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CherylL.Reid,MD
GeneralConsiderations
Physiology&
Etiology
ClinicalFindings
Treatment
Prognosis
ESSENTIALSOFDIAGNOSIS
Pregnancy
Historyofheartdisease
Symptomsandsignsofheartdisease
Echocardiographicevidenceofheartdisease
Heartdisease(mostfrequentlycongenitalorvalvulardiseases)occursin1–4%ofpregnancies,andtheincidenceisincreasing.Theuniquehemodynamicchangesassociatedwithpregnancymakediagnosisandmanagementofheartdiseaseinpregnantpatientsachallengetothephysicians,whomustconsidernotonlythepatientbutalsotheriskstothefetus.
DanzellJD:
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97.
A.CARDIOVASCULARPHYSIOLOGYOF“NORMAL”PREGNANCY
Normalpregnancyisaccompaniedbysignificantphysiologicchanges,althoughunderlyingmechanismsremainvirtuallyunknown(Table31–1).Thenormalsignsandsymptomsassociatedwithpregnancymayobscurethediagnosisofheartdiseaseduringthattime.Theclinicianmust,therefore,haveathoroughknowledgeofthesenormalchangesandtheaspectsofthehistoryandphysicalexaminationthatsuggestthepresenceofheartdisease.
Table31–1.Cardiovascularchangesinnormalpregnancy.
1.Bloodvolume—Theincreaseinmaternalbloodvolumebeginsasearlyasthesixthweekofpregnancy,peaksatapproximately32weeksofgestation,andstaysatthatlevel(40–50%higherthanpregestationallevels)untildelivery.Theplasmavolumeshowsamorerapidandsignificantrisethantheredbloodcellmass,accountingfortheappearanceofphysiologicanemiaduringpregnancy.Theincreasedbloodvolumeismaintaineduntilafterdelivery,whenaspontaneousdiuresisoccurs.Thisrapidpostpartumchangeinbloodvolumeisacriticalperiodforpatientswithunderlyingheartdisease.
2.Cardiacfunction—Normalpregnancyischaracterizedbyenhancedmyocardialperformance.Numerousstudieshaveshownagradualincreaseinleftventricularsystolicfunctionattributedtoleftventricularafterloadreductionduetothelow-resistancerunoffoftheplacenta.Thenriseinleftventricularsystolicfunctionbeginsinearlypregnancy,peaksinthetwentiethweek,andthenremainsconstantuntildelivery.
3.Cardiacoutput—Oneofthemostsignificantchangesduringpregnancyistheincreaseincardiacoutput,whichbeginstoriseduringthefirsttrimesterandpeaksattwenty-fifthandthirty-fifthweeksofgestation.Totalcardiacoutputincreasesupto50%overpregestationallevels.Cardiacoutputistheproductofstrokevolumeandheartrate.Duringtheearlypartofpregnancy,theincreaseincardiacoutputispredominantlytheresultofanincreaseinstrokevolume,augmentedbyincreasedintrinsicmyocardialcontractility.Aspregnancyadvances,heartrateincreasesandstrokevolumemildlydecreases.Theincreasedcardiacoutputinlatepregnancyismaintainedbecauseoftheincreasedheartrate.
Auniqueaspectofpregnancyisthehemodynamicchangesinducedbyachangeinapatient'
sposition.Whenthepatientisinthesupineposition,thegraviduterusinducesprofoundmechanicalcompressionoftheinferiorvenacava,decreasingvenousreturntotheheart,andthus,cardiacoutput.Achangefromthesupinetotheleftlateralpositionresultsina25–30%increaseincardiacoutputbecauseofanincreaseinstrokevolume.
4.Intravascularpressuresandvascularresistance—Systolicanddiastolicpressuresdropduringpregnancy.Asmalldecreaseinsystolicbloodpressurebeginsinthefirsttrimester,peaksatmidgestation,andreturnstonearprepregnancylevelsatterm.Thediastolicbloodpressuredecreasesmorethanthesystolicbloodpressure,duetoasignificantfallinsystemicvascularresistance,andresultsinawiderpulsepressure.Thesystemicbloodpressureincreasesduringpregnancywiththepatient'
sageandparity.Italsovarieswiththepatient'
sposition.Thehighestlevelsarerecordedearlyinthepregnancywhenthepatientisupright,andlowestwhensheissupine.Duringthelatterpartofpregnancytheeffectofpositiononsystemicbloodpressuredependsontherelativedegreesofinferiorvenacavaandaorticcompression.Totalvascularresistance,includingboththesystemicandthepulmonary,decreaseduringpregnancy.Themechanismforthefallinresistancesispoorlyunderstoodbutisattributedtothelow-resistancecirculationofthepregnantuterusandtohormonalchangesassociatedwithpregnancy.
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ChangesinHemodynamics,ventricularremodeling,andventricularcontractilityduringnormalpregnancy:
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B.ETIOLOGYANDSYMPTOMATOLOGY
1.Congenitalheartdisease—Asmedicalandsurgicaltreatmentofsuchpatientshasimproved,congenitalheartdiseaseisfoundmorefrequentlyduringpregnancy.Asaresult,morewomenwitheitheruncorrectedorsurgicallycorrectedcongenitalheartdiseasesaresurvivingintotheadulthood(Table31–2).
Table31–2.Commoncongenitalabnormalitiesfoundinpregnantwomen.
Onlyafewconditionsplaceapatientatahighrisktoadviseagainstpregnancy(Table31–3).Amajorityofthepatientswithmildtomoderateacyanoticcongenitalheartdiseasetoleratepregnancy,labor,anddeliverywell.Treatmentshouldinvolvefrequentcounselingbytheobstetricianandcardiologist.Inseverecases,physicalactivityandsaltintakelimitation,earlytreatmentofanyinfection,heartfailure,andarrhythmiashouldbeundertaken.Cesareandeliveryshouldbereservedonlyforobstetricindicationsbecausemostpatientscanbesafelydeliveredvaginally.
Table31–3.Relativecontraindicationstopregnancy.
High-riskpatientswithseverecyanoticcongenitalheartdisease,decreasedfunctionalcapacity,orEisenmenger'
ssyndromeshouldbeadvisedagainstpregnancy.Antibioticprophylaxisforbacterialendocarditisisrecommendedinmostpatientswithcongenitalheartdisease.Theriskoffetalmalformationintheoffspringshouldbeconsideredcarefully.
a.Atrialseptaldefect—Secundumatrialseptaldefectisthemostcommoncongenitalcardiacabnormalityencounteredduringpregnancy.Patientswithuncomplicatedatrialseptaldefectsusuallytoleratepregnancywithlittleproblem.Patientsmaynotbeabletotoleratetheacutebloodlossthatcanoccuratthetimeofdeliverybecauseofincreasedshuntingfromlefttorightcausedbysystemicvasoconstrictionassociatedwithhypotension.Theincidenceofsupraventriculararrhythmiasmayincreaseinolderpregnantpatients,whichmayresultinrightventricularfailureandvenousstasisleadingtoparadoxicalemboli.Low-doseaspirin,oncedailyafterthefirsttrimesteruntildeliverymayhelppreventclotformation.Pulmonaryhypertensionfromanatrialseptaldefectusuallyoccurslateinlife,pastthechildbearingyears.Bacterialendocarditisprophylaxisisrecommendedonlyforostiumprimumdefectduetoassociatedaorticvalveabnormality.Vaginaldeliveryispreferredovercesarean.Riskintheoffspringisabout2.5%.
b.Ventricularseptaldefect—Mostisolatedventricularseptaldefectshaveclosedbyadulthood.Womenwithventricularseptaldefectsgenerallyfarewellinpregnancy.Congestiveheartfailureandarrhythmiaarereportedonlyinpatientswithdecreasedleftventricularsystolicfunctionpriortopregnancy.Endocarditisprophylaxisduringdelivery,preferablyvaginal,isrecommended.
c.Patentductusarteriosus—Mostpatientswithapatentductusarteriosusundergosurgicalrepairinchildhood.Anormalpregnancycanbeexpectedinpatientswithsmall-to-moderateshuntsandnoevidenceofpulmonaryhypertension.Patientswithalargepatentductusarteriosus,elevatedpulmonaryvascularresistance,andareversedshuntareatgreatestriskforcomplicationsduringpregnancy.Thedecreasedsystemicvascularresistanceassociatedwithpregnancyincreasestheright-to-leftshuntandtheintrauterineoxygendesaturation.Patientsdevelopingheartfailurearetreatedwithdigoxinanddiuretics.Thepreferredmodeofdeliveryisvaginalinmostpatientswithendocarditisprophylaxisandhemodynamicmonitoringconsideredatthetimeofdelivery.Theriskinanoffspringisabout4%.
d.Pulmonicstenosis—Thenaturalhistoryofpulmonicstenosisfavorssurvivalintoadulthoodevenwithsevereobstructiontorightventricularoutflow.Mild-to-moderatepulmonicstenosis(peakgradient£
100mmHg)usuallypresentsnoincreasedriskduringpregnancy.Patientswithseverepulmonicstenosismayoccasionallytoleratepregnancywithoutdevelopingcongestiveheartfailure.Vaginaldeliveryistoleratedwell.Idealtreatmentconsistingofballoonvalvuloplastyshouldbeperformedbeforegestation.Theriskintheoffspringisabout3.5%.
e.Coarctationoftheaorta—Inuncomplicatedcoarctationoftheaorta,pregnancyisusuallysafeforthemotherbutmaybeassociatedwithfetalunderdevelopmentbecauseofthediminisheduterinebloodflow.Thebloodpressuremaydecreaseslightly,asduringnormalpregnancy,butstillremainselevated.MaternaldeathsinthesepatientsareusuallytheresultofaorticruptureorcerebralhemorrhagefromanassociatedberryaneurysmofthecircleofWillis.Patientswiththegreatestriskduringpregnancyarethosewithseverehypertensionorassociatedcardiacabnormalities,sucha