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RadhakRishnan RamaRaj, mdDepartment of Internal Medicine, University ofArizona College of Medicine, Tucson, AZ
Vincent L. soRReLL, mdProfessor of Clinical Medicine, Pediatrics, andRadiology, The Allan C. Hudson and Helen LovaasEndowed Chair of Cardiovascular Imaging, Sectionof Cardiology, University of Arizona Sarver HeartCenter, Tucson, AZ
Peripartum cardiomyopathy:Causes, diagnosis, and treatment
AbstrAct
Peripartum cardiomyopathy is a life-threatening condi-
tion of unknown cause that occurs in previously healthywomen during the peripartum period. It is characterizedby left ventricular dysfunction and symptoms of heartfailure that can arise in the last trimester of pregnancyor up to 5 months after delivery. We review its possiblecauses and how to recognize and manage it.
KEY POINts
Heightened suspicion is important when a pregnantwoman presents with signs of heart failure, becauseearly diagnosis allows proven treatment to be started.
Standard heart failure therapy should be started in post-partum patients with this disease, using available localprotocols.
Pregnant women should not receive angiotensin-con-verting enzyme inhibitors, angiotensin receptor blockers,or warfarin because of potential teratogenic effects.
An initial left ventricular end-systolic dimension less than
5.5 cm, a left ventricular ejection fraction greater than30%, and a low cardiac troponin level may predicta better outcome.
Subsequent pregnancies carry a high risk of relapse,even in women who have fully recovered leftventricular function.
CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 76 NUMBER 5 MAY 2009 289
Heart ailure during pregnancy wsoz s s 1849, b ws s
sb s sv om o omo
h o h 1930s.1
I 1971, Dmks 2 sb 27 s who s h m wh om, bomooh s, osvh , m h somperipar-tum cardiomyopathy.
Th Eo So o Coo3 m omoh s om o omoh h sswh ss o h h s moh o o wh 5 mohs o v.
Pm omoh s v
b b h. Th NoHos Dsh Sv (19902002) s m h os 1 v 2,289 vbhs h U Ss.4 Th ss s o b mo ommo A Amwom.1 Th vs oh oos: s hhs H, wh 1 s 300 vbhs, whh s 10 ms hh h h U Ss.5 Th so o sh vo ms .
Ahoh os ss h h ws 50%, mo os show
o b 0 o 5% h U Ss, hhh mbs h os ks bo bs.5,69
What causes It?
Pm omoh s o s om o oh m mo ss sso wh h s.Ahoh sv sb oo mh sms hv b ss, o o hm s
. Som sss bow.
mEdIcAl PrOblEms IN PrEgNANcY
doi:10.3949/ccjm.76a.08004
EDUCATIONAL OBJECTIVE: Readers will suspect peripartum cardiomyopathy in patients who developunexplained heart ailure symptoms during the peripartum periodCREDIT
CME
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290 CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 76 NUMBER 5 MAY 2009
PErIPArtum cArdIOmYOPAthY
MoriiMos hs b o o omo bos o h h v s whm omoh,10,11 wh s
mho vb moso mo m, oss, boss. Thv o mos s whm omoh om 8.8%o 78% ss.12,13 O h ohh, h s o bs o moso os o h oom o m omoh.7
crioropi vir iioA v o, hoo mmsos mh o h s o
s v ss os, o v so.
Bm 14 o vovs B19,hm hs vs 6, Es B vs, oomovs DNA omo b os sms om 8 (31%) o 26 swh m omoh h ws s so mmohsoo wh s fmmo.
Kh 15 o, s wh vo om b omo b os, h h m v joo mov hos whom h v s ws (om 50.2% bo o 58.1%w, P < .001), whs s hos whom h vs ss (om 54.3%bo o 51.4% w, P < .01).
L Hb16 o h m vo wos mos h w x m wh oxskvs hovs h h w wh o .
cimrimI homo hmsm, s omh s k s h moh (ov vs), somms ovok mmsos.17,18
As vw b As ,19 h smom s wh m omo h hs b o o o obos hh s, whh o s smom s wh oh omo h. Mos o hs bos s o m hm ss os o 37, 33,
25 kD. Th h boo hs
s hs hh v o mohmsm moo s, bom oko, ow vs o CD4+ CD25o o T s.
Wh ,20 s om Soh A , Mozmbq, H, o h hqs vs o mmoob s w sm sbo swh m omoh, svo h oh oo.
apopoi ifmmioAooss (omm h) o mos os h mob o ossv mo s o.21 Exms m ss h o
oss o mos hs o m omoh.22
Fs Fs s o s h k o ooss. Sw ,23 s , osv, o s om Soh A, oow 100s wh m omoh o6 mohs. D hs m 15 s , hos who h s hhsm vs o Fs/Ao 1 ( P < .05). I hsm s, sm vs o C v o mo oss o h (mkso fmmo) w v owh hh v msos ow v jo os so.
I h Ss o L VDso,24 vs o mo oss o h k 6 s s s h o h sso o.
a borm momi rpoD , boo vom o s. I o, o s s bs o xo o vs smoohms. Th ss vom o s s vsb hoh o h v om h s o h moh s. C o hs s mxmm o 20wks o .25
Th s v sso s o h h ms osm o s o bs o
h o ss.26,27
the deah rae
in hi dieae
i much lower
han previouly
repored
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CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 76 NUMBER 5 MAY 2009 291
rAmArAJ ANd sOrrEll
Or poib orOh ossb oo os o,28,29 x,30 mm omxs,31
ox shs,32 mm
s,33 soh,34 o kos.35
WhO Is at RIsK?
Dmks os2 ss h oowsk os o m omoh:
MAv m (hoh hss o , h s hh wom ov 3036)M
PmsGso hsoA Am .
clInIcal eatuRes
Pm omoh vovs v sso so wom wh ohso o h ss. I b oso oh ss o omoh b s.2
Diagnostic criteria o m o moh ( ms b s) 37:
C vo h smoh o o wh 5 mohs ov
No b s o h No ozb h ss bo hs moh o A jo o o ss h 45%, oh ombo o M mo osho o ss h 30% so mso h 2.7 m/m2.
Symptoms of heart failure sh s s,zzss, m, oho o v om s. Tho, wom whom m o moh s vo m os h sm oms o b om. Th s om s hoh o b o hv o s b h s o oso, so o ss o h hm omh ow s.38 Ph m os oxm wo hs o hh wom.39 Nvhss, sw
oh h smoms vo s
ohws om , hs shoom h vso.40
Pmo m ws s sm om 106 s 2007 s Ch
.41 Th so ws sm oh o osv h b ws hhvb; 17% o ss w os m 83% osm. Th m oss ws 28 6 s. L v o mos om omz 51% osvv s. Ths s w smo hos ss.2,36 Is, h v jo o omzo 23% o A oho.23
Thromboembolism b soo m omoh. Hmoss
hs m b ssmoms o mo mbosm.42
Cardiac arrhythmias s s hv so b o.43
A latent form o m omo h who s ss smoms hs b o.8
Preeclampsia should be excluded o hbss o hso hs xmo, ss mm s . Pms o s 20 wks o so s h z b hh boo ss, o h , sw, s wh , h hs, hs vso. Delayed diagnosis may be associated withhigher rates of illness and death; ho,hss sho os m o moh m wh x smoms. Ahoh h smomso h b o om hos o , hhsso h.44 Th ms h oss o x oh ss o omoh oom v sso so bhooh. Whh omobos sho b o hs s s soovs, s o oomm .45,46
Ro o ri MRIM so m (MRI) m bs s omm oo o osm omoh, m ovo b mo h mhsms
vov. I ms ob sm
In chimerim,
feal cell ake
up reidence in
he moher, or
vice vera
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292 CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 76 NUMBER 5 MAY 2009
PErIPArtum cArdIOmYOPAthY
mo oo, h z h mom.47
Fhmo, os h m (wh om) h
h o mo oss, , mosvs shm. Mos hs ovssbo h sbm wh o o b k , whs shmhs vs sbo sboo sm oo.48
Kwo 49 sb whm omoh whos mo m ws mos b o s hm o h v. Thsms mov sh ws wh b bok, os o bok
(ARB), sooo (Ao), h o ov.
L 50 vo s MRIo bos o h bom , whhm b mh mo s h b bos.
Qsos m bo MRI h hoo oos mos o om hm.
ManageMentO POstPaRtuM caRdIOMyOPathy
hr ir rm ri prWh os ss o ms , h w o h s s wsos o wh h o h moh. Co o mm wh sss ( ob s m m m)s ss, wh h moo. Angiotensin-converting enzyme (ACE)inhibitors and ARBs are contraindicatedin pregnancy bs h s bhs, hoh h h m
ms o osm wom wh h. Th o s o h so h ms, whoh hz b hoso,oohmos , bss.51,52 Howv, s s s sk o momos v s ms xos o ACE hbos.53
Digoxin, beta-blockers, loop diuretics,and drugs that reduce afterload sh shz s hv b ovo b s h mss o m
h o h .44
B boks hv so v o s wh h , b hhv o b s m o moh. Nvhss, b boks hv
o b s wom wh h so who kow vs s o h s, s k hss o o oss o o shm s.46,54
hr ir rm poprmA v, h m s oh o o wom wh omoh. ACE inhibitors and ARBs. Th os s o h h mxmm h
sv os. Diuretics v o smom . Spironolactone or digoxin s s s who hv Nw Yok H Asso o ss III o IV smoms. Th o whsooo s 25 m/ oso oh s s mxmz. Th o whox s h ows os o ob b sm ox v, whh sho bk ss h 1.0 /mL. I h DsIvso Go ,55 sm oxvs o 0.5 o 0.8 /mL (0.61.0 mo/L)w mos b, vs o 1.1 o 1.5/mL (1.41.9 mo/L) w sso wh s hs o h . Beta-blockers omm o m omoh,44 s h movsmoms, jo o, svv.
Nosv b boks sh s vo(Co) sv os sh s mooos (Too XL) hv show b.
Th o os s vo 25 m w (50 m w o s) omooo s 100 m o .
aioio rmD , h sk o hombomboomos ss o hh o os o oo os II, VII, VIII, X, o sm bo. Th sk m ss o 6 wks osm.1 Css o ,vos, homboss hv b o wom wh m omo h, h sk m b o h o hmb m sso s
o h s o bo.56,57
Periparum
cardiomyopa
hy can be
diagnoed only
if oher caue
of cardio
myopahy are
aben
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CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 76 NUMBER 5 MAY 2009 293
rAmArAJ ANd sOrrEll
Ps wh v o ssm mbo sm, wh sv v soo om homboss, shov oo.5658 Aoo
sho b o o om v o s om.
W w h ss o h W VssAs R C Ejo Fo, whh sho m whh wbs v h o sok s whjo os o ss h 35%.
W s soos b hmoh h so hmss ho s o .59,60 Howv, s om h Am Co o Co
o h Am H Assoo oh mm o s wh h vvsss h w s obb s h s 6 wks o so, b h s sk o mboh h w s kbw 6 12 wks o so.61 Ths so s w s v s h so h mss bms b so swh o hsv wks bo v. Uoh o ow mo wh h b s . Howv, show b o so, w bv s so sho b om o h sk o h .
cri rpioPs wh sv h s mx m h s o o svv o mov h qo . Howv, w h 3,000 hs vb o so wow . Tho, v sss vs
s b o so.
62
Ps wh smom v hhms sho b os o b o mo.63
nw rm Pentoxifylline mov ooms, v o, smoms wh o ovo h sm o sv s.64
Intravenous immunoglobulin movh jo o sv ss65,66
so mk h vs o fmm
o oks, m hoox.67
Immunosuppressive therapy os o hv ov o, b o b o s s wh ov mos.
Gv h vos oo mhsms om omoh, s k hmmossso w h s. F hmo, who omz ,m ssss m m f h os o h ss.
Ivsos hv mhsz h o o v o bo s m mosssv m, s h mm v vs, wh sbsqoo mo o.28,68
Bromocriptine (Po).Pm
omoh vos m b o hv omo s o o s3, o h xsso v o hs D omo h om o o 16 kD oo om o o. 29Tho, s h hb o s o m s ov h o m omoh. Bs o hs o,wo s wh m omohw wh bomo, hboo o so, h show ooov.69 W q osv omz oo ss o ov h b o bok o swh m omoh. Other proposed therapies mh oss,70 ss,71 mooobos,72 o b,73 mmo soo,74 h hss,75 omos.76
how o o r?Ps wh m omoh whoov om v o s o wh ow os obm (Dobx) b ow o h soh m 6 o 12 mohs.46
natuRal cOuRse
I s o s wh vos s o omoh, hos wh m o moh h sbs b ooss,wh 94% svv 5 s.7
Ahoh vos os hv show
h h os o m o
supec
periparum
cardiomyop
ahy in any
periparum
paien wih
unexplained
ympom of
hear failure
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PErIPArtum cArdIOmYOPAthY
In pregnancy,
we mu alo
conider he
welfare of he
feu
moh s s o h oh sz o om wh 6 mohs, sossb h v o mo o ov bo 6 mohs,
h ss o m hsos o hs.54
Ekm 36 o h, o 100 s wh m omoh hU Ss, h o 2 s, 9 h 4 h v h s. How v, 54 h ov om v o, ov ws mo k hos wh jo o h30% oss.Th o sohso ws 43%, h o w ws 13%. Th o s
v ws 40%, om wh h o o 30.2%.
I os, 98 s H, hh ws 15.3% m oow o 2.2 s, o bo 28% h om v o 6mohs.5
PROgnOstIc actORs
Troponin T. H 41 o h hsm oo T oo m s 2 wks h os o momoh o vs wh h v jo o 6 mohs.Howv, h ssv ws ow: oo Too o mo h 0.04 /mL ss v so wh ssv o o 55%. Th s ws91%. QRS duration o 120 ms o mo hs b s o o h. PooQRS o hs b show o b sk o o h s h ss o s wh shm oshm .77
Heart dimensions and ejection fractionh oos v sv ss. Fos omzo o v o w v sso mso o 5.5 m oss78 v jo o h 27%78 o 30%.36
I osv s,79 osho o 20% o mo v
so mso o 6 m o mo
h m o oss s h sk o ss v so ho.Oh os ssssm ssowh k o ov w v
so mso h 5.6 m, v hombs, A Am .6
RIsK O RelaPse
Ev ov o vo, sbsq s sko s o m omoh.A s H oow 99 s, 15 owhom bm . Eh o hwom who bm x
wos h o msso so.80
O sx Soh A wom who hNw Yok H Assoo ss I smomswho bm , wo wh8 wks o v, h oh o o o hv h smoms.81
I h U Ss, Ekm 82 44 wom wh m omo h who bm . O hs, 28h ov sso o, wh joos o 50% o hh bo bom , 16 h o. Th joo boh os h sbs q , b h s o b mo h 20% o 6 (21%), o. I os, h so o bmo h 20% 5 (31%), 3 (19%) .
Ps who ov om v o hv om v o sv obmh m k oh s, b h sho b w o h sk o v wh ov v o.46,82
Dob 83 om obmsss hooh o ms mxmoo o sv sx woms wh m omoh, o wh sbsqov o v o.
Bs o hs , o ommoso h s h oow:
I v o hs ov, sbsq s o o
, b h sho b
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rAmArAJ ANd sOrrEll
o h, hoh h sk s ow, so bs.I v o hs ov , om obm sss
hooh. I h voo sos o obm s o m, h s b os s
bov; h v oo sos o obm s bom, hh sk s mo s oomm.
I v o hs o ov , h sk s hh, sbsq s o omm.
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ADDRESS: Radhakrishnan Ramaraj, MD, Department of Internal Medicine,University of Arizona College of Medicine, 1501 North Campbell Avenue,
Tucson, AZ 85724; E-mail [email protected].