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PERIPARTUM CARDIOMYOPATHY
DR NISHANT TYAGI
MODERATOR : DR B N SHAHI
Escorts Heart Institute And Research CentreEscorts Heart Institute And Research Centre
Review of Cardiac Changes inReview of Cardiac Changes in
PregnancyPregnancy
 Increase in blood volume by 20 – 100 %Increase in blood volume by 20 – 100 %
 Starts as early as 6Starts as early as 6thth
weekweek
 Rises rapidly thru 32-34 weeks then a modest riseRises rapidly thru 32-34 weeks then a modest rise
 Net result = 1100 – 1600 cc increase above baselineNet result = 1100 – 1600 cc increase above baseline
 Increase in TBV due to:Increase in TBV due to:
 Increased vascular capacitanceIncreased vascular capacitance
 Systemic vasodilatationSystemic vasodilatation
Review cont….Review cont….
 CO rises by ~ 50 %CO rises by ~ 50 %
 Due to 3 important factors:Due to 3 important factors:
 Preload is increased due to increase in TBVPreload is increased due to increase in TBV
 Afterload is reduced due to decreased SVRAfterload is reduced due to decreased SVR
 Maternal HR rises 15-20 bpmMaternal HR rises 15-20 bpm
*Chapman et al.*Chapman et al. Kidney Int 1998; 54:2056Kidney Int 1998; 54:2056
What is a PPCM?What is a PPCM?
 PPCM was defined clinically as the onset of cardiac failure with noPPCM was defined clinically as the onset of cardiac failure with no
identifiable cause in the last month of pregnancy or within fiveidentifiable cause in the last month of pregnancy or within five
months after delivery, in the absence of heart disease before themonths after delivery, in the absence of heart disease before the
last month of pregnancy.last month of pregnancy.
* In 1997, National Heart, Lung, and Blood Institute (NHLBI) workshop* In 1997, National Heart, Lung, and Blood Institute (NHLBI) workshop
 Stricter echocardiographic criteria have been recommendedStricter echocardiographic criteria have been recommended
 EF < 45%,EF < 45%,
 FS < 30% or both, andFS < 30% or both, and
 LVED > 2.7 cm per square meter of BSALVED > 2.7 cm per square meter of BSA
* Obstet Gynecol 1999;94:311-316* Obstet Gynecol 1999;94:311-316
Criteria for DiagnosisCriteria for Diagnosis
 4 Criteria
 Development of HF in the last month of pregnancy, or
within 5 months postpartum
 Absence of a determinable cause for cardiac failure
 Absence of heart disease before last month of
pregnancy
 LV functional impairment demonstrated on Echo
Pregnancy-associated cardiomyopathy:Pregnancy-associated cardiomyopathy:
clinical characteristics and a comparisonclinical characteristics and a comparison
between early and late presentation.between early and late presentation.
  Circulation.  2005; 111(16):2050-5Circulation.  2005; 111(16):2050-5 Elkayam et al.Elkayam et al.
 123 women123 women
 100 traditional PPCM100 traditional PPCM
 23 pregnancy-associated cardiomyopathy earlier than the last23 pregnancy-associated cardiomyopathy earlier than the last
gestational month.gestational month.
 associated conditionsassociated conditions
 gestational hypertension (43%)gestational hypertension (43%)
 tocolytic therapy (19%), andtocolytic therapy (19%), and
 twin pregnancy (13%)twin pregnancy (13%)
 Normal LV recovery - 54%Normal LV recovery - 54%
 Maternal mortality - 9%.Maternal mortality - 9%.
 A comparison showed no differences in age, race, associatedA comparison showed no differences in age, race, associated
conditions, LV EF at diagnosis, its rate and time of recovery, andconditions, LV EF at diagnosis, its rate and time of recovery, and
maternal outcome.maternal outcome.
 These 2 conditions may represent a continuum of a spectrum of theThese 2 conditions may represent a continuum of a spectrum of the
same disease.same disease.
Cont…..Cont…..
 Overall responsibleOverall responsible
for 10,000 deaths andfor 10,000 deaths and
46,00046,000
hospitalizations eachhospitalizations each
yearyear
 Wide age range 20-Wide age range 20-
6060
*Dec et al. N Engl J Med 1994; 331:1564*Dec et al. N Engl J Med 1994; 331:1564
 Common Sx:Common Sx:
 Progressive dyspneaProgressive dyspnea
with exertionwith exertion
 Impaired exerciseImpaired exercise
capacitycapacity
 OrthopneaOrthopnea
 Paroxysmal nocturnalParoxysmal nocturnal
dyspneadyspnea
 Peripheral edemaPeripheral edema
*Felker et al. N Engl J Med 2000; 342:1077*Felker et al. N Engl J Med 2000; 342:1077
Causes of Cardiomyopathies
50
9
5 4 4 4 3 3 1
10
0
10
20
30
40
50
60Idiopathic
Ischem
ic
H
eartD
isease
Infiltrative
D
isease
P
eripartum
H
TN
H
IV
C
onnective
Tissue
S
ubstance
A
buse
D
oxorubicin
O
ther
Peripartum CardiomyopathyPeripartum Cardiomyopathy
 4% of all4% of all
cardiomyopathiescardiomyopathies
 1:4000 to 1:150001:4000 to 1:15000
deliveriesdeliveries
 DilatedDilated
CardiomyopathyCardiomyopathy
Five-year prospective study of the incidenceFive-year prospective study of the incidence
and prognosis of PPCM at a singleand prognosis of PPCM at a single
institution.institution.
  MayoMayo ClinClin Proc.  2005; 80(12):1602-6Proc.  2005; 80(12):1602-6 Department of Adult Medicine, HospitalDepartment of Adult Medicine, Hospital
Albert Schweitzer, HaitiAlbert Schweitzer, Haiti
 1 case per 300 live births,1 case per 300 live births,
 28% of 92 patients regained normal LV function at 6 m28% of 92 patients regained normal LV function at 6 m
 mortality - 15.3%mortality - 15.3%
 difference in echo at diagnosis between deceased patients anddifference in echo at diagnosis between deceased patients and
survivors was not statistically significant: mean LVED (6.2 vs 5.8survivors was not statistically significant: mean LVED (6.2 vs 5.8
cm; P=.08), EF (22% vs 25%; P=.12), FS (16% vs 15%; P=.46 )cm; P=.08), EF (22% vs 25%; P=.12), FS (16% vs 15%; P=.46 )
 echo features at diagnosis were unable to predict death, although aecho features at diagnosis were unable to predict death, although a
statistically significant difference occurred at diagnosis between thestatistically significant difference occurred at diagnosis between the
recovered group and nonrecovered group for mean EF (28% vsrecovered group and nonrecovered group for mean EF (28% vs
23%; P<.001) and FS (17% vs 14%; P=.004)23%; P<.001) and FS (17% vs 14%; P=.004)
Should we be concerned?Should we be concerned?
 Yes!Yes!
 Pregnancy Related Mortality SurveillancePregnancy Related Mortality Surveillance
1991-19991991-1999
 Leading Causes of Maternal Mortality:Leading Causes of Maternal Mortality:
 Embolism – 20%Embolism – 20%
 Hemorrhage – 17%Hemorrhage – 17%
 Hypertension – 16%Hypertension – 16%
 Peripartum Cardiomyopathy- 9%*Peripartum Cardiomyopathy- 9%*
EtiologyEtiology
 Multiple studies have attempted toMultiple studies have attempted to
elucidate a distinct etiology…..all haveelucidate a distinct etiology…..all have
failedfailed
 Theories:Theories:
 MyocarditisMyocarditis
 Abnormal Immune ResponseAbnormal Immune Response
 GeneticsGenetics
 Latent cardiomyopathyLatent cardiomyopathy
Myocarditis?Myocarditis?
 Nairobi Study1986Nairobi Study1986
 11 African women with PPCM11 African women with PPCM
 Endocardial biopsies done on all elevenEndocardial biopsies done on all eleven
 5 showed evidence of “healing myocarditis”5 showed evidence of “healing myocarditis”
 Presence of inflammatory cellsPresence of inflammatory cells
 NecrosisNecrosis
 Fibrous remodelingFibrous remodeling
 9 patients finished study9 patients finished study
 75% of myocarditis group developed persistent HF75% of myocarditis group developed persistent HF
 80% of patients without myocarditis improved80% of patients without myocarditis improved
**Sanderson et al. Br Heart J 1986: 56:285
Myocarditis? Cont…Myocarditis? Cont…
 Another study 1986:Another study 1986:
 84 women with cardiomyopathies84 women with cardiomyopathies
 14 diagnosed as being PPCM14 diagnosed as being PPCM
 29% of patients with PPCM were found to have29% of patients with PPCM were found to have
myocarditismyocarditis
 Only 9% of idiopathic CM related to myocarditisOnly 9% of idiopathic CM related to myocarditis
*O’Connell et al. J AM Coll Cardiol 1986; 8:52*O’Connell et al. J AM Coll Cardiol 1986; 8:52
Myocarditis? Cont….Myocarditis? Cont….
 33rdrd
Study 1990:Study 1990:
 18 patients with PPCM18 patients with PPCM
 14 due to myocarditis14 due to myocarditis
 10 of these received immunosuppressive Tx over 6-810 of these received immunosuppressive Tx over 6-8
weeks, then tapered over 6-8 weeksweeks, then tapered over 6-8 weeks
 9 of 10 improved on therapy9 of 10 improved on therapy
 However, 4 of 4 not receiving therapy also improvedHowever, 4 of 4 not receiving therapy also improved
**Midei et al. Circulation 1990; 81:922Midei et al. Circulation 1990; 81:922
Myocarditis? Cont….Myocarditis? Cont….
 1994 Retrospective study1994 Retrospective study
 34 patients diagnosed with PPCM34 patients diagnosed with PPCM
 Researches found lower incidence ofResearches found lower incidence of
myocarditis than previously reportedmyocarditis than previously reported
 8.8 % due to myocarditis8.8 % due to myocarditis
 * Rizeq et al. Am J Cardiol 1994; 74:474* Rizeq et al. Am J Cardiol 1994; 74:474
Myocarditis? Cont….Myocarditis? Cont….
 In 1995 a randomized trial ofIn 1995 a randomized trial of
immunosuppressive Tx in a related populationimmunosuppressive Tx in a related population
with myocarditis did not prove efficacy in thewith myocarditis did not prove efficacy in the
Myocarditis Treatment TrialMyocarditis Treatment Trial,,
*Mason JW , N Engl J Med 1995;333:269-275.*Mason JW , N Engl J Med 1995;333:269-275.
 In 2001 a similar fashion therapy with immuneIn 2001 a similar fashion therapy with immune
globulin did not improve outcomes in aglobulin did not improve outcomes in a
randomized trial of patients with acuterandomized trial of patients with acute
cardiomyopathy (IMAC Trial)cardiomyopathy (IMAC Trial)
*McNamara et al, Circulation 2001;103:2254-2259.*McNamara et al, Circulation 2001;103:2254-2259.
Abnormal Immune Response?Abnormal Immune Response?
 Maternal immunologic response to a fetalMaternal immunologic response to a fetal
antigen?antigen?
 Fetal cells may escape into the maternalFetal cells may escape into the maternal
circulation without being rejected.circulation without being rejected.
 May become lodged in cardiac tissue.May become lodged in cardiac tissue.
 May trigger immune responseMay trigger immune response
*Nelson et al. J Am Med Womens Assoc 1998; 53:31*Nelson et al. J Am Med Womens Assoc 1998; 53:31
Immune Response? Cont….Immune Response? Cont….
 Disproved 1990., Nigerian StudyDisproved 1990., Nigerian Study
 39 women with PPCM39 women with PPCM
 No differences between subjects and controlsNo differences between subjects and controls
in levels of:in levels of:
 Serum ImmunoglobulinsSerum Immunoglobulins
 Circulating Immune ComplexesCirculating Immune Complexes
 Cardiac muscle antibodiesCardiac muscle antibodies
*Cenac et al. Int J Cardiol 1990; 26:49*Cenac et al. Int J Cardiol 1990; 26:49
Peripartum cardiomyopathy: inflammatory markersPeripartum cardiomyopathy: inflammatory markers
as predictors of outcome in 100 prospectivelyas predictors of outcome in 100 prospectively
studied patients.studied patients.EurEur Heart J.  2006; 27(4):441-6Heart J.  2006; 27(4):441-6
 levels of C-reactive protein correlated positively withlevels of C-reactive protein correlated positively with
baseline LVED (P=0.0026) and LVES(P=0.0012)baseline LVED (P=0.0026) and LVES(P=0.0012)
diameters and inversely with LVEF (P=0.015).diameters and inversely with LVEF (P=0.015).
 Patients who died presented with significantly lowerPatients who died presented with significantly lower
mean EF and higher Fas/Apo-1 plasma values (P<0.05)mean EF and higher Fas/Apo-1 plasma values (P<0.05)
 Baseline Fas/Apo-1 and higher NYHA FC were the onlyBaseline Fas/Apo-1 and higher NYHA FC were the only
predictors of mortalitypredictors of mortality
GeneticsGenetics
 Several case reports publishedSeveral case reports published
 1963, Pierce et al. reported that 3 of 17 patients with PPCM had1963, Pierce et al. reported that 3 of 17 patients with PPCM had
definitive FH of same conditiondefinitive FH of same condition
 Also, 1976Also, 1976 StrungStrung documented male relatives of female patients withdocumented male relatives of female patients with
PPCM as also having cardiomyopathies.PPCM as also having cardiomyopathies.
 1984 Voss et al.1984 Voss et al. reported a patient who died from PPCM as didreported a patient who died from PPCM as did
her mother and two of her sistersher mother and two of her sisters
 1993 Massad et al.1993 Massad et al. reported 16 y.o girl with PPCM followingreported 16 y.o girl with PPCM following
molar preg. Sister later received cardiac transplant for PPCM.molar preg. Sister later received cardiac transplant for PPCM.
Latent cardiomyopathyLatent cardiomyopathy
 Hypertension duringHypertension during
pregnancy, pre-pregnancy, pre-
eclampsia, are frequenteclampsia, are frequent
in women with PPCM andin women with PPCM and
do support thatdo support that
hemodynamic stresseshemodynamic stresses
may play a rolemay play a role
 However, absence ofHowever, absence of
cardiac symptoms incardiac symptoms in
PPCM until thePPCM until the
postpartum period arguespostpartum period argues
against the "latentagainst the "latent
cardiomyopathy" theory.cardiomyopathy" theory.
Risk FactorsRisk Factors
 Age >30 yearsAge >30 years
 MultiparityMultiparity
 Pregnancy with multiplePregnancy with multiple
fetusesfetuses
 H/O Preeclampsia,H/O Preeclampsia,
eclampsia, or postpartumeclampsia, or postpartum
HTNHTN
 Long term tocolyticLong term tocolytic
therapy (>4weeks)therapy (>4weeks)
 African DescentAfrican Descent
 Maternal cocaine abuseMaternal cocaine abuse
Clinical PresentationClinical Presentation
 Symptoms:Symptoms:
 Paroxysmal NocturnalParoxysmal Nocturnal
DyspneaDyspnea
 Dyspnea on ExertionDyspnea on Exertion
 CoughCough
 OrthopneaOrthopnea
 Chest PainChest Pain
 Abdominal DiscomfortAbdominal Discomfort
 PalpitationPalpitation
 Signs:Signs:
 Raised JVPRaised JVP
 CardiomegalyCardiomegaly
 Gallop RhythmGallop Rhythm
 Holosystolic murmurHolosystolic murmur
 EdemaEdema
0
10
20
30
40
50
60
70
80
90
Parox.Noct.Dys...
Dyspnea
on
Exertion
CoughOrthopnea
ChestPain
Abdom
inalPain
Palpations
PPCM Symptoms
DiagnosisDiagnosis
 EKGEKG
 Two-dimensional echocardiogramTwo-dimensional echocardiogram
 Lab: CBC, BNP, TSH, FerritinLab: CBC, BNP, TSH, Ferritin
 CXRCXR
 ? Myocardial biopsy? Myocardial biopsy
EKG ChangesEKG Changes
 Sinus TachycardiaSinus Tachycardia
 Nonspecific STNonspecific ST
changeschanges
 LV HypertrophyLV Hypertrophy
Chest X-rayChest X-ray
 Pulmonary EdemaPulmonary Edema
 Venous congestionVenous congestion
 Enlarged CardiacEnlarged Cardiac
SilhouetteSilhouette
 R/O PER/O PE
EchocardiogramEchocardiogram
 Spherical LVSpherical LV
 Mitral and TricuspidMitral and Tricuspid
regurgitationregurgitation
 Left AtrialLeft Atrial
enlargementenlargement
 EF <55%EF <55%
TreatmentTreatment
 Delivery (consider elective caesarian with invasiveDelivery (consider elective caesarian with invasive
hemodynamic monitoring of the mother)hemodynamic monitoring of the mother)
 Similar to other forms of CHFSimilar to other forms of CHF
 DiureticsDiuretics
 ß-blockersß-blockers
 ACE - IACE - I
 DigoxinDigoxin
 AnticoagulantsAnticoagulants
** Must consider pregnancy class/breast-feeding harm
potential!
Pregnancy Drug Class ReviewPregnancy Drug Class Review
 Category A: Controlled studies in pregnant women fail to demonstrate a riskCategory A: Controlled studies in pregnant women fail to demonstrate a risk
to the fetus in the first trimester with no evidence of risk in later trimesters.to the fetus in the first trimester with no evidence of risk in later trimesters.
The possibility of harm appears remoteThe possibility of harm appears remote
 Category B: Presumed safety based on animal studies, with no controlledCategory B: Presumed safety based on animal studies, with no controlled
studies in pregnant women,studies in pregnant women, oror animal studies have shown an adverseanimal studies have shown an adverse
effect that was not confirmed in controlled studies in women in the firsteffect that was not confirmed in controlled studies in women in the first
trimester and there is no evidence of a risk in later trimesterstrimester and there is no evidence of a risk in later trimesters..
 Category C:Category C: Studies in women and animals are not availableStudies in women and animals are not available oror studies instudies in
animals have revealed adverse effects on the fetus and there are noanimals have revealed adverse effects on the fetus and there are no
controlled studies in women. Drugs should be given only if the potentialcontrolled studies in women. Drugs should be given only if the potential
benefits justify the potential risk to the fetusbenefits justify the potential risk to the fetus
 Category D: There is positive evidence of human fetal risk (unsafe),Category D: There is positive evidence of human fetal risk (unsafe),
however in some cases such as a life-threatening illness the potential riskhowever in some cases such as a life-threatening illness the potential risk
may be justified if there are no other alternativesmay be justified if there are no other alternatives
 Category X: Highly unsafe: risk of use outweighs any potential benefit.Category X: Highly unsafe: risk of use outweighs any potential benefit.
Drugs in this category are contraindicated in women who are or mayDrugs in this category are contraindicated in women who are or may
become pregnantbecome pregnant
DrugsDrugs
 Digoxin Class CDigoxin Class C
 Symptomatic controlSymptomatic control
 Requires levelRequires level
monitoringmonitoring
 Therapeutic levels 0.7-Therapeutic levels 0.7-
1.21.2
DiureticsDiuretics
 Lasix Class CLasix Class C
 Reserved for cardiacReserved for cardiac
conditionsconditions
 Not recommended inNot recommended in
PIHPIH
 May decreaseMay decrease
placental perfusionplacental perfusion
 Thiazide DiureticsThiazide Diuretics
 Reserved for cardiacReserved for cardiac
conditionsconditions
 Not recommended inNot recommended in
PIHPIH
 Thrombocytopenia hasThrombocytopenia has
been reported inbeen reported in
breast feeding infantsbreast feeding infants
VasodilatorsVasodilators
 Hydralazine Class CHydralazine Class C
 Compatible withCompatible with
breastfeedingbreastfeeding
 ACE InhibitorsACE Inhibitors
 Class D in 2Class D in 2ndnd
/3/3rdrd
trimesterstrimesters
 Reserved forReserved for
postpartum use-postpartum use-
compatible with BFcompatible with BF
 Renal toxicity inRenal toxicity in
infants exposed ininfants exposed in
uteroutero
Beta-BlockersBeta-Blockers
 Class CClass C
 Compatible with breast feedingCompatible with breast feeding
 Has been shown to cause IUGR in someHas been shown to cause IUGR in some
infants in utero.infants in utero.
AntiarrhythmicsAntiarrhythmics
 Presenting with sudden death or VT with hemodynamicPresenting with sudden death or VT with hemodynamic
compromise – ICDcompromise – ICD
 VT without hemodynamic compromise , managementVT without hemodynamic compromise , management
can be tempered somewhat because of the potentialcan be tempered somewhat because of the potential
transient nature of the myopathy and amiodaronetransient nature of the myopathy and amiodarone
therapy should be startedtherapy should be started
 Asymptomatic NSVT, no amiodarone therapy, focus onAsymptomatic NSVT, no amiodarone therapy, focus on
correction of metabolic abnormalities and consider thecorrection of metabolic abnormalities and consider the
addition of a beta receptor antagonistaddition of a beta receptor antagonist
AnticoagulantsAnticoagulants
 Heparin Class CHeparin Class C
 Short half life-can beShort half life-can be
discontinued prior todiscontinued prior to
delivery to preventdelivery to prevent
maternal hemorrhagematernal hemorrhage
 Not excreted in breastNot excreted in breast
milkmilk
 Warfarin Class DWarfarin Class D
 Contraindicated inContraindicated in
pregnancypregnancy
 Safe in breast feeding.Safe in breast feeding.
Not excreted in breastNot excreted in breast
milk.milk.
Indications:-Indications:-
1.evidence of a systemic embolus1.evidence of a systemic embolus
2.severe left ventricular dysfunction with2.severe left ventricular dysfunction with
documented mural thrombusdocumented mural thrombus
Cardiac TransplantationCardiac Transplantation
 Estimated that transplant is performed in up toEstimated that transplant is performed in up to
1/3 of PPCM patients1/3 of PPCM patients
 Pts should be strongly advised against futurePts should be strongly advised against future
pregnancies.pregnancies.
 Increased risk of HTN, preeclampsia, and pretermIncreased risk of HTN, preeclampsia, and preterm
laborlabor
 Also at risk for graft failure due to recurrentAlso at risk for graft failure due to recurrent
disease.disease.
*Scott et al. Obstet Gynecol 1993; 82:324*Scott et al. Obstet Gynecol 1993; 82:324
Differential DiagnosisDifferential Diagnosis
 PIHPIH
 However, HF associated with PIH representsHowever, HF associated with PIH represents
a diastolic failure, vs. systolic in PPCMa diastolic failure, vs. systolic in PPCM
 Pulmonary EmbolismPulmonary Embolism
 Again, usually ruled out by CXRAgain, usually ruled out by CXR
 If still suspicious, can order spiral CTIf still suspicious, can order spiral CT
PrognosisPrognosis
 Mortality estimates range from 25-50%.Mortality estimates range from 25-50%.
 Most deaths occur within 3 monthsMost deaths occur within 3 months
postpartumpostpartum
 Deaths usually caused by:Deaths usually caused by:
 Progressive pump failureProgressive pump failure
 ArrhythmiasArrhythmias
 Thromboembolic eventsThromboembolic events
Prognostic value of echocardiographyPrognostic value of echocardiography
Chapa JB et al,Chapa JB et al, ObstetObstet GynecolGynecol.  2005; 105(6):1303-8.  2005; 105(6):1303-8
 FS and LVED, at the time of diagnosis and at follow-up.FS and LVED, at the time of diagnosis and at follow-up.
LVD was defined by echo as FS < 30% and LVED > 4.8LVD was defined by echo as FS < 30% and LVED > 4.8
cmcm
 Of 32 patientsOf 32 patients
 13 (41%) recovered13 (41%) recovered
 19 (59%) - persistent LVD19 (59%) - persistent LVD
 Those who did not recover had a higher LVED and aThose who did not recover had a higher LVED and a
lower FS at diagnosislower FS at diagnosis
 A FS< 20% and a LVED > 6 cm at the time of diagnosisA FS< 20% and a LVED > 6 cm at the time of diagnosis
was associated with a more than 3-fold higher risk forwas associated with a more than 3-fold higher risk for
persistent LVDpersistent LVD
Risk stratification with DSERisk stratification with DSEDorbalaDorbala
S et al, J Am SocS et al, J Am Soc EchocardiogrEchocardiogr.  2005; 18(1):45-8.  2005; 18(1):45-8  
 7 women with PPCM and (mean LVEF 25.3 +/- 9.5%)7 women with PPCM and (mean LVEF 25.3 +/- 9.5%)
 6 underwent DSE at baseline and a follow-up resting6 underwent DSE at baseline and a follow-up resting
echo at a mean of 4.7 +/- 0.9 months after initialecho at a mean of 4.7 +/- 0.9 months after initial
presentation.presentation.
 The mean EF improved significantly from baselineThe mean EF improved significantly from baseline
(~25.3 ) to maximal inotropic contractile reserve (~ 53.8 )(~25.3 ) to maximal inotropic contractile reserve (~ 53.8 )
(P = .0004) and at follow-up ( ~ 53.0 ) (P = .006).(P = .0004) and at follow-up ( ~ 53.0 ) (P = .006).
Importantly, LVEF at maximal inotropic contractileImportantly, LVEF at maximal inotropic contractile
reserve and at follow-up (5.6 months) did not differreserve and at follow-up (5.6 months) did not differ
significantly (P = .5)significantly (P = .5)
Future Pregnancies?Future Pregnancies?
 Most experts agree that patients shouldMost experts agree that patients should
avoid future pregnancy if LV dysfunctionavoid future pregnancy if LV dysfunction
is persistent greater than 6 monthsis persistent greater than 6 months
Maternal and Fetal Outcomes ofMaternal and Fetal Outcomes of
Subsequent Pregnancies in Women withSubsequent Pregnancies in Women with
PPCMPPCM
Elkayam et al,Elkayam et al, N Engl J Med 344:1567, 2001N Engl J Med 344:1567, 2001
44 women who had had PPCM and had a total of 60 subsequent44 women who had had PPCM and had a total of 60 subsequent
pregnanciespregnancies
RECOVERED 28RECOVERED 28 LVD 16LVD 16
reduction in EFreduction in EF ~ 56 % to~ 49% P=0.002~ 56 % to~ 49% P=0.002 ~36% to ~32 %, P=0.08)~36% to ~32 %, P=0.08)
symptoms of HFsymptoms of HF 21 %21 % 44 %44 %
Maternal mortalityMaternal mortality nilnil 19 %19 %
premature deliverypremature delivery 11%11% 37%37%
therapeutic abortionstherapeutic abortions 4%4% 25%25%
Who recovered
Who did not
 Lampert et al.,who, with the use of DSE,Lampert et al.,who, with the use of DSE,
reported impaired contractile reserve in patientsreported impaired contractile reserve in patients
who had appeared to recover from PPCM andwho had appeared to recover from PPCM and
who had normal EF at rest. Thus, thewho had normal EF at rest. Thus, the
hemodynamic stress of a pregnancy mayhemodynamic stress of a pregnancy may
unmask impaired contractile reserve that is notunmask impaired contractile reserve that is not
apparent at rest.apparent at rest.
 Lampert et al. Am J Obstet Gynecol 1997;176:189-195Lampert et al. Am J Obstet Gynecol 1997;176:189-195
Future Pregnancies cont…Future Pregnancies cont…
 Highly IndividualHighly Individual
 Patient education of risksPatient education of risks
 If future pregnancy desired:If future pregnancy desired:
 Maternal Echocardiogram per trimesterMaternal Echocardiogram per trimester
 Serial sonograms for growthSerial sonograms for growth
PPCM EF < 45%,
FS < 30% or both, and
LVED > 2.7 cm per sq m of BSA
HF in last m of preg, or < 5 m pp
Absence of a determinable cause of HF
Absence of heart disease before
LVD demonstrated on Echo
4% of all cardiomyopathy
1:4000 to 1:15000 deliveries
Dilated Cardiomyopathy
Age >30 years
Multiparity
Pregnancy with multiple fetuses
H/O Preeclampsia, eclampsia, or HTN
Long term tocolytic therapy (>4weeks)
African Descent
PND
DOE
Cough
Orthopnea
Chest Pain
Abdominal Discomfort
Palpitation
incidence
Risk Factors
Diagnostic Criteria
symptoms
investigations
prognosis
Future preg.
EKG,
CXR,
Echo.,
DSE
CBC,
Trop T,
BNP,
TSH
25-50 % recover
15-20% mortality
Rest persistent LV dys.
Those who recovered
~ 20% will have symptoms of HF
Mortality nil
Those with LVD
~ 44% symptoms of HF
~20% mortality
Thanks

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Peripartum Cardiomyopathy: A Review

  • 1. PERIPARTUM CARDIOMYOPATHY DR NISHANT TYAGI MODERATOR : DR B N SHAHI Escorts Heart Institute And Research CentreEscorts Heart Institute And Research Centre
  • 2. Review of Cardiac Changes inReview of Cardiac Changes in PregnancyPregnancy  Increase in blood volume by 20 – 100 %Increase in blood volume by 20 – 100 %  Starts as early as 6Starts as early as 6thth weekweek  Rises rapidly thru 32-34 weeks then a modest riseRises rapidly thru 32-34 weeks then a modest rise  Net result = 1100 – 1600 cc increase above baselineNet result = 1100 – 1600 cc increase above baseline  Increase in TBV due to:Increase in TBV due to:  Increased vascular capacitanceIncreased vascular capacitance  Systemic vasodilatationSystemic vasodilatation
  • 3. Review cont….Review cont….  CO rises by ~ 50 %CO rises by ~ 50 %  Due to 3 important factors:Due to 3 important factors:  Preload is increased due to increase in TBVPreload is increased due to increase in TBV  Afterload is reduced due to decreased SVRAfterload is reduced due to decreased SVR  Maternal HR rises 15-20 bpmMaternal HR rises 15-20 bpm
  • 4.
  • 5.
  • 6. *Chapman et al.*Chapman et al. Kidney Int 1998; 54:2056Kidney Int 1998; 54:2056
  • 7.
  • 8.
  • 9.
  • 10.
  • 11. What is a PPCM?What is a PPCM?  PPCM was defined clinically as the onset of cardiac failure with noPPCM was defined clinically as the onset of cardiac failure with no identifiable cause in the last month of pregnancy or within fiveidentifiable cause in the last month of pregnancy or within five months after delivery, in the absence of heart disease before themonths after delivery, in the absence of heart disease before the last month of pregnancy.last month of pregnancy. * In 1997, National Heart, Lung, and Blood Institute (NHLBI) workshop* In 1997, National Heart, Lung, and Blood Institute (NHLBI) workshop  Stricter echocardiographic criteria have been recommendedStricter echocardiographic criteria have been recommended  EF < 45%,EF < 45%,  FS < 30% or both, andFS < 30% or both, and  LVED > 2.7 cm per square meter of BSALVED > 2.7 cm per square meter of BSA * Obstet Gynecol 1999;94:311-316* Obstet Gynecol 1999;94:311-316
  • 12. Criteria for DiagnosisCriteria for Diagnosis  4 Criteria  Development of HF in the last month of pregnancy, or within 5 months postpartum  Absence of a determinable cause for cardiac failure  Absence of heart disease before last month of pregnancy  LV functional impairment demonstrated on Echo
  • 13. Pregnancy-associated cardiomyopathy:Pregnancy-associated cardiomyopathy: clinical characteristics and a comparisonclinical characteristics and a comparison between early and late presentation.between early and late presentation.   Circulation.  2005; 111(16):2050-5Circulation.  2005; 111(16):2050-5 Elkayam et al.Elkayam et al.  123 women123 women  100 traditional PPCM100 traditional PPCM  23 pregnancy-associated cardiomyopathy earlier than the last23 pregnancy-associated cardiomyopathy earlier than the last gestational month.gestational month.  associated conditionsassociated conditions  gestational hypertension (43%)gestational hypertension (43%)  tocolytic therapy (19%), andtocolytic therapy (19%), and  twin pregnancy (13%)twin pregnancy (13%)  Normal LV recovery - 54%Normal LV recovery - 54%  Maternal mortality - 9%.Maternal mortality - 9%.  A comparison showed no differences in age, race, associatedA comparison showed no differences in age, race, associated conditions, LV EF at diagnosis, its rate and time of recovery, andconditions, LV EF at diagnosis, its rate and time of recovery, and maternal outcome.maternal outcome.  These 2 conditions may represent a continuum of a spectrum of theThese 2 conditions may represent a continuum of a spectrum of the same disease.same disease.
  • 14. Cont…..Cont…..  Overall responsibleOverall responsible for 10,000 deaths andfor 10,000 deaths and 46,00046,000 hospitalizations eachhospitalizations each yearyear  Wide age range 20-Wide age range 20- 6060 *Dec et al. N Engl J Med 1994; 331:1564*Dec et al. N Engl J Med 1994; 331:1564  Common Sx:Common Sx:  Progressive dyspneaProgressive dyspnea with exertionwith exertion  Impaired exerciseImpaired exercise capacitycapacity  OrthopneaOrthopnea  Paroxysmal nocturnalParoxysmal nocturnal dyspneadyspnea  Peripheral edemaPeripheral edema
  • 15. *Felker et al. N Engl J Med 2000; 342:1077*Felker et al. N Engl J Med 2000; 342:1077
  • 16. Causes of Cardiomyopathies 50 9 5 4 4 4 3 3 1 10 0 10 20 30 40 50 60Idiopathic Ischem ic H eartD isease Infiltrative D isease P eripartum H TN H IV C onnective Tissue S ubstance A buse D oxorubicin O ther
  • 17. Peripartum CardiomyopathyPeripartum Cardiomyopathy  4% of all4% of all cardiomyopathiescardiomyopathies  1:4000 to 1:150001:4000 to 1:15000 deliveriesdeliveries  DilatedDilated CardiomyopathyCardiomyopathy
  • 18. Five-year prospective study of the incidenceFive-year prospective study of the incidence and prognosis of PPCM at a singleand prognosis of PPCM at a single institution.institution.   MayoMayo ClinClin Proc.  2005; 80(12):1602-6Proc.  2005; 80(12):1602-6 Department of Adult Medicine, HospitalDepartment of Adult Medicine, Hospital Albert Schweitzer, HaitiAlbert Schweitzer, Haiti  1 case per 300 live births,1 case per 300 live births,  28% of 92 patients regained normal LV function at 6 m28% of 92 patients regained normal LV function at 6 m  mortality - 15.3%mortality - 15.3%  difference in echo at diagnosis between deceased patients anddifference in echo at diagnosis between deceased patients and survivors was not statistically significant: mean LVED (6.2 vs 5.8survivors was not statistically significant: mean LVED (6.2 vs 5.8 cm; P=.08), EF (22% vs 25%; P=.12), FS (16% vs 15%; P=.46 )cm; P=.08), EF (22% vs 25%; P=.12), FS (16% vs 15%; P=.46 )  echo features at diagnosis were unable to predict death, although aecho features at diagnosis were unable to predict death, although a statistically significant difference occurred at diagnosis between thestatistically significant difference occurred at diagnosis between the recovered group and nonrecovered group for mean EF (28% vsrecovered group and nonrecovered group for mean EF (28% vs 23%; P<.001) and FS (17% vs 14%; P=.004)23%; P<.001) and FS (17% vs 14%; P=.004)
  • 19. Should we be concerned?Should we be concerned?  Yes!Yes!  Pregnancy Related Mortality SurveillancePregnancy Related Mortality Surveillance 1991-19991991-1999  Leading Causes of Maternal Mortality:Leading Causes of Maternal Mortality:  Embolism – 20%Embolism – 20%  Hemorrhage – 17%Hemorrhage – 17%  Hypertension – 16%Hypertension – 16%  Peripartum Cardiomyopathy- 9%*Peripartum Cardiomyopathy- 9%*
  • 20. EtiologyEtiology  Multiple studies have attempted toMultiple studies have attempted to elucidate a distinct etiology…..all haveelucidate a distinct etiology…..all have failedfailed  Theories:Theories:  MyocarditisMyocarditis  Abnormal Immune ResponseAbnormal Immune Response  GeneticsGenetics  Latent cardiomyopathyLatent cardiomyopathy
  • 21. Myocarditis?Myocarditis?  Nairobi Study1986Nairobi Study1986  11 African women with PPCM11 African women with PPCM  Endocardial biopsies done on all elevenEndocardial biopsies done on all eleven  5 showed evidence of “healing myocarditis”5 showed evidence of “healing myocarditis”  Presence of inflammatory cellsPresence of inflammatory cells  NecrosisNecrosis  Fibrous remodelingFibrous remodeling  9 patients finished study9 patients finished study  75% of myocarditis group developed persistent HF75% of myocarditis group developed persistent HF  80% of patients without myocarditis improved80% of patients without myocarditis improved **Sanderson et al. Br Heart J 1986: 56:285
  • 22. Myocarditis? Cont…Myocarditis? Cont…  Another study 1986:Another study 1986:  84 women with cardiomyopathies84 women with cardiomyopathies  14 diagnosed as being PPCM14 diagnosed as being PPCM  29% of patients with PPCM were found to have29% of patients with PPCM were found to have myocarditismyocarditis  Only 9% of idiopathic CM related to myocarditisOnly 9% of idiopathic CM related to myocarditis *O’Connell et al. J AM Coll Cardiol 1986; 8:52*O’Connell et al. J AM Coll Cardiol 1986; 8:52
  • 23. Myocarditis? Cont….Myocarditis? Cont….  33rdrd Study 1990:Study 1990:  18 patients with PPCM18 patients with PPCM  14 due to myocarditis14 due to myocarditis  10 of these received immunosuppressive Tx over 6-810 of these received immunosuppressive Tx over 6-8 weeks, then tapered over 6-8 weeksweeks, then tapered over 6-8 weeks  9 of 10 improved on therapy9 of 10 improved on therapy  However, 4 of 4 not receiving therapy also improvedHowever, 4 of 4 not receiving therapy also improved **Midei et al. Circulation 1990; 81:922Midei et al. Circulation 1990; 81:922
  • 24. Myocarditis? Cont….Myocarditis? Cont….  1994 Retrospective study1994 Retrospective study  34 patients diagnosed with PPCM34 patients diagnosed with PPCM  Researches found lower incidence ofResearches found lower incidence of myocarditis than previously reportedmyocarditis than previously reported  8.8 % due to myocarditis8.8 % due to myocarditis  * Rizeq et al. Am J Cardiol 1994; 74:474* Rizeq et al. Am J Cardiol 1994; 74:474
  • 25. Myocarditis? Cont….Myocarditis? Cont….  In 1995 a randomized trial ofIn 1995 a randomized trial of immunosuppressive Tx in a related populationimmunosuppressive Tx in a related population with myocarditis did not prove efficacy in thewith myocarditis did not prove efficacy in the Myocarditis Treatment TrialMyocarditis Treatment Trial,, *Mason JW , N Engl J Med 1995;333:269-275.*Mason JW , N Engl J Med 1995;333:269-275.  In 2001 a similar fashion therapy with immuneIn 2001 a similar fashion therapy with immune globulin did not improve outcomes in aglobulin did not improve outcomes in a randomized trial of patients with acuterandomized trial of patients with acute cardiomyopathy (IMAC Trial)cardiomyopathy (IMAC Trial) *McNamara et al, Circulation 2001;103:2254-2259.*McNamara et al, Circulation 2001;103:2254-2259.
  • 26. Abnormal Immune Response?Abnormal Immune Response?  Maternal immunologic response to a fetalMaternal immunologic response to a fetal antigen?antigen?  Fetal cells may escape into the maternalFetal cells may escape into the maternal circulation without being rejected.circulation without being rejected.  May become lodged in cardiac tissue.May become lodged in cardiac tissue.  May trigger immune responseMay trigger immune response *Nelson et al. J Am Med Womens Assoc 1998; 53:31*Nelson et al. J Am Med Womens Assoc 1998; 53:31
  • 27. Immune Response? Cont….Immune Response? Cont….  Disproved 1990., Nigerian StudyDisproved 1990., Nigerian Study  39 women with PPCM39 women with PPCM  No differences between subjects and controlsNo differences between subjects and controls in levels of:in levels of:  Serum ImmunoglobulinsSerum Immunoglobulins  Circulating Immune ComplexesCirculating Immune Complexes  Cardiac muscle antibodiesCardiac muscle antibodies *Cenac et al. Int J Cardiol 1990; 26:49*Cenac et al. Int J Cardiol 1990; 26:49
  • 28. Peripartum cardiomyopathy: inflammatory markersPeripartum cardiomyopathy: inflammatory markers as predictors of outcome in 100 prospectivelyas predictors of outcome in 100 prospectively studied patients.studied patients.EurEur Heart J.  2006; 27(4):441-6Heart J.  2006; 27(4):441-6  levels of C-reactive protein correlated positively withlevels of C-reactive protein correlated positively with baseline LVED (P=0.0026) and LVES(P=0.0012)baseline LVED (P=0.0026) and LVES(P=0.0012) diameters and inversely with LVEF (P=0.015).diameters and inversely with LVEF (P=0.015).  Patients who died presented with significantly lowerPatients who died presented with significantly lower mean EF and higher Fas/Apo-1 plasma values (P<0.05)mean EF and higher Fas/Apo-1 plasma values (P<0.05)  Baseline Fas/Apo-1 and higher NYHA FC were the onlyBaseline Fas/Apo-1 and higher NYHA FC were the only predictors of mortalitypredictors of mortality
  • 29. GeneticsGenetics  Several case reports publishedSeveral case reports published  1963, Pierce et al. reported that 3 of 17 patients with PPCM had1963, Pierce et al. reported that 3 of 17 patients with PPCM had definitive FH of same conditiondefinitive FH of same condition  Also, 1976Also, 1976 StrungStrung documented male relatives of female patients withdocumented male relatives of female patients with PPCM as also having cardiomyopathies.PPCM as also having cardiomyopathies.  1984 Voss et al.1984 Voss et al. reported a patient who died from PPCM as didreported a patient who died from PPCM as did her mother and two of her sistersher mother and two of her sisters  1993 Massad et al.1993 Massad et al. reported 16 y.o girl with PPCM followingreported 16 y.o girl with PPCM following molar preg. Sister later received cardiac transplant for PPCM.molar preg. Sister later received cardiac transplant for PPCM.
  • 30. Latent cardiomyopathyLatent cardiomyopathy  Hypertension duringHypertension during pregnancy, pre-pregnancy, pre- eclampsia, are frequenteclampsia, are frequent in women with PPCM andin women with PPCM and do support thatdo support that hemodynamic stresseshemodynamic stresses may play a rolemay play a role  However, absence ofHowever, absence of cardiac symptoms incardiac symptoms in PPCM until thePPCM until the postpartum period arguespostpartum period argues against the "latentagainst the "latent cardiomyopathy" theory.cardiomyopathy" theory.
  • 31. Risk FactorsRisk Factors  Age >30 yearsAge >30 years  MultiparityMultiparity  Pregnancy with multiplePregnancy with multiple fetusesfetuses  H/O Preeclampsia,H/O Preeclampsia, eclampsia, or postpartumeclampsia, or postpartum HTNHTN  Long term tocolyticLong term tocolytic therapy (>4weeks)therapy (>4weeks)  African DescentAfrican Descent  Maternal cocaine abuseMaternal cocaine abuse
  • 32. Clinical PresentationClinical Presentation  Symptoms:Symptoms:  Paroxysmal NocturnalParoxysmal Nocturnal DyspneaDyspnea  Dyspnea on ExertionDyspnea on Exertion  CoughCough  OrthopneaOrthopnea  Chest PainChest Pain  Abdominal DiscomfortAbdominal Discomfort  PalpitationPalpitation  Signs:Signs:  Raised JVPRaised JVP  CardiomegalyCardiomegaly  Gallop RhythmGallop Rhythm  Holosystolic murmurHolosystolic murmur  EdemaEdema
  • 34. DiagnosisDiagnosis  EKGEKG  Two-dimensional echocardiogramTwo-dimensional echocardiogram  Lab: CBC, BNP, TSH, FerritinLab: CBC, BNP, TSH, Ferritin  CXRCXR  ? Myocardial biopsy? Myocardial biopsy
  • 35. EKG ChangesEKG Changes  Sinus TachycardiaSinus Tachycardia  Nonspecific STNonspecific ST changeschanges  LV HypertrophyLV Hypertrophy
  • 36. Chest X-rayChest X-ray  Pulmonary EdemaPulmonary Edema  Venous congestionVenous congestion  Enlarged CardiacEnlarged Cardiac SilhouetteSilhouette  R/O PER/O PE
  • 37. EchocardiogramEchocardiogram  Spherical LVSpherical LV  Mitral and TricuspidMitral and Tricuspid regurgitationregurgitation  Left AtrialLeft Atrial enlargementenlargement  EF <55%EF <55%
  • 38. TreatmentTreatment  Delivery (consider elective caesarian with invasiveDelivery (consider elective caesarian with invasive hemodynamic monitoring of the mother)hemodynamic monitoring of the mother)  Similar to other forms of CHFSimilar to other forms of CHF  DiureticsDiuretics  ß-blockersß-blockers  ACE - IACE - I  DigoxinDigoxin  AnticoagulantsAnticoagulants ** Must consider pregnancy class/breast-feeding harm potential!
  • 39. Pregnancy Drug Class ReviewPregnancy Drug Class Review  Category A: Controlled studies in pregnant women fail to demonstrate a riskCategory A: Controlled studies in pregnant women fail to demonstrate a risk to the fetus in the first trimester with no evidence of risk in later trimesters.to the fetus in the first trimester with no evidence of risk in later trimesters. The possibility of harm appears remoteThe possibility of harm appears remote  Category B: Presumed safety based on animal studies, with no controlledCategory B: Presumed safety based on animal studies, with no controlled studies in pregnant women,studies in pregnant women, oror animal studies have shown an adverseanimal studies have shown an adverse effect that was not confirmed in controlled studies in women in the firsteffect that was not confirmed in controlled studies in women in the first trimester and there is no evidence of a risk in later trimesterstrimester and there is no evidence of a risk in later trimesters..  Category C:Category C: Studies in women and animals are not availableStudies in women and animals are not available oror studies instudies in animals have revealed adverse effects on the fetus and there are noanimals have revealed adverse effects on the fetus and there are no controlled studies in women. Drugs should be given only if the potentialcontrolled studies in women. Drugs should be given only if the potential benefits justify the potential risk to the fetusbenefits justify the potential risk to the fetus  Category D: There is positive evidence of human fetal risk (unsafe),Category D: There is positive evidence of human fetal risk (unsafe), however in some cases such as a life-threatening illness the potential riskhowever in some cases such as a life-threatening illness the potential risk may be justified if there are no other alternativesmay be justified if there are no other alternatives  Category X: Highly unsafe: risk of use outweighs any potential benefit.Category X: Highly unsafe: risk of use outweighs any potential benefit. Drugs in this category are contraindicated in women who are or mayDrugs in this category are contraindicated in women who are or may become pregnantbecome pregnant
  • 40. DrugsDrugs  Digoxin Class CDigoxin Class C  Symptomatic controlSymptomatic control  Requires levelRequires level monitoringmonitoring  Therapeutic levels 0.7-Therapeutic levels 0.7- 1.21.2
  • 41. DiureticsDiuretics  Lasix Class CLasix Class C  Reserved for cardiacReserved for cardiac conditionsconditions  Not recommended inNot recommended in PIHPIH  May decreaseMay decrease placental perfusionplacental perfusion  Thiazide DiureticsThiazide Diuretics  Reserved for cardiacReserved for cardiac conditionsconditions  Not recommended inNot recommended in PIHPIH  Thrombocytopenia hasThrombocytopenia has been reported inbeen reported in breast feeding infantsbreast feeding infants
  • 42. VasodilatorsVasodilators  Hydralazine Class CHydralazine Class C  Compatible withCompatible with breastfeedingbreastfeeding  ACE InhibitorsACE Inhibitors  Class D in 2Class D in 2ndnd /3/3rdrd trimesterstrimesters  Reserved forReserved for postpartum use-postpartum use- compatible with BFcompatible with BF  Renal toxicity inRenal toxicity in infants exposed ininfants exposed in uteroutero
  • 43. Beta-BlockersBeta-Blockers  Class CClass C  Compatible with breast feedingCompatible with breast feeding  Has been shown to cause IUGR in someHas been shown to cause IUGR in some infants in utero.infants in utero.
  • 44. AntiarrhythmicsAntiarrhythmics  Presenting with sudden death or VT with hemodynamicPresenting with sudden death or VT with hemodynamic compromise – ICDcompromise – ICD  VT without hemodynamic compromise , managementVT without hemodynamic compromise , management can be tempered somewhat because of the potentialcan be tempered somewhat because of the potential transient nature of the myopathy and amiodaronetransient nature of the myopathy and amiodarone therapy should be startedtherapy should be started  Asymptomatic NSVT, no amiodarone therapy, focus onAsymptomatic NSVT, no amiodarone therapy, focus on correction of metabolic abnormalities and consider thecorrection of metabolic abnormalities and consider the addition of a beta receptor antagonistaddition of a beta receptor antagonist
  • 45. AnticoagulantsAnticoagulants  Heparin Class CHeparin Class C  Short half life-can beShort half life-can be discontinued prior todiscontinued prior to delivery to preventdelivery to prevent maternal hemorrhagematernal hemorrhage  Not excreted in breastNot excreted in breast milkmilk  Warfarin Class DWarfarin Class D  Contraindicated inContraindicated in pregnancypregnancy  Safe in breast feeding.Safe in breast feeding. Not excreted in breastNot excreted in breast milk.milk. Indications:-Indications:- 1.evidence of a systemic embolus1.evidence of a systemic embolus 2.severe left ventricular dysfunction with2.severe left ventricular dysfunction with documented mural thrombusdocumented mural thrombus
  • 46. Cardiac TransplantationCardiac Transplantation  Estimated that transplant is performed in up toEstimated that transplant is performed in up to 1/3 of PPCM patients1/3 of PPCM patients  Pts should be strongly advised against futurePts should be strongly advised against future pregnancies.pregnancies.  Increased risk of HTN, preeclampsia, and pretermIncreased risk of HTN, preeclampsia, and preterm laborlabor  Also at risk for graft failure due to recurrentAlso at risk for graft failure due to recurrent disease.disease. *Scott et al. Obstet Gynecol 1993; 82:324*Scott et al. Obstet Gynecol 1993; 82:324
  • 47. Differential DiagnosisDifferential Diagnosis  PIHPIH  However, HF associated with PIH representsHowever, HF associated with PIH represents a diastolic failure, vs. systolic in PPCMa diastolic failure, vs. systolic in PPCM  Pulmonary EmbolismPulmonary Embolism  Again, usually ruled out by CXRAgain, usually ruled out by CXR  If still suspicious, can order spiral CTIf still suspicious, can order spiral CT
  • 48. PrognosisPrognosis  Mortality estimates range from 25-50%.Mortality estimates range from 25-50%.  Most deaths occur within 3 monthsMost deaths occur within 3 months postpartumpostpartum  Deaths usually caused by:Deaths usually caused by:  Progressive pump failureProgressive pump failure  ArrhythmiasArrhythmias  Thromboembolic eventsThromboembolic events
  • 49. Prognostic value of echocardiographyPrognostic value of echocardiography Chapa JB et al,Chapa JB et al, ObstetObstet GynecolGynecol.  2005; 105(6):1303-8.  2005; 105(6):1303-8  FS and LVED, at the time of diagnosis and at follow-up.FS and LVED, at the time of diagnosis and at follow-up. LVD was defined by echo as FS < 30% and LVED > 4.8LVD was defined by echo as FS < 30% and LVED > 4.8 cmcm  Of 32 patientsOf 32 patients  13 (41%) recovered13 (41%) recovered  19 (59%) - persistent LVD19 (59%) - persistent LVD  Those who did not recover had a higher LVED and aThose who did not recover had a higher LVED and a lower FS at diagnosislower FS at diagnosis  A FS< 20% and a LVED > 6 cm at the time of diagnosisA FS< 20% and a LVED > 6 cm at the time of diagnosis was associated with a more than 3-fold higher risk forwas associated with a more than 3-fold higher risk for persistent LVDpersistent LVD
  • 50. Risk stratification with DSERisk stratification with DSEDorbalaDorbala S et al, J Am SocS et al, J Am Soc EchocardiogrEchocardiogr.  2005; 18(1):45-8.  2005; 18(1):45-8    7 women with PPCM and (mean LVEF 25.3 +/- 9.5%)7 women with PPCM and (mean LVEF 25.3 +/- 9.5%)  6 underwent DSE at baseline and a follow-up resting6 underwent DSE at baseline and a follow-up resting echo at a mean of 4.7 +/- 0.9 months after initialecho at a mean of 4.7 +/- 0.9 months after initial presentation.presentation.  The mean EF improved significantly from baselineThe mean EF improved significantly from baseline (~25.3 ) to maximal inotropic contractile reserve (~ 53.8 )(~25.3 ) to maximal inotropic contractile reserve (~ 53.8 ) (P = .0004) and at follow-up ( ~ 53.0 ) (P = .006).(P = .0004) and at follow-up ( ~ 53.0 ) (P = .006). Importantly, LVEF at maximal inotropic contractileImportantly, LVEF at maximal inotropic contractile reserve and at follow-up (5.6 months) did not differreserve and at follow-up (5.6 months) did not differ significantly (P = .5)significantly (P = .5)
  • 51.
  • 52. Future Pregnancies?Future Pregnancies?  Most experts agree that patients shouldMost experts agree that patients should avoid future pregnancy if LV dysfunctionavoid future pregnancy if LV dysfunction is persistent greater than 6 monthsis persistent greater than 6 months
  • 53. Maternal and Fetal Outcomes ofMaternal and Fetal Outcomes of Subsequent Pregnancies in Women withSubsequent Pregnancies in Women with PPCMPPCM Elkayam et al,Elkayam et al, N Engl J Med 344:1567, 2001N Engl J Med 344:1567, 2001 44 women who had had PPCM and had a total of 60 subsequent44 women who had had PPCM and had a total of 60 subsequent pregnanciespregnancies RECOVERED 28RECOVERED 28 LVD 16LVD 16 reduction in EFreduction in EF ~ 56 % to~ 49% P=0.002~ 56 % to~ 49% P=0.002 ~36% to ~32 %, P=0.08)~36% to ~32 %, P=0.08) symptoms of HFsymptoms of HF 21 %21 % 44 %44 % Maternal mortalityMaternal mortality nilnil 19 %19 % premature deliverypremature delivery 11%11% 37%37% therapeutic abortionstherapeutic abortions 4%4% 25%25%
  • 55.  Lampert et al.,who, with the use of DSE,Lampert et al.,who, with the use of DSE, reported impaired contractile reserve in patientsreported impaired contractile reserve in patients who had appeared to recover from PPCM andwho had appeared to recover from PPCM and who had normal EF at rest. Thus, thewho had normal EF at rest. Thus, the hemodynamic stress of a pregnancy mayhemodynamic stress of a pregnancy may unmask impaired contractile reserve that is notunmask impaired contractile reserve that is not apparent at rest.apparent at rest.  Lampert et al. Am J Obstet Gynecol 1997;176:189-195Lampert et al. Am J Obstet Gynecol 1997;176:189-195
  • 56. Future Pregnancies cont…Future Pregnancies cont…  Highly IndividualHighly Individual  Patient education of risksPatient education of risks  If future pregnancy desired:If future pregnancy desired:  Maternal Echocardiogram per trimesterMaternal Echocardiogram per trimester  Serial sonograms for growthSerial sonograms for growth
  • 57. PPCM EF < 45%, FS < 30% or both, and LVED > 2.7 cm per sq m of BSA HF in last m of preg, or < 5 m pp Absence of a determinable cause of HF Absence of heart disease before LVD demonstrated on Echo 4% of all cardiomyopathy 1:4000 to 1:15000 deliveries Dilated Cardiomyopathy Age >30 years Multiparity Pregnancy with multiple fetuses H/O Preeclampsia, eclampsia, or HTN Long term tocolytic therapy (>4weeks) African Descent PND DOE Cough Orthopnea Chest Pain Abdominal Discomfort Palpitation incidence Risk Factors Diagnostic Criteria symptoms investigations prognosis Future preg. EKG, CXR, Echo., DSE CBC, Trop T, BNP, TSH 25-50 % recover 15-20% mortality Rest persistent LV dys. Those who recovered ~ 20% will have symptoms of HF Mortality nil Those with LVD ~ 44% symptoms of HF ~20% mortality