Download Peripartum cardiomyopathy- a pregnancy related disorder

Peripartum cardiomyopathy- a pregnancy related disorder
A Review
Dr I.Kjaev, Dr S.Jovanova
Abstract
Peripartum cardiomyopathy is a pregnancy related disorder of unknown origin, it is an
Idiopathic heart failure that occurs in the absence of any determinable heart disease in the
last month of pregnancy and within 5 months of delivery.
Worldwide incidence varies among nations with the highest prevalence in Nigeria 1%
and
Haiti 0,33%.These numbers are far lower in more industrialized countries like South
Africa 0,1% and the USA 1 in 3000-4000 deliveries.
Prognostic criteria depend on the normalization of the left ventricular size and function
within 6 months of delivery. Up to 50% of patients with Peripartum
cardiomyopathy (PPCM) recovers with out complications even though this may not be
apparent in the first 6 months. Mortality ranges between 3,3 – 9,6% in well developed
countries.
The incidence in Macedonia is unknown. A lack of well organized multidisciplinary
approach leads to misdiagnosis and late discovery.
Key words: cardiomyopathy , pregnancy, ventricular end-systolic dimensions, left
ventricular ejection fraction, postpartum period, epidemiology, heart failure, therapy,
pregnancy complications, prognosis, consecutive pregnancy
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Introduction
Peripartum or sometimes called postpartum cardiomyopathy ( PPCM) is a serious life
Treating diseases that occurs during pregnancy or during the post partum period1.Heart
Failure during pregnancy has been discussed and recognized as early as in the 19th
Century, however the first steps towards understanding the complex of the issue were
done in the late 1930 and with the pioneer work of Demakis et al.
His criteria for the diagnosis of PPCM included, development of cardiac failure in the last
month of pregnancy or within 5 months after delivery, absence of determinable etiology
for the cardiac failure and absence of demonstrable heart disease prior to the last month
of pregnancy.
The clinical form of peripartal cardiomyopathy is similar to other
cardiomyopathies the main difference is the rapid onset of symptoms, previously normal
healthy individuals can acquire a condition that could in some circumstances only be
managed by heart transplantation. Recent studies have shown that an estimated 80% of
symptomatic patients recover, however completed recover is only expected in 30% of the
cases with normalization of the left ventricular function and size2.
A central element in the establishing the right diagnose is an ejection fraction of
less then 45% or the combination of an M-mode fractional shortening of less than 30%
and an end-diastolic dimension greater than 2,7sm/m2.
Symptoms that are often seen with this condition include dyspnea, cough,
orthopnea, hemoptysis, chest and abdominal discomfort. These are symptoms commonly
found in normal pregnancy which makes an early diagnose very difficult to make. Most
2
affected patients have New York Heart Association (NYHA) class III or IV function.3
Many differential diagnosis can be found the fulfill the previously stated symptoms, those
would include preeclampsia, eclampsia, pulmonary embolus, pneumonia or systemic
infection. Diagnosis and postpartum monitoring are helped by, echocardiography, MRI,
endometrial biopsy, chest x-ray, electrocardiography and others. Epidemiological
findings suggest that black race, multiparty, maternal age >30 years, twin gestation and
history of hypertension ,preeclampsia or eclampsia are associated with higher incidence
of peripartum
cardiomyopathy4,5.The incidence of PPCM is quoted as 1 in 3000-4000 deliveries for the
USA and Europe,1-1000 deliveries in South Africa and 1-300 in Haiti2
Materials and Methods
Information was gathered through Pub med Central. Key words used were peripartum
cardiomyopathy and postpartum cardiomyopathy. Most of the selected literature
represent small study groups and most of the information a not sufficient enough to
establish a good overview.
Discussion
The over goal of this research is to establish a clear algorithm of early discovery and
treatment. Multidisciplinary treatment which would include a gynecologist,
anestihiologyst
and a cardiologist would certainly help. The reality is that the delay between the first
symptoms and the diagnosis can range from a few weeks to several months in as much as
30% of all cases.6
3
The right way to start understanding peripartum cardiomyopathy is to find the
causes, although it seems to be an idiopathic disease several theories exist.
-
Inflammation High concentrations of tumor necrosis factor –α(TNFα),intertferon-
γ,interleukin -6,CRP and Fas/aptosis antigen 1(Apo-1) in PPCM7 suggest an
underlying inflammatory process for the pathophisiological development of PPCM.
-
Myocarditis Most of the patients that suffer from peripartal cardiomyopathy have
been found to have myocardial inflammation reports range from 29% - 100%3.It is
still unclear whether an actual infection is a trigger to this condition because most of
the studies were done at different times from the episode of cardiomyopathy. Future
research is needed for a clear answer.
-
Fetal Chimerism Fetal cells escaping in to the maternal circulation during
pregnancy are usually destroyed by the mothers immune system. In rare
circumstances a weakened immune system during pregnancy would allow these cells
to settle in the maternal heart and so triggering a response when the immune system
has normalized 8.This casual relationship is still yet to be confirmed
-
Genetics associations Several family mutations such as MYH7,SCN5A and
PSEN2 and sporadic mutations in MYH6 and TNNT2 DCM were found in patients
with peripartum cardiomyoptahy. However if these findings are true they would pave
a way to genetic screening test for PPCM.
Clinical symptoms
Most of the early symptoms such as dyspnea, orthopnea, dizziness , pedal edema
occur in normal pregnancies. That’s why it’s difficult to diagnose PPCM. Dyspnea in
normal pregnancy is thought to be due to hyperventilation caused by the effects of
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progesterone and also due to the pressure of the growing uterus9 .Peripheral edema is
also common among pregnant patients. Among a lot of other symptoms that might
be involved it is worth mentioning thromboembolism, cardiac arrhythmias,
preexisting hypertension and preeclampsia . Preeclampsia is commonly seen in
pregnancy, symptoms like high blood pressure, protein in the urine,swelling,sudden
weight gain, headaches and blurred vision that occur after the 20th week of
gestation. Some reports exist about a latent form of cardiomyopathy with out
significant signs and symptoms10 .
Doctors should consider peripartum cardiomyoptahy in any patient with
unexplained symptoms, delayed diagnosis may be associated with higher rates of
illness and death. The aim should be focused on excluding other causes of
cardiomyopathy and to confirm left systolic dysfunction by echocardiography.
The diagnosis and postpartum monitoring are facilitated by echocardiography, ECG,
MRI, endometrial biopsy and laboratory test.
Echocardiography is the single most effective test in establishing a
diagnosis. Common findings include globally decreased contractility and LV
enlargement without hyperttophy11
Follow up studies have shown that patients with higher LVEF at presentation
portends better cure rates and shorter recovery time.12It is also true that those patients
with
high initial LVEF are less likely to have a relapse during a subsequent
pregnancy.13Echocardiography as a non invasive test can help diagnose all
symptomatic patients with undiagnosed PPCM and so treatment can start
sooner. Follow up echocardiography is usually done before discharge from the
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hospital , at 6 weeks and 6 months postpartum and annually therafeter.14
MRI – enables the precise diagnosis of myocarditis,necrosis and LV thrombi and can
acquire accurate measurements of ventricular volumes, however it’s and expensive
technique that is not as effective as echocardiography.
ECG commonly seen changes are left ventricular hypertrophy and ST-T wave
abnormalities.14 Less common signs include atrial fibrillation ,flutter, prolonged PR
and QRS intervals.
Chest Radiography When evaluating new onset of dyspnea,tachycardia or hypoxia,
obtain a chest radiograph to detect pulmonary edema. This should be
preformed with abdominal shielding to evaluate the etiology of hypoxia and exclude
pneumonia. Fetal radiation exposure with 2 maternal chest radiographs with
abdominal shielding is about 0,00007 rads. The accepted limit of fetal radiation
exposure is 5 rads.
Endometrial biopsy is highly specific technique for diagnosing myocarditis, however
because it is an invasive procedure it is not the first line diagnostic
tool. Endometrial biopsy might be considered when myocarditis is suspected or when
no
improvement is seen 2 weeks after heart failure therapy.3
Cardiac protein assays They are not a standard diagnostic tool , however a recent
prospective trail of 106 patients with high troponin T levels ( > 0,4ng/ml) witin 2
weeks of peripartum cardiomyopathy onset significantly predicted smaller LVEF
and persistent LV dysfunction at 6 months follow up ( P< 0,001)15
Therapy
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Much research has been done in trying to find the right treatment. It is specific
because the setting is in the peripartum period were some drugs are not
recommended. A well balanced and multidisciplinary approach is needed that would
involve cardiovascular medicine,obstetrics,immunology,pathology and others. The
goal of the therapies is similar like a other condition of cardiomyopathy , that is
through pharmacological therapy to resolve the issue .
It is also worth mentioning that patient education is essential,every
treatment option should be first discussed with the patient so that she can feel that she
is
actively involved with therapy. One must remember that a healthy fetus depends on a
healthy mother. Medications should only be used when the benefit to the mother is
clear. Some of the medications used have clear teratogenicity so careful use is
warranted.
Diuretics – reduce preload and treat pulmonary congestion or peripheral edema,
both hydrochlorthiazide and furosemide are safe during pregnancy and lactation. The
known side effects include uterine hypoperfusion and maternal metabolic acidosis16 ,
as with any medication caution is needed.
ACE inhibitors –are considered first line therapies for heart failure therapy but they
are known to be contraindicated in pregnancy17. They can be used when the
pregnancy has ended but breast feeding should also be stopped.
Vasodilatators – Hydralazine is safe during pregnancy and is the primary
vasodilatotor drug antepartum11, in more severe case the use of nitroglycerin is
warranted.
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Digoxin – an inotropic and dromotropic agent is safe to use antepartum,because of
few available options during that period digoxin is an easy choice18
Inotropic agents – the use of dobutamin and dopamine is warranted only in cases of
severe low output, as soon as a patient is stable (hemodinamicaly) different treatment
option should be used.14
Beta- blockers – these drugs can be a second line of therapy during pregnancy when
clinically indicated. All are compatible with breast feeding
Calcium Channel blockers – are found to reduce the interlukin -6 levels in heart
failure
patients19,some caution is needed due to uterine hypoperfusion.
Arrhythmia management – atrial fibrillation is the most common arrhythmia in
patients
with peripartum cardiomyopathy.20 Quinidine and proacinamide are relatively safe to
use in puerperium and can therefore can be considered as a first line therapy 21,
digoxin can also be used.
Anticoagulants – Peripartum cardiomyopathy is associated with high rate of
tromboembolic complications, heart faiulure and pregnancy21 are both independent
risk
factors. Cases of arterial or venous thrombosis have been reported in as much as 50%
of women with PPCM. Because pregnancy is a hypercoagulable state once the
diagnosis of PPCM has been made prophylactic anticoagulant therapy should be
given during pregnancy. Full dose anticoagulation should be initiated ante partum for
women with deep venous thrombosis,atrial fibrillation, ventricular thrombi , embolic
events or when the ejection fraction is less then 30%.Treatment should be continued
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at least 6 weeks postpartum.
Other agents – Several studies are currently being done to find new drugs or
treatment options for PPCM, those include immunosuppressive drugs, intravenous
immunoglobulin, pentoxifylline( antiplatelet) and bromcriptine.
Mechanical Circulatory Support and Transplantation
In some severe cases mechanical circulatory support or even transplantation may be
needed. Both intra-aortic balloon pumps and LV assist devices ( LVADs) have been
used to bridge transplantation.22 .In recent years bridging the recovery via prolonged
circulatory support has helped to dramatically decrease the percentage of PPCM
requiring transplantation from 33% to 4%-7%14 .The potential for organ rejection in
peripartum cardiomyopathy is high due to the increased prevalence of autoimmune
mechanisms.
Route of delivery
Vaginal deliveries are preferred because they are associated with much lower rates of
complications, such as endometritis and embolism. Cesarean section is responsible
for
75% of incidents with embolism. Vaginal deliveries are not associated with the post
operative third spacing of fluid that occurs after cesarean deliveries. Unless the
mother
is decompensating, managing her medically and waiting for a spontaneous vaginal
delivery is reasonable. However if she is not responding to medical therapy or if the
fetus must
be delivered for obstetrics reasons, the best plan is to induce the labor with the goal
of vaginal delivery. Effective control of maternal pain during delivery is
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paramount. Regional anesthesia such as epidural or spinal is not associated with
myocardial depression as observed with inhaled anesthetics. The patient should not be
allowed to push, the uterus can expel the fetus without maternal pushing. The use of
low-forceps or vacuum can be used in the final stages of delivery.
Prognosis
Recovery from PPCM is defined as a recovery of LVEF to >0,50 or improvement by
>20.Recovery usually occurs between 3 and 6 months post partum, but in some rare
circumstances as late as 48 months postpartum23.The estimated mortality rate
associated with PPCM in the US is 6%-10%24.Death usually occurs within 30 days.
Even after a complete recovery from peripartum cardiomyopathy, the risk of
recurrence in subsequent pregnancy remains high and LVEF once improved can
worsen again. At this moment in time subsequent pregnancy is not advised for a
patient that had an episode of PPCM.
Conclusion
Periaprtum cardiomyopathy is a potentially life-treating illness which usually arises
shortly after delivery. Early diagnosis is prognostic ally important in these young
women. Hence the possibility of PPCM should be kept in mind by the doctor as a
possible complication of the postpartum period, wherever there are symptoms of heart
failure. Management of peripartum cardiomyopathy should first aim at improving
heart-failure symptoms through conventional therapies and then administering target
therapies. Luckily despite a high risk of recurrence in subsequent pregnancies many
patients with peripartum cardiomyopathy recover within 3 to 6 months.
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