlevels,liverchemistrytests,andthyroidstimulatinghormone
levels.Althoughthyroiddiseaseisanuncommoncauseofheartfailure,hypothyroidismand

hyperthyroidismarepotentiallyreversiblecausesofheartfailurewithappropriatetreatment.Coronary
diseasecausesapproximatelytwothirdsofcasesofheartfailure,andanacutecoronarysyndrome
shouldbesuspectedasacauseofnewheartfailureoranexacerbatingfactorinpatientswith
preexistingheartfailure.Ifanacutecoronarysyndromeissuspectedasprecipitatingheartfailure,
measurementoftroponinlevelsmaybeuseful.However,troponinlevelsareoccasionallymildly
elevatedinpatientswithanexacerbationofheartfailureowingtowallstressandsubendocardial
ischemiaorwithacutemyocarditis.Anelevatedtroponinleveldoesnotguidetherapyinthissetting
butcanbeusedasamarkerofmoreprogressiveheartfailureandworseprognosis.Routineevaluation
forunusualcausesofheartfailure,includinghemochromatosis,Wilsondisease,multiplemyeloma,
andmyocarditis,shouldnotbeperformed.Anevaluationforotherunusualcausesofheartfailure
shouldnotbeperformedroutinelyinallpatientsbutshouldonlybeperformedwhenthereare
suggestionsofspecificdiseasesbyhistoryorphysicalexamination.
Themostimportantdiagnostictestintheevaluationofheartfailureistransthoracicechocardiography.
Anechocardiogramwillgiveanassessmentofejectionfractionaswellasinformationaboutpossible
causes.Forexample,identificationofwallmotionabnormalitiesincreasessuspicionforcoronary
arterydiseaseandmyocardialischemia.Echocardiographyalsoallowsassessmentforaorticand
mitralvalvedisease,withthecaveatthatmitralregurgitationisoftencausedbytheremodeling
processofheartfailureandisthereforesecondarytotheheartfailureratherthantheprimarycause
(functionalmitralregurgitation).Additionally,theleftventricularenddiastolicdimensioncanbe
helpfulforevaluatingthechronicityofthediseaseprocessaswellasprognosis.Patientswithacute
heartfailuresyndromesandadilatedleftventriclelikelyhaveachronicdiseaseprocesswithdelayed
onsetorrecognitionofsymptoms.Asmallleftventricle(particularlywithoutwallthinning)is
associatedwithagreaterchanceofrecoveryofejectionfractioncomparedwithamarkedlydilated
leftventricle.Also,acombinationoffindingsonechocardiographymayprovideacluetothecauseof
heartfailure.Forexample,leftventricularhypertrophyandbiatrialenlargementinapatientwith
reducedejectionfractionsuggestsarestrictivecardiomyopathy.Theleftventricularhypertrophyof
HFpEFisusuallysecondarytohypertensionandshouldnotbeconfusedwiththeseverehypertrophy
characteristicofhypertrophiccardiomyopathy(seeMyocardialDisease).PatientswithHFpEFoften
havemildtomoderateleftventricularhypertrophy(<15mminanyregion).
Cardiacmagneticresonance(CMR)imagingisusedincreasinglyintheevaluationofpatientswith
heartfailure.CMRimagingcanbeusedtoassesswallmotionabnormalities,globalwallfunction,and
viability.Additionally,itcanbeusedtoassesstissueperfusion,tissueinjury(inflammationor
necrosis),fibrosis,infiltration(sarcoidoramyloid),orirondeposition.
Endomyocardialbiopsyisrarelyindicatedfortheevaluationofacuteheartfailure.Patientswith
progressiveheartfailureonmedicaltherapywhohavemalignantarrhythmiasshouldundergobiopsy
toevaluateforgiantcellmyocarditis.Biopsyisalsoreasonableforpatientswithnewonsetheart
failureunresponsivetostandardmedicaltherapy.Endomyocardialbiopsycanassistinthediagnosis
ofamyloidosisandhemochromatosis,whicharediffuseprocessesamenabletodiagnosisbybiopsy
techniquessarcoidosis,ontheotherhand,canbequitepatchyandislesslikelytobediscoveredon
endomyocardialbiopsy.
Thereisnoroleforroutinerightheartcatheterizationforthediagnosisormanagementofpatients
withheartfailure.Inpatientsadmittedtothehospitalwithheartfailure,routinerightheart
catheterizationhasnotbeendemonstratedtodecreaseeithermortalityorrehospitalizationrates
comparedwithusualcare.Rightheartmonitoringcanbehelpfulinpatientswithadvancedheart
failurewhoarerefractorytomedicaltherapy.Symptomatichypotensionandworseningkidney
functionmaybesuggestiveoflowcardiacoutputbutcouldalsobecausedbyinfectionorprogression
ofdisease.Arightheartcatheterizationdirectlymeasuringcardiacoutputandfillingpressurescan
guidetherapytowardimprovinghemodynamics(higherstrokevolumeandlowerfillingpressures)
withinotropicagentsand/ormoreaggressivediuresisifthefillingpressuresarehigh.Rightheart
catheterizationisalsoindicatedinpatientsbeingevaluatedforhearttransplantation.Pulmonary

hypertensionisariskfactorforpooroutcomesfollowinghearttransplantationbecausetheright
ventricleofthedonorheartisnotaccustomedtopumpingagainsthighpulmonarypressuresandmay
fail.

EvaluationforIschemia
RelatedQuestion
Question47
Althoughcoronaryarterydiseaseisthemostcommoncauseofheartfailure,owingtoexpenseand
radiationexposure,theroutineinvestigationforcoronarydiseasebystresstestingorcardiac
catheterizationorotherimagingmodalities(suchasCMRimaging,PET,orCT)isnolonger
consideredpartoftheroutineevaluationofallpatientswithnewlydiagnosedheartfailure.Cardiac
catheterizationshouldbeperformedinpatientspresentingwithanginaorsignificantischemia.
Additionally,cardiaccatheterizationisrecommendedforpatientspresentingwithchestpainthatmay
ormaynotbeofcardiacoriginandthosewithpreviouslydiagnosedcoronaryarterydiseasewithout
chestpainiftheyareeligibleforrevascularization.Noninvasivestresstestingisreasonableinpatients
withahistoryofcoronaryarterydiseasetoevaluateforreversibleischemia,asrevascularizationcan
dramaticallyimproveleftventricularfunction.Additionally,patientswithmultipleriskfactorsfor
coronarydiseaseshouldundergononinvasivetestingtoevaluateforsignsofischemia.Ifstresstesting
identifiessignificantischemicmyocardium,coronaryangiographyshouldbeconsidered.

KeyPoints
Btypenatriureticpeptidelevelscanbeusefultodistinguishcardiacfromnoncardiaccausesof
dyspneaintheurgentcaresetting.
Inpatientswithheartfailure,withtheexceptionofthyroiddisease,anextensiveevaluationof
unusualcausesofheartfailureshouldnotbeperformedunlesstherearesuggestionsofspecific
diseasesbyhistoryorphysicalexamination.
Themostimportantdiagnostictestintheevaluationofheartfailureistransthoracic
echocardiography,whichallowsassessmentofleftventricularejectionfractionaswellas
informationregardingpotentialcauses,clinicalcourse,andprognosis.

MedicalTherapyforSystolicHeartFailure
RelatedQuestion
Question95
ThetreatmentofpatientswithHFrEF(systolicheartfailure)comprisestreatmentoftheacute
exacerbationfollowedbyinstitutionoflongtermtherapytodecreasemorbidityandmortalityand
improvesymptomsinpatientswithchronicheartfailure.Theinitialtherapyforpatientspresenting
withacuteheartfailureandvolumeoverloadisadiuretic.AnACEinhibitororangiotensinreceptor
blocker(ARB)shouldalsobestartedunlessthepatienthassymptomatichypotension.Oncetheacute
heartfailureepisodehasstabilized,allpatientsshouldbetreatedwithablocker.
Thelongtermtherapyofheartfailureisbasedonthepatient'sfunctionalstatusasmeasuredbyNew
YorkHeartAssociation(NYHA)functionalclass(Table15)andsignsandsymptomsofvolume
overload.InadditiontoACEinhibitorsandblockers,othertreatmentsforheartfailurethathave
beenshowntodecreasemortalityandfuturehospitalizationsincludealdosteroneantagonistsand,
specificallyforblackpatients,hydralazineisosorbidedinitrate(Table16).Severaladditional

medicationshavebeenshowntoimprovesymptomsbuthavenoeffectonmortality.

ACEInhibitorsandAngiotensinReceptorBlockers
RelatedQuestion
Question9
ACEinhibitorshavebeenshowntodecreasemortality,decreasesymptomsofheartfailure,improve
functionalcapacity,andimproveleftventricularejectionfraction.AllpatientswithHFrEFshouldbe
startedonanACEinhibitor.Thepatientshouldbestartedonalowdose,whichcanbeuptitratedas
toleratedbasedonbloodpressureandsymptoms.HigherdosesofACEinhibitorscomparedwith
lowerdoseshavebeenshowntodecreaseheartfailurehospitaladmissionsbutnotmortality.In
patientswithbaselinehypotension,itisimportanttoinitiateACEinhibitortherapy,butthedose
shouldnotbemaximizedpriortoinitiatingblockadebecausethecombinationofbothagentsis
superiortotherapywitheitheronealone.CautionshouldbeusedwheninitiatingACEinhibitor
therapyinpatientswithchronickidneydisease,andthepatient'skidneyfunctionshouldbemonitored
closely.However,thepresenceofkidneydysfunctionshouldnotbeconsideredacontraindicationto
theinitiationoftheseagentsrecentguidelinessuggestinitiatingtherapyinpatientswithaserum
creatininelevelbelow3.0mg/dL(265.2mol/L).
AcommonadverseeffectofACEinhibitorsisadry,nonproductivecough,whichoccursinupto
20%ofpatients.Forthesepatients,itisreasonabletoswitchtoanARBinstead.Lessinformation
regardingmortalityisavailableforARBs,sotheyshouldnotbeusedasfirstlinetherapy.Other
commonadverseeffectsofbothdrugsincludehyperkalemiaand,occasionally,worseningkidney
function.InpatientswithangioedemawhiletakingACEinhibitors,ARBsshouldnotbeusedasan
alternativebecausetherearereportsofangioedemaalsooccurringwiththeseagents.

Blockers
RelatedQuestion
Question54
BlockersshouldbestartedinallpatientswithHFrEFafteracutedecompensationistreatedandthe
patientishemodynamicallystable.Thesedrugsblocktheadverseeffectsofchronicneurohormonal
activationoncardiacfunction.Threeblockershavebeenshowntodecreasemortality,reduceheart
failuresymptoms,andimproveleftventricularejectionfractioninpatientswithHFrEF:metoprolol
succinate,carvedilol,andbisoprolol.Itisimportanttouseoneofthesethreeagentsbecausetheyare
theonlyonesthathaveademonstratedbenefitinpatientswithheartfailure.Otherblockers,
includingshortactingmetoprolol(metoprololtartrate),havenotshownsimilarbenefit.Somepatients
experienceincreasedfatigueonblockade,butthevastmajorityexperienceanimprovementinheart
failuresymptoms.

InitiatingandManagingACEInhibitorandBlockerTherapy
PatientswithacuteheartfailureandvolumeoverloadshouldinitiallybestartedonanACEinhibitor.
Typically,ashortactingagentsuchascaptoprilshouldbeusedindivideddailydosessothatifthe
patientexperiencessymptomatichypotension,theeffectwillbetransient.ACEinhibitorsshouldbe
titratedbasedonbloodpressureandthepresenceorabsenceofadverseeffects.Forpatientswithnew
onsetheartfailureandvolumeoverload,ablockershouldnotbeinitiateduntilthepatientis
euvolemicorclosetoeuvolemic.

IncontrasttoACEinhibitors,inwhichthedosecanberapidlytitratedupward,blockersshouldbe
startedataverylowdoseoncepatientsareeuvolemicbecausetheseagentshaveanegativeinotropic
effect.Insteadofuptitratingthedrugonadailybasis,titrationofablockershouldbeperformed
slowlyat1to2weekintervals,onanoutpatientbasis.Anumberofstudieshavedemonstrateda
doseresponseeffectwithblockers.Highdosescomparedwithlowdosesofblockershavebeen
showntobemorebeneficialforbothmortalityreductionandthereductionofheartfailuresymptoms.
Althoughpatientsareoftendischargedonlowdoses,theseagentsshouldbeuptitratedtothe
maximaltolerateddosesafterthepatienthasbeendischarged(Table17).Limitationstomaximalup
titrationincludesymptomatichypotensionandbradycardia.Oncetheheartrateisbelow60/min,the
currentdosecanbemaintained.AhistoryofCOPDisnotacontraindicationtoinitiatingblocker
therapy,andthereisnoevidencethatthenonselectiveblockersarenottoleratedinthesepatients.

Diuretics
Diureticsarethemainstayoftherapyforsymptomsofheartfailureassociatedwithvolumeoverload.
Toavoidhypovolemia,thelowestdoseofdiureticnecessaryshouldbeused.Loopdiureticsarethe
mostcommonlyusedagents.Inpatientswithrefractoryheartfailure,theadditionofathiazide
diureticisoccasionallyusedtoaugmenttheeffectsoftheloopdiuretic.Thereisnoadvantagetoa
continuousintravenousinfusionversusbolustherapyindecompensatedheartfailure.Adverseeffects
ofdiureticsincludehypokalemia,hypomagnesemia,andworseningkidneyfunction.Aselectrolyte
abnormalitiescanleadtomalignantarrhythmias,electrolytesshouldbefrequentlymeasuredin
patientsreceivinghighdoses.Additionally,patientsshouldbecounseledtorestricttheirsodiumand
fluidintake.

Digoxin
Digoxinhasbeenusedfordecadesforthetreatmentofheartfailure.Digoxinhasnotbeenshownto
reducemortalitybutdoesdecreasehospitalizationsforHFrEFincomparisonwithplacebo.Inshort
termtrials,digoxinhasbeenshowntoimproveheartfailuresymptoms,qualityoflife,andexercise
tolerance.Thewithdrawalofdigoxininpatientsisassociatedwithincreasingheartfailuresymptoms.
Therapywithdigoxinshouldbecloselyfollowed.Itisreasonabletocheckaserumdigoxinlevel
whenapatientisstable.Patientswithkidneyimpairment,lowbodymass,andolderagehavereduced
metabolismofdigoxinandcanquicklydevelopatoxiclevel.Itisimportanttocheckadigoxinlevel
inpatientswithworseningkidneyfunction.Retrospectiveanalyseshaveshownthatserumlevels
greaterthan1ng/mL(1.28nmol/L)areassociatedwithincreasedriskofmortality,mostcommonly
relatedtoarrhythmias.

AldosteroneAntagonists
Aldosteroneantagonists(spironolactone,eplerenone)havebeenstudiedinpatientswithheartfailure
andNYHAfunctionalclassIItoIVsymptomsandhavebeenshowntoreducemortalityand
morbidity.ForpatientswithclassIIsymptoms,thebenefithasbeenshownonlyinthosewitha
historyofpriorhospitalizationoranelevatedBNPlevel.Theprincipalsideeffectoftheseagentsis
hyperkalemia.Spironolactoneandeplerenonehavenotbeencomparedwithoneanother,butin
clinicaltrials,gynecomastiaoccursspecificallywithspironolactone.Becauseoftheriskofkidney
dysfunctionandhyperkalemia,thesedrugsshouldbeusedonlyinpatientswithaserumcreatinine
levelbelow2.5mg/dL(221mol/L)inmenorbelow2.0mg/dL(176.8mol/L)inwomen,andwith
aserumpotassiumlevelbelow5.0mEq/L(5.0mmol/L).Additionally,ifthepatientisonpotassium
supplementation,thisshouldbediscontinuedwhentherapyisinitiated.Electrolytesandkidney
functionshouldbechecked1weekafterinitiationoftherapyandbecloselymonitoredovertime.
Aldosteroneantagonistsshouldbeusedverycautiouslyinelderlypatients.Thesedrugsarenot
effectiveasdiureticsatthedosesusedinheartfailuretherapy(12.525mg/dforspironolactone,2550

mg/dforeplerenone).

IsosorbideDinitrateandHydralazine
RelatedQuestion
Question106
Thecombinationofisosorbidedinitrateandhydralazineisanalternativetherapyforpatientswith
heartfailurewhohavekidneydysfunctionthatlimitstherapywitheitherACEinhibitorsorARBs.In
thissetting,thiscombinationisusedforitsvasodilatingproperties.Morerecently,basedon
retrospectivedatafromearlierclinicaltrials,aclinicaltrialinblackpatientswithheartfailureand
reducedejectionfractionandNYHAclassIIIandIVsymptomswasperformedthatdemonstrateda
reductioninmortalitywithaspecificformulationofthiscombinationcomparedwithplacebo.There
wasahighincidenceofadverseeffects(primarilyperipheraledema,dizziness,gastrointestinal
symptoms,andheadaches)anddrugwithdrawal.Forblackpatients,studieshavedemonstrated
improvementsinqualityoflifeinadditiontoamortalitybenefit.Notethatthiscombinationwas
studiedasanadditionaltherapyforpatientsalreadyonanACEinhibitororanARBandablocker,
notasareplacementtherapy,andshouldonlybeinstitutedaftertheseagentshavebeenmaximized.

CalciumChannelBlockers
Becauseoftheirvasodilatingeffects,calciumchannelblockershavebeencloselystudiedfortheir
potentialroleinthemanagementofheartfailure.Unfortunately,thenondihydropyridinecalcium
channelblockers(forexample,diltiazemorverapamil)alsohavemyocardialdepressionactivityand
havebeendemonstratedtoeitherhavenobenefitorworseoutcomesinpatientswithheartfailure.
Patientswhohavebeentreatedforhypertensionwithdiltiazemorverapamilshouldhavethoseagents
discontinuedonceadiagnosisofheartfailurehasbeenmade.Thesecondgenerationdihydropyridine
calciumchannelblockers,suchasamlodipineandfelodipine,havebeenshowntobesafeinpatients
withheartfailure,butdonotreducemorbidityormortality.Forpatientswhoarestillhypertensiveon
highdosesofACEinhibitorsandblockers,aperipherallyactingdihydropyridinecalciumchannel
blockercanbeusedasanantihypertensiveagent.

KeyPoints
Initialtherapyforallpatientswithheartfailurewithreducedejectionfractionshouldincludean
ACEinhibitorthosewithvolumeoverloadshouldbegivenadiuretic,andoncetheacuteheart
failureepisodehasstabilized,allpatientsshouldbeplacedonablocker.
Blockersinthetreatmentofheartfailureshouldbestartedataverylowdoseanduptitrated
slowly,at1to2weekintervals.
AldosteroneantagonistsshouldbestartedinpatientswithNewYorkHeartAssociationclassII
toIVsymptomswithappropriatekidneyfunctionandapotassiumlevelbelow5.0mEq/L(5.0
mmol/L).
Theadditionofisosorbidedinitrateandhydralazinetostandardheartfailuretherapyis
associatedwithimprovementsinqualityoflifeandamortalitybenefitinblackpatients.

ManagementofHeartFailurewithPreservedEjectionFraction
RelatedQuestion
Question15

ACEinhibitors,ARBs,blockers,andaldosteroneantagonistshavebeenstudiedinpatientswith
HFpEF.Unfortunately,noneoftheseagentshavedemonstratedanyclinicalbenefitcomparedwith
placebo.Atthistime,nomedicationshavedemonstratedareductioninmortalityinthispatient
population.TherapyforHFpEFshouldinsteadbebasedontreatingthecausesandsymptomsofthe
heartfailure.HypertensionisacommoncauseofHFpEF,andaggressivecontrolofbloodpressureis
necessary.Additionally,controllingtachycardiacanbehelpfulinpatientswithatrialarrhythmias.
PatientswithHFpEFareoftenquitevolumesensitive,withasmalltherapeuticwindowbetween
hypovolemiaandhypervolemia.Judicioususeofdiureticstomaintaineuvolemiaisimportant.These
patientsshouldbeencouragedtomonitortheirweightclosely,assmalldifferencesinvolumecan
quicklycausevolumeoverloadandsubsequenthospitaladmissions.

DeviceTherapy
Suddencardiacdeathisthecauseofdeathinapproximately50%ofpatientswithheartfailure.The
onlyreliablepredictorofanarrhythmiceventisleftventricularejectionfraction.Forthisreason,
implantablecardioverterdefibrillators(ICDs)areusedforprimarypreventionofsuddencardiacdeath
inpatientswithheartfailureandlowejectionfraction.

ImplantableCardioverterDefibrillatorforPreventionofSuddenCardiacDeath
RelatedQuestion
Question76
Inpatientswithmildtomoderateheartfailuresymptomsandleftventricularejectionfractionless
thanorequalto35%,placementofanICDreducesmortalitycomparedwithmedicaltherapyor
placeboinpatientswithbothischemicandnonischemicetiologies(Table18).ICDsareindicatedfor
patientswithNYHAfunctionalclassIIandIIIsymptoms,ejectionfractionlessthanorequalto35%
onguidelinedirectedmedicaltherapy,andalifeexpectancyofatleast1year.Aspatientswithclass
IVsymptomshaveareducedlifeexpectancy,ICDsarenotindicatedinthispopulationexceptin
patientswhoareawaitingtransplantationorundergoplacementofamechanicalcirculatorydevice.
ICDsshouldonlybeplacedafterpatientsareonguidelinedirectedmedicaltherapy.Forpatientswith
recentonsetofheartfailurewhohaveareasonablechanceofrecoveryoffunction,oneshouldwait
upwardsof6monthswiththepatientonadequatemedicaltherapyandthenreassessventricular
functiontodetermineiftheejectionfractionisstilllessthan35%priortoimplantation.Specifically,
patientstreatedwithrevascularizationforcoronaryarterydiseasemayhaveimprovementinejection
fraction,whichshouldberemeasuredafterrevascularization.

CardiacResynchronizationTherapy
RelatedQuestion
Question56
Inapproximately30%ofpatients,heartfailureprogressionisaccompaniedbydyssynchrony
(dysfunctionalventricularelectromechanicalcoordination)manifestedbyprolongationoftheQRS
durationoraleftbundlebranchblock.Biventricularpacing,orcardiacresynchronizationtherapy
(CRT),involvespacingtherightandleftventriclessimultaneously.Inadditiontotheusualplacement
ofapacerleadintheapexoftherightventricle,anadditionalleadisplacedthroughthecoronary
sinusdownacoronaryveinonthelateralwalloftheleftventricle.Thissimultaneouspacinghasbeen

demonstratedtoincreaseejectionfraction,decreaseheartfailuresymptoms,andreducemortalityin
patientswithHFrEFandECGevidenceofdyssynchrony.The2013AmericanCollegeofCardiology
Foundation/AmericanHeartAssociationheartfailuremanagementguidelinemakesastrong
recommendationwithstrongsupportingevidenceforCRTtherapyinpatientswithanejection
fractionlessthanorequalto35%,NYHAfunctionalclassIIItoIVsymptomsonguidelinedirected
medicaltherapy,andleftbundlebranchblockwithQRSdurationgreaterthanorequalto150msec
(seeTable18).AstrongrecommendationwithweakerevidenceisprovidedforpatientswithNYHA
functionalclassIIsymptoms.
PatientswithNYHAclassIsymptomscausedbyischemiaandaleftbundlebranchblockwithaQRS
durationgreaterthan150msechaveshownsomebenefitwithCRTtherapy,buttheriskbenefitratio
ishighowingtoadverseeffectsofthetherapy,anditiscurrentlyaclassIIbrecommendation.
Adverseeffectsincludeinfectionatthesiteofthedevice,inappropriatefirings,andoccasional
tricuspidvalveregurgitation.

KeyPoints
AnimplantablecardioverterdefibrillatorisrecommendedforpatientswithNewYorkHeart
AssociationclassIIorIIIheartfailure,aleftventricularejectionfractionlessthanorequalto
35%aftertreatmentwithguidelinedirectedmedicaltherapy,andalifeexpectancyofatleast1
year.
CardiacresynchronizationtherapyisrecommendedforpatientswithNewYorkHeart
AssociationclassIItoIVheartfailure,aleftventricularejectionfractionlessthanorequalto
35%onguidelinedirectedmedicaltherapy,andaleftbundlebranchblockwithQRSduration
greaterthanorequalto150msec.

AssessmentofChronicHeartFailure
Patientswithchronicheartfailureshouldbeseriallyassessedforprogressionofdiseaseinthe
outpatientsetting.Ateachvisit,itisimportanttoassesscurrentsymptomsandfunctionalcapacity,
volumestatus,andtheadequacyofthemedicaltherapy(bothappropriatedosesandtheappropriate
medicationsasheartfailureprogresses).Ofequalorgreaterimportanceisrepeatedpatienteducation,
includingremindingpatientstotaketheirmedicationsasprescribed,measuretheirweightdaily,avoid
dietarysodium,watchtheirfluidintake,andexerciseregularly.Patientswhoappropriatelytaketheir
medicationsandavoidsodiumandexcessfluidintakecangreatlyimprovetheirfunctionalstatus.
Despitemultiplestudiesdemonstratingthebenefitofmedicaltherapiesinheartfailure,fewerthan
60%ofpatientsaredischargedfromthehospitalonACEinhibitorandblockertherapy.Itis
importanttoreviewmedicationsateveryvisittoensurethatpatientsareontheappropriatetherapy.

SerialBTypeNatriureticPeptideAssessment
SerialassessmentofBNPlevelsinpatientswithchronicheartfailurehavebeenevaluatedinanumber
ofstudies.AlthoughhigherBNPlevelsareassociatedwithincreasedmortality,changeinlevelinan
individualpatientdoesnotpredictprogressionofdisease.Additionally,thereisnoevidenceofbenefit
tousingBNPforseriallyfollowingpatientstoassessvolumestatusorfordoseadjustmentof
medications.

EchocardiographyinChronicHeartFailure
Echocardiographyshouldbeperformedinpatientswithsevereleftventriculardysfunctionafter
optimizationofmedicaltherapytodetermineiftheleftventricularejectionfractionhasimprovedto

above35%beforeconsiderationofICDimplantation.Forpatientswithchronicheartfailurewhoare
clinicallystable,echocardiographyrarelyprovidesdiagnosticbenefit,andobtainingannual
echocardiogramsisnotlikelytochangetherapyoroutcome.Forpatientshospitalizedwithacuteheart
failure,obtainingarepeatechocardiogramtoevaluateleftventricularfunctionorforworsening
valvularabnormalitiesisreasonable.Ifapatienthasprogressiveheartfailuresymptomsasan
outpatient,arepeatechocardiogramcanbehelpfultoevaluateforprogressivevalvularabnormalities,
newwallmotionabnormalities,oranincreaseinleftventricularsizethatmayaltertreatmentand
affectprognosis.

AssessingPrognosis
Multipleretrospectivestudieshavebeenperformedlookingatmethodstoevaluateprognosisin
patientswithheartfailure.Current1yearsurvivalratesforpatientsundergoinghearttransplantation
orplacementofaleftventricularassistdevicearebetween85%and90%.Patientswithahigherrisk
ofdeathshouldbeconsideredforthesetherapies.ImportantriskfactorsfordeathincludeNYHAclass
IVsymptoms,repeathospitalizations,hyponatremia(serumsodium<133mEq/L[133mmol/L]),
worseningkidneyfunction,higherdosesofdiuretics,intoleranceofACEinhibitorsorblockers,and
arrhythmiasresultinginICDfirings.Patientswithmultipleriskfactorsshouldbereferredtoaheart
failurecardiologistforfurtherevaluation.Additionally,occasionaldiscussionswithpatientsregarding
endoflifeissuesandtheirwishesforadvancedheartfailuretherapyshouldbeinitiatedwhilethe
patientisstillstable.Patients'advancedcareplansoftenchangeovertime.
Cardiopulmonaryexercisetestingisroutinelyperformedtoassessprognosisinpatientsbeing
evaluatedfortransplantation.Patientswithalowoxygenconsumption(peakO2consumption<14
mL/kg/min)orahighratioofventilationtocarbondioxideproduction(V
E/V
CO2>34)haveapoor
1yearprognosis.
TheSeattleHeartFailuremodel(www.SeattleHeartFailureModel.org)isanonlineprogramthatuses
clinicalcharacteristicstopredictoutcomesinpatientswithheartfailure.Thismodelcanbeusedto
helpassessprognosisbasedonclinicalcharacteristicsandcanbeusedtoguidepatientsastheyask
questionsabouttheirprognosis.

KeyPoints
Patientswithchronicheartfailureshouldbeseenregularlyforassessmentofclinicalstatusas
wellasongoingpatienteducationregardingtakingmedicationsasprescribed,measuringtheir
weightdaily,reducingdietarysodiumandavoidingexcessfluidintake,andexercising
regularly.
Inpatientswithchronicheartfailurewhoareclinicallystable,annualormorefrequentfollow
upechocardiographyrarelyprovidestherapeuticordiagnosticbenefitandisnotrecommended.

InpatientManagementofHeartFailure
AcuteDecompensatedHeartFailure
Patientswithheartfailureadmittedtothehospitalusuallyhavesymptomsofvolumeoverloadasthe
primaryconcern.Reasonstoadmitpatientsincludeprogressiveheartfailuresymptomswithdyspnea
atrest,aninabilitytorespondtooraldiuretics,recurrentICDfirings,symptomsofischemia,
worseningkidneyfunction,andsignsofpoorperfusion(suchascoolextremities,alowpulse
pressure,orpulsusalternans).Therapyisprimarilyfocusedondiuresis.Additionalevaluationshould
beperformedtodeterminethereasonsforthedecompensation,includingareviewofmedicationsand
whetherthepatientwastakinghisorhermedicationsproperly,anechocardiogramtolookfor

reversiblecausesofworseningfunction,and,ifappropriate,anevaluationforischemia.Generally,the
initialdoseofintravenousdiureticshouldbeatleastequivalenttothetotaldailyoraldose.Ifthe
patientdoesnotrespondappropriatelytothatdose,rapiduptitrationshouldbeperformedtoassistin
fluidremoval.Thepatient'susualoutpatientmedications(forexample,ACEinhibitor,blocker)
shouldbecontinuedunlessthepatientishypotensiveordemonstratessignsofpoorperfusion,in
whichcasedosereductionordiscontinuationofboththeACEinhibitorandblockershouldbe
considered.Inpatientswithsignsoflowoutputheartfailure(hypotension,worseningkidneyorliver
function,coolextremities),theblockershouldbediscontinued.
Patientsshouldbeadequatelydiuresedduringthehospitalization.Orthopneaandanelevatedcentral
venouspressurearesuggestiveofelevatedfillingpressures.Patientsoftenhavesymptomatic
improvementbeforetheyareeuvolemic,andstrivingtoachieveeuvolemiamayresultinareduction
inreadmissionrates.Volumestatuscanbedifficulttoassessandacarefulphysicalexaminationis
required.Patientsshouldbeassessedforsignsofvolumeoverloadeveniftheyhavereachedagoalor
dryweight,asthisisnotareliablemeasureofeuvolemia.Findingssupportiveofeuvolemiainclude
anestimatedcentralvenouspressurebelow10cmH2O,noorthopnea,absenceofperipheraledema,
andreduceddyspnea.Despitetheuseofhighdoseintravenousdiuretictherapyandimprovementin
volumestatusduringhospitalization,manypatientsaredischargedbeforeachievingeuvolemicor
decongestedstatus.Objectivemarkersofdecongestionincludeneturineoutput,weightloss,evidence
ofhemoconcentration(increaseinhematocritandhemoglobinlevels),andreductioninBNPlevel.
Thesemarkershavebeenassociatedwithlowerratesofrehospitalizationforheartfailureinshort
termfollowup.
Ultrafiltrationcanoccasionallybeusedinpatientsadmittedwithvolumeoverloadwhenroutine
diuresisfailsorpatientsareunresponsivetodiuretictherapy.Arecentrandomizedtrialof
ultrafiltrationversusintravenousdiureticsinpatientswithdecompensatedheartfailureandworsening
kidneyfunctionshowednodifferenceinweightlossbutworsenedkidneyfunctioninpatientstreated
withultrafiltration.
Worseningkidneyfunction,typicallydefinedasaserumcreatinineincreaseof0.3mg/dL(26.52
mol/L)orgreaterduringhospitalizationforheartfailure,isacommoncomplication,occurringinup
to25%ofpatients.Thiseventoftentriggerschangesinmedicaltherapy,includingreductionor
cessationofdiuretics,ACEinhibitorsorARBs,oraldosteroneantagonists.Retrospectivestudieshave
foundthatthiscomplicationisassociatedwithworsesurvivallongterm,butnotherapieshavebeen
showntopreventorreduceworseningkidneyfunctioninpatientswithheartfailure.
Vasopressinantagonistscanbeusedforthetreatmentofpatientswithhypervolemicoreuvolemic
hyponatremia.Theyarebeneficialforcorrectinghyponatremiabuthavenotdemonstratedany
mortalitybenefits.

CardiogenicShock
RelatedQuestions
Question23
Question89
Cardiogenicshockisdefinedbypersistent,symptomatichypotensionandendorgandysfunction.
Patientshaveacutekidneyfailure,evidenceofliverdysfunctionwithelevatedaminotransferase
levels,poorperipheralperfusionwithcoolextremities,anddecreasedmentalstatus.Cardiogenic
shockrequiresintensivetherapywithintravenousvasopressors.Patientswhoremaininshockdespite
intravenoustherapyandwithworseningorganfunctionshouldbeconsideredformechanicalsupport.
Itisimportanttoquicklyruleoutreversiblecausesinpatientswithcardiogenicshock.Reversible

causesincludeacutemyocardialinfarctionventricularseptalorfreewallruptureandacutevalvular
regurgitation,possiblyrelatedtopapillarymusclerupture,infection,orascendingaorticarch
aneurysmwithdissectionoftheaorticvalve.Bedsideechocardiographycanbehelpfulinidentifying
structuralcausesofcardiogenicshock.
Theinitialtherapyforcardiogenicshockincludesvasoactivemedicationstoincreasecardiacoutput
andraisebloodpressurethroughperipheralvasoconstriction(Table19).Patientswithcardiogenic
shocksecondarytoprogressiveheartfailurearegenerallygivenaninotropicagent,suchas
dobutamineormilrinone(clearedbythekidneys).Patientswithperipheralvasoconstriction(increased
systemicvascularresistance)oftenbenefitfromtheadditionofapurevasodilatorsuchassodium
nitroprusside.Placementofarightheartcathetercanbehelpfultoassessfillingpressures,cardiac
output,andsystemicvascularresistancetohelpchoosetheappropriatemedicalregimen.Althoughthe
routineplacementofarightheartcatheterforpatientsadmittedwithheartfailurehasnotbeenshown
toimproveoutcomes,itshouldbeconsideredtoassistintherapeuticdecisionmakinginpatientswith
cardiogenicshock.
Forpatientswithsymptomatichypotensionandendorgandysfunctiondespitevasopressortherapy,
mechanicaltherapyshouldbeconsidered.Mechanicalsupportoptionsincludeintraaorticballoon
pumpsandpercutaneousorsurgicallyimplantedshorttermmechanicalventricularassistdevices
(VADs).Theseassistdeviceshavecathetersthatareplacedintothevascularsystem(leftatriumor
ventricle)theythenpumpthebloodintotheaorta,essentiallyassistingthefailingleftventricle.
Thesedevicesaugmentcardiacoutputandimproveendorganperfusion.Becauseallofthesedevices
requirelargecatheters,acommoncomplicationisvascularcompromiseatthepointofinsertion.

StrategiestoPreventReadmission
RelatedQuestion
Question34
AtmanyU.S.hospitals,heartfailureisthemostcommondischargediagnosis.Currently,30day
readmissionratesaregreaterthan20%,andreducingtheseadmissionsisamajorfocusofstudyand
resources.Studiesevaluatingdiuresishaveshownthatgreaterfluidremovalduringthehospitalization
isassociatedwithalengtheningofthetimetoreadmission.Additionally,itisimportantthatpatients
aredischargedonappropriatemedicaltherapy,includinganACEinhibitorandablocker.Early
physicianfollowup,ideallywithin7daysofdischarge,hasalsobeenassociatedwithareductionin
readmissions.
Becauseitisoftendifficulttoschedulepatientsforanofficevisitwithin1weekofdischarge,
multidisciplinaryheartfailureclinicshavebeencreatedbyanumberofhospitals.Theseprograms
oftenincludetelephonemonitoringofsignsandsymptoms,evaluatingwhetherpatientsareactually
takingtheirmedications,educatingpatientsonsaltandfluidrestriction,andprovidingamechanism
forearlyfollowupafterhospitalization.

KeyPoints
Inhospitalizedpatientswithacutevolumeoverload,theinitialdoseofintravenousdiuretic
shouldbeatleastequivalenttothetotaldailyoraldoserapiduptitrationshouldbeperformed
ifneededtoassistinfluidremoval.
Reversiblecausesofcardiogenicshockincludeacutemyocardialinfarction,ventricularseptal
orfreewallrupture,andacutevalvularregurgitation.
Inpatientsdischargedwithadiagnosisofheartfailure,earlyphysicianfollowup,ideallywithin
7daysofdischarge,hasbeenassociatedwithareductioninhospitalreadmissions.

AdvancedRefractoryHeartFailure
Oncepatientshaveprogressedtoadvancedheartfailure,thetherapeuticoptionsarelimitedto
inotropictherapy,hearttransplantation,mechanicalcirculatorysupport,andpalliativecare.Heart
transplantationisthebestoptionforpatientswithendstageheartfailure,with50%survivalrates
approaching13years,butislimitedbydonoravailabilitysuchthatonly2000transplantsare
performedintheUnitedStatesannually.Candidatesfortransplantarethereforethoroughlyevaluated
forcomorbiditiesthatmaylimitsurvival.Theupperagelimitforhearttransplantis65to70years.
Patientswithkidneydysfunction,diabeteswithendorganmanifestations,malignancy,chronic
infection,orothercomorbiditiesareoftendeniedtransplant.Optionsforpatientswhoarenot
candidatesforhearttransplantationincludemechanicalcirculatorysupportasdestinationtherapyand
inotropictherapy.However,inotropictherapydoesnotdecreasemortalityandmayactuallyincrease
it.Thesurvivalofinotropicdependentpatientsislessthan10%at1year.

MechanicalCirculatorySupport
RelatedQuestion
Question69
Withthedevelopmentofcontinuousflowleftventricularassistdevices(LVADs),thesurvivalof
patientswithadvancedheartfailureafterimplantationofthesedeviceshasdramaticallyimproved.
Thepumpsaresurgicallyinsertedintotheleftventricle,andthebloodispumpedthroughthedevice
fromtheleftventricletotheaorta.Thepatientshavealine(driveline)comingthroughtheskin
throughwhichthepoweristransmittedtothepump.NinetypercentofpatientsreceivinganLVADas
abridgetohearttransplantationarealiveat1year.Forpatientswhoarenotcandidatesfortransplant
andhaveanLVADplacedasdestinationtherapy,thesurvivalat2yearsismorethan60%and
improving.Complicationsrelatedtothesedevicesincludeischemicandhemorrhagicstroke(>10%of
patients),drivelinerelatedinfections(approximately30%),andgastrointestinalbleedingrelatedto
arteriovenousmalformations(20%insomereports).

ManagementofPostTransplantPatients
RelatedQuestion
Question102
Theprognosisofhearttransplantrecipientshasimprovedgreatlyinrecentyears.Mostpatientshave
nofunctionallimitationsandreturntoanormalqualityoflife.Patientstypicallybeginonathreedrug
immunosuppressionregimenearlyaftertransplantationthatincludesacalcineurininhibitor
(cyclosporineortacrolimus),anantiproliferativeagent(mycophenolatemofetil,sirolimus,or
everolimus),andprednisone.Mostcenterstrytoweanpatientsoffofprednisoneby1year.
Immunosuppressivemedicationsareassociatedwithanumberofadverseeffects,including
hypertension(>90%ofpatients)andnewonsetdiabetes(20%ofpatients).Duringthefirstyearpost
transplant,whiledosesofimmunosuppressantsarehigh,patientshaveanincreasedriskforinfection.
Rejectionoccursinapproximately20%ofpatientsinthefirstyearbutisalmostnonexistentafterthe
firstyearunlessapatientstopstakingimmunosuppressants.Signsofrejectionincludeheartfailure
andatrialarrhythmias(typicallyatrialflutter).However,mostpatientswithrejectionmanifestno
clinicalsymptoms,necessitatingroutinesurveillancewithendomyocardialbiopsy.Thesurveillance
intervalvarieshowever,mostcentersperformbiopsiesbetween1and5yearsaftertransplant.
Thelongtermcomplicationsofhearttransplantincludecardiacallograftvasculopathyand

malignancy.Cardiacallograftvasculopathyoccursinmorethan50%ofthepatientsbythefifthyear
aftertransplant.Itischaracterizedbydiffuseintimalthickeningofthecoronaryarteriesthatstarts
distallyandprogressesproximally.Forthisreason,theusualtherapiesforcoronarydisease,suchas
percutaneouscoronaryinterventionandcoronaryarterybypassgrafting,areusuallynotbeneficial.
Lymphoproliferativedisordersandskincancerarethemostcommonmalignancies.
Becausetheheartintransplantpatientsisdenervated,theyusuallydonotexperiencetypicalischemic
chestpain,leadingtoatypicalpresentationsofcoronaryarterydiseaseandacutecoronarysyndromes.
Additionally,withoutvagalinnervation,heartratestendtorunbetween90/minand110/min.Heart
transplantpatientshaveamarkedresponsetoadenosinebutarenotresponsivetodigoxinoratropine.
Fortransplantpatientspresentingwithatrialarrhythmias,cautionshouldbeusedbeforegiving
adenosinetodiagnosethearrhythmiabecauseitmaycauseprolongedatrioventricularconduction
block.

KeyPoints
Cardiacallograftvasculopathyoccursinmorethan50%ofhearttransplantrecipientsbythe
fifthyearaftertransplantbecauseofitsdiffusenature,revascularizationisusuallynot
beneficial.
Becausetheheartintransplantpatientsisdenervated,theyusuallydonotexperiencetypical
ischemicchestpain,leadingtoatypicalpresentationsofcoronaryarterydiseaseandacute
coronarysyndromes.

SpecificCardiomyopathies
Themostcommoncauseofheartfailureiscoronaryarterydisease.Othercommoncausesinclude
hypertensionandidiopathiccardiomyopathy.Approximately10%ofpatientswithheartfailurehave
heartfailurerelatedtoaspecificetiology.Thisincludesmedicationinducedcardiomyopathies
(primarilychemotherapeuticagents),myocarditis,amyloidosis,sarcoidosis,infectiousetiologiessuch
asHIV,peripartumcardiomyopathies,andalcoholorotherdruginducedcardiomyopathies.Others
willbediscussedlater.Restrictivecardiomyopathieswithsuchcausesaschemotherapeuticagents,
amyloidosis,andsarcoidosisarediscussedinMyocardialDisease.Peripartumcardiomyopathyis
discussedinPregnancyandCardiovascularDisease.

TakotsuboCardiomyopathy
Takotsubo,orstressinduced,cardiomyopathyisasyndromeofreversibleventricularsystolic
dysfunctionusuallyprecipitatedbyanacuteemotionalorphysiologicstress.Althoughtakotsubo
cardiomyopathywasinitiallydescribedinelderlywomenfollowingintenseemotionalstress,the
syndromemayoccurinmenandinsomepatientsanantecedentstressmaynotbeidentifiable.Itis
believedtobecausedbysympatheticmediatedmyocyteinjury,buttheprecisepathogenesisis
unknown.Itoftenmimicsanacutemyocardialinfarctionwithelevatedtroponinlevelsand
electrocardiographicchanges,butitisusuallyassociatedwithnormalcoronaryarteries.Thehallmark
iswallmotionabnormalitiesthatextendbeyondasinglecoronaryterritory,identifiedby
echocardiographyorotherimagingstudy.Forexample,onleftventriculogram,theapexoftheheart
willbehypokineticandthemidheartwillcontractnormally.Characteristicelectrocardiographic
changesincludeSTsegmentelevationanddiffusedeepTwaveinversionswithsomeprolongationof
theQTcinterval.Takotsubocardiomyopathyisusuallyassociatedwithrecoveryofsystolicfunction
intheacuteperiod.Nevertheless,thesepatientsshouldbetreatedwithACEinhibitorsandblockers
acutely.Thereisnoacceptedlengthoftimetocontinuethistherapyinpatientswhoseleftventricular
functionreturnstonormal.Fortherarepatientwhodoesnotrecover,thistherapyshouldbe
continued.

AcuteMyocarditis
Myocarditisusuallypresentswithheartfailuresymptomsoverafewdaystoweeks.Occasionally,
patientshavesymptomsforseveralmonthsbeforeheartfailureisdiscovered.Theclassicpresentation
ofviralmyocarditisincludesaviralprodromewithfever,myalgia,andupperrespiratorysymptoms,
butaprodromeisnotrequiredforthediagnosis.Patientspresentwithdyspnea,chestpain,and
arrhythmias.ECGabnormalitiesareoftenpresent,alongwithevidenceofmyocardialdamagewith
elevatedtroponinlevels.
Variousinfectiouspathogenscancausemyocarditis.Themostcommoncausesareadenovirus,
coxsackievirus,andenterovirus.Thepathogenesisofmyocarditisisunclearandmayinvolvedirect
infectionofthemyocardiumwiththevirusoranimmunesystemresponsetotheinfection.
Endomyocardialbiopsycandefinemyocarditiswithevidenceofmyocardialnecrosis,degeneration,
orboth,withanadjacentinflammatoryinfiltrate.Indicationsforendomyocardialbiopsyinclude
ventriculararrhythmia,highgradeconductionblock(typeIIorIII)orlackofresponsetousualheart
failuretherapy.
Therapyforacutemyocarditisissupportiveandconsistsofusualheartfailuretherapy.Placebo
controlledimmunosuppressivetrialshavenotdemonstratedimprovementsinmortalityorejection
fraction.Patientsoftentakemonths(612)torecoverleftventricularfunction.Approximately50%of
patientseventuallyrecovercardiacfunctiontherefore,itisimportanttowaitandnotplaceanICDfor
theusualindications(ejectionfraction<35%andNYHAclassIIorIIIsymptoms)untilatleast6
monthsofheartfailuretherapy.

GiantCellMyocarditis
Giantcellmyocarditisisanacute,rapidlyprogressiveformofmyocarditisassociatedwithventricular
arrhythmiasandprogressivecardiacdysfunctiondespitemedicaltherapy.Forunclearreasons,this
processusuallyoccursinpersonsyoungerthan40years.Theunderlyingmechanismisunknownbut
isthoughttobeautoimmune.Onendomyocardialbiopsy,thepathognomonicgiantcellisseen.
Unlikeotherformsofmyocarditis,aggressiveimmunosuppressivetherapyhasbeenshownto
improvesurvivalbutthisprocessisstilloftenfatal.Thesepatientsshouldbeconsideredforcardiac
transplantationbutoftenneedtobebridgedwithVADs.Therearecasereportsofgiantcell
myocarditisrecurringposttransplantation.

TachycardiaMediatedCardiomyopathy
Sustainedtachyarrhythmiaforweekstomonthscanproducecardiomyopathy.Themostcommon
arrhythmiasimplicatedareatrialfibrillation,atrialflutter,andtachycardia.Patientswithveryfrequent
prematureventricularcontractions(10,000/d)candevelopcardiomyopathy.Controllingheartrate
withmedicationsorablationoftenresultsinimprovementandresolutionofheartfailureforthese
patients.Patientswithtachycardiashouldbeevaluatedforhyperthyroidism.

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Next:MyocardialDisease
Notes
Chapter04
0Notes
HeartFailure
Questions
ReferenceRanges