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thoracentesis and paracentesis mayprovide temporary symptomatic relief of dyspnea.Care mustbe
takentoavoidrapidfluidremovalandhypotension.
End-of-life considerations should be strongly considered in patients with advanced HF who are
refractorytotherapy.Discussionsregardingthediseasecourse,treatmentoptions,survival,functional
status, andadvancedirectives shouldbeaddressed earlyinthetreatmentofthepatient withHF.For
those with end-stage disease (stage D, NYHA class IV) with multiple hospitalizations and severe
declineintheirfunctionalstatusandqualityoflife,hospiceandpalliativecareisrecommended.
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AcuteHeartFailureandCardiogenicPulmonaryEdema
GENERALPRINCIPLES
Acuteheartfailure(AHF)resultsfromasuddenincreaseinintracardiacpressureoracutemyocardial
dysfunctionleadingtodecreasedperipheralperfusionandcardiogenicpulmonaryedema(CPE).CPE
occurswhenthepulmonarycapillarypressureexceedstheforcesthatmaintainfluidwithinthevascular
space(serumoncoticpressureandinterstitialhydrostaticpressure).
Increased pulmonary capillary pressure may be caused by LV failure of any cause, obstruction to
transmitralflow(e.g.,mitralstenosis,atrialmyxoma),orrarely,pulmonaryveno-occlusivedisease.
Alveolar flooding and impairment of gas exchange follow accumulation of fluid in the pulmonary
interstitium.
DIAGNOSIS
ClinicalPresentation
Clinical manifestationsofAHFandCPEmayoccurrapidlyandincludedyspnea,anxiety,cough,and
restlessness.
Thepatientmayexpectoratepinkfrothyfluid.
Physicalsignsofdecreasedperipheralperfusion,pulmonarycongestion,hypoxemia,useofaccessory
respiratorymuscles,andwheezingareoftenpresent.
DiagnosticTesting
Radiographic abnormalities include cardiomegaly, interstitial and perihilar vascular engorgement,
KerleyBlines,andpleuraleffusions.
Theradiographicabnormalitiesmayfollowthedevelopment ofsymptomsbyseveralhours,andtheir
resolutionmaybeoutofphasewithclinicalimprovement.
TREATMENT
Placingthepatientinasittingpositionimprovespulmonaryfunction.
Bedrest,paincontrol,andreliefofanxietycandecreasecardiacworkload.
Noninvasivepositive-pressureventilationispreferredandmayhaveparticularlyfavorableeffectsin
thesettingofpulmonaryedema.68Mechanicalventilationisindicatedifoxygenationisinadequateor
hypercapniaoccurs.
Precipitatingfactorsshouldbe identifiedandcorrectedbecauseresolution ofpulmonaryedemacan
oftenbeaccomplishedwithcorrectionoftheunderlyingprocess.Themostcommonprecipitantsare:
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Severehypertension
MIormyocardialischemia(particularlyifassociatedwithMR)
Acutevalvularregurgitation
New-onsettachyarrhythmiasorbradyarrhythmias
VolumeoverloadinthesettingofsevereLVdysfunction
Medications
FurosemideisavenodilatorthatdecreasespulmonarycongestionwithinminutesofIVadministration,
well before itsdiureticactionbegins.Aninitial dose of40–80 mgIV should begivenover several
minutesandcanbeincreasedbasedonresponsetoamaximumof200mginsubsequentdoses.
Nitroglycerin is a venodilator that can potentiate the effect of furosemide. IV administration is
preferabletooralandtransdermalformsbecauseitcanberapidlytitrated.
Supplementaloxygenshouldbeadministeredinitiallytoraisethearterialoxygentensionto>60mm
Hg.
Inotropic agents may be necessary for treatment of AHF and CPE in patients with concomitant
hypotensionorshock.
Dobutamine andmilrinone are positive inotropes, chronotropes, andarterial vasodilators. Major
drawbacksincludearrhythmiasandhypotension.
Norepinephrine, rather than dopamine (Table 5-5), should be used for stabilization of the
hypotensive HF patient.Although a large randomized trial foundnomortality difference between
dopamineandnorepinephrineinacohortofundifferentiatedshockpatients,thereweremoreadverse
events (primarily arrhythmic) in the dopamine group, and subgroup analysis of those with
cardiogenicshockshowedanincreasedrateofdeathat28daysinthedopaminegroup.
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ParenteralvasodilatorssuchassodiumnitroprussideshouldbereservedforpatientswithsevereHF
not responding to oral medications. Sodium nitroprusside is a direct arterial vasodilator with less
potent venodilatory properties. It is particularly effective in patients who have concomitant
hypertensionorsevereaortic/mitralvalveinsufficiency.
Sodium nitroprosside should be used carefully in patients with myocardial ischemia because of
potentialreductioninregionalmyocardialbloodflow(coronarysteal).
Parenteral agents should be started atlow doses, titrated to the desired hemodynamic effect, and
discontinuedslowlytoavoidreboundvasoconstriction.Continuoushemodynamicmonitoringshould
beutilizedtohelpguidetherapy.
Theinitialdoseof0.25μg/kg/mincanbetitrated(maximumdoseof10μg/kg/min)tothedesired
hemodynamiceffectoruntilhypotensiondevelops.
Thehalf-lifeofnitroprussideis 1–3 minutes,andits metabolismresults inthe release ofcyanide,
whichismetabolizedbythelivertothiocyanateandisthenexcretedviathekidney.
Toxic levels of thiocyanate (>10 mg/dL) may develop in patients with renal insufficiency.
Thiocyanate toxicitymay manifest as nausea,paresthesias, mental status changes, abdominalpain,
andseizures.
Methemoglobinemiaisararecomplicationoftreatmentwithnitroprusside.
Epinephrine may be considered in patients with refractory cardiogenic shock; however, its use has
beenassociatedwith increasedmortality.Escalationoftherapytoincludeepinephrineshouldprompt
considerationofMCS.
SPECIALCONSIDERATIONS
Right heart catheterization (e.g., Swan-Ganz catheter) may be helpful in cases where a prompt
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response to therapy does not occur by allowing differentiation between cardiogenic and
noncardiogenic causes of pulmonary edema via measurement of central hemodynamics and cardiac
output.Itmaythenbeusedtoguidesubsequenttherapy.Theroutineuseofrightheartcatheterizationin
acuteHFpatientsisnotbeneficial.
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An intra-aortic balloon pump (IABP) can be considered for temporary hemodynamic support in
patients who have failed pharmacologic therapies and have transient myocardial dysfunctionor are
awaitingadefinitiveproceduresuchasanLVADortransplantation.Severeaortoiliacatherosclerosis
andmoderatetosevereaorticvalveinsufficiencyarecontraindicationstoIABPplacement.
Percutaneous LVADs provide short-term hemodynamic support for patients in cardiogenic shock.
These devices have been shown to provide superior hemodynamic effects compared with IABP.
However, use of percutaneous LVADs compared with IABP did not improve 30-day survival in
criticallyillpatients.
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Cardiomyopathy
DilatedCardiomyopathy
GENERALPRINCIPLES
Definition
Dilatedcardiomyopathy(DCM)isadiseaseofcardiacmusclecharacterizedbydilationofthecardiac
chambersandreductioninventricularcontractilefunction.
Epidemiology
DCMisthemostcommonformofnonischemiccardiomyopathyandisresponsibleforapproximately
10,000deathsand46,000hospitalizationseachyear.ThelifetimeincidenceofDCMisabout30cases
per100,000persons.
Pathophysiology
DCM may be secondary to progression of any process that affects the myocardium, and dilation is
directly relatedtoneurohormonal activation.Familial DCM accounts for upto50% ofcasesandis
likelyunderestimated.
4,72
Dilationofthecardiacchambersandvaryingdegreesofhypertrophyareanatomichallmarks.Tricuspid
and mitral regurgitation are common because of the effect of chamber dilation on the valvular
apparatus.
Atrialandventriculararrhythmiasarepresentinasmanyasone-halfofthesepatientsandcontribute
tothehighincidenceofsuddendeathinthispopulation.
DIAGNOSIS
ClinicalPresentation
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Patientsmostoftenpresentwithtypicalfeaturesofheartfailure.
DiagnosticTesting
IMAGING
DiagnosisofDCMcanbeconfirmedwithechocardiographyorcardiacMRI.
DIAGNOSTICPROCEDURES
EndomyocardialbiopsyprovideslittleinformationthataffectstreatmentofpatientswithDCMsandisnot
routinelyrecommended.11EndomyocardialbiopsyforDCMisrecommendedinclinicalscenariosthat
mayresultinthediagnosisofatreatableformofacutemyocarditis,including:
New-onsetHFof<2weeksindurationwithnormal-sizedordilatedleftventricle andhemodynamic
compromise.
New-onsetHFof2 weeks to 3 months in duration associated with a dilated leftventricle and new
ventriculararrhythmias,high-gradeatrioventricularblock(typeIIsecond-degreeorthird-degree),and
failuretorespondtousualcare.
TREATMENT
Medications
ThemedicalmanagementofsymptomaticpatientsisidenticaltothatforHFrEFfromothercauses.This
consistsofcontrollingtotalbodysodiumandvolumeandpharmacotherapyincludingβ-blockers,ARNI
(orACEinhibitorsorARBs),MRAs,andSGLT2inhibitors.
Immunosuppressivetherapywithagentssuchasprednisone,azathioprine,andcyclosporineforbiopsyproven myocarditis has been advocated by some, but efficacy has not been established, with the
possibleexceptionoftheveryrarepatientwithgiantcellmyocarditis.
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NonpharmacologicTherapies
NonpharmacologictherapiesforDCMareidenticaltothoseforHFrEFingeneraland,whenindicatedby
practiceguidelines,includeICDimplantation,CRT,andtemporaryMCS.
SurgicalManagement
Cardiac transplantation should be considered for selected patients with HF due to DCM that is
refractorytomedicaltherapy.
LVADplacementmaybenecessaryforstabilizationofpatientsinwhomcardiactransplantationisan
optionorinselectpatientswhoarenoteligiblefortransplantation.
HeartFailureWithPreservedEjectionFraction
GENERALPRINCIPLES
Definition
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HFpEF, also called diastolic HF, referstotheclinical syndromeofHFinthepresenceofpreserved
systolicfunction(LVEF≥50%).
Diastolic dysfunction refers to an abnormality in the mechanical function of the heart during the
relaxation phase of the cardiac cycle, resulting in elevated filling pressures and impairment of
ventricularfilling.
Epidemiology
AlmosthalfofpatientsadmittedtothehospitalwithHFhaveanormalornear-normalEF.
HFpEFismostprevalentinolderwomen,mostofwhomhavehypertensionand/ordiabetesmellitus.
ManyofthesepatientsalsohaveCADand/orAF.
Etiology
ThevastmajorityofpatientswithHFpEFhavehypertensionandLVhypertrophy.
Myocardial disorders associated with HFpEF include restrictive cardiomyopathy, obstructive and
nonobstructive hypertrophic cardiomyopathy, infiltrative cardiomyopathies, and constrictive
pericarditis.
Pathophysiology
ReducedventricularcomplianceandelastanceplayamajorroleinthepathophysiologyofHFpEF.
FactorscontributingtotheclinicalHFpEFsyndromeincludeabnormalsodiumhandlingbythekidneys,
atrial dysfunction, autonomic dysfunction, increased arterial stiffness, pulmonary hypertension,
sarcopenia,obesity,deconditioning,andothercomorbidities.
DIAGNOSIS
DiagnosisisbasedonechocardiographiccriteriaandDopplerfindingsofnormalLVEFandimpaired
diastolic relaxation and elevated filling pressures. More sensitive echocardiographic parameters of
systolicfunction,suchasLVstrain,maybeabnormalinpatientswithHFpEF.
TREATMENT
No pharmacologic therapy has been shown in a randomized controlled trial to reduce mortality in
HFpEFpatients.TreatmentsthatreducethecombinedoutcomeofcardiacdeathandHFhospitalization
includeARNI,MRA,andSGLT2inhibitors.
Practice guidelinesforHFpEFemphasizebloodpressurecontrol,heartratecontrolor restorationof
sinusrhythminsymptomaticpatients,judiciousdiureticuse,andtreatmentofischemicheartdisease.
HypertrophicCardiomyopathy
GENERALPRINCIPLES
Definition
Hypertrophiccardiomyopathy(HCM)canbedefinedbroadlyasthepresenceofincreasedLVwall
thicknessthatisnotsolelyexplainedbyabnormalloadingconditions.Morespecifically,HCMisa
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geneticallydetermineddiseasewhereinsarcomeremutationsleadtoLVhypertrophyassociatedwith
nondilatedventricularchambersintheabsenceofanotherdiseasethatwouldbecapableofcausingthe
magnitudeofhypertrophypresentinagivenindividual.
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Epidemiology
HCMisthemostcommonlyinheritedheartdefect,occurringin1outof500individuals.
Approximately 500,000 people have HCM in the United States, although many are unaware. An
estimated 36% of young athletes who die suddenly have probable or definite HCM, making it the
leadingcauseofSCDinyoungpeopleintheUnitedStates.
Pathophysiology
HCMresultsfromamutationinageneencodingoneoftheproteinsinvolvedinessentialmyocardial
sarcomerefunctions.
Over50%ofclinicallyaffectedpatientshaveanidentifiedmutation.
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Themostcommonmutationsinvolvemyosinbinding proteinC(MYBPC3)andmyosinheavychain7
(MYH7).
The histopathologic change in HCM consists of hypertrophied myocytes arranged in a disorganized
mannerwithinterstitialfibrosis.
Thesechanges leadgrosslytomyocardialhypertrophy thatistypicallypredominantintheventricular
septum(asymmetricseptalhypertrophy)butmayinvolveanyandallventricularsegments.HCMcanbe
classified clinically according to the presence or absence of LV outflow tract (LVOT) obstruction.
Whenpresent,itistermedhypertrophicobstructivecardiomyopathy.
LVOTobstructionmayoccuratrestbutisenhancedbyfactorsthatincreaseLVcontractility(exercise),
decrease ventricular volume (e.g., Valsalva maneuver, volume depletion, large meal), or decrease
afterload(vasodilators).
Delayedventriculardiastolicrelaxationanddecreasedcomplianceare commonand,alongwithMR,
mayleadtopulmonarycongestion.
Myocardialischemiaiscommon,secondarytoamyocardialoxygensupply–demandmismatch.
Systolic anterior motion of the anterior leaflet ofthe mitral valve is often associated with MR and
likelydeterminestheseverityofLVOTobstruction.
DIAGNOSIS
HCMisusuallydiagnosedbymaximalLVwallthickness≥15mmintheabsenceofanotherdiseasethat
couldaccountforthedegreeofhypertrophy.
ClinicalPresentation
Presentation varies but may include exertional dyspnea, angina, fatigue, dizziness, syncope,
palpitations,orsuddendeath.
Suddendeath ismostcommoninchildrenandyoungadultsbetweentheagesof10and35 yearsand
oftenoccursduringorimmediatelyafterperiodsofstrenuousexertion.
PHYSICALEXAMINATION
Coarse systolic outflow murmur localized along the left sternal border that is accentuated by
maneuversthatdecreasepreload(e.g.,standing,Valsalva maneuver)andmaybeassociatedwitha
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forcefuldoubleortripleapicalimpulse.
Bisferiens(doublepeakpercardiaccycle)carotidpulsemayoccurinthepresenceofobstruction.
DiagnosticTesting
ELECTROCARDIOGRAPHY
TheECGofHCMisusuallyabnormalandinvariablysoinsymptomaticpatientswithLVOTobstruction.
ThemostcommonabnormalitiesareST-segmentandT-waveabnormalities,followedbyevidenceofLV
hypertrophy.TheECGinapical-variantHCMischaracterizedbylarge,invertedTwavesacrossthe
precordialleads.
IMAGING
Two-dimensional echocardiography and Doppler flow studies can establish the presence of a
significantLVoutflowgradientatrestorwithprovocation.
Additional riskstratification should be pursuedwith 24- to48-hour Holter monitoring andexercise
testing.
CardiacMRIisindicatedinpatientswithsuspectedHCMinwhomthediagnosiscannotbeconfirmed
withechocardiography.DelayedgadoliniumenhancementonMRIindicatesincreasedriskofSCD.
GENETICTESTING
GenetictestingforHCMiscommerciallyavailable.Genetictestingcanbeusedtoconfirmthediagnosis
ofHCMwhentheclinicalpresentationisunclearortofacilitatefamilyscreeningtodetermineat-risk
first-degreerelativesofanindexpatient.
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TREATMENT
Managementisdirectedtowardreliefofsymptomsandpreventionofsuddendeath.
Infective endocarditis prophylaxis remains controversial, and prophylactic antibiotics are no longer
recommendedbyguidelines.
Treatmentinasymptomatic individuals is controversial, andno conclusive evidence has been found
thatmedicaltherapyisbeneficial.
Mild-tomoderate-intensityrecreationalexerciseisbeneficial.
Participationinhigh-intensityrecreationalactivitiesormoderate-tohigh-intensitycompetitivesports
may increase the risk of sudden death. These activities should only be considered after a
comprehensiveevaluationandshareddiscussion.
Medications
Nonvasodilatingβ-blockersarefirst-lineagentstoreducesymptomsofHCMbyreducingmyocardial
contractilityandheartrate.
Nondihydropyridinecalciumchannelantagonists(verapamilanddiltiazem)mayimprovethesymptoms
ofHCMbyreducingmyocardialcontractilityandheartrate.Therapyshouldbeinitiatedatlowdoses,
withcarefultitrationinpatientswithoutflowobstruction.Thedoseshouldbeincreasedgraduallyover
severaldaystoweeksifsymptomspersist.
Disopyramide,anegativeinotropicagentthatresultsinloweringoftheLVOTgradient,maybeadded
forHCMpatientswhoremainsymptomaticdespitetheuseofβ-blockersandcalciumchannelblockers
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(aloneor incombination). Use requires monitoringofthe QTinterval, and concomitantuseof other
antiarrhythmicdrugsshouldbeavoided.
Diuretics may improve pulmonarycongestive symptoms in patients with elevatedpulmonary venous
pressures.TheseagentsshouldbeusedcautiouslyinpatientswithLVOTobstructionbecauseexcessive
preloadreductionworsenstheobstruction.
Vasodilatorsshouldbeavoided,duetotheriskofincreasingtheLVOTgradient.
Atrial and ventricular arrhythmias occur commonly in patients with HCM. Supraventricular
tachyarrhythmias are tolerated poorly. Cardioversion is indicated if hemodynamic compromise
develops.
Digoxin is relativelycontraindicatedbecause of its positive inotropic properties and potential for
exacerbatingventricularoutflowobstruction.
AF should be converted to sinus rhythm when possible, and anticoagulation is recommended if
paroxysmalorchronicAFdevelops.
Diltiazem,verapamil,orβ-blockerscanbe usedto controlthe ventricular response.Procainamide,
disopyramide, or amiodarone(see Chapter7, Cardiac Arrhythmias) maybe effective inthe chronic
suppressionofAF.
ICD implantation is recommended for patients with HCM and prior cardiac arrest, ventricular
fibrillation,orhemodynamicallysignificantventriculartachycardia.
ICDimplantationisreasonableinhigh-riskpatientswiththefollowing:
Priorcardiacarrest
Unexplainedsyncope
LVmaximalwallthickness>30mm
SuddendeathattributedtoHCMinoneormorefirst-degreerelatives
LVapicalaneurysm
LVsystolicdysfunctionwithLVEF<50%
NSVT
ExtensivedelayedgadoliniumenhancementonMRI
NonpharmacologicTherapiesforHCM
Dual-chamberpacing(seeChapter7,CardiacArrhythmias)improvessymptomsinsomepatientswith
HCM.AlterationoftheventricularactivationsequenceviaRVpacingmayminimizeLVOTobstruction
secondarytoasymmetricseptalhypertrophy.Only10%ofpatientswithHCMmeetthecriteriafor
pacemakerimplantation,andtheeffectondecreasingtheLVOTgradientisonly25%.Asubsetofpatients
withHCMmayderivesymptomaticbenefitfromdual-chamberpacingwithoutaneffectonsurvival.
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SurgicalManagement
Septal reduction therapy (surgical myectomy or catheter-based alcohol septal ablation) is
recommendedforthosewithLVOTobstructionandseveresymptomsdespitemedicaltherapy.
Septal myectomy is the most commonly performed surgical intervention in HCM. In experienced
centers,itisassociatedwithsymptomimprovementin95%ofpatientswith<1%operativemortality.
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Concomitant mitral valve intervention (mitral valve repair or replacement) is rarely required in
experiencedcentersbecauseMRgenerallyrespondswelltoseptalreduction.
Alcohol septal ablation, a catheter-based alternative to surgical myectomy, also provides relief of
obstructionandsymptomaticbenefitwithlowproceduralmortality,althoughitcanbeassociatedwith
heartblock,requiringpacemakerplacementinupto20%ofpatients.
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Cardiac transplantation should be considered for patients with refractory symptoms and end-stage
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HCM.
PatientEducation
Geneticcounselingandclinicalscreeningarerecommendedforfirst-degreerelativesofpatientswith
HCMbecauseitistransmittedasanautosomaldominanttrait.
RestrictiveCardiomyopathy
GENERALPRINCIPLES
Definition
Restrictive cardiomyopathy(RCM)is characterizedbya rigidheartwithpoor ventricularfilling but
generallyanondilatedLVandnormalLVEF.RightHFsymptomsoftenpredominate.
RCM may be primary, including conditions such as idiopathic RCM, endomyocardial fibrosis, and
Löeffler endocarditis, or secondary to either infiltrative conditions (amyloidosis, sarcoidosis,
hypereosinophilicsyndrome)orstoragediseases (Fabrydisease, hemochromatosis,andtheglycogen
storagediseases).
ConstrictivepericarditismaypresentsimilarlytoRCMbutisadiseasewhereinthepericardiumlimits
diastolic filling. Constriction carries a different prognosis and therapy, and the distinction between
constrictionandRCMisessential.
Pathophysiology
Inamyloidosis,misfoldedprotein(amyloid)depositsinthecardiacinterstitium,interruptingthenormal
myocardialcontractileunitsandcausingrestriction.Mostcommonly,themisfoldedproteiniseitherAL
orTTR.
In sarcoidosis, granulomatousinfiltrationofthemyocardiumisoften subclinicalandmorecommonly
presentswith arrhythmias or conductionsystemdisease;however,inup to5% ofsarcoidosiscases,
restrictionoccurs.
Inhemochromatosis,excessironisdepositedinthecardiomyocytesarcoplasm,ultimatelyovercoming
antioxidant capacity and resulting in lipid peroxidation and membrane permeability. Injury occurs
initiallyintheepicardiumandthenlater inthe myocardium andendocardium,with systolic function
initiallypreserved.
Fabry disease, an X-linked genetic disorder, is characterized by deficientactivity of the lysosomal
enzyme α-galactosidase A, resulting in lysosomal accumulation of globotriaosylceramide in tissues.
Morethanhalfofpatientsdevelopcardiomyopathy,typicallywithLVhypertrophyandRCM.
DIAGNOSIS
DiagnosticTesting
ELECTROCARDIOGRAPHY
TheclassicECGfindinginamyloidosisislowvoltage(despiteechocardiographicevidenceof
ventricularthickening)withpoorR-waveprogression.Insarcoidosis,conductiondiseaseisoften
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present.
IMAGING
In RCM, echocardiography with Doppler analysis often demonstrates thickened myocardium with
normal or abnormal systolic function, abnormal diastolic filling patterns, and evidence of elevated
intracardiac pressure. Compared with constrictive pericarditis, respiratory variation is less marked
andtissueDopplervelocitiesarereduced.
Cardiac MRI and PET are useful diagnostic tools for patients with cardiac sarcoidosis because
granulomas,inflammation,andedemamaybe seen,whichappeartoimprovewiththerapy.81Cardiac
MRIisalsousefulinthediagnosisofamyloidosis.
Bone-seeking radiotracers have strong avidity for TTR-variant amyloid, andbone scintigraphywith
99mtechnetium-labeledpyrophosphateishighlysensitiveandspecificforTTR-cardiacamyloidosis.
DIAGNOSTICPROCEDURES
Oncardiaccatheterization,elevatedandequalizedRVandLVfillingpressuresareseenwithaclassic
“dip-and-plateau” pattern inthe RV and LV pressure tracing. Although pericardial constriction may
produce similar findings, absence of ventricular interdependence identifies RCM as opposed to
constriction.
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RVendomyocardialbiopsyshouldbeconsideredinpatientsinwhomadiagnosisisnotestablishedor
wherecharacterizationofaproteinspecieswillaltertherapy,asincardiacamyloidosis.
TREATMENT
Specifictherapyaimedatameliorationoftheunderlyingcauseshouldbeinitiated.
InpatientswithALcardiacamyloidosis,chemotherapytoreduceALproductionshouldbepursuedin
conjunctionwithahematologist.
In patients with TTR amyloidosis, tafamidis has been shown to reduce all-cause mortality and HF
hospitalizationsandimprovefunctionalstatusandqualityoflife.
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Heartorheart-livertransplantationmaybeconsideredfortreatmentrefractorycardiacamyloidosis.
Cardiac hemochromatosis may respond to reduction of total body iron stores via phlebotomy or
chelationtherapywithdeferoxamine.
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Cardiacsarcoidosismayrespondtoglucocorticoidtherapyorotherimmunomodulatorytherapies.
Fabrydiseasemaybetreatedwithrecombinantα-galactosidaseAenzymereplacementtherapy.
85,86
Digoxin should be avoided in patients with AL cardiac amyloidosis because digoxin is bound
extracellularlybyamyloidfibrils andmaycausehypersensitivityandtoxicity.87β-Blockers shouldbe
avoidedinpatientswithcardiacamyloidosis.
PeripartumCardiomyopathy
GENERALPRINCIPLES
Definition
Peripartumcardiomyopathy(PPCM)isdefinedasLVsystolicdysfunctiondiagnosedinthelastmonthof
pregnancyupto5monthspostpartum,withanincidence1in1000–4000pregnanciesintheUnited
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