42 JAPI april 2013 VOL.

61

Review Article

Peripartum Cardiomyopathy
* ** ***
VN Mishra , Nalini Mishra , Devanshi
Abstract
Peripartum cardiomyopathy (PPCM) is a rare type of cardiomyopathy of unknown aetiology associated with
significant mortality and morbidity and characterized by heart failure in late pregnancy or puerperium. Recently
PPCM workshop committee has recommended inclusion of echocardiographic features of LV dysfunction to redefine
PPCM. Subsequent pregnancies are associated with a very high mortality in these patients and hence should be
avoided. Women with PPCM continue to have significant mortality despite the use of conventional drugs for
managing heart failure. Use of newer drugs such as immunoglobulin, pentoxifylline, bromocriptine, and cabergoline
along with newer interventions such as plasmapheresis, immunoadsorption, ventricular assist devices and last but not
the least the heart transplantation hold promise for future.
hypertension. Ninety percent
Introduction Risk of PPCM occurs within two

P eripartum cardiomyopathy (PPCM) is a rare but critical disorder

causing heart failure in women in late pregnancy or puerperium. It was
Fact months of delivery.
Myocarditis : Association
first described in the 18th century but ors between myocarditis and
was recognized as a separate clinical entity in 1930. In 1971 Demakis PPCM was suggested by some
Incidence of peripartum
et al described 27 patients who presented during the puerperium investigators who reported a
cardiomyopathy has been
with cardiomegaly, abnormal electrocardiographic findings, and high incidence of myocarditis
congestive heart failure, and named the syndrome The peripartum found to be greater 7
in
on endomyocardial biopsy in
cardiomyopathy. Following the recommendations of workshop multiparous women and in patients with peripartum
1
committee on PPCM that echocardiographic features of the left those with advanced maternal cardiomyopathy, later reports
8
ventricular dysfunction should be incorporated to diagnose PPCM. age. Multifetal pregnancy however found a comparable
The definition of PPCM was modified which now includes following (twin and triplets), incidence in age and sex
four criteria, three clinical and one echocardiographic preeclampsia, and gestational matched nonpregnant group
hypertension appear to have a with idiopathic dilated
1. Development of heart failure during last trimester ofhigher incidence.9 African cardiomyopathy.
11
The
pregnancy or first six months post partum. prevalence of myocarditis in
American race, Obesity,
2. Absence of any identifiable cause for cardiac failure. Maternal cocaine and alcohol patients with peripartum
cardiomyopathy ranged from
3. Absence of any recognizable heart disease prior to lastabuse, smoking and long term
tocolytic therapy have been 8.8% to 78% in different
trimester of pregnancy. studies. On the other hand, the
attributed as risk factors for
4. Echocardiographic criteria- Demonstrable echocardiographic PPCM. Role of Selenium and presence or absence of
proof of left ventricular systolic dysfunction. Ejection fraction Zinc deficiency in PPCM is myocarditis alone does not
10 predict the outcome of
less than 45%, left ventricular fractional shortening less than controversial. peripartum cardiomyopathy.
30% or left ventricular end-diastolic dimension>2.7cm/m square
2
Etiopat According to this hypothesis
after a cardio tropic viral
of body surface area.
hogene infections pathologic immune
Epidemiology response occurs that is
Peripartum Cardiomyopathy is relatively rare condition. The sis probably inappropriately
precise incidence in India is not known, an incidence of one case per directed against native cardiac
Peripartum
1374 live births has been reported from a tertiary care hospital from cardiomyopathy is generally tissue proteins, leading to
12
3
South India. The National Hospital Discharge ventricular dysfunction.
considered a form of
Survey (19902002) estimated that it occurs in one in every 2,289 live idiopathic Bultmann et al found
primary
births in the United States. The prevalence is reported to be one case myocardial parvovirus B19, human herpes
disease virus, Epstein-Barr virus and
per 6000 live births in Japan. The disease appears to be more associated with the pregnant
common in African American women. The rate varies in other state. cytomegalovirus
Although several
populations; in South Africa reported incidence is higher 1 in 1000 plausible DNA in endomyocardial
etiologic
4
live birth. A much higher incidence of I in 300 live births has been mechanisms biopsy specimens from 8
have been
5
reported from Haiti, and an extremely high rate of 1% has been suggested, none of them is (31%) of 26 patients with
6 peripartum cardiomyopathy
reported from Nigeria. The higher prevalence in developingdefinite.
that was associated
countries may be attributed to environmental, ecological, and Nutritional : Many immunohistologically with
cultural puerperal and post puerperal practices besides diagnostic nutritional disorders have
interstitial inflammation.
criteria and reporting standards. been suggested as causes, but Khl et al found, that in
other than salt overload, none patients with viral infection
* ** has been validated by confirmed by
ProfessorDept of Medicine, Associate Professor Dept of O and G,
****
Intern, Pt JNM Medical College and BRAM Hospital, Raipur, epidemiological studies. endomyocardial biopsy, the
Chattisgarh Higher incidence in African median left ventricular
Received: 03.02.2011; Accepted: 14.11.2011 countries has been attributed ejection fraction improved in
to the consumption of kanwa, those in whom the virus was
a tradition for 40 post-partum cleared, whereas it was found
days. Kanwa is a dry salt and to be decreased in those in
causes hypervolemia and
where the virus persisted. of
Chimerism : In a phenomenon called Chimerism, there is mixture provoking
268 JAPI april 2013 VOL. 61
JAPI april 2013 VOL. 61
The serum from patients with peripartum cardiomyopathy has
been found to contain autoantibodies in high titers, which are not
present in serum from patients with idiopathic cardiomyopathy.
Most of these antibodies are against normal human cardiac tissue
proteins of 37, 33, and 25 kD. Multiparity is a risk factor for the
development of this disorder, suggesting that previous exposure
to foetal or paternal antigen may elicit an abnormal myocardial
inflammatory response. The timing of presentation in the
immediate postpartum period supports an autoimmune
13
pathogenesis. Adaptive changes in the maternal immune
system allow the foetal tolerance necessary for successful
pregnancy and include the induction of suppressor cells. These
adaptive changes likely underlie the clinical observation that
autoimmune disorders such as multiple sclerosis which affect
women during their reproductive years typically have lower
relapse rates during pregnancy itself, with a marked increase in
the immediate postpartum period. The majority of cases of
peripartum cardiomyopathy present in early postpartum period
of the same pregnancy during which the restoration of the
maternal immune system results in an increase in autoimmune
exacerbations in other disorders.
Apoptosis and inflammation : Apoptosis (programmed cell death)
of cardiac myocytes occurs in heart failure and may contribute to
progressive myocardial dysfunction. Experiments in mice suggest
that apoptosis of cardiac myocytes has a role in peripartum
cardiomyopathy. Plasma levels of C-reactive protein and tumour
necrosis factor alpha (markers of inflammation) were found to be
elevated and correlated with higher left ventricular dimensions
and lower left ventricular ejection fractions at presentation. Recent
studies have indicated that increased proteolytic cathepsin D
activity in cardiomyocyte result in 16kDa prolactin fragment with
anti-angiogenic and apoptotic properties which may contribute to
14
development of this disease. Pharmacological blockade of
prolactin might emerge as a novel disease specific therapeutic
15
option in near future. Another observation that there is elevation
in the concentration of the inflammatory cytokines such as
tumour necrosis factor-alpha in post partum period has been
attributed as a causative factor by some workers.
16
An abnormal hemodynamic response : during pregnancy blood
volume and cardiac output increases. In addition the after load
decreases because of the relaxation of vascular smooth muscle.
The increase in volume and cardiac output causes transient and
reversible hypertrophy of the left ventricle to meet the needs of
the mother and foetus. The transient left ventricular systolic
dysfunction during the third trimester and early postpartum
period returns to baseline once the cardiac output decreases,
17
given the hemodynamic stress decompensation usually occurs
during pregnancy in women with previous subclinical ischemic
or myopathic heart disease in last trimester of pregnancy but in
cases of PPCM because of some unknown familial, genetic or
environmental factors this decompensation starts late in gestation
and in more than 75% cases it is diagnosed only during post
18
partum period, but the same argument stands against this
theory as well.
Latent idiopathic Cardiomyopathy : According to this theory
unmasking of latent idiopathic dilated cardiomyopathy is brought
to fruition by the hemodynamic stresses of pregnancy.
Hypertension during pregnancy, and pre-eclampsia, are both
reported in a higher frequency in women with peripartum
cardiomyopathy and support that hemodynamic stresses may
play a role. However keeping the normal hemodynamic changes
of pregnancy in mind, it is well known that most women with
compromised cardiac function such as valvular heart disease
JAPI april 2013 VOL. 61
genotype, sometimes response.
an immune
43
present with symptoms of heart failure by the end of the
second trimester. Absence of cardiac symptoms in PPCM until
the postpartum period argues against the latent
cardiomyopathy theory.
Pathological Changes
In a patient of PPCM after post mortem the heart specimen
appear pale, soft, dilated and heavier in comparison to normal
heart. Cardiac chambers quite often show mural thrombi, Gray
white patches of endocardial thickening are usually seen at the
site of mural thrombi. Heart valves and coronary arteries appear
normal, pericardial effusion is occasionally seen. On
histopathological examination evidence of degeneration, fibrosis,
interstitial oedema, and fatty and mononuclear cell infiltration
along with sparse or abundant collection of eosinophils in
myocardium is seen, myocardial hypertrophy is also found in
some patients. Electron microscopy reveals varying degree of
enlargement, destruction or fragmentation of myofibrils, increase
in size and number of mitochondria, along with the deposition of
19
glycogen and some abnormal proteinacious material. On
histochemical examination of myocardial cells, the most important
observation is the presence of sarcoplasmic fat vacuoles
containing triglyceride without any increase of the lipofuscin or
amyloid. Besides these, the low levels of plasma albumin and pre
albumin, selenium and zinc have also been observed as
20
mentioned earlier.
Clinical Features
Symptoms of PPCM are the same as in patients with systolic
dysfunction who are not pregnant. New or rapid onset of the
following symptoms require prompt evaluation: cough,
orthopnea, paroxysmal nocturnal dyspnoea, fatigue, palpitations,
weight gain, haemoptysis, chest pain, and unexplained abdominal
pain. Physical examination often reveals enlarged heart,
tachycardia, and decreased pulse oximetry. Blood pressure may be
normal, elevated jugular venous pressure, third heart sound, and
loud pulmonic component of the second heart sound are usually
found, mitral and/or tricuspid regurgitation, and pulmonary
rales are also noted. Worsening of peripheral oedema, ascites and
hepatomegaly are quite often seen. Arrhythmias are commonly
found which may be responsible for embolic phenomenon
peripheral or pulmonary. In some patients small to moderate
pericardial effusion may be found.
Presence of elevated blood pressures (systolic >140 mm Hg
and/ or diastolic >90 mm Hg) and proteinuria suggests
preeclampsia. Overall clinical presentation and hemodynamic
changes are indistinguishable from those found in other forms
21
of dilated cardiomyopathy. Few patients with high output
22
heart failure have also been reported.
Diagnosis
Diagnosis of PPCM requires a very high level of suspicion by
treating physicians and obstetrician.They should consider
peripartum cardiomyopathy in any peripartum patient with
unexplained symptoms of heart failure.The greatest dilemma is
the lack of specific clinical criteria allowing distinction between
PPCM presenting with the new onset heart failure and other
causes of systolic dysfunction.Therefore all other possible
causes of dilated heart with heart failure must be thoroughly
excluded before accepting clinical diagnosis of PPCM.
Lab Investigations
X-ray chest : may reveal cardiomegaly and pulmonary venous
269
44
congestion with interstitial or alveolar oedema.Occasionally
pleural effusion may be found. As x-ray chest is not essential
to diagnose PPCM, its routine use during pregnancy should
be discouraged and if needed may be done using an
abdominal shield.
ECG : may be normal or might show sinus tachycardia,
atrial fibrillation, nonspecific ST segment changes, conduction
abnormalities and other types of arrhythmias.
Echocardiography : Echocardiography remains an important tool
for evaluation and follow up for women with postpartum
cardiomyopathy. The finding of a decrease in myocardial systolic
function, as manifested by a decrease in left ventricular ejection
fraction or fractional shortening is essential to the diagnosis. Left
ventricular dilatation is also frequently evident, particularly in
those women presenting late. Mild compensatory left ventricular
hypertrophy can be seen, however, marked increases in LV wall
thickness may suggest primary hypertrophic cardiomyopathy, an
entity with a very distinct natural history and prognosis. A small
pericardial effusion may be seen in the early and immediate
postpartum period. Valvular morphology is generally normal;
however, with marked LV enlargement mitral regurgitation
secondary to annular dilatation may be seen, tricuspid and
pulmonary regurgitation are also seen some times. Overall
echocardiographic features of postpartum cardiomyopathy are
indistinguishable from those of primary non-ischemic dilated
23
cardiomyopathy.
Endomyocardial biopsy : The endomyocardial biopsy may
show features of myocarditis, as many cases of PPCM seem to
be related to myocarditis, but the decision for biopsy should
be taken after thorough discussion between patient and
treating physicians. Biopsies in patients with peripartum
cardiomyopathy have the highest yield when performed early
after the onset of symptoms; however there is paucity of
literature on this aspect of diagnostic modality.
Viral and bacterial titer and cultures : such as coxasckie B virus
antibody titer should be considered in selected cases, these have
more research implications rather than real diagnostic one.
MRI : Magnetic resonance imaging (MRI) may be used as a
complementary tool to diagnose peripartum cardiomyopathy, and
it may prove to be important in identifying the mechanisms
involved. It can measure global and segmental myocardial
contraction, and it can characterize the myocardium. Delayed
contrast enhancement (with gadolinium) can help to differentiate
the type of myocyte necrosis, i.e. myocarditis vs. ischemia.
Myocarditis has a nonvascular distribution in the subepicardium
with a nodular or band -like pattern, whereas ischemia has a
vascular distribution in a subendocardial or transmural location.
Cardiac MRI can be used to guide biopsy to the abnormal area,
which may be much more useful than the blind biopsy. Recently
Cardiac Magnetic Resonance Imaging (CMRI) has been used in
24
prognostication in PPCM.
Right heart catheterization : In women with persistent heart
failure, hemodynamic instability or evidence of an organ
dysfunction, right heart catheterization to assess the filling
pressures and cardiac output should be considered. It will
also demonstrate enlargement of all chambers of heart
predominantly the left ventricle.
Biochemical evaluation : Usually reveals little or no elevation
in creatinine kinase, or cardiac troponin. In women with acute
heart failure and hemodynamic compromise, assessment of
liver function tests and renal function provides an assessment
of organ perfusion.
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270 JAPI april 2013
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Differential Diagnosis
PPCM should be differentiated from other forms of
Cardiomyopathy. The most common and confusing being
Idiopathic Dilated Cardiomyopathy (IDCM), though PPCM is
identical to IDCM in many ways, most researchers now agree that
25
it is a distinct clinical entity. PPCM occurs at a younger age and
is generally associated with better prognosis, it occur mostly post
partum (78-93%) whereas IDCM usually manifests by the second
trimester. Higher incidence of myocarditis is found in PPCM
along with unique sets of antigen and antibodies against
myocardium which is not seen in IDCM. Heart size returns to
normal after delivery in more number of PPCM patients as
compared to IDCM and contrary to IDCM, PPCM may lead to
26
rapid worsening of clinical course and poor outcome. Other
common causes of heart failure, such as valvular heart diseases,
coronary artery disease including acute myocardial infarction,
pulmonary thromboembolism, severe eclampsia and pneumonia
should be excluded on the basis of history, physical examination
and investigations.
The most common alternative diagnosis is the occult valvular
heart disease which can be effectively ruled out by transthoracic
echocardiography. The finding of normal systolic function
excludes postpartum cardiomyopathy and should lead to an
evaluation for forms of high output failure such as anaemia and
thyrotoxicosis. Although ischemic heart disease is uncommon in
this population, women with significant risk factors such as Type I
diabetes should have at least a non-invasive assessment for
coronary ischemia, and if questions persist should undergo
angiography. In women with persistent heart failure,
hemodynamic instability or evidence of an organ dysfunction,
right heart catheterization to assess filling pressures and cardiac
output should be considered.
Treatment
Management of Heart failure
1. During Pregnancy - Early diagnosis and prompt treatment
are the keys to optimize pregnancy outcome. When
considering diagnostic tests or treatment during pregnancy,
the welfare of the foetus should always be considered along
with that of the mother. Patients with severe forms of heart
failure will require ICCU management, with monitoring of
arterial blood pressure (ABP), central venous pressure (CVP),
and sometimes pulmonary artery catheter (PAC).
Coordinated management with specialists team is essential.
Angiotensin converting enzyme (ACE) inhibitors and ARBs
are contraindicated in pregnancy because these can cause
27
birth defects, although remaining the main treatment
option for postpartum women with heart failure.
The teratogenic effects occur particularly in the second
and third trimester, characterized by foetal hypotension,
pulmonary hypoplasia, oligohydramnios, anuria, and
renal tubular dysplasia. However a recent study
suggested risk of malformations even after first trimester
exposure to ACE inhibitors.
Digoxin, loop diuretics, sodium restriction and drugs that
reduce afterload such as hydralazine and nitrates have been
proven to be safe and are the mainstays of medical therapy of
heart failure during pregnancy. Digoxin is effective due to its
inotropic and rate reducing effect. Diuretics are useful
because of the preload reduction along with salt restriction.
They are relatively safe in pregnancy and lactation; however
one should be cautious regarding volume depletion which
may result in to dehydration causing uterine hypoperfusion
28
and foetal distress. Calcium channel blockers were not used
earlier because of fear of negative contractile effects and
potential risk of uterine hypoperfusion but recently
Amlodepine has been found to improve survival in non
 29
ischemic Cardiomyopathy patients. Beta-blockers have
strong evidence of efficacy in patients with heart failure, but
they have not been tested in peripartum cardiomyopathy.
Nevertheless, beta-blockers have long been used in pregnant
women with hypertension without any known adverse
effects on the foetus, and patients taking these agents prior to
diagnosis can continue to use them safely.
2. During Post Partum period- Treatment is identical to that
for nonpregnant women with dilated Cardiomyopathy.
ACE inhibitors and ARBs are useful. The usual target
dose is one half the maximum antihypertensive doses.
Diuretics are given for symptomatic relief; spironolactone
or digoxin is used in patients who have New York Heart
Association class III or IV symptoms. The dose of
spironolactone is 25 mg/day after dosing of other drugs
is maximized. The goal with digoxin therapy is the lowest
daily dose to obtain a detectable serum digoxin level,
which should be kept at less than 1.0 ng/ml, Beta-
blockers are recommended as they improve symptoms,
ejection fraction, and survival. Non selective beta-blockers
such as carvedilol and selective ones such as metoprolol
have shown benefit. The goal dosage is carvedilol 25 mg
twice a day or metoprolol 100 mg once a day.
Anticoagulant Therapy
Due to high risk of venous and arterial thrombosis
anticoagulation with subcutaneous heparin should be instituted
in these patients more so in bedridden patients, those with LVEF
<35%, presence of atrial fibrillation, mural thrombi, obese patients
and those with history of thromboembolism. Hypercoagulable
state of pregnancy coupled with stasis of blood due to ventricular
dysfunction makes PPCM patients prone for thrombus formation.
This situation may persists up to six weeks after postpartum,
hence use of heparin is advocated in ante partum period and that
of heparin or warfarin in the post partum period, as warfarin is
contraindicated in pregnancy because of its teratogenic effect
while use of both heparin and warfarin is safe in lactation.
30
Patients with evidence of systemic embolism, with severe left
ventricular dysfunction or documented cardiac thrombosis,
should receive anticoagulation. Anticoagulation should be
continued until return of normal left ventricular function is
documented.
Antiarrhythmic Drugs
As in other forms of non-ischemic dilated cardiomyopathy,
ventricular arrhythmias can be an important clinical issue. No
antiarrhythmic agent is completely safe during pregnancy
quinidine and procainamide should be tried first because of their
31
higher safety profile. Beta blockers may be useful, for atrial
32
arrhythmias digoxin may be considered. Patients presenting
with sudden death or ventricular tachycardia with hemodynamic
compromise, strong consideration of an implantable cardioverter
defibrillator (ICD) is warranted due to the potential for a fatal
recurrence. For patients presenting with symptomatic ventricular
tachyarrhythmia which are hemodynamically well tolerated,
management can be tempered somewhat by the potential
transient nature of the myopathy and amiodarone therapy at 200
to 400 mg orally every six hourly is an alternative. If left
JAPI april 2013 VOL. 61
46
favourable and comparable long term survival in both the
41,42
groups. However, as fewer than 3,000 hearts are available
45
ventricular function recovers, the risk of serious arrhythmic
event is markedly diminished and amiodarone therapy can be
discontinued. For patients with asymptomatic non-sustained
ventricular tachyarrhythmia we should not initiate
amiodarone therapy, but focus on correction of metabolic
abnormalities and consider the addition of a beta receptor
antagonist if not already being utilized.
Newer Treatment Modalities
Pentoxifylline : Treatment with pentoxifylline, a xanthine
derived agent known to inhibit the production of tumour
necrosis factor alpha, has been shown to improve functional
class and left ventricular function in patients with idiopathic
dilated cardiomyopathy. Similar to other aetiologies of left
ventricular dysfunction, elevated levels of TNF alpha has been
33
found in these patients.
Role of Bromocriptine and Cabergoline : Most recently few
studies have suggested the role of prolactin breakdown products
in the aetiology of PPCM. Prolactin secretion can be reduced with
34
bromocriptine which had beneficial effects in a small study. In
one case study with use of cabergoline which is a strong and long
lasting antagonist of prolactin significant improvement in left
35
ventricular functions were reported. It is premature to comment
about usefulness of these drugs at present but it seems that they
might become important treatment modality in management of
PPCM in times to come.
Immune Modulating Therapy : Given the inflammatory nature of
peripartum cardiomyopathy and the occasional appearance of
myocarditis on endomyocardial biopsy, immunosuppressive and
immune modulatory therapy has been utilized. Intravenous
immunoglobulin improved the ejection fraction in several studies
36
and also markedly reduced the levels of inflammatory cytokines.
Plasmapharesis has also been utilized effectively for this purpose,
and may be an alternative to immune globulin therapy in
peripartum cardiomyopathy. Recently a small controlled study of
immunoadsorption therapy in idiopathic dilated cardiomyopathy
demonstrated significant improvements in left ventricular
function, suggesting that therapies directed against humoral
autoimmunity may have a significant role in the treatment of this
disorders. Other proposed therapies which might be useful are
calcium channel antagonists, statins, monoclonal antibodies and
interferon beta.
Other Interventions and Devices : Because the disease is
reversible in good number of patients, the temporary use of an
intraaortic balloon pump or left ventricular assist devices may
37
help in stabilizing critical patients. Extracorporeal membrane
oxygenation has been tried successfully in some patients as a
38
bridge to recovery. Ventricular tachycardia leading to cardiac
39
arrest has been reported in PPCM patients, to avoid such
situation increasing use of automated implantable cardioverter
defibrillator (AICD) is being tried.
40
In extreme situations
multiorgan support systems such as ventilator therapy,
continuous venovenous hemodialysis may be required besides
circulatory assist devices. Plasmapharesis and
immunoadsosorption therapies are other newer techniques which
have been tried for immunomodulation in these patients.
Cardiac transplantation : Patients with severe heart failure who
does not respond despite maximal drug therapy may be
considered for cardiac transplantation to survive because of high
risk of mortality. Two reports comparing results of cardiac
transplantation in age matched females with peripartum
cardiomyopathy and idiopathic cardiomyopathy showed
271
for transplantation worldwide per year this procedure
remains more of a theoretical consideration rather than a day
to day treatment option.
Managing hemodynamic stress of delivery for patients
presenting during the late stages of pregnancy, due consideration
should be given to limiting the hemodynamic stress of the
delivery. Compensated patients may undergo vaginal delivery
with appropriate monitoring. For those patients late in pregnancy
with more significant hemodynamic compromise, elective
caesarean with invasive hemodynamic monitoring of the mother
should be considered. Single shot spinal anaesthesia is currently
not preferred because of severe consequences like cardiac arrest
and pulmonary oedema. Controlled epidural analgesia (ER) is a
43
safe and effective method in this situation.
Follow-Up Management
Patients who show normal left ventricular function on
echocardiographic evaluation at rest or with low-dose
dobutamine stress test can be allowed to taper and then
discontinue heart failure treatment in 6 to 12 months. They are
encouraged to remain as active as their functional status allows,
however aerobic activities and heavy lifting are discouraged for at
least the first six months postpartum. Given the metabolic
demands of lactation, breast feeding is strongly discouraged in
more symptomatic patients. As pharmacologic therapy to the
patient can be passed on to the child in breast milk discouraging
the breast feeding in more functional patients. If breastfeeding is
considered in these women, it has to be with careful monitoring
of the baby. Echocardiogram should be repeated at 6 months post
delivery. For those patients with persistent cardiomyopathy beta
blockers may be added at this point if not already on therapy.
Prognosis
Although peripartum cardiomyopathy shares many features of
other forms of dilated cardiomyopathy, an important distinction is
that women with this disorder have a much higher rate of
spontaneous recovery of left ventricular function on
echocardiography in post partum period; nearly half of the
women will normalize their ejection fraction during follow-up
within six months. Prognosis is directly correlated to recovery of
left ventricular function. For those women whose LVEF
normalizes during follow-up, the prognosis is excellent as
without the stimulus of a subsequent pregnancy the chance of
development of heart failure or future LV dysfunction is minimal.
For those women whose left ventricular function does not recover,
prognosis remains guarded and mortality rates as high as 10-50%
44
has been reported. LV size is an important predictor, as women
presenting without significant LV dilatation appeared to have a
greater chance of spontaneous recovery during follow-up. In
contrast, women with marked LV dilatation at presentation
appeared to have a greater likelihood of developing into a chronic
cardiomyopathy. Initial NYHA class or hemodynamic status does
not seem to predict the likelihood of subsequent recovery. A
fractional shortening on echocardiogram less than 20% and a LV
end diastolic dimension greater than or equal to 6 cm was
associated with a threefold increase in persistent LV dysfunction.
Risks of Subsequent Pregnancies
In patients whose left ventricular function fails to normalize
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272 JAPI april 2013
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2. Selle T, Renger I, Labidi S et al; Reviewing peripartum
JAPI april 2013 VOL. 61
during follow-up, subsequent pregnancies carry a high risk of left
ventricular deterioration and progressive heart failure, and hence
pregnancy is strongly discouraged given the possible risk to the
life of the mother. Mortality was reported to be in the range of 8 to
17 percent in a large study in this group in comparison to 0 to 2%
in patients with normal left ventricular ejection fraction before the
45
subsequent pregnancy. Real problem lies in the issue of how to
advice 70 to 80% of women who seem to have a complete recovery
of left ventricular size and function following PPCM. In an
attempt to clarify the prognosis in these cases, women with a
history of PPCM underwent a dobutamine stress
echocardiography test to assess their left ventricular contractile
46
reserve (Lampert et al 1997). The results of this study indicated
that women with recovered PPCM has significantly less left
ventricular contractility reserve than a group of normal matched
controls. These finding are of concern and indicate a possible
though small risk in women with apparently recovered left
ventricular function. They should be explained duly during
counselling. A recent large retrospective survey suggests that in
women with normal ejection fractions at the time of subsequent
pregnancy, there was an approximate 21% risk of the
development of heart failure, and a drop in the mean ejection
fraction from 0.56 to 0.49. However no serious complications were
noted and the majority of these women had a successful delivery
at term with close and careful monitoring. This was in marked
contrast to the women with persistent LV dysfunction prior to
47
pregnancy in which 19% mortality was reported. Overall
recommendation clearly being that pregnancy is avoided in
women with persistent poor left ventricular function. Women
whose LV function normalizes should still be made aware of the
risk of possible recurrence, dobutamine stress test should be
performed and due counselling should be performed though in
the majority of these women successful pregnancy can be
accomplished with appropriate monitoring.
Conclusion
Peripartum cardiomyopathy is an uncommon but potential life
threatening cardiac failure of unknown aetiology, encountered
late in pregnancy or in the post partum period. Diagnosis of
PPCM should essentially include echocardiographic
substantiation of left ventricular dysfunction. Role of
endomyocardial biopsy in day to day diagnosis is controversial.
Usefulness of diuretics, vasodilators, digoxin, beta blockers and
anticoagulant in medical management is well established. ACE
inhibitors and ARB blockers should be avoided during pregnancy
but should be started in post partum period. In resistant cases
pentoxifylline, immunoglobulin and immunosuppressive drugs
may be used. Bromocriptine and cabergoline hold promise for the
future. Severe cases might require advanced life support systems
and even heart transplantation. Prognosis is linked to recovery of
left ventricular functions. Subsequent pregnancies are associated
with a very high mortality more so in those whose LV functions
do not improve even six month after puerperium hence
pregnancy should be avoided in this group. In patients with
normal cardiac function on echo evaluation subsequent
pregnancy may be considered under close multidisciplinary
supervision.
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