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42 JAPI APRIL 2013 VOL.

61
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.
*
Professor Dept of Medicine,
**
Associate Professor Dept of O and
G,
****
Intern, Pt JNM Medical College and BRAM Hospital, Raipur,
Chatisgarh
Received: 03.02.2011; Accepted: 14.11.2011
Introduction
P
eripartum cardiomyopathy (PPCM) is a rare but critical
disorder causing heart failure in women in late pregnancy
or puerperium. It was frst described in the 18
th
century but
was recognized as a separate clinical entity in 1930. In 1971
Demakis et al described 27 patients who presented during the
puerperium with cardiomegaly, abnormal electrocardiographic
findings, and congestive heart failure, and named the
syndrome The peripartum cardiomyopathy. Following the
recommendations of workshop committee on PPCM
1
that
echocardiographic features of the left ventricular dysfunction
should be incorporated to diagnose PPCM. The defnition of
PPCM was modifed which now includes following four criteria,
three clinical and one echocardiographic
1. Development of heart failure during last trimester of
pregnancy or frst six months post partum.
2. Absence of any identifable cause for cardiac failure.
3. Absence of any recognizable heart disease prior to last
trimester of pregnancy.
4. Echocardiographic criteria- Demonstrable echocardiographic
proof of left ventricular systolic dysfunction. Ejection fraction
less than 45%, left ventricular fractional shortening less than
30% or left ventricular end-diastolic dimension>2.7cm/m
square of body surface area.
2
Epidemiology
Peripartum Cardiomyopathy is relatively rare condition.
The precise incidence in India is not known, an incidence of one
case per 1374 live births has been reported from a tertiary care
hospital from South India.
3
The National Hospital Discharge
Survey (19902002) estimated that it occurs in one in every 2,289
live births in the United States. The prevalence is reported to be
one case per 6000 live births in Japan. The disease appears to be
more common in African American women. The rate varies in
other populations; in South Africa reported incidence is higher
1 in 1000 live birth.
4
A much higher incidence of I in 300 live
births has been reported from Haiti,
5
and an extremely high rate
of 1% has been reported from Nigeria.
6
The higher prevalence
in developing countries may be atributed to environmental,
ecological, and cultural puerperal and post puerperal practices
besides diagnostic criteria and reporting standards.
Risk Factors
Incidence of peripartum cardiomyopathy has been found to
be greater in multiparous women
7
and in those with advanced
maternal age.
8
Multifetal pregnancy (twin and triplets),
preeclampsia, and gestational hypertension appear to have a
higher incidence.
9
African American race, Obesity, Maternal
cocaine and alcohol abuse, smoking and long term tocolytic
therapy have been atributed as risk factors for PPCM. Role of
Selenium and Zinc defciency in PPCM is controversial.
10
Etiopathogenesis
Peripartum cardiomyopathy is generally considered a form
of idiopathic primary myocardial disease associated with the
pregnant state. Although several plausible etiologic mechanisms
have been suggested, none of them is defnite.
Nutritional : Many nutritional disorders have been suggested
as causes, but other than salt overload, none has been validated
by epidemiological studies. Higher incidence in African
countries has been atributed to the consumption of kanwa, a
tradition for 40 post-partum days. Kanwa is a dry salt and causes
hypervolemia and hypertension. Ninety percent of PPCM occurs
within two months of delivery.
Myocarditis : Association between myocarditis and PPCM
was suggested by some investigators who reported a high
incidence of myocarditis on endomyocardial biopsy in patients
with peripartum cardiomyopathy, later reports however found
a comparable incidence in age and sex matched nonpregnant
group with idiopathic dilated cardiomyopathy.
11
The prevalence
of myocarditis in patients with peripartum cardiomyopathy
ranged from 8.8% to 78% in diferent studies. On the other hand,
the presence or absence of myocarditis alone does not predict
the outcome of peripartum cardiomyopathy. According to
this hypothesis after a cardio tropic viral infections pathologic
immune response occurs that is probably inappropriately
directed against native cardiac tissue proteins, leading to
ventricular dysfunction.
12
Bultmann et al found parvovirus B19,
human herpes virus, Epstein-Barr virus and cytomegalovirus
DNA in endomyocardial biopsy specimens from 8 (31%) of 26
patients with peripartum cardiomyopathy that was associated
immunohistologically with interstitial inflammation. Khl
et al found, that in patients with viral infection confrmed by
endomyocardial biopsy, the median left ventricular ejection
fraction improved in those in whom the virus was cleared,
whereas it was found to be decreased in those in where the
virus persisted.
Chimerism : In a phenomenon called Chimerism, there is
mixture of genotype, sometimes provoking an immune response.
Review Article
Peripartum Cardiomyopathy
VN Mishra
*
, Nalini Mishra
**
, Devanshi
***
268 JAPI APRIL 2013 VOL. 61
JAPI APRIL 2013 VOL. 61 43
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 infammatory response. The timing of presentation
in the immediate postpartum period supports an autoimmune
pathogenesis.
13
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 afect
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 infammation : 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 infammation) 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 development of this disease.
14

Pharmacological blockade of prolactin might emerge as a novel
disease specifc therapeutic option in near future.
15
Another
observation that there is elevation in the concentration of the
infammatory cytokines such as tumour necrosis factor-alpha
in post partum period has been atributed 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 partum period,
18
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
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 efusion is occasionally seen. On
histopathological examination evidence of degeneration, fbrosis,
interstitial oedema, and faty and mononuclear cell infltration
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 myofbrils,
increase in size and number of mitochondria, along with the
deposition of glycogen and some abnormal proteinacious
material.
19
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 mentioned earlier.
20
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 efusion 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 of dilated
cardiomyopathy.
21
Few patients with high output heart failure
have also been reported.
22

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 specifc 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
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44 JAPI APRIL 2013 VOL. 61
congestion with interstitial or alveolar oedema.Occasionally
pleural efusion 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 fbrillation, nonspecifc 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 fnding 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 efusion 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
cardiomyopathy.
23

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 diferentiate
the type of myocyte necrosis, i.e. myocarditis vs. ischemia.
Myocarditis has a nonvascular distribution in the subepicardium
with a nodular or band-like patern, 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
prognostication in PPCM.
24
Right heart catheterization : In women with persistent heart
failure, hemodynamic instability or evidence of an organ
dysfunction, right heart catheterization to assess the flling
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 litle 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.
Diferential 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 it is a distinct clinical entity.
25
PPCM occurs at a younger age
and is generally associated with beter 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
rapid worsening of clinical course and poor outcome.
26
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 efectively ruled out by
transthoracic echocardiography. The fnding 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 signifcant 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 flling pressures and cardiac
output should be considered.
Treatment
Management of Heart failure
A. 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 birth defects,
27
although remaining the main
treatment option for postpartum women with heart failure.
The teratogenic efects 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 frst 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 efective due
to its inotropic and rate reducing efect. 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
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JAPI APRIL 2013 VOL. 61 45
and foetal distress.
28
Calcium channel blockers were not
used earlier because of fear of negative contractile efects
and potential risk of uterine hypoperfusion but recently
Amlodepine has been found to improve survival in non
ischemic Cardiomyopathy patients.
29
Beta-blockers have
strong evidence of efcacy 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
efects on the foetus, and patients taking these agents prior
to diagnosis can continue to use them safely.
B. 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 beneft. 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 efect 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 frst because of their
higher safety profle.
31
Beta blockers may be useful, for atrial
arrhythmias digoxin may be considered.
32
Patients presenting
with sudden death or ventricular tachycardia with hemodynamic
compromise, strong consideration of an implantable cardioverter
defbrillator (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
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 found in
these patients.
33
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
bromocriptine which had benefcial efects in a small study.
34
In
one case study with use of cabergoline which is a strong and long
lasting antagonist of prolactin signifcant improvement in left
ventricular functions were reported.
35
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 infammatory 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 and also markedly reduced the levels of infammatory
cytokines.
36
Plasmapharesis has also been utilized efectively
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 signifcant improvements
in left ventricular function, suggesting that therapies directed
against humoral autoimmunity may have a signifcant 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 help in stabilizing critical patients.
37
Extracorporeal
membrane oxygenation has been tried successfully in some
patients as a bridge to recovery.
38
Ventricular tachycardia
leading to cardiac arrest has been reported in PPCM patients,
39

to avoid such situation increasing use of automated implantable
cardioverter defbrillator (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
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46 JAPI APRIL 2013 VOL. 61
favourable and comparable long term survival in both the
groups.
41,42
However, as fewer than 3,000 hearts are available
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 signifcant 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 safe and efective method in this situation.
43

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 frst 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% has been reported.
44
LV size is an important predictor,
as women presenting without signifcant 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
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 subsequent pregnancy.
45
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 atempt 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 reserve (Lampert et al 1997).
46
The results of this study
indicated that women with recovered PPCM has signifcantly
less left ventricular contractility reserve than a group of normal
matched controls. These fnding 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 pregnancy in which 19% mortality was reported.
47

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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