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Review
Cardiology Department, Royal
Liverpool and Broadgreen
University Hospitals NHS Trust,
Liverpool, UK
Correspondence to
Dr J Pyatt, Consultant
Cardiologist, Cardiology
Department, Royal Liverpool and
Broadgreen University Hospitals
NHS Trust, Prescot Street,
Liverpool L7 8XP, UK;
jason.pyatt@rlbuht.nhs.uk
Received 29 December 2009
Accepted 28 August 2010
Published Online First
10 October 2010
34
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Peripartum cardiomyopathy: current understanding,
comprehensive management review and
new developments
Jason R Pyatt, Gopal Dubey
ABSTRACT
Peripartum cardiomyopathy (PPCM) is a rare and
potentially fatal disease which presents with symptoms
of heart failure primarily due to left ventricular (LV)
systolic dysfunction in the last month of pregnancy and
up to 5e6 months after delivery. PPCM is still regarded
as a disease of unknown aetiology, although recent
evidence suggests a role for a 16 kDa prolactin derivative
produced by proteolytic cleavage of prolactin secondary
to unbalanced oxidative stress present during late
pregnancy and early puerperium. The medical
management of PPCM is similar to other forms of non-
ischaemic dilated cardiomyopathy, but with the
management tailored to choose safe drugs in pregnancy
and lactation to minimise maternal and fetal morbidity.
There is an increased risk of venous thromboembolism,
and anticoagulation is recommended. About 30e50% of
the patients recover without complications, with their
baseline LV systolic function at rest returning to normal.
The risk of recurrence of PPCM is high, especially if the
LV systolic function has not fully recovered. However, for
those women who have normal LV systolic function as
demonstrated on echocardiography and dobutamine
stress test, the risk of severe cardiomyopathy including
death is relatively low in a subsequent pregnancy.
INTRODUCTION
Peripartum cardiomyopathy (PPCM) is a rare and
potentially fatal disease which presents with
symptoms of heart failure primarily due to left
ventricular (LV) systolic dysfunction presenting in
the last part (mean 32e38 weeks) of pregnancy and
up to 5e6 months after delivery.1e5 Usually, it
occurs early in the postpartum period, with about
45% in the first week and 75% within the first
month.3 Clinically, it is very similar to other forms
of non-ischaemic dilated cardiomyopathy except for
its unique relationship with pregnancy3 4 and the
higher likelihood for full recoverydthat is, normal-
isation of ejection fraction (EF) in almost half of the
cases.5 However, it can still result in chronic
disability and ultimately death in relatively young
women in their reproductive years. This emphasises
the need for a thorough understanding of PPCM, as
its care will transcend many different specialities
and mandates a multidisciplinary approach to its
management. The following review will provide
a comprehensive overview of the condition, espe-
cially focusing on its clinical features and manage-
ment during pregnancy, treatment after pregnancy,
and implications for future conception, as well as
highlighting exciting recent new developments.
AETIOLOGY
Despite extensive research into its underlying aeti-
ology, it is still not clear exactly how it occurs, so
PPCM is still regarded as a disease of unknown
aetiology.1e3 5 However, many studies lend support
to the idea of an inflammatory pathology leading to
myocarditis,6 due to either a viral infection2 4 7e10
or an autoimmune response in pregnancy against
maternal myocardium provoked by the release of
fetal antigen into maternal blood or by some, as
yet, unknown agents.11e14 None of these proposed
mechanisms are strongly supported by clinical
evidence, as there has been great variability in the
results of endomyocardial biopsies and in the
finding of autoantibodies against myocardium.
Endomyocardial biopsies of the reported cases have
shown features of myocarditis ranging between 9%
and 78%.12 15e17 However, there may be a role for
proinflammatory cytokines such as tumour
necrosis factor10 and interleukins (IL-1, IL-6) in the
aetiology as well.18e20
Recent evidence in an animal (mice) model
suggests a role for a 16 kDa prolactin derivative
produced by proteolytic cleavage of prolactin
secondary to unbalanced oxidative stress present
during late pregnancy and early puerperium.21 This
derivative has been found to be cardiotoxic, anti-
angiogenic, proapoptotic and proinflammatory,
which can potentially damage or impair metabo-
lism and contractility of cardiomyocytes.22e24 This
theory is further supported by small clinical reports
of postpartum women with PPCM responding
(recovery from PPCM) when treated with medi-
cines causing reduced secretion of prolactin from
posterior pituitary gland or working as a D2
receptor antagonist such as bromocriptine and
carbergoline.25e27
Other theories include impaired cardiac micro-
circulation, programmed cell death (apoptosis),2 5
deficiency of micronutrients such as selenium,
possible genetic links, and other environmental
factors.28e30 Indeed, in certain cultures where the
incidence of PPCM is high, certain cultural practices
performed during the puerperium such as
consuming lake salt or rock salt (known as ‘kanwa’
which has a particularly high sodium content) to
promote the flow of breast milk and the heating of
the body by sitting on a clay bed with a fire beneath
to keep warm (a belief felt to ward off infection)
have both been suggested as contributory factors in
its development as well.31 32
Overall it appears that there is a multifactorial
aetiology albeit currently unknown. However,
whatever the initial trigger or combination of
Postgrad Med J 2011;87:34e39. doi:10.1136/pgmj.2009.096594
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initiating processes, myocardial biopsy in PPCM shows damage
to myocardium with progressive death of cardiomyocytes and
destruction of the cytoskeleton of the heart, resulting in
progressive loss of heart muscle, ultimately leading to clinical
heart failure.33
EPIDEMIOLOGY
PPCM is not restricted to any particular childbearing age, but in
58% of cases the age at presentation was older than 30 years,
with primigravida occurring in approximately a third of women
(27e33%).3 Its highest incidence occurs in African women or
African American women from southern USA, with the lowest
incidence in Hispanic women in the USA. It has also been
reported in Caucasians, Japanese, Chinese, Indians and Korean
women. Recent reports suggest an estimated incidence of one
case per 299 live births in Haiti,34 35 one case per 1000 live births
in South Africa, and one case per 2289e4000 live births in the
USA.36 These more recent data from the USA suggest a higher
incidence than reported in earlier studies, especially in certain
ethnic groups.4 36 37 Its true incidence in the UK has not been
estimated.
On the basis of several large reported series of PPCM, the
following have been recognised as risk factors favouring devel-
opment and recurrence of PPCM: advanced maternal age, multi-
parity, Afro-American race, twin pregnancy, pre-eclampsia,
gestational hypertension1 2 4 5 and use of tocolysis (b2 stimu-
lants).38 PPCM appears not to have a hereditary association.12 18 39
CLINICAL PRESENTATION OF PPCM
Presentation of PPCM is similar to that of patients presenting
with LV systolic heart failure due to other causes. Usually
women in late pregnancy or early puerperium present with
palpitations, fatigue, shortness of breath at rest or on exertion,
cough, paroxysmal nocturnal dyspnoea, and orthopnoea.
DIAGNOSIS OF PPCM
It is largely a diagnosis of exclusion. Other causes of heart
disease such as congenital heart disease or acquired condi-
tionsdthat is, myocardial infarction causing LV dysfunction,
pulmonary hypertension or valvular heart diseasedmust be
ruled out first before making a diagnosis of PPCM.12 37 40 It is
then diagnosed in previously healthy women presenting with
symptoms of heart failure and evidence of decreased LV systolic
function from late pregnancy to early puerperium.
However, the diagnosis of PPCM poses many challenges,
as many women in the last month of normal pregnancy expe-
rience similar symptoms to that of early heart failure, such as
shortness of breath on exertion, nocturnal dyspnoea and cough,
fatigue, palpitations and pedal oedema, making differentiation
difficult.5 16 17 37 38 Clinical examination remains important for
making the diagnosis, and findings of a pulse rate >100 beats/min,
elevated jugular venous pressure, third heart sound and basal
crepitations on lung auscultation are abnormal in pregnancy and
suggest heart failure.
Although, PPCM presents in late pregnancy and early puer-
perium, early onset of pregnancy-associated cardiomyopathy has
been reported, albeit in relatively few women from one series.3
Also, recent observations from Haiti suggest that a subclinical
form of PPCM without overt clinical symptoms may exist. The
investigators identified four clinically normal postpartum
women with asymptomatic systolic dysfunction on echocardi-
ography, who subsequently either developed clinically detectable
dilated cardiomyopathy or improved their LV function and
Postgrad Med J 2011;87:34e39. doi:10.1136/pgmj.2009.096594
Review
completely recovered, suggesting a possible latent phase before
the development of overt heart failure or spontaneous
recovery.41 However, a proportion of these cases may represent
either subclinical dilated cardiomyopathy presenting in early
pregnancy for the first time or a viral myocarditis and so distinct
from true PPCM.
Therefore it is imperative to maintain a high index of suspi-
cion to identify cases of PPCM. Original diagnostic criteria for
PPCM were developed by Demakis et al in 1971.5 They did not
include echocardiographic findings because echocardiography
was not readily available at that time. In 1999, echocardio-
graphic criteria were incorporated in a new definition (box 1).12
All patients should have routine blood tests to exclude
anaemia, electrolyte disturbance, and kidney, liver and thyroid
dysfunction, including inflammatory markers, a septic screen
and viral serology. None of the routine or specific blood tests can
help in screening for or positively determining a diagnosis of
PPCM. Even cardiac markers such as troponins and creatinine
kinase-MB are not helpful alone in reaching a diagnosis.
A chest x-ray confers negligible radiation to the fetus at any
stage of gestation and should be performed in a woman
suspected of having PPCM, and no shielding is necessary.42
Radiological signs of heart failure such as cardiomegaly,
pulmonary congestion and pleural effusion may be found.
Again, the ECG is mostly non-specific. It may show sinus
tachycardia or other arrhythmias such as atrial fibrillation, atrial
flutter and ventricular tachycardia, as they all have been
reported in PPCM. An intraventricular block pattern, non-
specific ST-T changes and LV hypertrophy pattern may also be
present.43
Thus echocardiography is required to establish dilated cardiac
chambers and reduced LV systolic function and to differentiate
PPCM from other causes of heart failure, such as valve disease,
etc. The likelihood of recovery of cardiac function after pregnancy
is most strongly correlated with the degree of LV dilatation and
systolic function seen on echocardiography at diagnosis.44
The role of endomyocardial biopsy in the diagnosis of PPCM
is controversial.9 10 16 At most, it has a sensitivity of 50% and
a specificity of 99%.5 However, it is not routinely recommended,
as it is not widely available, is not specific to PPCM as it can be
positive for myocarditis in similar numbers of patients with
other forms of dilated cardiomyopathy, and can have a relatively
high complication rate.
Cardiac MRI is used widely in other forms of cardiomyopa-
thies particularly for diagnostic and prognostic purposes.
However, it cannot reveal a specific pattern in PPCM which
would help in differentiating it from other forms of cardi-
omyopathy.45e47 Coronary angiography is not routinely indi-
cated, as coronary arteries are usually normal in PPCM.
Early involvement of a cardiologist is recommended for facil-
itating the diagnosis. Timely diagnosis of PPCM is important, as
Box 1 Diagnostic criteria for peripartum cardiomyopathy
< Onset of heart failure in last month of pregnancy to
5e6 months post partum
< Without any other demonstrable cause of heart failure
< Absence of any heart disease before pregnancy
< Echocardiography criteria to include an ejection fraction
<45%, fractional shortening <30% or both, and end-diastolic
dimension (LVIDd) >2.7 cm/m2 body surface area
35
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Review
pregnancy in patients with dilated cardiomyopathy, especially
PPCM, is associated with high maternal and fetal morbidity.
Early diagnosis and prompt initiation of treatment are essential
to optimise pregnancy outcome.
GENERAL MANAGEMENT OF PPCM
This needs a multidisciplinary team approach. Involvement of
a cardiologist, obstetrician, intensivist, anaesthetist and paedia-
trician is imperative at the earliest possible stage of the disease to
reduce the mortality and morbidity associated with it. The type
and level of monitoring and care should be tailored according to
the severity of cardiac decompensation and response to the
treatment.48
In general, the medical management of PPCM is similar to
that of other forms of non-ischaemic dilated cardiomyopathy.
However, the management strategy must be tailored to choose
safe drugs in pregnancy and lactation to minimise maternal and
fetal morbidity. The aims of medical treatment should be to
reduce fluid and salt intake, increase myocardial contractility,
reduce cardiac preload and cardiac afterload, and prevent
complications and mortality, importantly, thromboembolism,
progressive heart failure and cardiac arrhythmia.
Fluid restriction to 2 litres per day and salt restriction (2e4 g
per day) is advisable to improve symptoms.5 49 Strict bed rest is
not recommended except when the patient cannot tolerate
mobility because of symptoms and severity of heart failure, as
this increases the risk of thromboembolism.50 Once symptoms
are improving with medical treatment, modest exercise is
recommended.17 50
Diuretics are safe agents which help to reduce pulmonary
congestion and relieve symptoms, as they reduce preload. In the
hospital setting, loop diuretics are safer to use,6 49 51 52 but, in
mild cases, thiazide diuretics are sufficient. Spironolactone may
not be safe based on animal studies, although data for pregnancy
in humans are limited.49
During pregnancy, nitrates and hydralazine are considered safe
drugs to reduce afterload. ACE inhibitors should not be used for
PPCM as, in the 2nd and 3rd trimester of pregnancy, they have
toxic effects on fetal kidneys, resulting in oligohydramnios,
hypocalvaria and sometimes fetal renal failure and death.53
However, ACE inhibitors should be used after birth or if PPCM is
diagnosed in the puerperium. Digoxin is safe during pregnancy
and lactation and is used to increase contractility of the failing
heart provided that the digoxin level is monitored and kept in
the therapeutic range.54 55
To reduce the risk of arrhythmia and sudden death and improve
long-term prognosis, a b blocker should be used if it is not
contraindicated. Also, it is important to address electrolyte
imbalance, which may predispose to arrhythmia, particularly as
low potassium and magnesium are common in pregnancy. The
benefit of b blockers to maternal health usually outweighs the
potential risk to the baby. This risk is of growth restriction and
low-birth-weight babies. Therefore, growth should be monitored
regularly in the 2nd and 3rd trimester.56 Specific antiarrhythmic
drugs, such as adenosine and flecainide, should be used cautiously,
mainly in the acute setting, and are generally well tolerated,
although their safety for the baby cannot be guaranteed.57 Routine
use of long-term antiarrhythmics should be avoided in PPCM
because of their proarrhythmic potential in LV impairment.
There is an increased risk of thromboembolism in most types
of dilated cardiomyopathy (especially if EF <35% and the
ventricles are severely dilated). Pregnancy and the puerperium
further increase this risk because of the hypercoaguable state of
pregnancy.58 59 To help this, anticoagulation is recommended.
36
Although warfarin is teratogenic in early pregnancy, its main
contraindication in PPCM is its risk of fetal cerebral haemor-
rhage in the second and third trimester. Thus heparins are
favoured in pregnancy, as they (unlike warfarin) do not cross the
placenta. The risk of thromboembolism is particularly high if
the patient is in atrial fibrillation, has a history of venous
thromboembolism, or echocardiography shows mural thrombus,
EF <35% or severely dilated LV. Low-molecular-weight heparins
(LMWH) are preferred because they have a lower risk of osteo-
porosis and of heparin-induced thrombocytopenia. They are
eliminated more rapidly in pregnancy, resulting in a more
common dosage regimen, and their dose is adjusted according to
body weight in early pregnancy. Currently, only dalteparin,
enoxaparin and tinzaparin are used in the UK and, in those with
additional risk factors for thromboembolism, the dose is
increaseddthat is enoxaparin 1 mg/kg twice daily. For specific
advice on dosing, refer to the guidelines of the Royal College of
Obstetricians and Gynaecologists.60
Finally, immunosuppressive and anti-inflammatory medicines
have not been shown to improve the outcome of PPCM and
therefore are not routinely recommended.16 61
SPECIFIC MANAGEMENT DURING PREGNANCY AND LABOUR
As PPCM poses high risks to both mother and fetus, intense
fetal and maternal monitoring is required during delivery.
If preterm (<37 weeks), the heart failure is well tolerated, the
patient is responding to medical treatment and cardiac status is
stable, the pregnancy should be allowed to go to term (ie,
37 weeks) with elective induction and vaginal delivery there-
after. However, for mothers with a new diagnosis of PPCM at
term, labour should be induced, or a caesarean section planned if
LV function is poor or deteriorating rapidly.8 38 56 Vaginal
delivery is preferred over caesarean section because of the
increased risk of pulmonary emboli and endometritis after
caesarean section.62
During vaginal delivery, the second stage of labour and labour
pain cause maximum haemodynamic and oxidative cardiac
stress, which can be minimised by using appropriate regional
anaesthetics and reducing the duration of the second stage of
labour. To relieve pain, continuous epidural or spinal anaesthetic
is preferred,38 63 and the second stage of labour can be shortened
by application of a vacuum device or forceps.17 38 49 This helps
to prevent acute cardiac decompensation during parturition.
However, caesarean section should be performed if there is an
obstetric indication or the patient is not responding to optimal
medical treatment and is in acute cardiac decompensation or
expected to progress to acute cardiac decompensation during
parturition.12
In postpartum-onset PPCM, medical management remains
the same as for pregnancy-onset PPCM. However, ACE inhibi-
tors or angiotensin receptor blockers can be used to reduce the
afterload, as they have been shown to improve mortality in all
heart failure patients with LV systolic improvement.17 49
Warfarin can also be used for anticoagulation, but should not be
started until 5e7 days postnatally to reduce complications of
delayed postpartum haemorrhage. In the interim between
delivery and the start of warfarin, LMWH should be prescribed.
It should be noted that heparin and warfarin, b blockers, digoxin
and some ACE inhibitors (namely captopril and enalopril) are
safe during breast feeding.
It is imperative to give contraceptive advice, as patients
should not become pregnant again for at least a year or until LV
function has returned to normal. Progestogenic methods of
contraception, in particular, the Mirena intrauterine system and
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Implanon are the most efficacious and safe methods of contra-
ception for women who have had PPCM. They also have the
added advantage of reducing menstrual loss, which is often
increased in patients receiving warfarin. The combined oral
contraceptive pill is contraindicated because of its thrombogenic
potential. Barrier methods have a high failure rate.
MONITORING AND FOLLOW-UP
Patients should be monitored on a regular basis to ascertain the
response to treatment, with titration of their drugs according to
guidelines as required. Monitoring includes echocardiographic
assessment of LV systolic function, symptoms and complica-
tions of PPCM, and side effects of treatment. In patients who
have shown complete recovery, annual echocardiographic
assessment is recommended.64
Currently, there is little evidence or consensus to guide
discontinuation of medical treatment once recovery is complete.
It is often recommended that ACE inhibitors and b blockers
should be continued for at least 1 year after complete recovery
clinically and echocardiographically, although this is not based
on research evidence at the current time.
Patients refractory to medical treatment and showing
progressive deterioration of LV systolic function on echocardi-
ography should have early evaluation for heart transplantation.
In severe cases of end stage heart failure and deteriorating
haemodynamic function not responding to intensive inotropic
support, an LV mechanical assist device can be implanted as
a bridge to recovery or while awaiting cardiac transplant.65e67
Patients of young age, minimal end organ damage, and recent-
onset PPCM have a more favourable outcome after cardiac
transplant than older patients who are late presenters.68
PROGNOSIS AND RECOMMENDATIONS FOR FUTURE
PREGNANCY
About 30e50% of patients with PPCM recover without
complications, with their baseline LV systolic function at rest
returning to normal.69 If LV systolic function does not return to
normal within 6 month post partum, it usually indicates irre-
versible cardiomyopathy and portends a worse prognosis.
Patients may require more than 12 months to recover, and
improvement has been seen even in the second and third years
after diagnosis, confirming that the recovery period is not only
restricted to the first 6e12 months post partum. Therefore it is
recommended to continue treatment and follow-up for
a considerable period of time to achieve maximum benefit.70
Patients with recovery of LV function have been shown by
a dobutamine test to have a reduced contractile reserve.71
Although such a test may be useful to predict complications,
including deterioration of LV function during subsequent preg-
nancy, the data are derived from only a single-centre study with
relatively low numbers of women.
In terms of the predictive value of echocardiographic param-
eters, an initial LV end systolic dimension of 5.5 cm or less and
EF >27% have both been shown to predict normalisation of LV
function after pregnancy.72 Similarly, fractional shortening
<20% and left ventricular internal dimension in diastole
(LVIDd) >6 cm at the time of diagnosis indicate a more than
threefold higher risk of progressing to persistent LV dysfunction
later on.73 However, the overall prognostic value of echocardi-
ography in PPCM seems to be relatively poor owing to the small
numbers of patients studied.73 74
It has been suggested that the risk of recurrence in women
who have completely recovered LV function after their previous
Postgrad Med J 2011;87:34e39. doi:10.1136/pgmj.2009.096594
Review
pregnancy is lower than previously believed.75 In this retro-
spective study, subsequent pregnancy was strongly associated
with further decline in LV function which could induce clinical
heart failure and, in some cases, prove fatal. Heart failure
occurred in 21% of subjects who had normal LV function before
their further pregnancy compared with 44% of those who had
decreased function, and all the deaths occurred in the group with
abnormal LV function before subsequent pregnancy. Further-
more, recovery of LV function was more likely in those women
with EF >30% at their first diagnosis of PPCM. Therefore LV
systolic function seems to be a major prognostic factor and forms
the basis of decision-making when counselling patients with
peripartum cardiomyopathy about the risks during a future
pregnancy.
Ideally, every woman planning a future pregnancy should
have echocardiography performed, and, even if it is normal, they
ought to have dobutamine stress echocardiography as well.
Women with a full recovery of LV function on both echocardi-
ography and dobutamine stress test can be advised that the risks
of major complications are relatively low (w35% risk of recur-
rence but with low mortality and the majority of women
experiencing a successful pregnancy).
NEW DEVELOPMENTS
In two cases reported from Germany, the patients showed very
good improvement in cardiac function when treated with
bromocriptine.27 As yet, bromocriptine has not been established
as a treatment option because of lack of evidence from clinical
trials and the potential for serious complications such as
myocardial infarction.76e79 A randomised control trial on the use
of bromocriptine in PPCM is on-going in Haiti and South Africa.
However, at present, bromocriptine is not recommended until
the results of these clinical trials are known.
Pentoxifylline, a vasodilator drug that also inhibits tumour
necrosis factor a, has been shown to improve outcome in a small
study when added to conventional treatment.80 In addition,
intravenous immunoglobulin therapy has been found to be
beneficial in a selected group of patients (those with proven
myocarditis of autoimmune origin) in improving symptoms and
LV function.61 80
AREAS OF UNCERTAINTY
Collaborative and multicentre prospective, randomised and
double-blind international studies would be of great help
in finding the correct aetiology, diagnostic methods, best
management options and outcomes (mortality and morbidity).
Main messages
< Peripartum cardiomyopathy is a rare and potentially fatal
disease of pregnancy and the postnatal period.
< It presents with symptoms of heart failure and may rapidly
progress to acute cardiac decompensation and life-threatening
heart failure.
< Thromboembolism and cardiac arrhythmia are common
complications.
< Treatment is generally the same as for heart failure with left
ventricular systolic dysfunction with some possible excep-
tions because of the risks of certain drugs to the unborn child.
< In the presence of persistent heart failure, further pregnancy is
not recommended.
37
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Review
Current research questions
< To more precisely define the triggering factors involved in its
aetiology
< Identification and treatment of the disease in the very early
stage before significant myocardial damage occurs
< To further explore the role of prolactin in its pathogenesis and
trial the use of prolactin blockade
Key references
< Demakis JG, Rahimtoola SH. Peripartum cardiomyopathy.
Circulation 1971;44:964e8.
< Fett JD. Understanding peripartum cardiomyopathy, 2008.
Int J Cardiol 2008;130:1e2.
< Hilfiker-Kleiner D, Silva K, Drexeler H. Peripartum
cardiomyopathy: Recent insight into its pathophysiology.
Trends Cardiovasc Med 2008;18:173e9.
< Elkayam U, Akhter MW, Singh H, Khan S, Bitar F, Hameed A,
et al. Pregnancy-associated cardiomyopathy: clinical
characteristics and a comparison between early and late
presentation. Circulation 2005;111:2050e5.
< Sliva K, Tibazarwa K, Hilfiker-Kleiner D. Management of
peripartum cardiomyopathy. Curr Heart Fail Rep
2008;5:238e44.
Specifically, it is not yet clear whether immunosuppression,
immunoadsorption, apheresis, antiviral treatment or suppression
of proinflammatory cytokines will have a role in the manage-
ment of PPCM. Finally, the role of prolactin and its suppression
by bromocriptine or carbergoline is yet to be fully elucidated.
MULTIPLE-CHOICE QUESTIONS (TRUE (T)/FALSE (F); ANSWERS
AFTER THE REFERENCES)
1. Peripartum cardiomyopathy (PPCM)
A. Is a rare disease.
B. Is a disease of only middle-aged women.
C. Commonly presents in late pregnancy only.
D. Commonly presents up to 12 month post partum.
E. Always leads to full recovery of cardiac function.
2. Which of the following statements are true or false about
PPCM?
A. It is mainly associated with viral infection.
B. 16 kDa prolactin derivative could have a role.
C. Malnutrition is not associated with PPCM.
D. Certain cultural practices are associated with increased
incidence.
E. Autoimmunity may have a role.
3. Which of the following statements are true/false?
A. Its true incidence in UK is one in a thousand live births.
B. It has an hereditary association in Hispanics.
C. Advanced maternal age is a recognised risk factor.
D. Rarely occurs in young primigravida.
E. Tocolysis is a recognised risk factor.
4. The following are recognised features of PPCM
A. Evidence of diastolic heart failure must be present on
echocardiography.
38
B. EF <45% and fractional shortening >30%.
C. Presence of a history of heart disease rules out a diagnosis of
PPCM.
D. The cardiac markers CK-MB and troponin must be elevated.
E. Endomyocardial biopsy is always indicated in severe cases.
5. In relation to the management of PPCM the following are
correct
A. Its general management is similar to ischaemic cardiomyo-
pathy.
B. Premature induction of labour is the treatment of choice.
C. Caesarean section is preferred in selected cases.
D. ACE inhibitors are not safe to use post partum.
E. Digoxin is safe to use during pregnancy.
6. Which of the following statements are true or false about
PPCM?
A. Bromocriptine has been established as a treatment option.
B. Pentoxifylline generally improves outcome.
C. Immunoglobulin may have a role in proven cases of
myocarditis of autoimmune origin.
D. Cardiac transplantation is recommended in refractory cases.
E. Future pregnancy should be discouraged in all cases.
Competing interests None.
Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES
1. Demakis JG, Rahimtoola SH. Peripartum cardiomyopathy. Circulation
1971;44:964e8.
2. Homans DC. Peripartum cardiomyopathy. N Engl J Med 1985;312:1432e7.
3. Elkayam U, Akhter MW, Singh H, et al. Pregnancy-associated cardiomyopathy:
clinical characteristics and a comparison between early and late presentation.
Circulation 2005;111:2050e5.
4. Cunningham FG, Pritchard JA, Hankins GD, et al. Peripartum heart failure: idiopathic
cardiomyopathy or compounding cardiovascular events? Obstet Gynecol
1986;67:157e68.
5. Demakis JG, Rahimtoola SH, Sutton GC, et al. Natural course of peripartum
cardiomyopathy. Circulation 1971;44:1053e61.
6. Melvin KR, Richardson PJ, Olsen EG, et al. Peripartum cardiomyopathy due to
myocarditis. N Engl J Med 1982;307:731e4.
7. Cénac A, Gaultier Y, Devillechabrolle A, et al. Enterovirus infection in peripartum
cardiomyopathy. Lancet 1988;2:968e9.
8. Midei MG, DeMent SH, Feldman AM, et al. Peripartum myocarditis and
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Postgrad Med J 2011;87:34e39. doi:10.1136/pgmj.2009.096594 39

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Peripartum cardiomyopathy: current

understanding, comprehensive
management review and new developments
Jason R Pyatt and Gopal Dubey

Postgrad Med J 2011 87: 34-39 originally published online October 10,
2010
doi: 10.1136/pgmj.2009.096594

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