Anda di halaman 1dari 8


discussions, stats, and author profiles for this publication at:

Peripartum cardiomyopathy: Current

understanding, comprehensive management
review and...

Article in Postgraduate medical journal · October 2010

DOI: 10.1136/pgmj.2009.096594 · Source: PubMed


33 117

2 authors, including:

Jason Pyatt
Royal Liverpool and Broadgreen University Hospitals NHS Trust


All content following this page was uploaded by Jason Pyatt on 20 May 2014.

The user has requested enhancement of the downloaded file.

Downloaded from on May 30, 2011 - Published by


Peripartum cardiomyopathy: current understanding,

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

Cardiology Department, Royal ABSTRACT AETIOLOGY

Liverpool and Broadgreen Peripartum cardiomyopathy (PPCM) is a rare and Despite extensive research into its underlying aeti-
University Hospitals NHS Trust, ology, it is still not clear exactly how it occurs, so
Liverpool, UK potentially fatal disease which presents with symptoms
of heart failure primarily due to left ventricular (LV) PPCM is still regarded as a disease of unknown
Correspondence to systolic dysfunction in the last month of pregnancy and aetiology.1e3 5 However, many studies lend support
Dr J Pyatt, Consultant up to 5e6 months after delivery. PPCM is still regarded to the idea of an inflammatory pathology leading to
Cardiologist, Cardiology as a disease of unknown aetiology, although recent myocarditis,6 due to either a viral infection2 4 7e10
Department, Royal Liverpool and
evidence suggests a role for a 16 kDa prolactin derivative or an autoimmune response in pregnancy against
Broadgreen University Hospitals
NHS Trust, Prescot Street, produced by proteolytic cleavage of prolactin secondary maternal myocardium provoked by the release of
Liverpool L7 8XP, UK; to unbalanced oxidative stress present during late fetal antigen into maternal blood or by some, as pregnancy and early puerperium. The medical yet, unknown agents.11e14 None of these proposed
management of PPCM is similar to other forms of non- mechanisms are strongly supported by clinical
Received 29 December 2009
Accepted 28 August 2010 ischaemic dilated cardiomyopathy, but with the evidence, as there has been great variability in the
Published Online First management tailored to choose safe drugs in pregnancy results of endomyocardial biopsies and in the
10 October 2010 and lactation to minimise maternal and fetal morbidity. finding of autoantibodies against myocardium.
There is an increased risk of venous thromboembolism, Endomyocardial biopsies of the reported cases have
and anticoagulation is recommended. About 30e50% of shown features of myocarditis ranging between 9%
the patients recover without complications, with their and 78%.12 15e17 However, there may be a role for
baseline LV systolic function at rest returning to normal. proinflammatory cytokines such as tumour
The risk of recurrence of PPCM is high, especially if the necrosis factor10 and interleukins (IL-1, IL-6) in the
LV systolic function has not fully recovered. However, for aetiology as well.18e20
those women who have normal LV systolic function as Recent evidence in an animal (mice) model
demonstrated on echocardiography and dobutamine suggests a role for a 16 kDa prolactin derivative
stress test, the risk of severe cardiomyopathy including produced by proteolytic cleavage of prolactin
death is relatively low in a subsequent pregnancy. 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,
INTRODUCTION which can potentially damage or impair metabo-
Peripartum cardiomyopathy (PPCM) is a rare and lism and contractility of cardiomyocytes.22e24 This
potentially fatal disease which presents with theory is further supported by small clinical reports
symptoms of heart failure primarily due to left of postpartum women with PPCM responding
ventricular (LV) systolic dysfunction presenting in (recovery from PPCM) when treated with medi-
the last part (mean 32e38 weeks) of pregnancy and cines causing reduced secretion of prolactin from
up to 5e6 months after delivery.1e5 Usually, it posterior pituitary gland or working as a D2
occurs early in the postpartum period, with about receptor antagonist such as bromocriptine and
45% in the first week and 75% within the first carbergoline.25e27
month.3 Clinically, it is very similar to other forms Other theories include impaired cardiac micro-
of non-ischaemic dilated cardiomyopathy except for circulation, programmed cell death (apoptosis),2 5
its unique relationship with pregnancy3 4 and the deficiency of micronutrients such as selenium,
higher likelihood for full recoverydthat is, normal- possible genetic links, and other environmental
isation of ejection fraction (EF) in almost half of the factors.28e30 Indeed, in certain cultures where the
cases.5 However, it can still result in chronic incidence of PPCM is high, certain cultural practices
disability and ultimately death in relatively young performed during the puerperium such as
women in their reproductive years. This emphasises consuming lake salt or rock salt (known as ‘kanwa’
the need for a thorough understanding of PPCM, as which has a particularly high sodium content) to
its care will transcend many different specialities promote the flow of breast milk and the heating of
and mandates a multidisciplinary approach to its the body by sitting on a clay bed with a fire beneath
management. The following review will provide to keep warm (a belief felt to ward off infection)
a comprehensive overview of the condition, espe- have both been suggested as contributory factors in
cially focusing on its clinical features and manage- its development as well.31 32
ment during pregnancy, treatment after pregnancy, Overall it appears that there is a multifactorial
and implications for future conception, as well as aetiology albeit currently unknown. However,
highlighting exciting recent new developments. whatever the initial trigger or combination of

34 Postgrad Med J 2011;87:34e39. doi:10.1136/pgmj.2009.096594

Downloaded from on May 30, 2011 - Published by


initiating processes, myocardial biopsy in PPCM shows damage completely recovered, suggesting a possible latent phase before
to myocardium with progressive death of cardiomyocytes and the development of overt heart failure or spontaneous
destruction of the cytoskeleton of the heart, resulting in recovery.41 However, a proportion of these cases may represent
progressive loss of heart muscle, ultimately leading to clinical either subclinical dilated cardiomyopathy presenting in early
heart failure.33 pregnancy for the first time or a viral myocarditis and so distinct
from true PPCM.
EPIDEMIOLOGY Therefore it is imperative to maintain a high index of suspi-
PPCM is not restricted to any particular childbearing age, but in cion to identify cases of PPCM. Original diagnostic criteria for
58% of cases the age at presentation was older than 30 years, PPCM were developed by Demakis et al in 1971.5 They did not
with primigravida occurring in approximately a third of women include echocardiographic findings because echocardiography
(27e33%).3 Its highest incidence occurs in African women or was not readily available at that time. In 1999, echocardio-
African American women from southern USA, with the lowest graphic criteria were incorporated in a new definition (box 1).12
incidence in Hispanic women in the USA. It has also been All patients should have routine blood tests to exclude
reported in Caucasians, Japanese, Chinese, Indians and Korean anaemia, electrolyte disturbance, and kidney, liver and thyroid
women. Recent reports suggest an estimated incidence of one dysfunction, including inflammatory markers, a septic screen
case per 299 live births in Haiti,34 35 one case per 1000 live births and viral serology. None of the routine or specific blood tests can
in South Africa, and one case per 2289e4000 live births in the help in screening for or positively determining a diagnosis of
USA.36 These more recent data from the USA suggest a higher PPCM. Even cardiac markers such as troponins and creatinine
incidence than reported in earlier studies, especially in certain kinase-MB are not helpful alone in reaching a diagnosis.
ethnic groups.4 36 37 Its true incidence in the UK has not been A chest x-ray confers negligible radiation to the fetus at any
estimated. stage of gestation and should be performed in a woman
On the basis of several large reported series of PPCM, the suspected of having PPCM, and no shielding is necessary.42
following have been recognised as risk factors favouring devel- Radiological signs of heart failure such as cardiomegaly,
opment and recurrence of PPCM: advanced maternal age, multi- pulmonary congestion and pleural effusion may be found.
parity, Afro-American race, twin pregnancy, pre-eclampsia, Again, the ECG is mostly non-specific. It may show sinus
gestational hypertension1 2 4 5 and use of tocolysis (b2 stimu- tachycardia or other arrhythmias such as atrial fibrillation, atrial
lants).38 PPCM appears not to have a hereditary association.12 18 39 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
Presentation of PPCM is similar to that of patients presenting Thus echocardiography is required to establish dilated cardiac
with LV systolic heart failure due to other causes. Usually chambers and reduced LV systolic function and to differentiate
women in late pregnancy or early puerperium present with PPCM from other causes of heart failure, such as valve disease,
palpitations, fatigue, shortness of breath at rest or on exertion, etc. The likelihood of recovery of cardiac function after pregnancy
cough, paroxysmal nocturnal dyspnoea, and orthopnoea. is most strongly correlated with the degree of LV dilatation and
systolic function seen on echocardiography at diagnosis.44
DIAGNOSIS OF PPCM The role of endomyocardial biopsy in the diagnosis of PPCM
It is largely a diagnosis of exclusion. Other causes of heart is controversial.9 10 16 At most, it has a sensitivity of 50% and
disease such as congenital heart disease or acquired condi- a specificity of 99%.5 However, it is not routinely recommended,
tionsdthat is, myocardial infarction causing LV dysfunction, as it is not widely available, is not specific to PPCM as it can be
pulmonary hypertension or valvular heart diseasedmust be positive for myocarditis in similar numbers of patients with
ruled out first before making a diagnosis of PPCM.12 37 40 It is other forms of dilated cardiomyopathy, and can have a relatively
then diagnosed in previously healthy women presenting with high complication rate.
symptoms of heart failure and evidence of decreased LV systolic Cardiac MRI is used widely in other forms of cardiomyopa-
function from late pregnancy to early puerperium. thies particularly for diagnostic and prognostic purposes.
However, the diagnosis of PPCM poses many challenges, However, it cannot reveal a specific pattern in PPCM which
as many women in the last month of normal pregnancy expe- would help in differentiating it from other forms of cardi-
rience similar symptoms to that of early heart failure, such as omyopathy.45e47 Coronary angiography is not routinely indi-
shortness of breath on exertion, nocturnal dyspnoea and cough, cated, as coronary arteries are usually normal in PPCM.
fatigue, palpitations and pedal oedema, making differentiation Early involvement of a cardiologist is recommended for facil-
difficult.5 16 17 37 38 Clinical examination remains important for itating the diagnosis. Timely diagnosis of PPCM is important, as
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. Box 1 Diagnostic criteria for peripartum cardiomyopathy
Although, PPCM presents in late pregnancy and early puer-
perium, early onset of pregnancy-associated cardiomyopathy has < Onset of heart failure in last month of pregnancy to
been reported, albeit in relatively few women from one series.3 5e6 months post partum
Also, recent observations from Haiti suggest that a subclinical < Without any other demonstrable cause of heart failure
form of PPCM without overt clinical symptoms may exist. The < Absence of any heart disease before pregnancy
investigators identified four clinically normal postpartum < Echocardiography criteria to include an ejection fraction
women with asymptomatic systolic dysfunction on echocardi- <45%, fractional shortening <30% or both, and end-diastolic
ography, who subsequently either developed clinically detectable dimension (LVIDd) >2.7 cm/m2 body surface area
dilated cardiomyopathy or improved their LV function and

Postgrad Med J 2011;87:34e39. doi:10.1136/pgmj.2009.096594 35

Downloaded from on May 30, 2011 - Published by


pregnancy in patients with dilated cardiomyopathy, especially Although warfarin is teratogenic in early pregnancy, its main
PPCM, is associated with high maternal and fetal morbidity. contraindication in PPCM is its risk of fetal cerebral haemor-
Early diagnosis and prompt initiation of treatment are essential rhage in the second and third trimester. Thus heparins are
to optimise pregnancy outcome. favoured in pregnancy, as they (unlike warfarin) do not cross the
placenta. The risk of thromboembolism is particularly high if
GENERAL MANAGEMENT OF PPCM the patient is in atrial fibrillation, has a history of venous
This needs a multidisciplinary team approach. Involvement of thromboembolism, or echocardiography shows mural thrombus,
a cardiologist, obstetrician, intensivist, anaesthetist and paedia- EF <35% or severely dilated LV. Low-molecular-weight heparins
trician is imperative at the earliest possible stage of the disease to (LMWH) are preferred because they have a lower risk of osteo-
reduce the mortality and morbidity associated with it. The type porosis and of heparin-induced thrombocytopenia. They are
and level of monitoring and care should be tailored according to eliminated more rapidly in pregnancy, resulting in a more
the severity of cardiac decompensation and response to the common dosage regimen, and their dose is adjusted according to
treatment.48 body weight in early pregnancy. Currently, only dalteparin,
In general, the medical management of PPCM is similar to enoxaparin and tinzaparin are used in the UK and, in those with
that of other forms of non-ischaemic dilated cardiomyopathy. additional risk factors for thromboembolism, the dose is
However, the management strategy must be tailored to choose increaseddthat is enoxaparin 1 mg/kg twice daily. For specific
safe drugs in pregnancy and lactation to minimise maternal and advice on dosing, refer to the guidelines of the Royal College of
fetal morbidity. The aims of medical treatment should be to Obstetricians and Gynaecologists.60
reduce fluid and salt intake, increase myocardial contractility, Finally, immunosuppressive and anti-inflammatory medicines
reduce cardiac preload and cardiac afterload, and prevent have not been shown to improve the outcome of PPCM and
complications and mortality, importantly, thromboembolism, therefore are not routinely recommended.16 61
progressive heart failure and cardiac arrhythmia.
Fluid restriction to 2 litres per day and salt restriction (2e4 g SPECIFIC MANAGEMENT DURING PREGNANCY AND LABOUR
per day) is advisable to improve symptoms.5 49 Strict bed rest is As PPCM poses high risks to both mother and fetus, intense
not recommended except when the patient cannot tolerate fetal and maternal monitoring is required during delivery.
mobility because of symptoms and severity of heart failure, as If preterm (<37 weeks), the heart failure is well tolerated, the
this increases the risk of thromboembolism.50 Once symptoms patient is responding to medical treatment and cardiac status is
are improving with medical treatment, modest exercise is stable, the pregnancy should be allowed to go to term (ie,
recommended.17 50 37 weeks) with elective induction and vaginal delivery there-
Diuretics are safe agents which help to reduce pulmonary after. However, for mothers with a new diagnosis of PPCM at
congestion and relieve symptoms, as they reduce preload. In the term, labour should be induced, or a caesarean section planned if
hospital setting, loop diuretics are safer to use,6 49 51 52 but, in LV function is poor or deteriorating rapidly.8 38 56 Vaginal
mild cases, thiazide diuretics are sufficient. Spironolactone may delivery is preferred over caesarean section because of the
not be safe based on animal studies, although data for pregnancy increased risk of pulmonary emboli and endometritis after
in humans are limited.49 caesarean section.62
During pregnancy, nitrates and hydralazine are considered safe During vaginal delivery, the second stage of labour and labour
drugs to reduce afterload. ACE inhibitors should not be used for pain cause maximum haemodynamic and oxidative cardiac
PPCM as, in the 2nd and 3rd trimester of pregnancy, they have stress, which can be minimised by using appropriate regional
toxic effects on fetal kidneys, resulting in oligohydramnios, anaesthetics and reducing the duration of the second stage of
hypocalvaria and sometimes fetal renal failure and death.53 labour. To relieve pain, continuous epidural or spinal anaesthetic
However, ACE inhibitors should be used after birth or if PPCM is is preferred,38 63 and the second stage of labour can be shortened
diagnosed in the puerperium. Digoxin is safe during pregnancy by application of a vacuum device or forceps.17 38 49 This helps
and lactation and is used to increase contractility of the failing to prevent acute cardiac decompensation during parturition.
heart provided that the digoxin level is monitored and kept in However, caesarean section should be performed if there is an
the therapeutic range.54 55 obstetric indication or the patient is not responding to optimal
To reduce the risk of arrhythmia and sudden death and improve medical treatment and is in acute cardiac decompensation or
long-term prognosis, a b blocker should be used if it is not expected to progress to acute cardiac decompensation during
contraindicated. Also, it is important to address electrolyte parturition.12
imbalance, which may predispose to arrhythmia, particularly as In postpartum-onset PPCM, medical management remains
low potassium and magnesium are common in pregnancy. The the same as for pregnancy-onset PPCM. However, ACE inhibi-
benefit of b blockers to maternal health usually outweighs the tors or angiotensin receptor blockers can be used to reduce the
potential risk to the baby. This risk is of growth restriction and afterload, as they have been shown to improve mortality in all
low-birth-weight babies. Therefore, growth should be monitored heart failure patients with LV systolic improvement.17 49
regularly in the 2nd and 3rd trimester.56 Specific antiarrhythmic Warfarin can also be used for anticoagulation, but should not be
drugs, such as adenosine and flecainide, should be used cautiously, started until 5e7 days postnatally to reduce complications of
mainly in the acute setting, and are generally well tolerated, delayed postpartum haemorrhage. In the interim between
although their safety for the baby cannot be guaranteed.57 Routine delivery and the start of warfarin, LMWH should be prescribed.
use of long-term antiarrhythmics should be avoided in PPCM It should be noted that heparin and warfarin, b blockers, digoxin
because of their proarrhythmic potential in LV impairment. and some ACE inhibitors (namely captopril and enalopril) are
There is an increased risk of thromboembolism in most types safe during breast feeding.
of dilated cardiomyopathy (especially if EF <35% and the It is imperative to give contraceptive advice, as patients
ventricles are severely dilated). Pregnancy and the puerperium should not become pregnant again for at least a year or until LV
further increase this risk because of the hypercoaguable state of function has returned to normal. Progestogenic methods of
pregnancy.58 59 To help this, anticoagulation is recommended. contraception, in particular, the Mirena intrauterine system and

36 Postgrad Med J 2011;87:34e39. doi:10.1136/pgmj.2009.096594

Downloaded from on May 30, 2011 - Published by


Implanon are the most efficacious and safe methods of contra- pregnancy is lower than previously believed.75 In this retro-
ception for women who have had PPCM. They also have the spective study, subsequent pregnancy was strongly associated
added advantage of reducing menstrual loss, which is often with further decline in LV function which could induce clinical
increased in patients receiving warfarin. The combined oral heart failure and, in some cases, prove fatal. Heart failure
contraceptive pill is contraindicated because of its thrombogenic occurred in 21% of subjects who had normal LV function before
potential. Barrier methods have a high failure rate. 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
Patients should be monitored on a regular basis to ascertain the
with EF >30% at their first diagnosis of PPCM. Therefore LV
response to treatment, with titration of their drugs according to
systolic function seems to be a major prognostic factor and forms
guidelines as required. Monitoring includes echocardiographic
the basis of decision-making when counselling patients with
assessment of LV systolic function, symptoms and complica-
peripartum cardiomyopathy about the risks during a future
tions of PPCM, and side effects of treatment. In patients who
have shown complete recovery, annual echocardiographic
Ideally, every woman planning a future pregnancy should
assessment is recommended.64
have echocardiography performed, and, even if it is normal, they
Currently, there is little evidence or consensus to guide
ought to have dobutamine stress echocardiography as well.
discontinuation of medical treatment once recovery is complete.
Women with a full recovery of LV function on both echocardi-
It is often recommended that ACE inhibitors and b blockers
ography and dobutamine stress test can be advised that the risks
should be continued for at least 1 year after complete recovery
of major complications are relatively low (w35% risk of recur-
clinically and echocardiographically, although this is not based
rence but with low mortality and the majority of women
on research evidence at the current time.
experiencing a successful pregnancy).
Patients refractory to medical treatment and showing
progressive deterioration of LV systolic function on echocardi-
ography should have early evaluation for heart transplantation.
In two cases reported from Germany, the patients showed very
In severe cases of end stage heart failure and deteriorating
good improvement in cardiac function when treated with
haemodynamic function not responding to intensive inotropic
bromocriptine.27 As yet, bromocriptine has not been established
support, an LV mechanical assist device can be implanted as
as a treatment option because of lack of evidence from clinical
a bridge to recovery or while awaiting cardiac transplant.65e67
trials and the potential for serious complications such as
Patients of young age, minimal end organ damage, and recent-
myocardial infarction.76e79 A randomised control trial on the use
onset PPCM have a more favourable outcome after cardiac
of bromocriptine in PPCM is on-going in Haiti and South Africa.
transplant than older patients who are late presenters.68
However, at present, bromocriptine is not recommended until
the results of these clinical trials are known.
PROGNOSIS AND RECOMMENDATIONS FOR FUTURE Pentoxifylline, a vasodilator drug that also inhibits tumour
PREGNANCY necrosis factor a, has been shown to improve outcome in a small
About 30e50% of patients with PPCM recover without study when added to conventional treatment.80 In addition,
complications, with their baseline LV systolic function at rest intravenous immunoglobulin therapy has been found to be
returning to normal.69 If LV systolic function does not return to beneficial in a selected group of patients (those with proven
normal within 6 month post partum, it usually indicates irre- myocarditis of autoimmune origin) in improving symptoms and
versible cardiomyopathy and portends a worse prognosis. LV function.61 80
Patients may require more than 12 months to recover, and
improvement has been seen even in the second and third years AREAS OF UNCERTAINTY
after diagnosis, confirming that the recovery period is not only Collaborative and multicentre prospective, randomised and
restricted to the first 6e12 months post partum. Therefore it is double-blind international studies would be of great help
recommended to continue treatment and follow-up for in finding the correct aetiology, diagnostic methods, best
a considerable period of time to achieve maximum benefit.70 management options and outcomes (mortality and morbidity).
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- Main messages
nancy, the data are derived from only a single-centre study with
relatively low numbers of women. < Peripartum cardiomyopathy is a rare and potentially fatal
In terms of the predictive value of echocardiographic param- disease of pregnancy and the postnatal period.
eters, an initial LV end systolic dimension of 5.5 cm or less and < It presents with symptoms of heart failure and may rapidly
EF >27% have both been shown to predict normalisation of LV progress to acute cardiac decompensation and life-threatening
function after pregnancy.72 Similarly, fractional shortening heart failure.
<20% and left ventricular internal dimension in diastole < Thromboembolism and cardiac arrhythmia are common
(LVIDd) >6 cm at the time of diagnosis indicate a more than complications.
threefold higher risk of progressing to persistent LV dysfunction < Treatment is generally the same as for heart failure with left
later on.73 However, the overall prognostic value of echocardi- ventricular systolic dysfunction with some possible excep-
ography in PPCM seems to be relatively poor owing to the small tions because of the risks of certain drugs to the unborn child.
numbers of patients studied.73 74 < In the presence of persistent heart failure, further pregnancy is
It has been suggested that the risk of recurrence in women not recommended.
who have completely recovered LV function after their previous

Postgrad Med J 2011;87:34e39. doi:10.1136/pgmj.2009.096594 37

Downloaded from on May 30, 2011 - Published by


B. EF <45% and fractional shortening >30%.

Current research questions C. Presence of a history of heart disease rules out a diagnosis of
< To more precisely define the triggering factors involved in its D. The cardiac markers CK-MB and troponin must be elevated.
aetiology E. Endomyocardial biopsy is always indicated in severe cases.
< Identification and treatment of the disease in the very early
stage before significant myocardial damage occurs 5. In relation to the management of PPCM the following are
< To further explore the role of prolactin in its pathogenesis and correct
trial the use of prolactin blockade A. Its general management is similar to ischaemic cardiomyo-
B. Premature induction of labour is the treatment of choice.
C. Caesarean section is preferred in selected cases.
Key references D. ACE inhibitors are not safe to use post partum.
E. Digoxin is safe to use during pregnancy.
< Demakis JG, Rahimtoola SH. Peripartum cardiomyopathy.
Circulation 1971;44:964e8. 6. Which of the following statements are true or false about
< Fett JD. Understanding peripartum cardiomyopathy, 2008. PPCM?
Int J Cardiol 2008;130:1e2. A. Bromocriptine has been established as a treatment option.
< Hilfiker-Kleiner D, Silva K, Drexeler H. Peripartum B. Pentoxifylline generally improves outcome.
cardiomyopathy: Recent insight into its pathophysiology. C. Immunoglobulin may have a role in proven cases of
Trends Cardiovasc Med 2008;18:173e9. myocarditis of autoimmune origin.
< Elkayam U, Akhter MW, Singh H, Khan S, Bitar F, Hameed A, D. Cardiac transplantation is recommended in refractory cases.
et al. Pregnancy-associated cardiomyopathy: clinical E. Future pregnancy should be discouraged in all cases.
characteristics and a comparison between early and late
Competing interests None.
presentation. Circulation 2005;111:2050e5.
< Sliva K, Tibazarwa K, Hilfiker-Kleiner D. Management of Provenance and peer review Not commissioned; externally peer reviewed.
peripartum cardiomyopathy. Curr Heart Fail Rep
1. Demakis JG, Rahimtoola SH. Peripartum cardiomyopathy. Circulation
Specifically, it is not yet clear whether immunosuppression, 1971;44:964e8.
2. Homans DC. Peripartum cardiomyopathy. N Engl J Med 1985;312:1432e7.
immunoadsorption, apheresis, antiviral treatment or suppression 3. Elkayam U, Akhter MW, Singh H, et al. Pregnancy-associated cardiomyopathy:
of proinflammatory cytokines will have a role in the manage- clinical characteristics and a comparison between early and late presentation.
ment of PPCM. Finally, the role of prolactin and its suppression Circulation 2005;111:2050e5.
4. Cunningham FG, Pritchard JA, Hankins GD, et al. Peripartum heart failure: idiopathic
by bromocriptine or carbergoline is yet to be fully elucidated. cardiomyopathy or compounding cardiovascular events? Obstet Gynecol
MULTIPLE-CHOICE QUESTIONS (TRUE (T)/FALSE (F); ANSWERS 5. Demakis JG, Rahimtoola SH, Sutton GC, et al. Natural course of peripartum
cardiomyopathy. Circulation 1971;44:1053e61.
AFTER THE REFERENCES) 6. Melvin KR, Richardson PJ, Olsen EG, et al. Peripartum cardiomyopathy due to
1. Peripartum cardiomyopathy (PPCM) myocarditis. N Engl J Med 1982;307:731e4.
A. Is a rare disease. 7. Cénac A, Gaultier Y, Devillechabrolle A, et al. Enterovirus infection in peripartum
cardiomyopathy. Lancet 1988;2:968e9.
B. Is a disease of only middle-aged women. 8. Midei MG, DeMent SH, Feldman AM, et al. Peripartum myocarditis and
C. Commonly presents in late pregnancy only. cardiomyopathy. Circulation 1990;81:922e8.
D. Commonly presents up to 12 month post partum. 9. Zimmermann O, Kochs M, Zwaka TP, et al. Myocardial biopsy based classification
and treatment in patients with dilated cardiomyopathy. Int J Cardiol
E. Always leads to full recovery of cardiac function. 2005;104:92e100.
10. Fett JD. Inflammation and virus in dilated cardiomyopathy as indicated by
2. Which of the following statements are true or false about endomyocardial biopsy. Int J Cardiol 2006;112:125e6.
PPCM? 11. Ansari AA, Fett JD, Carraway RE, et al. Autoimmune mechanisms as the basis for
A. It is mainly associated with viral infection. human peripartum cardiomyopathy. Clin Rev Allergy Immunol 2002;23:301e24.
12. Pearson GD, Veille JC, Rahimtoola S, et al. Peripartum cardiomyopathy:National
B. 16 kDa prolactin derivative could have a role. Heart, Lung and Blood Institute and Office of rare Diseases. (National Institute of
C. Malnutrition is not associated with PPCM. Heart). Workshop recommendations and review. JAMA 2000;283:1183e8.
D. Certain cultural practices are associated with increased 13. Bozkurt B, Villaneuva FS, Holubkov R, et al. Intravenous immune globulin in the
therapy of peripartum cardiomyopathy. J Am Coll Cardiol 1999;34:177e80.
incidence. 14. Warraich RS, Sliwa K, Damasceno A, et al. Impact of pregnancy-related heart
E. Autoimmunity may have a role. failure on humoral immunity: clinical relevance of G3-subclass immunoglobulins in
peripartum cardiomyopathy. Am Heart J 2005;150:263e9.
3. Which of the following statements are true/false? 15. Huerta EM, Erice A, Espino RF, et al. Post-partum cardiomyopathy and acute
myocarditis. Am Heart J 1985;110:1079e81.
A. Its true incidence in UK is one in a thousand live births. 16. Rizeq MN, Rickenbacher PR, Fowler MB, et al. Incidence of myocarditis in
B. It has an hereditary association in Hispanics. peripartum cardiomypathy. Am Heart J 1994;74:474e7.
C. Advanced maternal age is a recognised risk factor. 17. Sliwa K, Fett JD, Elkayam U. Peripartum cardiomyopathy. Lancet
D. Rarely occurs in young primigravida. 18. Witlin AG, Mable WC, Sibai BM. Peripartum cardiomyopathy:an ominous diagnosis.
E. Tocolysis is a recognised risk factor. Am J Obstet Gynaecol 1997;176:182e8.
19. Ludwig P, Fischer E. Peripartum cardiomyopathy. Aust N J Z Obstet Gynaecol
4. The following are recognised features of PPCM 1997;37:156e60.
20. Silva K, Skudicky D, Bergemann A, et al. Peripartum cardiomyopathy: analysis of
A. Evidence of diastolic heart failure must be present on clinical outcome, left ventricular function, plasma level of cytokines and FAS/APO-1.
echocardiography. J Am Coll Cardiol 2000;35:701e5.

38 Postgrad Med J 2011;87:34e39. doi:10.1136/pgmj.2009.096594

Downloaded from on May 30, 2011 - Published by


21. Hilfiker-Kleiner D, Silva K, Drexeler H. Peripartum cardiomyopathy: Recent insight 53. Shotan A, Widerhorn J, Hurst A, et al. Risks ofangiotensin-converting enzyme
into its pathophysiology. Trends Cardiovasc Med 2008;18:173e9. inhibition during pregnancy: experimental and clinical evidence, potential
22. Corbacho AM, Martinez De La Escalera G, Clapp C. Roles of prolactin and related mechanisms, and recommendations for use. Am J Med 1994;96:451e6.
members of the prolactin/growth lactogen family in angiogenesis. J Endocrinol 54. Rathore SS, Wang Y, Krmholz HM. Sex based difference in the effect of digoxin for
2002;173:219e38. the treatment of heart failure. N Engl J Med 2002;347:1403e11.
23. Hilfiker-Kleiner D, Kaminski K, Podewki E, et al. A cathepsin derived 16kDa 55. Adams KF Jr, Patterson JH, Gattis WA. Relationship of serum digoxin concentration
form of prolactin mediates postpartum cardiomyopathy. Cell 2007; to mortality and morbidity in women in the digitalis investigation group trial: A
128:589e600. retrospective analysis. J Am Coll Cardiol 2005;46:497e504.
24. Tabruyn SP, Soelet CM, Rentier-Delrue F, et al. The antiangiogenic factor 16K 56. Heider AL, Kuller JA, Strauss RA, et al. Peripartum cardiomyopathy: a review of the
human prolactin induces caspase-dependent apoptosis by a mechanism that requires literature. Obstet Gynecol Surv 1999;54:5260e340.
activation of nuclear factor-kappa B. Mol Endocrinol 2003;7:1815e23. 57. Page RL. Treatment of arrhythmias during pregnancy. Am Heart J 1995;130:871e6.
25. De Jong JS, Rietveld K, Van Lochem LT, et al. Rapid left ventricular recovery after 58. Heit JA, Kobbervig CE, James AH, et al. Trends in the incidence of venous
carbergoline treatment in a patient with peripartum cardiomyopathy. Eur J Heart Fail thromboembolism during pregnancy or postpartum: a 30 year population based
2009;11:220e2. study. Ann Intern Med 2005;143:697e706.
26. Habedank D, Kuhenle Y, Elgeti T, et al. Recovery from peripartum cardiomyopathy 59. James AH, Jamison MG, Brancazio LR, et al. Venous thromboembolism during
after treatment with bromocriptine. Eur J Heart Fail 2008;10:1149e51. pregnancy and the postpartum period: incidence, risk factors, and mortality. Am J
27. Hilfiker-Kleiner D, Meyer GP, Schieffer E, et al. Recovery from postpartum Obstet Gynecol 2006;194:1311e15.
cardiomyopathy in 2 patients by blocking prolactin release with bromcriptine. J Am 60. Royal College of Obstetricians & Gynaecologists. Reducing the Risk of
Coll Cardiol 2007;50:2354. Thromboembolism During Pregnancy, Birth and the Puerperium. Guideline no. 37.
28. Phillips SD, Warnes CA. Peripartum cardiomyopathy: current therapeutic London: RCOG, 2009.
perspectives. Curr Treat Options Cardiovasc Med 2004;6:481e8. 61. McNamara DM, Holubkov R, Starling RC, et al. Controlled trial of intravenous
29. Veille JC, Zaccaro D. Peripartum cardiomyopathy: summary of an immune globulin in recent-onset dilated cardiomyopathy. Circulation
international survey on peripartum cardiomyopathy. Am J Obstet Gynecol 2001;103:2254e9.
1999;181:315e19. 62. Velickovic IA, Leicht CH. Peripartum cardiomyopathy and cesarean section: report
30. Cénac A, Simonoff M, Moretto P, et al. A low plasma selenium is a risk factor for of two cases and literature review. Arch Gynecol Obstet 2004;270:307e10.
peripartum cardiomyopathy. A comparative study in Saharan Africa. Int J Cardiol 63. Zangrillo A, Landoni G, Pappalardo F, et al. Different anesthesiological management
1992;36:57e9. in two high risk pregnant women with heart failure undergoing emergency cesarean
31. Trevitt L. Attitudes and customs in child birth among Hausa women in Zaria City. section. Minerva Anestesiol 2005;71:227e36.
Savanna 1973;2:223e6. 64. Goland S, Modi K, Bitar F, et al. Clinical profile and predictors of complications in
32. Davidson NM, Trevitt L, Parry EH. Peripartum cardiac failure an explanation for the peripartum cardiomyopathy. J Card Fail 2009;15:645e50.
observed geographic distribution in Nigeria. Bull World Health Organ 65. Benlolo S, Lefoll C, Katchatouryan V, et al. Successful use of levosimendan in
1974:51:203e8. a patient with peripartum cardiomyopathy. Anesth Analg 2004;98:822e4.
33. Fett JD. Understanding peripartum cardiomyopathy, 2008. Int J Cardiol 66. Aziz TM, Burgess MI, Acladious NN, et al. Heart transplantation for peripartum
2008;130:1e2. cardiomyopathy: a report of three cases and a literature review. Cardiovasc Surg
34. Fett JD, Christie LG, Carraway RD. Five years prospective study of incidences and 1999;7:565e7.
prognosis of peripartum cardiomyopathy at a single institution. Mayo Clinc Proc 67. Oosterom L, de Jonge N, Kirkels J, et al. Left ventricular assist device as a bridge to
2005;80:1602e6. recovery in a young woman admitted with peripartum cardiomyopathy. Neth Heart J
35. Fett JD, Carraway RD, Dowell DL, et al. Peripartum cardiomyopathy in the 2008;16:426e8.
Hospital Albert Schweitzer District of Haiti. Am J Obstet Gynecol 68. Aravot DJ, Banner NR, Dhalla N, et al. Heart transplantation for peripartum
2002;186:1005e10. cardiomyopathy. Lancet 1987;2:1024.
36. Mielniczuk LM, Williams K, Davis DR. Frequency of peripartum cardiomyopathy. 69. Whitehead SJ, Berg CJ, Chang J. Pregnancy related mortality due to
Am J Cardiol 2006;97:1765e8. cardiomyopathy: United States, 1991e1997. Obstet Gynecol 2003;102:1326e31.
37. Brar SS, Khan SS, Sandhu GK, et al. Incidence, mortality and racial differences in 70. Fett JD, Sanon H, Thelisma E, et al. Recovery from severe heart failure following
peripartum cardiomyopathy. Am J Cardiol 2007;100:302e4. peripartum cardiomyopathy. Int J Gynecol Obstet 2009;104:125e7.
38. Lampert MB, Hibbard J, Weinert L, et al. Peripartum heart failure associated with 71. Dorbala S, Brozena S, Zeb S, et al. Risk stratification of the women with peripartum
prolonged tocolytic therapy. Am J Obstet Gynecol 1993;168:493e5. cardiomyopathy at initial presentation: a dobutamine stress echocardiography study.
39. Pierce JA, Price BO, Joyce JW. Familial occurence of postpartal heart failure. Arch J Am Soc Echocardiography 2005;18:45e8.
Intern Med. 1963;111:651e5. 72. Duran N, Gunes H, Duran I, et al. Predictors of prognosis in patients with peripartum
40. Lampert MB, Lang RM. Peripartum cardiomyopathy. Am Heart J 1995;130:860e70. cardiomyopathy. Int J Gynaecol Obstet 2008;101:137e40.
41. Fett JD, Christie LG, Carraway RD, et al. Unrecognised peripartum cardiomyopathy 73. Chapa JB, Heiberger HB, Weinert L, et al. Prognostic value of echocardiography in
in Haitian women. Int J Gyanecology Obstetetrics 2005;90:161e6. peripartum cardiomyopathy. Obstet Gynecol 2005;105:1303e8.
42. Toppenberg KS, Hill DA, Miller DP. Safety of radiographic imaging during pregnancy. 74. Lampbert MB, Weinert L, Hibbard J, et al. Contractile reserve in patient with
Am Fam Physician 1999;59:1813e18. peripartum cardiomyopathy and recorded left ventricular function. Am J Obstet
43. Murali S, Baldisseri MR. Peripartum cardiomyopathy. Crit Care Med Gynecol 1997;176:189e95.
2005;33:S340e6. 75. Elkayam U, Tummala PP, Rao K, et al. Maternal and fetal outcomes of subsequent
44. Witlin AG, Mabie WC, Sibai BM. Peripartum cardiomyopathy: a longitudinal pregnancies in women with peripartum cardiomyopathy. N Engl J Med
echocardiographic study. Am J Obstet Gynecol 1997;177:1129e32. 2001;344:1567e71.
45. Marmursztejn J, Vignaux O, Goffinet F, et al. Delayed-enhanced cardiac 76. Patti G, Nasso G, D’Ambrosio A, et al. Myocardial infarction during pregnancy and
magnetic resonance imaging features in peripartum cardiomyopathy. Int J Cardiol postpartum: a review. G Ital Cardiol 1999;29:333e8.
2009;137:e63e4. 77. Roth A, Elkayam U. Acute myocardial infarction associated with pregnancy. Ann
46. Frederic M, Christophi L, Pascal DG, et al. Characteristic of peripartum Intern Med 1996;125:751e76.
cardiomyopathy by cardiac magnetic resonance imaging. Euro Radiol 78. Goland S, Schwarz ER, Siegel RJ, et al. Pregnancy-associated spontaneous coronary
2008;18:2765e9. artery dissection. Am J Obstet Gynecol 2007;197:e11e13.
47. Mouquet F, Lions C, de Groote P, et al. Characterisation of peripartum 79. Hopp L, Weisse AB, Lffy L. Acute Myocardial infarction in a healthy mother using
cardiomyopathy by cardiac magnetic resonance imaging. Eur Radiol bromocriptine to suppress lactation. Can J Cardiol 1996;12:415e18.
2008;18:2765e9. 80. Sliwa K, Skudicky D, Candy G, et al. The addition of pentoxifylline to conventional
48. Sliva K, Tibazarwa K, Hilfiker-Kleiner D. Management of peripartum cardiomyopathy. therapy improves outcome in patients with peripartum cardiomyopathy. Eur J Heart
Curr Heart Fail Rep 2008;5:238e44. Fail 2002;4:305e9.
49. Task force on the management of cardiovascular diseases during
pregnancy of the European society of cardiology. Expert consensus document
on the management of the cardiovascular disease during pregnancy. Eur Heart J ANSWERS
50. Burch GE, McDonald CD, Walsh JJ. The effect of prolonged bed rest on post partal
cardiomyopathy. Am Heart J 1971;81:186e201.
1. A (T); B (F); C (F); D (F); E (F)
51. Van Hoeven KH, Kitsis RN, Katz SD, et al. Peripartum versus idiopathic dilated 2. A (F); B (T); C (F); D (T); E (T)
cardiomyopathy in young women-a comparison of clinical, pathologic and prognostic 3. A (F); B (F); C (T); D (F); E (T)
features. Int J Cardiol 1993;40:57e65. 4. A (F); B (F); C (F); D (F); E (F)
52. Amos AM, Jaber WA, Russell SD. Improved outcomes in peripartum 5. A (F); B (F); C (T); D (F); E (T)
cardiomyopathy with contemporary. Am Heart J 2006;152:509e13. 6. A (F); B (F); C (T); D (T); E (F)

Postgrad Med J 2011;87:34e39. doi:10.1136/pgmj.2009.096594 39

Downloaded from on May 30, 2011 - Published by

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,
doi: 10.1136/pgmj.2009.096594

Updated information and services can be found at:

These include:
References This article cites 79 articles, 19 of which can be accessed free at:

Email alerting Receive free email alerts when new articles cite this article. Sign up in
service the box at the top right corner of the online article.


To request permissions go to:

To order reprints go to:

To subscribe to BMJ go to:

View publication stats