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European Heart Journal (2011) 32, 3147–3197

doi:10.1093/eurheartj/ehr21

E!" #$%&E'%E!

E!" #uideline on the *ana+e*ent o

-ardioa-ular dieae durin+ pr e+nan-

he a or-e on the ana+e*ent o "ardioa-ular &ieae

durin+ re+nan- o the European !o-iet o "ardiolo+ (E!")

Endored 5 the European !o-iet o #ne-olo+ (E!#), the 6o-iation or

European aediatri- "ardiolo+ (6E"), and the #er*an !o-iet or #ender

edi-ine (e#)

6uthor/0a or-e e*5er: era 8e+it;a+roe ("hairperon) (#er*an)


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314 E!" #uideline

&o-u*ent 8eie?er: Hel*ut =au*+artner ("# 8eie? "oordinator) (#er*an), "hriti &eaton ("# 8eie?

"oordinator) ($A), "arlo 6+uiar (ortu+al), a??ar 6l6ttar (ran-e), 6n+ele 6lono #ar-ia (!pain), 6nna

6ntoniou (#ree-e), %oan "o*an (8o*ania), $ri Elaa* ($!6), i+uel 6n+el #o*e!an-he (!pain), ina

#ot-hea (=ul+aria), &enie HilierAleiner (#er*an), 8o5ert #a5or Ai (Hun+ar), 6nataia Aitiou

(#r ee-e), Aaren . !. Aonin+ (0he etherland), #re+or L. H. 'ip ($A), 6thanaio anoli (#ree-e),

6leCandre e5aaa (ran-e), %eta intale ('atia), arie"laude ori-e (ran-e), =ar5ara J. ulder (0he

etherland), 6+neM a>uet (=el+iu*), !uanna ri-e ($A), !ilia #. riori (%tal), aria J. !alador (!pain),

6raha* !hotan (%rael), "andi-e A. !ileride ("anada), !en I. !ou5@ (&en*ar), JoN r+%n+ol !tein

(6utria), ilar orno (!pain), iel ejltrup (&en*ar), iona Baler ($A), "arole Barne ($!6).

he di-loure or* o the author and reie?er are aaila5le on the E!" ?e5ite ???.e-ardio.or+/+ u ideline

: : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : :: : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : Ae?ord re+nan- @ "ardioa-ular dieae @ #uideline @ 8i ae*ent @ ana+e*ent @ "on+ential heart

dieae @ alular heart dieae @ Hpertenion @ Heart ailure @ 6rrhth*ia

0a5le o "ontent

1. rea*5le . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .310

2. #eneral -onideration . . . . . . . . . . . . . . . . . . . . . . . . . .311

2.1. %ntrodu-tion . . . . . . . . . . . . . . . . . . . . . . . . . . . . .311

2.2. ethod . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .311

2.3. Epide*iolo+ . . . . . . . . . . . . . . . . . . . . . . . . . . . .311

2.4. Hae*odna*i-, hae*otati-, and *eta5oli- alteration

durin+ pre+nan- . . . . . . . . . . . . . . . . . . . . . . . . . .311

2.. #eneti- tetin+ and -ounellin+ . . . . . . . . . . . . . . . .312

2.O. "ardioa-ular dia+noi in pre+nan- . . . . . . . . . . . .312

2.7. etal ae*ent . . . . . . . . . . . . . . . . . . . . . . . . . .314

2.. %nterention in the *other durin+ pre+nan- . . . . . . .31

2.9. i*in+ and *ode o delier: ri or *other and -hild .31

2.10. %ne-tie endo-arditi . . . . . . . . . . . . . . . . . . . . . .31O

2.11. 8i eti*ation: -ontraindi-ation or pre+nan- . . . .317

2.12. ethod o -ontra-eption and ter*ination o pre+nan- , and in itro ertiliation . . . . . . . . . . . . . .319

2.13. #eneral re-o**endation . . . . . . . . . . . . . . . . . . .31O0

3. "on+enital heart dieae and pul*onar hpertenion . . . . .31O0

3.1. aternal hi+h ri -ondition PBorld Health

Ir+aniation (%%%) – %7Q ee alo !e-tion 2.11R . . . . . . . .31O0

3.2. aternal lo? and *oderate ri -ondition (Borld Health

Ir+aniation %, %%, and %%%Q ee alo a5le O and 7) . . . . . .31O3

3.3. !pe-ii- -on+enital heart dee-t . . . . . . . . . . . . . . . .31O3

3.4. 8e-o**endation or the *ana+e*ent o -on+enital

heart dieae . . . . . . . . . . . . . . . . . . . . . . . . . . . .31OO

4. 6orti- dieae . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .31OO

4.1. aternal and oprin+ ri . . . . . . . . . . . . . . . . . . .31OO

4.2. !pe-ii- ndro*e . . . . . . . . . . . . . . . . . . . . . . . .31OO

4.3. ana+e*ent . . . . . . . . . . . . . . . . . . . . . . . . . . . . .31O74.4. 8e-o**endation or the *ana+e*ent o aor ti- dieae .31O

. alular heart dieae . . . . . . . . . . . . . . . . . . . . . . . . . . .31O

.1. !tenoti- ale leion . . . . . . . . . . . . . . . . . . . . . . .31O

.2. 8e+ur+itant leion . . . . . . . . . . . . . . . . . . . . . . . . .31O9

.3. alular atrial i5rillation (natie ale) . . . . . . . . . . .3170

.4. rotheti- ale . . . . . . . . . . . . . . . . . . . . . . . . . .3170

.. e-hani-al prothei and anti-oa+ulation . . . . . . . . .3170

.O. 8e-o**endation or the *ana+e*ent o alular heart

dieae . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .3172

O. "oronar arter dieae and a-ute -oronar ndro*e . . . .3173

O.1. aternal and oprin+ ri . . . . . . . . . . . . . . . . . . .3173

O.2. ana+e*ent . . . . . . . . . . . . . . . . . . . . . . . . . . . . .3174

O.3. 8e-o**endation or the *ana+e*ent o -oronar

arter dieae . . . . . . . . . . . . . . . . . . . . . . . . . . . .3174

7. "ardio*opathie and heart ailure . . . . . . . . . . . . . . . . .3174

7.1. eripartu* -ardio*opath . . . . . . . . . . . . . . . . . . .3174

7.2. &ilated -ardio*opath . . . . . . . . . . . . . . . . . . . . .317O

7.3. Hpertrophi- -ardio*opath . . . . . . . . . . . . . . . . .317O

7.4. 8 e-o**endation or the *ana+e*ent o heart ailur e .3177

. 6rrhth*ia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .3177

.1. 6rrhth*ia ao-iated ?ith tru-tural and -on+enital

heart dieae . . . . . . . . . . . . . . . . . . . . . . . . . . . .3177

.2. !pe-ii- arrhth*ia . . . . . . . . . . . . . . . . . . . . . . . .3177

.3. %nterentional therap: -atheter a5lation . . . . . . . . . .3179

.4. %*planta5le -ardioerterdei5rillator . . . . . . . . . . . . .3179

.. =radarrhth*ia . . . . . . . . . . . . . . . . . . . . . . . . . .3179

.O. 8e-o**endation or the *ana+e*ent

o arrhth*ia . . . . . . . . . . . . . . . . . . . . . . . . . . . .310

9. Hpertenie diorder . . . . . . . . . . . . . . . . . . . . . . . . . .310

9.1. &ia+noi and ri ae*ent . . . . . . . . . . . . . . . . .311

9.2. &einition and -laii-ation o hpertenion in

pre+nan- . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .311

9.3. ana+e*ent o hpertenion in pre+nan- . . . . . . . . .311

9.4. onphar*a-olo+i-al *ana+e*ent and preention o

hpertenion in pre+nan- . . . . . . . . . . . . . . . . . . . .312

9.. har*a-olo+i-al *ana+e*ent o hpertenion in

pre+nan- . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .312

9.O. ro+noi ater pre+nan- . . . . . . . . . . . . . . . . . . . .313

9.7. 8e-o**endation or the *ana+e*ent

o hpertenion . . . . . . . . . . . . . . . . . . . . . . . . . . .313

10. enou thro*5oe*5oli* durin+ pre+nan- and the

puerperiu* . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .313

10.1. Epide*iolo+ and *aternal ri . . . . . . . . . . . . . . .313

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E!" #uideline 3149

10.2. 8i a-tor or pre+nan-related enou thro*5o

e*5oli* and ri tratii-ation . . . . . . . . . . . . . . . .314

10.3. reention o enou thro*5oe*5oli* . . . . . . . . .314

10.4. ana+e*ent o a-ute enou thro*5oe*5oli* . . .31

10.. 8e-o**endation or the preention and *ana+e*ent

o enou thro*5oe*5oli* in pre+nan- and

puerperiu* . . . . . . . . . . . . . . . . . . . . . . . . . . . . .317

11. &ru+ durin+ pre+nan- and 5reateedin+ . . . . . . . . . . . .317

11.1. #eneral prin-iple . . . . . . . . . . . . . . . . . . . . . . . .317

11.2. 8e-o**endation or dru+ ue . . . . . . . . . . . . . . .31

12. 6-no?led+e*ent . . . . . . . . . . . . . . . . . . . . . . . . . . .3191

13. 8eeren-e . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .3191

'it o ta5le

a5le 1. "lae o re-o**endation

a5le 2. 'eel o eiden-e

a5le 3. Eti*ated etal and *aternal ee-tie doe or ariou

dia+noti- and interentional radiolo+ pro-edure

a5le 4. redi-tor o *aternal -ardioa-ular eent and ri

-ore r o* the "688E# tuda5le . redi-tor o *aternal -ardioa-ular eent identiied

in -on+ential heart dieae in the ;6H686 and Ahair tud

a5le O. odiied BHI -laii-ation o *aternal -ardioa-ular

ri: prin-iple

a5le 7. odiied BHI -laii-ation o *aternal -ardioa-ular

ri: appli-ation

a5le . aternal predi-tor o neonatal eent in ?o*en ?ith

heart dieae

a5le 9. #eneral re-o**endation

a5le 10. 8e-o**endation or the *ana+e*ent o -on+enital

heart dieae

a5le 11. 8e-o**endation or the *ana+e*ent o aorti- dieae

a5le 12. 8e-o**endation or the *ana+e*ent o alular heart

dieaea5le 13. 8e-o**endation or the *ana+e*ent o -or onar

arter dieae

a5le 14. 8e-o**endation or the *ana+e*ent o -ardio*opa

thie and heart ailure

a5le 1. 8e-o**endation or the *ana+e*ent o arrhth*ia

a5le 1O. 8e-o**endation or the *ana+e*ent o hpertenion

a5le 17. "he- lit or ri a-tor or enou thr o*5oe*5oli*

a5le 1. realen-e o -on+enital thro*5ophilia and the ao-i

ated ri o enou thro*5oe*5oli* durin+ pre+nan-

a5le 19. 8i +roup a--ordin+ to ri a-tor: deinition and pre

entie *eaur e

a5le 20. 8e-o**endation or the preention and *ana+e*ent

o enou thro*5oe*5oli* in pre+nan- and puerperiu*

a5le 21. 8e-o**endation or dru+ ue

655reiation and a-r on*

6= a*5ulator 5lood preure *onitorin+

6"" 6*eri-an "olle+e o "ardiolo+

6"E an+iotenin-onertin+ en*e

6"! a-ute -oronar ndro*e

6 atrial i5rillation

6H6 6*eri-an Heart 6o-iation

a a-tiated partial thro*5oplatin ti*e

68= an+iotenin re-eptor 5lo-er

6! aorti- tenoi

6!& atrial eptal dee-t

6 atrioentri-ular

6!& atrioentri-ular eptal dee-t

=% 5od *a indeC

= =tpe natriureti- peptide

= 5lood preure

"&" "enter or &ieae "ontrol

"H6&! -on+etie heart ailure, hpertenion, a+e

(.7 ear), dia5ete, tr o e

"% -oniden-e interal

"I -ardia- output

"o6 -oar-tion o the aorta

" -o*puted to*o+r aph"& -ardioa-ular dieae

&= diatoli- 5lood preure

&" dilated -ardio*opath

& deep enou thr o*5oi

E"# ele-tr o-ardio+ra*

E eje-tion ra-tion

E!" European !o-iet o "ardiolo+

E!H European !o-iet o Hpertenion

E!%" European !o-iet o %ntenie "are edi-ine

&6 ood and &ru+ 6d*initration

H" hpertrophi- -ardio*opath

%"& i*planta5le -ardioerterdei5rillator

%8 international nor*alied ratio

i.. intraenou'BH lo? *ole-ular ?ei+ht heparin

' let entri-ular

'E let entri-ular eje-tion ra-tion

'II let entri-ular outlo? tra-t o5tru-tion

8% *a+neti- reonan-e i*a+in+

! *itral tenoi

pro= ter*inal pro =tpe natriureti- peptide

LH6 e? Lor Heart 6o-iation

I6" oral anti-oa+ulant

6H pul*onar arterial hpertenion

6 pul*onar arter preure

"% per-utaneou -oronar interention

" peripartu* -ardio*opath

! pul*onar ale tenoi8 ri+ht entri-ular

!= toli- 5lood preure

! upraentri-ular ta-h-ardia

#6 -o*plete tranpoition o the +reat arterie

8 tri-upid re+ur+itation

$H unra-tionated heparin

!& entri-ular eptal dee-t


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310 E!" #uideline

entri-ular ta-h-ardia

E enou thr o*5oe*5oli*

BHI Borld Health Ir+aniation

1. r ea*5le

#uideline u**arie and ealuate all aaila5le eiden-e, at the

ti*e o the ?ritin+ pro-e, on a parti-ular iue ?ith the ai* o

aitin+ phi-ian in ele-tin+ the 5et *ana+e*ent trate+ie

or an indiidual patient, ?ith a +ien -ondition, tain+ into

a--ount the i*pa-t on out-o*e, a ?ell a the ri – 5eneit

ratio o parti-ular dia+noti- or therapeuti- *ean. #uideline

are no u5titute 5ut are -o*ple*ent or teCt5oo and -oer

the European !o-iet o "ardiolo+ (E!") "ore "urri-ulu*

topi-. #uideline and re-o**endation hould help the

phi-ian to *ae de-iion in their dail pra-ti-e. Ho?eer , the

inal de-iion -on-ernin+ an indiidual patient *ut 5e *ade 5

the reponi5le phi-ian().

6 +reat nu*5er o #uideline hae 5een iued in re-ent ear

5 the E!" a ?ell a 5 other o-ietie and or+aniation.

=e-aue o the i*pa-t on -lini-al pra-ti-e, >ualit -riteria or the

deelop *ent o +uideline hae 5een eta5lihed in order to

*ae all de-iion tranparent to the uer. he re-o**endation

or or *ulatin+ and iuin+ E!" #uideline -an 5e ound on

the E!" ?e 5ite (http://?? ?.e -a r dio.or+/+uideline ure/e-

+u id eline/ a5out/a+e/r ule?r itin+.a pC). E!" #uideline

repreent the o i-ial poition o the E!" on a +ien to pi- and are

r e+ular l updated.

*5er o thi a or-e ?ere ele-ted 5 the E!" to rep reent proeional

ed ?ith the *edi-al -are o patient ?ith thi patholo+. !ele-ted eCpert in the

ndertoo a -o* prehenie reie? o the pu5lihed eiden-e or dia+noi,

e *ent, and/or preention o a +ien -ondition a--ordin+ to E!" "o**ittee

a-ti-e #uideline ("#) poli-. 6 -riti-al

ealuation o dia+noti- and therapeuti- pro-edure ?a per

or*ed in-ludin+ ae*ent o the ri – 5eneit ratio.

Eti*ate o eCpe-ted health out-o*e or lar+er population

?ere in-luded, ?here data eCit. he leel o eiden-e and

the tren+th o re-o**endation o parti-ular treat*ent option

?ere ?ei+hed and +raded a--ordin+ to predeined -ale,

a outlined in a5le 1 and 2.

he eCpert o the ?ritin+ and reie?in+ panel illed in de-lar a

tion o interet or* ?hi-h *i+ht 5e per-eied a real or poten

tial our-e o -onli-t o interet. hee or* ?ere -o*piled

into one ile and -an 5e ound on the E!" Be5 !ite (http://

?? ? .e-ardio.or+/+uideline). 6n -han+e in de-laration o inter

et that arie durin+ the ?ritin+ period *ut 5e notiied to the E!"

and updated. he a or-e re-eied it entire inan-ial upport

ro* the E!" ?ithout an inole*ent ro* health-are indutr.

he E!" "# uperie and -oordinate the preparation o

ne? #uideline produ-ed 5 a or-e, eCpert +roup, or -on

enu panel. he "o**ittee i alo reponi5le or the endore

*ent pro-e o thee #uideline. he E!" #uideline under+o

eCtenie reie? 5 the "# and eCternal eCpert. 6ter appr opri

ate reiion it i approed 5 all the eCpert inoled in the a or-e. he inalied do-u*ent i approed 5 the "# or pu5li

-ation in the European Heart Journal.

he ta o deelopin+ #uideline -oer not onl the inte

+ration o the *ot re-ent reear-h, 5ut alo the -reation o edu

-ational tool and i*ple*entation pro+ra**e or the

re-o**endation. o i*ple*ent the +uideline, -ondened

po-et +uideline erion, u**ar lide, 5oolet ?ith eential

*ea+e, and an ele-troni- erion or di+ital appli-ation (*art

phone, et-.) are produ-ed. hee erion are a5rid+ed and, thu,

i needed, one hould al?a reer to the ull teCt erion ?hi-h i

reel aaila5le on the E!" ?e5ite.

he ational !o-ietie o the E!" are en-oura+ed to endore,

tranlate, and i*ple*ent the E!" #uideline. %*ple*entation

0a5le 1 "lae o r e-o**endation

Classes of

recommendationsDenition Suggested

wording to use

Class I Evidence and/or general agreement Is

recommended/is that a given treatment or

procedure indicated

is benecial, useful, eective.

Class II Conicting evidence

and/or a divergence of

opinion about the

usefulness/e!cac" of the

given treatment or

procedure.

Class IIa #eight of evidence/opinion is in Should beconsidered

favour of usefulness/e!cac".

Class IIb $sefulness/e!cac" is less well %a" beconsidered

established b" evidence/opinion.

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311E!" #uideline

0a5le 2 'eel o eiden-ethe hould 5e *ana+ed 5 interdi-iplinar tea*Q hi+h ri

patient hould 5e treated in pe-ialied -entreQ and dia+noti-

pro-edure and interention hould 5e peror*ed 5 pe-ialit

?ith +reat eCpertie in the indiidual te-hni>ue and eCperien-e

in treatin+ pre+nant patient. 8e+itrie and prope-tie tudie

are ur+entl needed to i*proe the tate o no?led+e.

2.2 ethodhe #uideline are 5aed on a te*ati- ear-h o the literature

o the lat 20 ear in the ational %ntitute o Health data5ae

(u5ed). he pu5li-ation and re-o**endation o the Euro

pean and 6*eri-an -ardiolo+i-al o-ietie are alo -onidered:

6*eri-an Heart 6o-iation/6*eri-an "olle+e o "ardiolo+

&evel ofData derived from multiple

randomi'ed clinical trialsEvidence (or meta)anal"ses.

Data derived from a single randomi'ed&evel ofclinical trial

Evidence *or large non)randomi'ed studies.

&evel ofConsensus of opinion of the

e+perts and/Evidence C

or small studies,retrospective studies,

registries.

**e are needed 5e-aue it ha 5een ho?n that the out-o*e o dieae *a 5e

a5l inluen-ed 5 the thorou+h appli-ation o -lini-al re-o**endation.

e and re+itrie are needed to eri that reallie dail pra-ti-e i in eepin+

?hat i re-o**ended in the +uideline, thu -o*pletin+ the loop 5et?een

l reear-h, ?ritin+ o +uideline, and i*ple*entin+ the* into -lini-al pra-ti-e.

he +uideline do not, ho?eer , oerride the indiidual r epon

i5ilit o health proeional to *ae appropriate de-iion in

the -ir-u*tan-e o the indiidual patient, in -onultation ?ith

that patient, and, ?here appropriate and ne-ear, the patient

+uar dian or -arer. %t i alo the health proeional

reponi5ilit to eri the rule and re+ulation appli-a5le to

dru+ and dei-e at the ti*e o pr e-ription.

2. #eneral -onideration

2.1 %ntr odu-tion6t preent, 0.2 – 4S o all pre+nan-ie in ?etern

indutrialied -ountrie are -o*pli-ated 5 -ardioa-ular

dieae ("&),1

and the nu*5er o the patient ?ho deelop

-ardia- pro 5le* durin+ pre+nan- i in-reain+. eerthele,

the nu*5er o u-h patient preentin+ to the indiidual

phi-ian i *all. Ho?eer, no?led+e o the ri ao-iated

?ith "& durin+ pre+nan- and their *ana+e*ent are o

piotal i*portan-e or adiin+ patient 5eore pre+nan-.

hereore, +uideline on dieae *an a+e*ent in pre+nan-

are o +reat relean-e. !u-h +uideline hae to +ie pe-ial

-onideration to the a-t that all *eaur e

-on-ern not onl the *other, 5ut the etu a ?ell. her eore,

the opti*u* treat*ent o 5oth *ut 5e tar+eted. 6 therap

aoura5le or the *other -an 5e ao-iated ?ith an i*pair*ent

o the -hild, and in eCtre*e -ae treat*ent *eaure ?hi-h

prote-t the urial o the *other -an -aue the death o theetu. In the other hand, therapie to prote-t the -hild *a

lead to a u5opti*al out-o*e or the *other. =e-aue prope-tie

or rando*ied tudie are la-in+, ?ith a e? eC-eption, re-

o**endation in thi +uideline *otl -orrepond to the eiden-e

leel ".

!o*e +eneral -on-luion hae arien ro* thee +uideline:

-ounellin+ and *ana+e*ent o ?o*en o -hild5earin+ a+e ?ith

upe-ted -ardia- dieae hould tart 5eore pre+nan- o--urQ

(6H6/6""),2

the E!" in 2003,3

the Borin+ #roup alular

Heart &ieae o the E!",4

the +uideline o the #er*an !o-iet

o "ardiolo+ (#er*an !o-iet o "ardiolo+),,O

and the E!"

a or-e on the ana+e*ent o alular Heart &ieae 2007.7

2.3 Epide*iolo+

he pe-tru* o "& in pre+nan- i -han+in+ and dier 5et?een -ountrie. %n the ?etern ?orld, the ri o "& in pre+

nan- ha in-reaed due to in-reain+ a+e at irt pre+nan- and

in-reain+ prealen-e o -ardioa-ular ri a-torTdia5ete,

hpertenion, and o5eit. 6lo the treat*ent o -on+enital heart

dieae ha i*proed, reultin+ in an in-reaed nu*5er o

?o*en ?ith heart dieae rea-hin+ -hild5earin+ a+e.

%n ?etern

-ountrie *aternal heart dieae i no? the *ajor -aue o

*aternal death durin+ pre+nan-.9

Hpertenie diorder are the *ot re>uent -ardioa-ular

eent durin+ pre+nan-, o--urrin+ in O – S o all

pre+nan-ie.10

%n the ?etern ?orld, -on+enital heart dieae i the *ot re>uent

-ardioa-ular dieae preent durin+ pre+nan- (7 – 2S),

?ith hunt leion predo*inatin+ (20 – OS).11,12 "on+enital

heart dieae repreent jut 9 – 19S outide Europe and

orth 6*eri-a. 8heu*ati- alular dieae do*inate in non

?etern -ountrie, -o*priin+ O – 9S o all -ardioa-ular

dieae in pre+nan-.11,12

"ardio*opathie are rare, 5ut repreent eere -aue o -ar

dioa-ular -o*pli-ation in pre+nan-. eripartu* -ardio*opa

th (") i the *ot -o**on -aue o eere -o*pli-ation.13

2.4 Hae*odna*i-, hae*otati-, and*eta5oli- alteration durin+ pr e+nan-re+nan- indu-e -han+e in the -ardioa-ular te* to *eet

the in-reaed *eta5oli- de*and o the *other and etu. he

in-lude in-reae in 5lood olu*e and -ardia- output ("I), andredu-tion in te*i- a-ular reitan-e and 5lood preure (=).

la*a olu*e rea-he a *aCi*u* o 40S a5oe 5aeline at 24

?ee +etation. 6 30 – 0S in-reae in "I o--ur in nor*al

pre+ nan-. %n earl pre+nan- in-reaed "I i pri*aril related

to the rie in troe olu*eQ ho?eer , in late pre+nan-, heart

rate i the *ajor a-tor. Heart rate tart to rie at 20 ?ee

and in-reae until 32 ?ee. %t re*ain hi+h 2 – da ater

delier. !te*i- = (!=) tpi-all all earl in +etation and

diatoli- = (&=) i uuall 10 **H+ 5elo? 5aeline in the

e-ond tri*eter. hi de-reae in = i -aued 5 a-tie

aodilatation, a-hieed


6/52

312 E!" #uideline

throu+h the a-tion o lo-al *ediator u-h a prota--lin and

nitri- oCide. %n the third tri*eter, the &= +raduall in-reae

and *a nor*alie to nonpre+nant alue 5 ter*.

he heart -an in-reae it ie 5 up to 30S, ?hi-h i partiall

due to dilatation. &ata re+ardin+ toli- and diatoli- un-tion

in pre+nan- are -ar-e. !toli- un-tion in-reae irt 5ut

*a de-reae in the lat tri*eter. 8eport on diatoli-

un-tion are -onli-tin+.

re+nan- indu-e a erie o hae*otati- -han+e, ?ith an

in-reae in -on-entration o -oa+ulation a-tor, i5rino+en, and

platelet adheiene, a ?ell a di*inihed i5rinoli, ?hi-h lead

to hper-oa+ula5ilit and an in-reaed ri o thr o*5oe*5oli-

eent. %n addition, o5tru-tion to enou return 5 the enlar+in+

uteru -aue tai and a urther rie in ri o thro* 5o

e*5oli*. aternal +lu-oe ho*eotai *a -han+e and

-holeterol leel

in-reae in adaptation to etal – *aternalneed.

hiolo+i-al -han+e that o--ur durin+ pre+nan- -an a e-t

a5orption, eC-retion, and 5ioaaila5ilit o all dru+.14

he

in-reaed intraa-ular 5lood olu*e partl eCplain the hi+her

doa+e o dru+ re>uired to a-hiee therapeuti- pla*a -on-en

tration, and the doe adaptation needed durin+ treat*ent. ore

oer, the raied renal peruion and the hi+her hepati- *eta5oli*

in-reae dru+ -learan-e. he altered phar*a-oineti- o dru+

ar in *a+nitude durin+ dierent ta+e o pre+nan-, *ain+

-areul *onitorin+ o the patient and doe adjut*ent ne-ear.

$terine -ontra-tion, poitionin+ (let lateral . upine), pain,

anCiet, eCertion, 5leedin+, and uterine inolution -aue i+nii-ant

hae*odna*i- -han+e durin+ la5our and potpartu*. 6naethe

ia, anal+eia, hae*orrha+e, and ine-tion *a indu-e additional

-ardioa-ular tre. != and &= in-reae 1 – 2S and 10

–

1S, repe-tiel, durin+ uterine -ontra-tion. !u-h in-reae are

ao-iated ?ith a rie in preure in the a*nioti- luid, and in the

intrathora-i- enou, -ere5ropinal, and eCtradural luid. "I

in-reae 5 1S in earl la5our, 5 2S durin+ ta+e 1, and 5

0S durin+ eCpulie eort.1

%t rea-he an in-reae o 0S

earl potpartu* due to autotranuion ao-iated ?ith uterine

inolution and reorption o le+ oede*a.

%n -on-luion, the phiolo+i-al adaptation to pre+nan- inlu

en-e the ealuation and interpretation o -ardia- un-tion and

-lini -al tatu.

2. #eneti- tetin+ and -ounellin+6n i*portant ape-t -on-ernin+ the -are o oun+ ?o*en ?ith

"& i the -onultation a5out the ri o inheritan-e o -ardia-

dee-t or their de-endant. he ri i raied i+nii-antl in -o*

parion ?ith parent ?ithout "& ?here the ri i 1S. %n

addition, there are lar+e dieren-e 5et?een ea-h o the heredi

tar heart dieae -ondition, and the ri or de-endant i

dependent on ?hether onl the *other, onl the ather, or 5oth

parent uer ro* hereditar -ardia- dee-t.1O

%n +eneral, the

ri i hi+her ?hen the *other i ae-ted rather than the

ather.1O

he re-urren-e ri arie 5et?een 3S and 0S depend

in+ on the tpe o *aternal heart dieae.

"hildren o parent ?ith a -ardioa-ular -ondition inherited in

an autoo*al do*inant *anner (e.+. aran ndro*e, hper

trophi- -ardio*opath, or lon+ U ndro*e) hae an inheri

tan-e ri o 0S, re+ardle o +ender o the ae-ted parent.

he inal phenotpe ?ill alo 5e deter*ined 5 in-o*plete pene

tran-e and pleiotropi- ee-t, and *a ar i+nii-antl. or

dee-t that are inherited in a pol+eni- *anner, re-urren-e ri

i le -learl deined. 6utoo*al re-eie and V-hr o*oo*al

re-eie inheritan-e are rar e.

#eneti- tetin+ *a 5e ueul:

@ in -ardio*opathie and -hannelopathie, u-h a lon+ Undr o*e

17

@ ?hen other a*il *e*5er are a e-ted

@ ?hen the patient ha d*orphi- eature, deelop*ental dela/

*ental retardation, or ?hen other non-ardia- -on+enital

a5nor*alitie are preent, in ndro*e u-h a in aran,

22>11 deletion, Billia* – =euren, 6la+ille, oonan, and

Holt – Ira* ndro*e.

or a teadil in-reain+ nu*5er o +eneti- dee-t, +eneti- -reen

in+ 5 -horioni- illou 5iop -an 5e oered in the 12th ?ee o

pre+nan-. 6ll ?o*en ?ith -on+enital heart dieae hould 5e

oered etal e-ho-ardio+raph in the 19th to 22nd ?ee o pre+

nan-. eaure*ent o nu-hal old thi-ne in the 12th to 13th

?ee o pre+nan- i an earl -reenin+ tet or ?o*en oer 3

ear o a+e. he enitiit or the preen-e o a i+nii-ant

heart dee-t i 40S, ?hile the pe-ii-it o the *ethod i 99S.

he in-iden-e o -on+enital heart dieae ?ith nor*al nu-hal

old thi-ne i 1/1000.1

he inheritan-e pattern dier a*on+ the dieae, and ther e

ore +eneti- -ounellin+ 5 a +eneti-it i hi+hl r e-o**ended

or patient and their a*il *e*5er.17

#eneti- tetin+ ater

-areul -ounellin+ ha the rationale o identiin+ atri a*pto

*ati- or dieaeree relatie and to +uide -lini-al ureillan-e or

dieae onet, there5 enhan-in+ preentie and treat*ent inter

ention. %t i ado-ated in patient ?ith no?n +eneti- diorder

and i *ore adia5le i treat*ent option are aaila5le.17

2.O "ardioa-ular dia+noiin pr e+nan-he ollo?in+ pro-edure are o relean-e or the dia+noi and

*ana+e*ent o "& in pre+nan-.

Hitor and -lini-al ineti+ation

an diorder -an 5e identiied 5 tain+ a -areul peronal and

a*il hitor, parti-ularl -ardio*opathie, the aran n

dro*e, -on+enital heart dieae, juenile udden death, lon+

U ndro*e, and -ate-hola*iner+i- entri-ular ta-h-ardia

() or =ru+ada ndro*e. %t i i*portant to a pe-ii-all

a5out poi5le udden death in the a*il. he ae*ent o

dpnoea i i*portant or dia+noi and pro+noi o ale

leion and or heart ailure. 6 thorou+h phi-al eCa*ination-oniderin+ the phiolo+i-al -han+e that o--ur durin+ pre+

nan- (!e-tion 2.4) i *andator, in-ludin+ au-ultation or

ne? *ur*ur, -han+e in *ur*ur, and looin+ or i+n o

heart ailure. Bhen dpnoea o--ur durin+ pre+nan- or

?hen a ne? patholo+i-al *ur*er i heard, e-ho-ardio+raph i

indi-ated. %t i -ru-ial to *eaure the =, in let lateral re-u*

5en- (ee !e-tion 9) uin+ a tandardied *ethod, and to

loo or proteinuria, epe-iall ?ith a hitor or a*il hitor


7/52

313E!" #uideline

o hpertenion or pree-la*pia. ICi*etr hould 5e per

or*ed in patient ?ith -on+enital heart dieae.

Ele-tro-ardio+ra ph

he +reat *ajorit o pre+nant patient hae a nor*al ele-tro-ar

dio+ra* (E"#). he heart i rotated to?ard the let and on the

ura-e E"# there i a 1 – 20 let aCi deiation. "o**on

indin+ in-lude tranient ! e+*ent and ?ae -han+e, the

preen-e o a U ?ae and inerted ?ae in lead %%%, an

attenuated U ?ae in lead 6, and inerted ?ae in lead 1,

2, and, o--aionall, 3. E"# -han+e -an 5e related to a +radual

-han+e in the poition o the heart and *a *i*i- let entri-ular

(') hpertroph and other tru-tural heart dieae.

Holter *onitorin+ hould 5e peror*ed in patient ?ith no?n

preiou paroC*al or peritent do-u*ented arrhth*ia P,

atrial i5rillation (6), or atrial lutterR or thoe reportin+

*p to* o palpitation.

E-ho-ardio+r aph

=e-aue e-ho-ardio+raph doe not inole eCpoure to radiation,

i ea to peror*, and -an 5e repeated a oten a needed, it ha

5e-o*e an i*portant tool durin+ pre+nan- and i the preerred

-reenin+ *ethod to ae -ardia- un-tion.

ranoeopha+eal e-ho-ardio+raph

ultiplane trandu-er hae *ade tranoeopha+eal e-ho-ardio

+raph a er ueul e-ho-ardio+raphi- *ethod in the ae*ent

o adult ?ith, or eCa*ple, -o*pleC -on+enital heart dieae.

ranoeopha+eal e-ho-ardio+raph, althou+h rarel re>uired, i

relatiel ae durin+ pre+nan-. he preen-e o to*a-h -on

tent, ri o o*itin+ and apiration, and udden in-reae in

intraa5do*inal preure hould 5e taen into a--ount, and etal

*onitorin+ peror*ed i edation i ued.

ECer-ie tetin+

ECer-ie tetin+ i ueul to ae o5je-tiel the un-tional

-apa-it, -hronotropi- and = repone, a ?ell a

eCer-ieindu-ed arrhth*ia. %t ha 5e-o*e an inte+ral part o

the ollo?up o +ro?n up -on+enital heart dieae patient a

?ell a patient ?ith a*pto*ati- alular heart dieae.19,20

%t

hould 5e peror*ed in patient ?ith no?n heart dieae, pre er

a5l prior to pre+nan- to ait in ri ae*ent.

hi "o**ittee re-o**end peror*in+ u5*aCi*al eCer-ie

tet to rea-h 0S o predi-ted *aCi*al heart rate in a*pto

*ati- pre+nant patient ?ith upe-ted "&. here i no eiden-e

dieae ?ith 5orderline or *ildl redu-ed 'E. u-lear -inti+ra

ph hould 5e aoided durin+ pre+nan- 5e-aue o radiation

eCpoure.

8adiation eCpoure

he ee-t o radiation on the etu depend on the radiation doe

and the +etational a+e at ?hi-h eCpoure o--ur. % poi5le, pro

-edure hould 5e delaed until at leat the -o*pletion o the

period o *ajor or+ano+enei (.12 ?ee ater *ene). her e

i no eiden-e o an in-reaed etal ri o -on+enital

*alor*ation, intelle-tual dia5ilit, +ro?th retri-tion, or

pre+nan- lo at doe o radiation to the pre+nant ?o*an o ,

0 *#22,23

(???.5t.-d -. +o/ radiat ion/p r enata lphi-ia n .apQ

a - -eed 31 I-to5er 2007). here * a 5e a *all in- reae in ri

(1:2000 . 1:3000) o -hildhood -an-er. he thre h old at ?hi-h an

in-reaed ri o -on+enital *al

or*ation o--ur ha not 5een deinitel deter*ined. !o*e ei

den-e u++et that ri o *alor*ation i in-reaed at doe

.100 *#, ?herea the ri 5et?een 0 and 100 *# i le

-lear. &urin+ the irt 14 da ater ertiliation, inta-t urial

?ithout etal a5nor*alit or death are the *ot liel out-o*e o

radiation eCpoure .0 *#. 6ter the irt 14 da, radiation

eCpoure .0 *# *a 5e ao-iated ?ith an in-reaed ri o

-on +enital *alor*ation, +ro?th retri-tion, and intelle-tual

dia5ilit. ot *edi-al pro-edure do not eCpoe the etu to

u-h hi+h leel o radiation (a5le 3). or the *ajorit o

dia+noti- *edi-al pro-edure, inolin+ doe to the etu o up

to 1 *#, the ao-iated ri o -hildhood -an-er are er

lo?. (&o-u*ento the Health rote-tion 6+en-. 8adiation,

"he*i-al and Eniron *ental Haard ar-h 2009. 8!E9

rote-tion o pre+nant patient durin+ dia+noti- *edi-al

eCpoure to ioniin+ r adiation. 6di-e ro* the Health

rote-tion 6+en-, he 8oal "olle+e o 8adiol

o+it, and the "olle+e o 8adio+rapher.)

0a5le 3 Eti*ated etal and *aternal ee-tie doe

or ariou dia+noti- and interentional r adiolo+

pro-edure

re-u*5ent --le er+o*etr appear to 5e the *ot -o*orta5le

*odalit, 5ut tread*ill ?alin+ or upri+ht --le er+o*etr *a

alo 5e ued. &o5uta*ine tre hould 5e aoided. % re pirator+a anali i ued, the li*it i a repirator eC-han+e ratio o

1.0. !tre e-ho-ardio+raph uin+ 5i--le er+o*etr *a add to

the dia+noti- pe-ii-it in dete-tin+ the preen-e and eCtent

o i-hae*ia in hi+h ri patient ?ith poi5le -oronar

arter dieae. hi -an alo 5e ueul prior to -on-eption to

ae *o

tion Plet entri-ular eje-tion ra-tion ('E)R, and alo in patient

?ith other -ardio*opathie, ?ith alular or -on+enital heart

aECpoure depend on the nu*5er o proje-tion or ie?.

" W -o*puted to*o+raphQ 6 W poteroanteriorQ "% W per-utaneou

-oronar interention.

rocedure-etal e+posure

%atern

al

Chestradiograph

(PA and lateral)


8/52


9/52

31E!" #uideline

appropriate interention that ideall ?ill preent adere etal

e>uelae.37,3

2. %nterention in the *other durin+ pr e+nan-

2..1 er-utaneou theraphe a*e retri-tion ?hi-h appl or dia+noti- -oronar an+io

+raph (ee !e-tion 2.O) are releant. % an interention i a5ol

utel ne-ear, the 5et ti*e to interene i -onidered to 5e

ater the ourth *onth in the e-ond tri*eter. = thi ti*e or+a

no+enei i -o*plete, the etal throid i till ina-tie, and the

olu*e o the uteru i till *all, o there i a +reater ditan-e

5et?een the etu and the -het than in later *onth. luoro-op

and -inean+io+raph ti*e hould 5e a 5rie a poi5le and the

+raid uteru hould 5e hielded ro* dire-t radiation. Heparin

ha to 5e +ien at 40 – 70 $/+, tar+etin+ an a-tiated

-lottin+ ti*e o at leat 200 , 5ut not eC-eedin+ 300 .

2..2 "ardia- ur+er ?ith -ardiopul*onar 5pa

aternal *ortalit durin+ -ardiopul*onar 5pa i no? i*ilar

to that in nonpre+nant ?o*en ?ho under+o -o*para 5le

-ardia- pro-edure.1

Ho?eer, there i i+nii-ant *or5idit

in-ludin+ late neurolo+i-al i*pair*ent in 3 – OS o -hildren,

and etal *ortalit re*ain hi+h.39

or thi reaon -ardia-

ur+er i re-o**ended onl ?hen *edi-al therap or

interentional pro -edure ail and the *other lie i

threatened. he 5et period or ur+er i 5et?een the 13th

and 2th ?ee.40,41

!ur+er durin+ the irt tri*eter -arrie

a hi+her ri o etal *alor *ation, and durin+ the third

tri*eter there i a hi+her in-i den-e o preter* delier

and *aternal -o*pli-ation. Be no? ro* preiou tudie

that +etational a+e ha a lar+e i*pa-t on neonatal out-o*e.42

8e-ent i*proe*ent in neonatal -are ha urther i*proedurial o pre*ature inant. 6t 2O ?ee, urial i

+enerall 0S, ?ith 20S hain+ eriou neurolo+i-al

i*pair*ent. or thi reaon, -aearean delier *a 5e

-onidered 5eore -ardiopul*onar 5pa i +etational a+e i

.2O ?ee.43

Bhether or not delier i adanta+eou or

the 5a5 at thi +etational a+e depend on eeral a-tor:

+ender, eti*ated ?ei+ht, prior ad*initration o -orti-oter oid

5eore delier, and the out-o*e tatiti- o the neonatal unit

-on-erned. Bhen +etational a+e i 2 ?ee or *ore, delier

5eore ur+er hould 5e -onidered. =eore ur+er a ull

-oure (at leat 24 h) o -orti-oteroid hould 5e ad*initered

to the *other, ?heneer poi5le. &urin+ -ardiopul*onar

5pa, etal heart rate and uterine tone hould 5e *onitored

in addition to tandard patient *onitorin+. u*p lo?

.

2. '/

*in/*2

and peruion preure.

70 **H+ are *andator to

*aintain ade>uate uteropla-ental 5lood lo?Q pulatile lo?,

althou+h -ontroerial, ee* *ore ee-tie or preerin+ uter

opla-ental 5lood lo?. aternal hae*ato-rit .2S i re-

o**ended to opti*ie the oC+en delier. or*other*i-

peruion, ?hen eai5le, i ado-ated, and tate o the art pH

*ana+e*ent i preerred to aoid hpo-apnia reponi5le or

uteropla-ental ao-ontri-tion and etal hpoCia. "ardiopul*on

ar 5pa ti*e hould 5e *ini*ied.44

2.9 i*in+ and *ode o delier: ri or *other and -hild

Hi+h ri delier

%ndu-tion, *ana+e*ent o la5our, delier, and potpartu* ur

eillan-e re>uire pe-ii- eCpertie and -olla5oratie *ana+e*ent

5 illed -ardiolo+it, o5tetri-ian, and anaetheiolo+it, ineCperien-ed *aternal – etal *edi-ine unit.

4,4O

i*in+ o delier

!pontaneou onet o la5our i appropriate or ?o*en ?ith

nor*al -ardia- un-tion and i preera5le to indu-ed la5our or

the *ajorit o ?o*en ?ith heart dieae. i*in+ i indiidualied,

a--ordin+ to the +raida -ardia- tatu, =ihop -ore (a -or e

5aed upon the tation o the preentin+ part and our -har a-ter

iti- o the -eriC: dilatation, ea-e*ent, -oniten-, and po

ition), etal ?ell5ein+, and lun+ *aturit. &ue to a la- o

prope-tie data and the inluen-e o indiidual patient -har a-ter

iti-, tandard +uideline do not eCit, and *ana+e*ent hould

thereore 5e indiidualied. %n ?o*en ?ith *ild unrepaired -on+e

nital heart dieae and in thoe ?ho hae under+one u--eul-ardia- ur+i-al repair ?ith *ini*al reidua, the *ana+e*ent o

la5our and delier i the a*e a or nor*al pre+nant ?o*en.

'a5our indu-tion

ICto-in and artii-ial rupture o the *e*5rane are indi-ated

?hen the =ihop -ore i aoura5le. 6 lon+ indu-tion ti*e

hould 5e aoided i the -eriC i unaoura5le. Bhile there i

no a5olute -ontraindi-ation to *iopr otol or dinoprotone,

there i a theoreti-al ri o -oronar aopa* and a lo? ri

o arrhth*ia. &inoprotone alo ha *ore proound ee-t on

= than prota+landin E1 and i thereore -ontraindi-ated in

a-tie "&. e-hani-al *ethod u-h a a ole -atheter ?ould

5e preera5le to phar*a-olo+i-al a+ent, parti-ularl in the

patient ?ith -anoi ?here a drop in te*i- a-ular reitan-eand/or = ?ould 5e detri*ental.

47

a+inal or -aearean delier

he preerred *ode o delier i a+inal, ?ith an indiidualied

delier plan ?hi-h inor* the tea* o ti*in+ o delier (pon

taneou/indu-ed), *ethod o indu-tion, anal+eia/re+ional anae

theia, and leel o *onitorin+ re>uired. %n hi+h ri leion,

delier hould tae pla-e in a tertiar -entre ?ith pe-ialit

*ultidi-iplinar tea* -are. a+inal delier i ao-iated ?ith

le 5lood lo and ine-tion ri -o*pared ?ith -aearean deli

er, ?hi-h alo in-reae the ri o enou thro*5oi and

thro*5oe*5oli*.4

%n +eneral, -aearean delier i reered

or o5tetri- indi-ation. here i no -onenu re+ardin+ a5olute

-ontraindi-ation to a+inal delier a thi i er *u-hdependent on *aternal tatu at the ti*e o delier and the

anti-ipated -ardiopul*onar toleran-e o the patient. "aearean

delier hould 5e -onidered or the patient on oral

anti-oa+ulant (I6") in preter* la5our, patient ?ith aran

ndro*e and an aorti- dia*eter .4 **, patient ?ith

a-ute or -hroni- aorti- die-tion, and thoe in a-ute intra-ta5le

heart ailure. "earean delier *a 5e -onidered in aran

patient ?ith an

aorti- dia*eter 40 – 4 **.7,49,0

(ee alo !e-tion4.3).


10/52

31O E!" #uideline

%n o*e -entr e, -aearean delier i ado-ated or ?o*en ?ith

eere aorti- tenoi (6!) and in patient ?ith eere or * o

pul *onar h per tenion (in-ludin+ Eien*en+er ndr o*e), or

a-ute heart ailur e.7,4O

(ee pe-ii- e-tion). "aearean delier

*a 5e -onider ed in patient ?ith *e-hani-al heart ale

prothee to preent pro 5le* ?ith planned a+inal delier . %n

u-h patient, a prolon+ed ?it-h to he par in/lo? *ole-ular

?ei+ht he par in

('BH) *a indeed 5e r e>uir ed or a lon+ ti*e 5e or e a+inal

5irth, par ti-ular l, ?hen the o5tetr i-al ituation i

unaour a5le. hi ?ould in-r eae the *aternal ri (ee alo

!e-tion . and .O).

Hae*odna*i- *onitorin+

!te*i- arterial preure and *aternal heart rate are *onitor ed,

5e-aue lu*5ar epidural anaetheia *a -aue hpotenion.

ule oCi*etr and -ontinuou E"# *onitorin+ are utilied a

re>uired. 6 !?an – #an -atheter or hae*odna*i- *onitorin+

i rarel i eer indi-ated due to the ri o arrhth*ia

proo-ation, 5leedin+, and thro*5oe*5oli- -o*pli-ation on

re*oal.1

6naetheia/anal+eia

'u*5ar epidural anal+eia i oten re-o**enda5le 5e-aue it

redu-e painrelated eleation o *patheti- a-tiit, redu-e

the ur+e to puh, and proide anaetheia or ur+er. "ontinuou

lu*5ar epidural anal+eia ?ith lo-al anaetheti- or opiate, or

-ontinuou opioid pinal anaetheia -an 5e ael ad*initered.

8e+ional anaetheia -an, ho?eer, -aue te*i- hpotenion

and *ut 5e ued ?ith -aution in patient ?ith o5tru-tie ale

leion. %ntraenou (i..) peruion *ut 5e *onitored -areull.2

'a5our

In-e in la5our, the ?o*an hould 5e pla-ed in a lateral de-u5itu

poition to attenuate the hae*odna*i- i*pa-t o uterine -ontra-tion.

3he uterine -ontra-tion hould de-end the etal

head to the perineu*, ?ithout *aternal puhin+, to aoid the

un?anted ee-t o the alala *anoeur e.4,

&elier *a 5e aited 5 lo? or-ep or a-uu* eCtra-tion.

8outine anti5ioti- prophlaCi i not re-o**ended. "ontinuou

ele-troni- etal heart rate *onitorin+ i r e-o**ended.

&elier in anti-oa+ulated ?o*en ?ith protheti- ale

I6" hould 5e ?it-hed to 'BH or unra-tionated heparin

($H) ro* the 3Oth ?ee. Bo*en treated ?ith 'BH hould

5e ?it-hed to i.. $H, at leat 3O h 5eore the indu-tion o

la5our or -aearean delier. $H hould 5e di-ontinued 4 – O

h 5eore planned delier, and retarted 4 – O h ater delier

i there are no 5leedin+ -o*pli-ation (ee alo !e-tion.). $r+ent delier in a patient ?ith a *e-hani-al ale tain+

thera peuti- anti-oa+ulation *a 5e ne-ear, and there i a

hi+h ri o eere *aternal hae*orrha+e. % e*er+ent delier i

ne-ear ?hile the patient i till on $H or 'BH, prota*ine

hould 5e -onidered. rota*ine ?ill onl partiall reere the

anti-oa+ulant ee-t o 'BH. %n the eent o ur+ent delier

in a patient on therapeuti- I6", -aearean delier i

preerred to redu-e the ri o intra-ranial hae*orrha+e in the

ull anti-oa+ulated etu. % e*er+ent delier i ne-ear, reh

roen pla*a hould 5e +ien prior to -aearean delier to

a-hiee a tar+et international

nor*alied ratio (%8) o Y2.4

Iral ita*in A (0. – 1 *+)*a

alo 5e +ien, 5ut it tae 4 – O h to inluen-e the %8. % the

*other ?a on I6" at the ti*e o delier, the anti-oa+ulated

ne?5orn *a 5e +ien reh roen pla*a and hould re-eie

ita*in A. he etu *a re*ain anti-oa+ulated or – 10

da ater di-ontinuation o *aternal I6".

entri-ular arrhth*ia durin+ pre+nan- and la5our

6r rhth*ia are the *ot -o**on -ar dia- -o*pli-ation durin+ pre+ nan- in ?o*en ?ith and ?ithout tru-tural heart

dieae.12,O,7

he *a *ani et or the irt ti*e dur in+ pr e+nan-, or

pre+nan- *a eCa-er5ate pr eeCitin+ arr hth*ia. – O0

he 200O

6""/6H6/ E!" +uideline or *ana+e*ent o patient ?ith

entri-ular arr hth *ia and the preention o udden -ardia-

death re-o**end that pre+nant ?o*en ?ith pr olon+ed U

ndro*e ?ho hae had *p to* 5eneit ro* -ontinued 5

5lo-er therap throu+hout pre+ nan-, durin+ delier, and

potpartu* unle there are deinite -ontr aindi-ation. $e o

55lo-er dur in+ la 5our doe not preent uterine

-ontra-tion and a+inal delier.O1

otpartu* -are6 lo? i.. inuion o oCto-in (

,

2 $/*in), ?hi-h aoid te*i-

hpotenion, i ad*initered ater pla-ental delier to preent

*aternal hae*orrha+e. rota+landin analo+ue are ueul to

treat potpartu* hae*orrha+e, unle an in-reae in pul*onar

arter preure (6) i undeira5le. ethler+onoine i -ontr a

indi-ated 5e-aue o the ri (.10S) o ao-ontri-tion and

hpertenion.O2,O3

eti-ulou le+ -are, elati- upport to-in+,

and earl a*5ulation are i*portant to redu-e the ri o

thro*5oe*5oli*. &elier i ao-iated ?ith i*portant hae*o

dna*i- -han+e and luid hit, parti-ularl in the irt 12 – 24

h,

?hi-h *a pre-ipitate heart ailure in ?o*en ?ith tru-tur al

heart dieae. Hae*odna*i- *onitorin+ hould thereore 5e

-ontinued or at leat 24 h ater delier.O4

=reateedin+

'a-tation i ao-iated ?ith a lo? ri o 5a-terae*ia e-ondar

to *atiti. %n hi+hl *pto*ati-/un?ell patient, 5ottle

eedin+ hould 5e -onidered.

2.10 %ne-tie endo-ar diti%ne-tie endo-arditi durin+ pre+nan- i rare, ?ith an eti*ated

oerall in-iden-e o 0.00OS (1 per 100 000 pre+nan-ie)O

and

an in-iden-e o 0.S in patient ?ith no?n alular or -on+enital

heart dieae.OO

he in-iden-e i hi+her in dru+ addi-t. atient

?ith the hi+het ri or ine-tie endo-arditi are thoe ?ith a

protheti- ale or protheti- *aterial ued or -ardia- ale

repair , a hitor o preiou ine-tie endo-arditi, and o*e

pe-ial patient ?ith -on+enital heart dieae.

2.10.1 rophlaCi

he a*e *eaure a in nonpre+nant patient ?ith re-ent *odi

i-ation o +uideline appl.O7

Endo-arditi prophlaCi i no? onl

re-o**ended or patient at hi+het ri o a>uirin+ endo-arditi

durin+ hi+h ri pro-edure, e.+. dental pro-edure. &urin+ delier

the indi-ation or prophlaCi ha 5een -ontroerial and, +ien the

la- o -onin-in+ eiden-e that ine-tie endo-arditi i related to

either a+inal or -aearean delier, anti5ioti- prophlaCi i not

re-o**ended durin+ a+inal or -aearean delier.O7,O


11/52

317E!" #uideline

2.10.2 &ia+noi and ri ae*ent

he dia+noi o ine-tie endo-arditi durin+ pre+nan-

inole the a*e -riteria a in the nonpre+nant patient.O7

%n

pite o pro +re in the dia+noi and treat*ent o ine-tie

endo-arditi, *aternal *or5idit and *ortalit re*ain hi+h,

reportedl 33S in one tud (*ainl due to heart ailure and

thro*5oe*5oli- -o* pli-ation).O9

etal *ortalit i alo hi+h at

29S. Heart ailure due to a-ute ale re+ur+itation i the *ot

-o**on -o*pli-ation, re>uirin+ ur+ent ur+er ?hen *edi-al

treat*ent -annot ta5ilie the patient.O7

"ere5ral and peripheral

e*5oliation are alo re >uent -o*pli-ation .

2.10.3 0r eat*ent

%ne-tie endo-arditi hould 5e treated the a*e ?a a in the

nonpre+nant patient, 5earin+ in *ind the etotoCi- ee-t o anti

5ioti- (ee !e-tion 11). % ine-tie endo-arditi i dia+noed, anti

5ioti- hould 5e +ien +uided 5 -ulture and anti5ioti- enitiit

reult and lo-al treat*ent proto-ol. 6nti5ioti- that -an 5e

+ien durin+ all tri*eter o pre+nan- are peni-illin, a*pi-illin,

a*oCi -illin, erthro*-in, *elo-illin, and -ephaloporin.70

6ll o the* are in-luded in +roup = o the ood and &ru+

6d*initration (&6) -laii-ation. an-o*-in, i*ipene*,

ria*pi-in, and tei-o planin are all +roup ", ?hi-h *ean ri

-annot 5e eC-luded and

their ri – 5eneit ratio *ut 5e -areull -onidered. here i

a deinite ri to the etu in all tri*eter o pre+nan-

?ith

and the hould thereore onl 5e ued or ital indi-ation.71

ale ur+er durin+ pre+nan- hould 5e reered or -ae

?here *edi-al therap ha ailed a per +uideline in nonpre+nant

patient.O7

6 ia5le etu hould 5e deliered prior to ur+er

?here poi5le (ee !e-tion 2..2).

or pr e+nan-

he ri o pre+nan- depend on the pe-ii- heart dieae and

o**ended. 6dole-ent hould 5e +ien adi-e on -ontr a-eption,

and pre+nan- iue hould 5e di-ued a oon a the 5e-o*e

pre+nan- and dru+ reie?ed o that thoe ?hi-h are -ontraindi

-ated in pre+nan- -an 5e topped or -han+ed to alternatie

?here poi5le (ee !e-tion 11.2, a5le 21). he ollo?up plan

hould 5e di-ued ?ith the patient and, i poi5le, her partner.

Bo*en ?ith i+nii-ant heart dieae hould 5e *ana+ed jointl

5 an o5tetri-ian and a -ardiolo+it ?ith eCperien-e in treatin+

pre+nant patient ?ith heart dieae ro* an earl ta+e. Hi+h

ri patient hould 5e *ana+ed 5 an eCpert *ultidi-iplinar

tea* in a pe-ialit -entre. 6ll ?o*en ?ith heart dieae hould

5e aeed at leat on-e 5eore pre+nan- and durin+ pre+nan-,

and hopital delier hould 5e adied.

2.11.2 8i ae*ent: eti*ation o *aternal and

oprin+ ri

o eti*ate the ri o *aternal -ardioa-ular -o*pli-ation ,

eeral approa-he are aaila5le. &ieaepe-ii- ri -an 5e

aeed, and i de-ri5ed in thee +uideline in the repe-tie

0a5le 4 redi-tor o *aternal -ardioa-ular eent

and ri -ore ro* the "688E# tud12

Prior cardiac event (heart #ail%re' transient ischaemic

attac' stroe &e#ore pregnancy or arrhythmia).

aseline *+,A #%nctional class -"" or cyanosis.

e#t heart o&str%ction (mitral valve area


12/52

31 E!" #uideline

0a5le O odiied BHI -laii-ation o *aternal

-ardioa-ular ri: prin-iple

0is5 class 0is5 of pregnanc" b" medical condition

I*o detecta&le increased ris o# maternal

mortality and no8mild increase in mor&idity.

IISmall increased ris o# maternal mortality or

moderate increase in mor&idity.

III

Signi;cantly increased ris o# maternal

mortality or severe mor&idity. =pert

co%nselling re$%ired. "# pregnancy is

decided %pon' intensive specialist

cardiac and o&stetric monitoring needed

I6

=tremely high ris o# maternal mortality

or severe mor&idity> pregnancy

contraindicated. "# pregnancy occ%rs

termination sho%ld &e disc%ssed. "#

odiied ro* horne et al.72

BHI W Borld Health Ir+aniation

appear alua5le to predi-t *aternal ri, althou+h

oer eti*ation -an o--ur .7,73

he "688E# ri -ore i

de-ri5ed in a5le 4. %n ?o*en ?ith -on+enital heart dieae, the

"688E# -ore12

*a alo 5e ao-iated ?ith a hi+her ri o late

-ardioa-ular eent pot pr e+nan-.74

he predi-tor ro* the

;6H686 tud7

(a5le ) hae not et 5een alidated in other

tudie. %t hould 5e noted that predi-tor and ri -ore r o*

the "688E# and ;6H686 tudie are hi+hl population

dependent. %*portant ri a-tor in-ludin+ pul*onar arterial

hpertenion (6H) and dilated aorta ?ere not identiied 5e-aue

the ?ere underrepreented in thee tudie. he "688E#

tud in-luded a->uired and -on+enital heart dieae, ?hile the;6H686 tud ineti+ated a population ?ith -on+enital heart

dieae onl.

he a or-e re-o**end that *aternal ri ae*ent i

-arried out a--ordin+ to the *odiied Borld Health Ir+aniation

(BHI) ri -laii-ation.72

hi ri -laii-ation inte+rate

all no?n *aternal -ardioa-ular ri a-tor in-ludin+ the

underlin+ heart dieae and an other -o*or5idit. %t in-lude

-ontraindi-a tion or pre+nan- that are not in-orporated in

the "688E# and ;6H686 ri -ore/predi-tor. he

+eneral prin-iple o

thi -laii-ation are depi-ted in a5le O. 6 pra-ti-al appli-ation

i +ien in a5le 7. %n ?o*en in BHI -la %, ri i er lo?,

and -ardiolo+ ollo?up durin+ pre+nan- *a 5e li*ited to

one or t?o iit. hoe in BHI %% are at lo? or *oderate ri,and ollo?up eer tri*eter i re-o**ended. or ?o*en in

BHI -la %%%, there i a hi+h ri o -o*pli-ation, and re>uent

(*onthl or 5i*onthl) -ardiolo+ and o5tetri- reie? durin+

pre+nan- i re-o**ended. Bo*en in BHI -la %7 hould 5e

adied a+aint pre+nan- 5ut, i the 5e-o*e pre+nant and ?ill

not -onider ter*ination, *onthl or 5i*onthl reie? i needed.

eonatal -o*pli-ation o--ur in 20 – 2S o patient ?ith heart

dieae12,O,7,7,7O

?ith a neonatal *ortalit 5et?een 1S and

4S.12,O,7

aternal and neonatal eent are hi+hl -orr elated.7

redi-tor o neonatal -o*pli-ation are lited in a5le .

0a5le 7 odiied BHI -laii-ation o *aternal

-ardioa-ular ri: appli-ation

Conditions in which pregnanc" ris5 is #/7 I

? :ncomplicated' small or mild9 p%lmonary stenosis

9 patent d%ct%s arterios%s9 mitral valve prolapse

? S%ccess#%lly repaired simple lesions (atrial orventric%lar septal de#ect' patent d%ct%s arterios%s'

anomalo%s p%lmonary veno%s drainage).

? Atrial or ventric%lar ectopic &eats' isolated

Conditions in which pregnanc" ris5 is #/7 II or III

#/7 II (i# other2ise 2ell and %ncomplicated)

? :noperated atrial or ventric%lar septal de#ect

? epaired tetralogy o# @allot

? 7ost arrhythmias

#/7 II8III (depending on individ%al)

? 7ild le#t ventric%lar impairment

? ,ypertrophic cardiomyopathy

? *ative or tiss%e valv%lar heart disease not considered

? 7ar#an syndrome 2itho%t aortic dilatation? Aorta


13/52

319E!" #uideline

0a5le aternal predi-tor o neonatal eent in

?o*en ?ith heart dieae

3. Smoing d%ring pregnancy

odiied ro* !iu et al.12

("688E# ineti+ator)Q Ahair et al.7O

Q &renthen/7

inlation, it i, ho?eer , not ?ithout ri in patient ?ith 6H, -a

noi, and ontan -ir-ulation. he ri *a 5e lo?er ?ith the

*ini *all inaie htero-opi- te-hni>ue u-h a the Eure

dei-e. Htero-opi- teriliation i peror*ed 5 inertin+ a

*etal *i-roinert or pol*er *atriC into the intertitial

portion o ea-h allopian tu5e. hree *onth ater

pla-e*ent, -orr e-t dei-e pla-e*ent and 5ilateral tu5al

o--luion are -onir*ed ?ith peli- i*a+in+. 6danta+e o

htero-opi- teriliation in-lude the a5ilit to peror* the

pro-edure in an outpatient ettin+ and ?ithout an in-iion. 6

diadanta+e i the 3 *onth

?aitin+ period until tu5al o--luion i -onir*ed.0

ae-to*

or the *ale partner i another ei-a-iou option, 5ut the lon+

ter* pro+noi o the e*ale partner *ut 5e taen into

a--ount a the *ale partner *a outlie her or *an ear.ieper et al. (;6H686 ineti+ator). #ien the la- o pu5lihed data a5out -ontra-eption in heart

LH6 W e? Lor Heart 6o-iation.

2.12 ethod o -ontra-eption andter*ination o pre+nan-, and in itro

ertiliation2.12.1 ethod o -ontra-eption

"ontra-eptie *ethod in-lude -o*5ined hor*onal -ontr a-ep

tie (oetro+en/pro+etin), pro+eto+enonl *ethod, intr auter

ine dei-e, and e*er+en- -ontra-eption. heir ue need to 5e

5alan-ed a+aint the ri o pre+nan-.

%n 2010, the "enter or &ieae "ontrol ("&") *odiied the

BHI u++etion or *edi-al eli+i5ilit -riteria or -ontr a-eptie

ue in ?o*en ?ith "&. Phttp: //???.-d - .+o/*?r/p reie?/

**?rht*l/rr9e02a13.ht*R. onthl inje-ta5le that -ontain

*edroCpro+eterone a-etate are inappropriate or patient ?ith

heart ailure 5e-aue o the tenden- or luid retention. 'o?

doe oral -ontra-eptie -ontainin+ 20 *+ o ethinl etradiol are

ae in ?o*en ?ith a lo? thro*5o+eni- potential, 5ut not in

dieae, adi-e hould 5e proided 5 phi-ian or

+nae-olo+it ?ith appropriate trainin+.

2.12.3 ethod o ter*ination o pr e+nan-

re+nan- ter*ination hould 5e di-ued ?ith ?o*en in ?ho*

+etation repreent a *ajor *aternal or etal ri. he irt tri*e

ter i the aet ti*e or ele-tie pre+nan- ter*ination, ?hi-h

hould 5e peror*ed in hopital, rather than in an outpatient

a-il it, o that all e*er+en- upport eri-e are

aaila5le. he *ethod, in-ludin+ the need or anaetheia, hould

5e -onidered on an indiidual 5ai. Hi+h ri patient hould 5e

*ana+ed in an eCperien-ed -entre ?ith onite -ardia- ur+er.

Endo-arditi pro phlaCi i not -onitentl re-o**ended 5

-ardiolo+it,1

5ut treat*ent hould 5e indiidualied.

#nae-olo+it routinel

adie anti5ioti- prophlaCi to preent pota5ortal endo*etriti,

?hi-h o--ur in – 20S o ?o*en not +ien anti5ioti-.2,3

&ilatation and ea-uation i the aet pro-edure in 5oth the

irt and e-ond tri*eter. % ur+i-al ea-uation i not eai5le

?o*en ?ith -o*pleC alular dieae.77,7

in the e-ond tri*eter, prota+landin E1 or E2, or *iopr otol,6part ro* 5arrier *ethod (-ondo*), the leonor+etr el

releain+ intrauterine dei-e i the aet and *ot ee-tie -ontr a

-eptie that -an 5e ued in ?o*en ?ith -anoti- -on+enital heart

dieae and pul*onar a-ular dieae. %t redu-e *entrual

5lood lo 5 40 – 0S and indu-e a*enorrhoea in a i+nii-ant

pro portion o uer.79

%t hould 5e 5orne in *ind that S o

patient eCperien-e aoa+al rea-tion at the ti*e o i*plantQ

thereore, or thoe ?ith hi+hl -o*pleC heart dieae (e.+. ontan,

Eien*en+er) intrauterine i*plant are indi-ated onl ?hen

pro+eter oneonl pill or der*al i*plant hae proed

una--epta5le and, i ued, the hould onl 5e i*planted in a

hopital eniron*ent. 6 -opper intrauterine dei-e i a--epta5le in non-anoti- or *ildl

-anoti- ?o*en. 6nti5ioti- prophlaCi i not re-o**ended at

the ti*e o inertion or re*oal in-e the ri o peli- ine-tion i

not in-reaed. % eC-eie 5leedin+ o--ur at the ti*e o *ene,

the dei-e hould 5e re*oed. %t i -ontraindi-ated in -anoti-

?o*en ?ith hae*ato -rit leel .S 5e-aue intrini-

hae*otati- dee-t in-reae the ri o eC-eie *entrual

5leedin+.

2.12.2 !teriliation

u5al li+ation i uuall a--o*plihed ael, een in relatiel hi+h

ri ?o*en. =e-aue o the ao-iated anaetheia and a5do*inal

a ntheti- prota+landin tru-turall related to prota+landin E1,

-an 5e ad*initered to ea-uate the uteru.4

hee dru+ are

a5or5ed into the te*i- -ir-ulation and -an lo?er te*i-

a

-ular reitan-e and =, and in-reae heart rate, ee-t that are

+reater ?ith E2 than ?ith E1.

$p to 7 ?ee +etation, *iepritone i an alternatie to

ur+er. Bhen prota+landin E -o*pound are +ien, te*i-

arterial oC+en aturation hould 5e *onitored ?ith a tran-u

taneou pule oCi*eter and norepinephrine inued at a rate that

upport the &=, ?hi-h rele-t te*i- a-ular reitan-e.

rota+landin -o*pound hould 5e aoided 5e-aue the -ani+nii-antl in-reae 6 and *a de-reae -oronar peruion.

!aline a5ortion hould 5e aoided 5e-aue aline a5orption -an

-aue eCpanion o the intraa-ular olu*e, heart ailure, and -lot

tin+ a5nor*alitie.

2.12.4 %n itro ertiliation

%n itro ertiliation *a 5e -onidered ?here the ri o the pro

-edure itel, in-ludin+ hor*onal ti*ulation and pre+nan-, i lo?.

hro*5oe*5oli* *a -o*pli-ate in itro ertiliation ?hen hi+h

oetradiol leel *a pre-ipitate a prothro*5oti- tate.O

1. aseline *+,A class -"" or cyanosis1/

/. 7aternal le#t heart o&str%ction1/'D

1/'!

5. 7%ltiple gestation1/'!

!. :se o# oral anticoag%lants d%ring pregnancy1/

D. 7echanical valve prosthesis!

http://www.cdc.gov/Mmwr/preview/http://www.cdc.gov/Mmwr/preview/http://www.cdc.gov/Mmwr/preview/http://www.cdc.gov/Mmwr/preview/


14/52

31O0 E!" #uideline

2.13 #eneral re-o**endation 3. "on+enital heart dieae and pul*onar hpertenion

0a5le 9 #eneral r e-o**endation

0ecommendations Classa &evel b

Pre9pregnancy ris assessment andco%nselling is indicated in all 2omen2ith no2n or s%spected congenital orac$%ired cardiovasc%lar and aortic

I C

is assessment sho%ld &eper#ormed in all 2omen 2ithcardiac diseases o# child&earing

I C

,igh ris patients sho%ld &e treatedin specialiEed centres &y a

I C

Genetic co%nselling sho%ld &e o##eredto 2omen 2ith congenital heartdisease or congenital arrhythmia'cardiomyopathies' aortic disease or

I C

chocardiography sho%ld &e per#ormedinany pregnant patient 2ith

I C

e#ore cardiac s%rgery a #%ll co%rseo# corticosteroids sho%ld &eadministered to the mother

I C

@or the prevention o# in#ectiveendocarditis in pregnancy thesame meas%res as in non9

I C

aginal delivery is recommended as;rst choice in most patients.

I C

7" (2itho%t gadolini%m) sho%ld &econsidered i# echocardiography is IIa C

"n patients 2ith severehypertension' vaginal delivery 2ithepid%ral analgesia and elective

IIa C

hen gestational age is at least /2ees' delivery &e#ore necessarys%rgery sho%ld &e considered.

IIa C

Caesarean delivery sho%ld &e

considered #or o&stetric indicationsor #or patients 2ith dilatation o# theascending aorta -5! mm'severe aortic stenosis' pre9term la&o%r2hile on oral anticoag%lants'isenmen er s ndrome or severe

IIa C

Caesarean delivery may &e consideredin 7ar#an patients 2ith an aortic IIb C

A chest radiograph' 2ith shielding o#the #et%s' may &e considered i# othermethods are not s%ccess#%l in

IIb C

Cardiac catheteriEation may &econsidered 2ith very strictindications' timing' and shielding o#

IIb C

CT and electrophysiologicalst%dies' 2ith shielding o# the#et%s' may &e considered in

IIb C

Coronary &ypass s%rgery or valv%lar

s%rgery may &e considered 2henconservative and medical therapyhas #ailed' in sit%ations thatthreaten the motherHs li#e and that

IIb C

Prophylactic anti&iotic therapy d%ringdelivery is not recommended.

III C

a"la o re-o**endation.

5'eel o eiden-e.

%n *an ?o*en ?ith -on+enital heart dieae, pre+nan- i ?ell

tolerated. he ri o pre+nan- depend on the underlin+

heart dieae a ?ell a on additional a-tor u-h a entri-ular

and alular un-tion, un-tional -la, and -anoi. he *i-arria+e rate i hi+her in *ore -o*pleC dieae (i+ure 1).

Oaternal

-ardia- -o*pli-ation are preent in 12S o -o*pleted pre+nan

-ie and are a+ain *ore r e>uent a the dieae 5e-o*e *ore

-o*pleC. atient ?ho eCperien-e -o*pli-ation durin+ pre+nan-

*a alo 5e at hi+her ri o late -ardia- eent a ter pre+nan-.74

Iprin+ -o*pli-ation, in-ludin+ oprin+ *ortalit (4S), ar e

*ore re>uent than in the +eneral population.

$uall, -on+enital heart dieae ?ill 5e no?n and dia+noed

5eore pre+nan-. repre+nan- ae*ent in-ludin+ *edi-al

hitor, e-ho-ardio+r aph, and eCer-ie tetin+ i indi-ated in all

patient, ?ith other dia+noti- tet indi-ated on an indiidual

preiou -ardia- eent are o parti-ular pro+noti- alue (ee

a5le 4 and ). 6lo =tpe natriureti- peptide (=)/ter*inal

pro =tpe natriureti- peptide (pro=) ae*ent *a 5e

helpul in ri tratii-ation. 6n eCer-ie tet 5eore pre+nan-

a-hiein+ ,70S o eCpe-ted ?orload, ho?in+ a drop in arterial

preure or a drop in oC+en aturation *a identi ?o*en at

ri o deelopin+ *pto* or -o*pli-ation durin+ pre+nan-.

&ia+noti- pro-edure that -an 5e ued durin+ pre+nan- ar e

outlined in !e-tion 2.O.21

or urther ri ae*ent ee

!e-tion 2.11.

3.1 aternal hi+h ri -ondition PBorldHealth Ir+aniation (%%%) – %Q ee alo!e-tion 2.11Ratient in LH6 -la %%%/%7 or ?ith eerel redu-ed un-tion o

the te*i- entri-le are at hi+h ri durin+ pre+nan-, alon+

?ith other pe-ii- -ondition di-ued 5elo?. %n addition,

o*e

3.1.1 ul*onar hpertenion

aternal ri

dierent pathophiolo+ie ?hi-h in-lude 6H, pul*onar hper

tenion related to let heart dieae, pul*onar hpertenion

related to lun+ dieae and/or hpoCia, -hroni- thro*5oe*5oli-

pul*onar hpertenion, and pul*onar hpertenion ?ith

un-lear and or *ultia-torial *e-hani*. 6H in-lude the idio

pathi- and herita5le or* o the dieae a ?ell a pul*onar

hpertenion ao-iated ?ith -on+enital heart dieae, ?ith or

" W-o*puted to*o+raphQ "& W -ardioa-ular dieaeQ 8% W *a+neti- ?ithout preiou -orre-tie ur+er. 6 *ean 6 Z2 **H+ at

ret i indi-atie o pul*onar hpertenion.7

6 hi+h *ater nalreonan-e i*a+in+ *ortalit ri i reported (30 – 0S in older erie and 17 – 33S


15/52

31O1E!" #uideline

100 6

I0 6

0 6

0 6

D0 6

!0 6

A&ortions

7iscarriages

Completed pregnancies

50 6

30 6

/0 6

10 6

0 6

i+ure 1 &itri5ution o *i-arria+e, -o*pleted pre+nan-ie (.20 ?ee pre+nan- duration), and ele-tie a5ortion or ea-h -on+enital

heart dieae eparatel and the oerall rate. 6!& W atrial eptal dee-tQ 6!& W atrioentri-ular eptal dee-tQ 6I! W aorti- tenoiQ

""#6 W -on+enital -orre-ted tranpoition o the +reat arterieQ "H& W -on+enital heart dieaeQ "oar-tation W aorti- -oar-tationQ

E5tein W E5tein ano*alQ Eien*en+er W Eien*en+er ndro*eQ ontan W patient ater ontan repairQ 6!& W pul*onar atreia

?ith entri-ular eptal dee-tQ ! W pul*onar ale tenoiQ #6 W -o*plete tranpoition o the +reat arterieQ I W tetralo+ o

allotQ !& W entri-ular eptal dee-t.

in *ore re-ent paper) in patient ?ith eere 6H and

Eien*en +er ndro*e.7,

aternal death o--ur in the lat

tri*eter o pre+nan- and in the irt *onth ater delier

5e-aue o pul *onar hpertenie -rie, pul*onarthro*5oi, or rer a-tor ri+ht heart ailure. hi o--ur een in

patient ?ith little or no di a5ilit 5eore or durin+ pre+nan-.

8i a-tor or *aternal death are: late hopitaliation, eerit

o pul*onar hpertenion, and +eneral anaetheia.7

he ri

pro5a5l in-reae ?ith *ore ele ated pul*onar preure.

Ho?eer, een *oderate or* o pul *onar a-ular dieae

-an ?oren durin+ pre+nan- a a reult o the de-reae in

te*i- a-ular reitan-e and oerload o the ri+ht entri-le,

and no ae -uto alue i no?n. Bhether the ri i alo

hi+h or -on+enital patient ater u--eul hunt -loure ?ith

*ildl eleated pul*onar preure Pe.+. ater atrial eptal dee-t

(6!&) -loure ?ith a *ean preure o 30 **H+R i not ?ell

no?n, 5ut thee ri are pro5a5l lo?er and pre+ nan- -an

5e -onidered ater a -areul ri ae*ent on the 5ai o allaaila5le dia+noti- *odalitie in a pe-ialied -entr e.

9

I5tetri- and oprin+ ri

eonatal urial rate are reported to 5e 7 –

9S.7

ana+e*ent

ollo?up. % pre+nan- o--ur, ter*ination hould 5e oered. %n

ie? o the ri o anaetheia thi hould 5e peror*ed in a

tertiar -entre eCperien-ed in the *ana+e*ent o 6H patient.

% patient -hooe to -ontinue pre+nan- depite the ri, the

hould 5e *ana+ed in a -entre ?ith eCpertie in 6H ?ith all

therapeuti- option aaila5le.O

Eer eort hould 5e *ade to

*aintain -ir-ulatin+ olu*e, and to aoid te*i- hpotenion,

hpoCia, and a-idoi ?hi-h *a pre-ipitate rera-tor heart

ailure. !upple*ental oC+en therap hould 5e +ien i there i

hpoCae*ia. %.. prota--lin or aeroolied iloprot hae 5een

o--aionall ued antenatall and peripartu* to i*proe hae*od

na*i- durin+ delier.90

%n patient ?ho are alread tain+ dru+

therap or 6H 5eore 5e-o*in+ pre+nant, -ontinuation o thi

therap hould 5e -onidered, 5ut patient hould 5e inor*ed

a5out the terato+eni- ee-t o o*e therapie, u-h a 5oentan.

Hae*odna*i- *onitorin+ 5 !?an – #an -atheter *a 5e

ao-iated ?ith eriou -o*pli-ation u-h a pul*onar arter

rupture, ?hile it utilit ha not 5een de*ontratedQ thereore, it

i rarel i eer indi-ated.

edi-al therap. %n patient ?here the indi-ation or anti-oa+ulation

outide pre+nan- i eta5lihed, anti-oa+ulation hould alo 5e*aintained durin+ pre+nan-.

9 %n 6H ao-iated ?ith -on+enital

-ardia- hunt in the a5en-e o i+nii-ant hae*opti, anti-oa+u

lant treat*ent hould 5e -onidered in patient ?ith pul*onar

arter thro*5oi or i+n o heart ailure. %n 6H ao-iated

?ith -onne-tie tiue diorder, anti-oa+ulant treat*ent hould

5e -onidered on an indiidual 5ai. %n 6H ao-iated ?ith

portal hpertenion, anti-oa+ulation i not re-o**ended in

patient ?ith in-reaed ri o 5leedin+.


16/52


17/52

31O3E!" #uideline

3.2 aternal lo? and *oderate ri-ondition (Borld Health Ir+aniation %,%%, and %%%Q ee alo a5le O and 7)%n patient ?ho hae under+one preiou u--eul ur+i-al repair

?ithout *e-hani-al heart ale i*plantation, pre+nan- i oten

?ell tolerated i eCer-ie toleran-e i +ood, entri-ular un-tion

i nor*al, and un-tional tatu i +ood. 6lthou+h patient needto 5e inor*ed a5out the (oten *all) additional ri,

pre+nan- hould not 5e di-oura+ed. atient hould 5e een

5 the end o the irt tri*eter and a ollo?up plan ?ith ti*e

interal or reie? and ineti+ation u-h a e-ho-ardio+ra*

deined. he ollo?up plan hould 5e indiidualied tain+ into

a--ount the -o*pleCit o the heart dieae and -lini-al tatu o

the patient. !o*e -on+enital -ondition *a deteriorate durin+

pre+nan-, thereore ollo?up ti*eline need to 5e leCi5le.

a+inal delier

-an 5e planned in *ot -ae.3,93,94

3.3 !pe-ii- -on+enital heart dee-t

3.3.1 6trial eptal dee-t

aternal ri

re+nan- i ?ell tolerated 5 *ot ?o*en ?ith an 6!&. he

onl -ontraindi-ation i the preen-e o 6H or Eien*en+er

ndro*e

(ee !e-tion 3.2.1 and 3.2.2).9

"loure o a hae*odna*i-all i+

3.3.2 entri-ular eptal dee-t

aternal ri

or lar+e entri-ular eptal dee-t (!&) ?ith pul*onar hper

tenion, ee *aternal hi+h ri -ondition (!e-tion 3.1). !*all peri

*e*5ranou !& (?ithout let heart dilatation) hae a lo? ri o

-o*pli-ation durin+ pre+nan-.9

"orre-ted !& hae a +ood

pro+noi durin+ pre+nan-, ?hen ' un-tion i preered. r e

pre+nan- ealuation o the preen-e o a (reidual) dee-t,

-ardia- di*enion, and an eti*ation o pul*onar preure i

re-o**ended.


ree-la*pia *a o--ur *ore oten than in the nor*al

population.9

ana+e*ent

$uall ollo?up t?i-e durin+ pre+nan- i ui-ient and pon

taneou a+inal delier i appro priate.

3.3.3 6trioentri-ular eptal dee-t

aternal ri

6ter -orre-tion, pre+nan- i uuall ?ell tolerated ?hen reidual

ale re+ur+itation i not eere and entri-ular un-tion i nor*al

(BHI ri -la %%). atient ?ith eere (reidual) let atrioentri

-ular (6) ale re+ur+itation ?ith *pto* and/or i*paired en

tri-ular un-tion hould 5e treated ur+i-all prepr e+nan-,7

nii-ant 6!& hould 5e peror*ed 5eore pre+nan-. aourin+ ale repair. or atrioentri-ular eptal dee-t (6!&)

hro*5oe*5oli- -o*pli-ation hae 5een de-ri5ed in up to

S.O

6rrhth*ia o--ur *ore oten than in health ?o*en,

?ith pul*onar hpertenion, ee *aternal hi+h ri -ondition

(!e-tion 3.1.1). "orre-tion o a hae*odna*i-all i+nii-ant19

epe-iall ?hen the 6!& i unrepaired or -loed at older a+e 6!& 5eore pre+nan- hould 5e -onidered. 6rrhth*ia

and the pre+nant ?o*an i .30 ear old.9,9O


%n ?o*en ?ith unrepaired 6!&, pree-la*pia and *all or +eta

tional a+e 5irth *a o--ur *ore r e>uentl. %n repaired 6!&, no

eCtra ri i en-ounter ed.

ana+e*ent

$uall ollo?up t?i-e durin+ pre+nan- i ui-ient. or a e-un

du* dee-t, -atheter dei-e -loure -an 5e peror*ed durin+ pre+

nan-, 5ut i onl indi-ated ?hen the -ondition o the *other i

deterioratin+ (?ith tranoeopha+eal or intra-ardia- e-ho-ardio

+raphi- +uidan-e). "loure o a *all 6!& or peritent ora*en

oale or the preention o paradoCi-al e*5oli i not indi-ated.

=e-aue o the in-reaed ri o paradoCi-al e*5oli*, in

?o*en ?ith a reidual hunt, preention o enou tai (ue o

-o*preion to-in+ and aoidin+ the upine poition) i i*por

tant, a i earl a*5ulation ater delier. or prolon+ed 5ed ret,

prophla-ti- heparin ad*initration hould 5e -onidered.97

&ili

+ent -are i i*portant to eli*inate air in i.. line ?hi-h -ould

lead to te*i- e*5oliation due to ri+httolet huntin+ durin+

la5our.

!pontaneou a+inal delier i in *ot -ae appro priate.

and ?orenin+ o LH6 -la a ?ell a ?orenin+ o 6 ale

re+ur+itation hae 5een de-ri5ed durin+ pre+nan-.

99

he ri o heart ailure i lo? and onl eCit in ?o*en ?ith eere re+ur

+itation or i*paired entri-ular un-tion.


I5tetri- -o*pli-ation are *ainl related to the ri o a-ute

heart ailure durin+ or jut ater delier and the depend on *p

to* and 6 durin+ pre+nan-. Iprin+ *ortalit ha 5een

reported in OS, pri*aril due to the o--urren-e o -o*pleC -on

+enital heart dieae.99

ana+e*ent

ollo?up. ollo?up durin+ pre+nan- i adia5le at leat on-e

ea-h tri*eter. "lini-al and e-ho-ardio+raphi- ollo?up i indi

-ated *onthl or 5i*onthl in patient ?ith *oderate or eer e

ale re+ur+itation or i*paired entri-ular un-tion. %n un-orre-ted

6!&, the ri o paradoCi-al e*5oliation eCit. or re-

o**ended preentie *eaure or thro*5oe*5oli*, ee

!e-tion 3.3.1.

&elier. !pontaneou a+inal delier i appropriate in *ot -ae.

3.3.4 "oar-tation o the aorta

aternal ri

re+nan- i oten ?ell tolerated in ?o*en ater repair o -oar -ta

tion o the aorta ("o6) (BHI ri -la %%). !i+nii-ant (re)


18/52

31O4 E!" #uideline

-oar-tation hould 5e -orre-ted 5eore pre+nan-. Bo*en ?ith

unrepaired natie "o6 and thoe repaired ?ho hae reidual

hpertenion, reidual "o6, or aorti- aneur* hae an

in-reaed ri o aorti- rupture and rupture o a -ere5ral

aneur* durin+ pre+nan- and delier. Ither ri a-tor or

thi -o*pli-ation in-lude aorti- dilatation and 5i-upid aorti-

ale, and the hould 5e looed or prepr e+nan-.


6n eC-e o hpertenie diorder and *i-arria+e ha 5een

reported.100,101

ana+e*ent

"loe ureillan-e o = i ?arranted, and re+ular ollo?up at leat

eer tri*eter i indi-ated. Hpertenion hould 5e treated,

althou+h a++reie treat*ent in ?o*en ?ith reidual -oar-tation

*ut 5e aoided to preent pla-ental hpoperuion. er-utaneou

interention or re"o6 i poi5le durin+ pre+nan-, 5ut it i

ao-iated ?ith a hi+her ri o aorti- die-tion than outide

pre+ nan- and hould onl 5e peror*ed i eere hpertenion

per it depite *aCi*al *edi-al therap and there i

*aternal or etal -o*pro*ie. he ue o -oered tent *a

lo?er the ri o die-tion.

&elier. !pontaneou a+inal delier i preerred ?ith ue o epi

dural anaetheia parti-ularl in hpertenie patient.

3.3. ul*onar ale tenoi and r e+ur+itation

aternal ri

ul*onar ale tenoi (!) i +enerall ?ell tolerated durin+

pre+nan-.102 – 104

Ho?eer , eere tenoi *a reult in -o*pli

-ation in-ludin+ ri+ht entri-ular (8) ailure and arrhth*ia. re

pre+nan- relie o tenoi (uuall 5 5alloon aluloplat)

hould 5e peror*ed in eere tenoi ( pea &oppler +radient

.O4 **H+).19,O,10

!eere pul*onar re+ur+itation ha 5een identiied a an inde

pendent predi-tor o *aternal -o*pli-ation, epe-iall in patient

?ith i*paired entri-ular un-tion.7O,10O

%n *pto*ati- ?o*en or

?hen 8 un-tion i a5nor*al due to eere pul*onar re+ur+ita

tion, prepre+nan- pul*onar ale repla-e*ent ( preera5l

5io prothei) hould 5e -onidered.


he in-iden-e o *aternal o5tetri- -o*pli-ation, parti-ularl

hpertenionrelated diorder in-ludin+ ( pre)e-la*pia, *a 5e

in-reaed in ?o*en ?ith !.103

he in-iden-e o oprin+ -o*pli

-ation alo appear to 5e hi+her than in the +eneral population.103

ul*onar re+ur+itation +enerall -arrie no additional oprin+

ri.

ana+e*ent

ollo?up. ild and *oderate ! are re+arded lo?ri leion

(BHI ri -lae % and %%) (a5le O and 7), and ollo?up on-e

eer tri*eter i ui-ient. %n patient ?ith eere !, *onthl

or 5i*onthl -ardia- ealuation in-ludin+ e-ho-ardio+raph ar e

adied to deter*ine -lini-al tatu and or ureillan-e o 8 un-

tion. &urin+ pre+nan- in eerel *pto*ati- ! not

repondin+ to *edi-al therap and 5ed ret, per-utaneou

aluloplat -an 5e underta en.

&elier. a+inal delier i aoured in patient ?ith noneere

!, or eere ! in LH6 -la %/%%. "aearean e-tion i

-onidered in patient ?ith eere ! and in LH6 -la %%%/%7

depite *edi-al therap and 5ed ret, in ?ho* per-utaneou

pul*onar alot o* -annot 5e peror*ed or ha ailed.

3.3.O 6orti- tenoi

"on+enital 6! i *ot oten -aued 5 a 5i-upid aorti- ale. herate o pro+reion o tenoi in thee oun+ patient i lo?er

than in older patient.107

=e-aue 5i-upid aorti- ale i ao-iated

?ith aorti- dilatation and aorti- die-tion, aorti- di*enion

hould 5e *eaured prepre+nan- and durin+ pre+nan-. he

ri o die-tion i in-reaed durin+ pre+nan- (ee alo !e-tion

4.3).10,109

6ll ?o*en ?ith a 5i-upid aorti- ale hould

under+o i*a+in+ o the a-endin+ aorta 5eore pre+nan-, and

ur+er hould 5e -onidered ?hen the aorti- dia*eter i

.0 **. or re-o**endation on the *ana+e*ent o pre+nant

?o*en ?ith 6!, ee !e-tion on alular heart dieae.

3.3.7 0etralo+ o allot

aternal ri

%n unrepaired patient, ur+i-al repair i indi-ated 5eore pre+nan-. Bo*en ?ith repaired tetralo+ o allot uuall toler ate

pre+nan- ?ell (BHI ri -la %%). "ardia- -o*pli-ation durin+

pre+nan- hae 5een reported in up to 12S o patient. 6rrhth

*ia and heart ailure in parti-ular *a o--ur.110

Ither -o*pli

-ation in-lude thro*5oe*5oli*, pro+reie aorti- root

dilatation, and endo-arditi. &un-tion o the ri+ht entri-le

and/or *oderate to eere pul*onar re+ur+itation are ri

a-tor or -ardioa-ular -o*pli-ation, and pre+nan- *a 5e

ao-iated ?ith a peritin+ in-reae in 8 ie. %n *pto*ati-

?o*en ?ith *ared dilatation o the ri+ht entri-le due to

eere pul*onar re+ur+itation, prepre+nan- pul*onar ale

repla-e*ent (ho*o+rat) hould 5e -onider ed.19


he ri o oprin+ -o*pli-ation i in-reaed.

ana+e*ent

ollo?up. ollo?up eer tri*eter i ui-ient in the *ajorit o

?o*en. %n ?o*en ?ith eere pul*onar re+ur+itation, *onthl

or 5i*onthl -ardia- ealuation ?ith e-ho-ardio+raph i indi

-ated. % 8 ailure o--ur durin+ pre+nan-, treat*ent ?ith diure

ti- hould 5e tarted and 5ed ret adied. ran-atheter ale

i*plantation or earl delier hould 5e -onidered in thoe

?ho do not repond to -oneratie treat*ent.

&elier. he preerred *ode o delier i a+inal in al*ot all

-ae.

3.3. E5tein ano*alaternal ri

%n ?o*en ?ith E5tein ano*al ?ithout -anoi and heart

ailure, pre+nan- i oten tolerated ?ell (BHI ri -la%%).

!*p to*ati- patient ?ith -anoi and/or heart ailure

hould 5e treated 5eore pre+nan- or -ounelled a+aint

pre+nan-. %n eere *pto*ati- tri-upid re+ur+itation (8),

repair hould 5e -onidered prepr e+nan-. he hae*odna*i-

pro5le* een durin+ pre+nan- depend lar+el on the eerit

o the 8 and


19/52

31OE!" #uideline

the un-tional -apa-it o the ri+ht entri-le.111,112

6n 6!& and

alo the Bol – arinon – Bhite ndro*e are -o**on

ao-i ated indin+. he in-iden-e o arrhth*ia *a rie

durin+ pre+ nan- and i ao-iated ?ith a ?ore pro+noi.111


he ri o pre*ature delier and etal *ortalit i eleated.112

ana+e*ent

ollo?up. Een eere 8 ?ith heart ailure -an uuall 5e *ana+ed

*edi-all durin+ pre+nan-. Bo*en ?ith E5tein ano*al and

interatrial huntin+ -an deelop hunt reeral and -anoi in

pre+nan-. here i alo a ri o paradoCi-al e*5oli (ee

!e-tion 3.4.2).

&elier. he preerred *ode o delier i a+inal in al*ot all

-ae.

3.3.9 0ranpoition o the +reat arterie

aternal ri

hou+h *an ?o*en tolerate pre+nan- relatiel ?ell, ater an

atrial ?it-h operation (!ennin+ or utard repair) patient haean in-reaed ri o deelopin+ -o*pli-ation u-h a arrhth*ia

(o*eti*e liethreatenin+), and heart ailure (BHI ri -la

%%%).93

!o*e o thee ?o*en ?ill hae underlin+ 5rad-ardia or

jun-tional rhth*. %n thee -enario, 55lo-er need to 5e

ued -autioul, i at all. 6n irreeri5le de-line in 8 un-tion

ha 5een de-ri5ed in 10S o -ae. atient ?ith *ore than *od

erate i*pair*ent o 8 un-tion or eere 8 hould 5e adied

a+aint pre+nan-.


ree-la*pia and pre+nan-indu-ed hpertenion a ?ell a

o prin+ -o*pli-ation are *ore oten en-ountered than in

nor*al pre+nan-.

ana+e*ent

ollo?up. %t i re-o**ended that patient ?ith a utard or

!ennin+ repair hae *onthl or 5i*onthl -ardia- and e-ho-ardio

+raphi- ureillan-e o *pto*, te*i- 8 un-tion, and heart

rhth*.

&elier. %n a*pto*ati- patient ?ith *oderate or +ood entri-u

lar un-tion, a+inal delier i adied. % entri-ular un-tion

deteriorate, an earl -aearean delier hould 5e planned to

aoid the deelop*ent or ?orenin+ o heart ailure.113

6rterial ?it-h oper ation

Inl *all erie o patient ?ith an arterial ?it-h operation and

pre+nan- hae 5een de-ri5ed o ar.114

he ri o pre+nan-

ee* lo? in thee patient ?hen there i a +ood -lini-al -ondition

pr epre+nan-. a+inal delier i adied.

3.3.10 "on+enitall -orre-ted tranpoition o the +r eat

arterie

aternal ri

%n patient ?ith -on+enitall -orre-ted tranpoition o the +reat

arterie (alo -alled atrioentri-ular and entri-uloarterial

di-ordan-e), ri depend on un-tional tatu, entri-ular un-

tion, preen-e o arrhth*ia, and ao-iated leion. atient

hae an in-reaed ri o deelopin+ -o*pli-ation u-h a arrhth

*ia (o*eti*e liethreatenin+), and heart ailure (BHI ri

-la %%%). hee patient are predipoed to deelopin+ 6

5lo-Q thereore, 55lo-er *ut 5e ued ?ith eCtre*e -aution.

6n irreeri5le de-line in 8 un-tion ha 5een de-ri5ed in 10S

o -ae.11,11O

atient ?ith LH6 un-tional -la %%% or %7, i*por

tant entri-ular dun-tion Peje-tion ra-tion (E) ,40SR, or

eere 8 hould 5e -ounelled a+aint pre+nan-.


he rate o etal lo i in-reaed.

ana+e*ent

ollo?up. %t i re-o**ended that patient hae re>uent e-ho ur

eillan-e o te*i- 8 un-tion (eer 4 – ?ee) and

ae *ent o *pto* and heart rhth*.

&elier. %n a*pto*ati- patient ?ith *oderate or +ood entri

-ular un-tion, a+inal delier i adied. % entri-ular un-tion

deteriorate, an earl -aearean delier hould 5e planned to

aoid the deelop*ent or ?orenin+ o heart ailure.

3.3.11 ontan -ir-ulation

aternal ri

6lthou+h u--eul pre+nan- i poi5le in ele-ted patient ?ith

intenie *onitorin+, thee are *oderate to hi+h ri pre+nan-ie

and patient hould 5e -ounelled -areull (BHI ri -la %%% or

%). here i pro5a5l a hi+her *aternal ri i the ontan -ir-uit i

not opti*al, and -areul ae*ent prepre+nan- i indi-ated.

6trial arrhth*ia and LH6 -la deterioration hae 5een

de-ri5ed.117,11

atient ?ith oC+en aturation ,S at ret,

depreed entri-ular un-tion, and/or *oderate to eere 6re+ur+itation or ?ith proteinloin+ enteropath hould 5e -oun

elled a+aint pre+nan-.


he oprin+ ri in-lude pre*ature 5irth, *all or +etational

a+e, and etal death in up to 0S.

ana+e*ent

ollo?up. %t i re-o**ended

Documents

12197 PREGN Guidelines Pregnancy FT