This document presents a case study of a 37-year-old woman admitted with symptoms of heart failure including shortness of breath and edema. She had been admitted previously during her last month of pregnancy and postpartum with similar symptoms. Testing showed reduced left ventricular function consistent with peripartum cardiomyopathy. The document then reviews peripartum cardiomyopathy including definition, risk factors, pathogenesis, clinical manifestations, diagnostic criteria, differential diagnosis, and management recommendations focusing on heart failure treatment.
2. CASE STUDY
A woman 37 years old admitted in MU-1 via OPD| patient has c/o SOBc fatigue and
body swelling from last 8 months
HOPI
Patient is in USDH 8 months back (At that time she was at her 8th month of gestation
when she started complaining of SOBc-III, fatigues and generalized body edema. SOB
was gradual in onset and progressive in nature, associated with extreme fatigue,
exaggerated on walking exertion and lying straight and relieved to some extent on
rest. It is also associated with generalized body edema that started from feet than
comes up over the upper body and eyes and facial puffiness. It was not associated
with any fever, cough, sputum or hemoptysis, chest pain, nausea, vomiting,
diaphoresis. Then she consulted her gynecologist, they have planned her C/sec
(limited history available for the indication) and discharged home. The patient was
once again complaining of shortness of breath and gen body edema referred to
nephro. Admitted there managed conservatively, discharged and sent home. Now
patient presented in medical OPD with same complain got admitted in medical unit 1
and further workup was done here.
PERIPARTUM CARDIOMYOPATHY BY DR. MARYAM IQBAL 2
3. Past Medical History
No significant history of IHD, DM,HTN
Past Surgical History
History of C/sec done at periphery 8 months back. Baby couldn't survived
Gynecological History
Not significant
Personal History
Patient is nonsmoker, with normal eating and normal bowel and bladder habits
Socioeconomic History
Patient has low socioeconomic status
Drug History
Patient has no history of any known drug allergies
ON EXAMINATION
(CASE STUDY) CONTINUED
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PERIPARTUM CARDIOMYOPATHY BY DR. MARYAM IQBAL
4. ON EXAMINATION
(CASE STUDY) CONTINUED
At presentation, Patient's vitals are:
Bp:110/70
Pulse: 96/min regular
Temp: Afebrile
SpO2: 94 % @RA
R.R: 22/min
On GPE: Gen body edema was present that includes bilateral pedal edema, facial
puffiness, eyelids and was pitting in nature.
Slightly pallor +ve on conjunctival examination
JVP was raised as well as prominent pulsations were seen
No history of jaundice, clubbing or any other significant positive findings
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PERIPARTUM CARDIOMYOPATHY BY DR. MARYAM IQBAL
5. ON CVS
Inspection
Apical impulse was shifted upward and laterally and same on palpation as
well.
Auscultation
S1 heard normal with normal intensity
S2 heard normal with normal intensity
S3 gallop rhythm was appreciated
Added sounds
A pansystolic murmur was appreciated at 5th intercostal space & left
parasternal border as well. (TR/ MR?) no association with posture/ breathing.
ON EXAMINATION
(CASE STUDY) CONTINUED
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PERIPARTUM CARDIOMYOPATHY BY DR. MARYAM IQBAL
6. On Resp
Normal vesicular breathing plus bilateral fine basal crackles present at bases
only; mild ronchi were also appreciated.
Rest: normal
On GIT
Abdomen soft non-tender. No fluid thrill or shifting dullnes.
BSA
Stitch line healed and clean.
CNS
15/15 GCS
Rest normal
ON EXAMINATION
(CASE STUDY) CONTINUED
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PERIPARTUM CARDIOMYOPATHY BY DR. MARYAM IQBAL
12. INTRODUCTI
ON
Peripartum cardiomyopathy (PPCM,
also called pregnancy-associated
cardiomyopathy) is a rare cause of
heart failure (HF) that affects women
late in pregnancy or in the early
puerperium.
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PERIPARTUM CARDIOMYOPATHY BY DR. MARYAM IQBAL
13. DEFINITION
The definition developed by the 2010 European Society of Cardiology (ESC) Working
Group on Peripartum Cardiology is the most widely used and has been included in
the 2018 ESC guidelines on management of cardiovascular diseases during
pregnancy. The definition is broad, as the Working Group sought to avoid
underdiagnosis of PPCM, and all three conditions must be met
●Development of HF in the last month of pregnancy (or toward the end of
pregnancy) or within five months following delivery.
●Absence of another identifiable cause for the HF.
●Left ventricular (LV) systolic dysfunction with an LV ejection fraction (LVEF) of less
than 45 percent, with or without LV dilation. The last criterion was added to prevent
the inclusion of patients with disorders with higher LVEF that mimic systolic HF [6,9].
Such disorders include accelerated hypertension, diastolic dysfunction, systemic
infection, pulmonary embolism, or complications of late pregnancy (eg, preeclampsia
or amniotic fluid embolus).
Some patients with higher LVEF (between 45 and 50 percent) may be diagnosed with
PPCM if they have typical clinical features; in such patients, the diagnosis should be
made after all other causes have been excluded. Prior definitions also exclude
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PERIPARTUM CARDIOMYOPATHY BY DR. MARYAM IQBAL
14. RISK FACTORS
The following are factors associated with increased risk of PPCM:
●Age greater than 30 years [3,4,10,12].
●African descent [26].
●Pregnancy with multiple gestation [10,27].
●Prior or concurrent preeclampsia, eclampsia, or postpartum
hypertension [28]. ●Maternal cocaine abuse [29].
●Long-term (>4 weeks) oral tocolytic therapy with beta adrenergic
agonists such as terbutaline [30].
●Parity ≥4 [31].
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15. PATHOGENESIS
Whereas no single unifying cause for PPCM has been identified, several
contributing pathogenic factors have been identified.
Experimental research suggests that in the setting of pregnancy-related
maternal cardiovascular changes, these multiple factors result in a common
final pathway with enhanced oxidative stress, cleavage of prolactin to an
angiostatic N-terminal 16 kDA prolactin fragment, and impaired vascular
endothelial growth factor (VEGF) signaling because of upregulated soluble
fms-like tyrosine kinase.
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16. CLINICAL MANIFESTATIONS
Timing of Presentation PPCM is less commonly seen before 36
weeks of gestation, and affected patients usually present
during the first month postpartum. Most women with PPCM are
diagnosed early after delivery during readmission after
discharge.
Sign & Symptoms Presentation of PPCM is variable and similar
to other forms of systolic HF due to cardiomyopathy. Patients
most commonly complain of dyspnea; other typical symptoms
include cough, orthopnea, paroxysmal nocturnal dyspnea,
pedal edema, and hemoptysis. Initial diagnosis may be delayed
since symptoms such as nonspecific fatigue, shortness of
breath, and pedal edema are similar to those that occur in
normal pregnancy but more pronounced. 16
PERIPARTUM CARDIOMYOPATHY BY DR. MARYAM IQBAL
17. PHYSICAL EXAMINATION NORMAL
PREGNANCY
In a normal pregnancy, as a result of the increase in endogenous progestins, respiratory
tidal volume is increased and patients have a tendency to hyperventilate. However, the rate
of respiration should be normal. Normal pregnancy is characterized by an exaggerated x
and y descent of the jugular venous waveform, but the jugular venous pressure should be
normal.
Cardiovascular Changes
Heart rate increases (10-20%)
Stroke Volume Increases (10%)
Cardiac output increases (30-50%)
Mean Arterial Pressure Decreases (10%)
Peripheral Resistance Decreases (35%)
Cardiac auscultation reveals a systolic ejection murmur at the lower left sternal edge, over
the pulmonary area, or both, in 96% of women during pregnancy. This pulmonic arterial
flow murmur tends to become quieter during inspiration. Diastolic murmurs warrant further
evaluation.
The first heart sound (S1) may be exaggerated, and the second heart sound (S2) split may
be more prominent due to an increased right-sided flow. An S3 has been described as a
normal finding in pregnancy. Peripheral edema occurs in approximately one third of healthy
gravid women
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18. NOW IN PPCM
In a patient with PPCM, signs of heart failure are the same as those in
nonpregnant patients with systolic dysfunction.
Tachycardia and decreased oxygen saturation could be present. Blood
pressure may be normal. Elevated blood pressures (systolic >140 mm
Hg and/or diastolic >90 mm Hg) and hyperreflexia with clonus
suggest preeclampsia.
Elevated jugular venous pressure, cardiomegaly, a third heart sound,
a loud pulmonic component of the second heart sound, mitral or
tricuspid regurgitation, pulmonary rales, worsening of peripheral
edema, ascites, arrhythmias, embolic phenomenon, and
hepatomegaly may be present.
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19. EMBOLISM
Signs and symptoms of systemic or pulmonary thromboembolism may be
present. Case series have reported varying rates of thromboembolism,and
further data are needed to quantify the risk of this complication [1].
Patients with PPCM and LVEF
Criteria for diagnosis — As noted above, the diagnosis of PPCM is based
upon three clinical criteria. INVESTIGATIONS: ECG Ecg findings in patients
with PPCM are nonspecific and include sinus tachycardia (or rarely atrial
fibrillation) and nonspecific ST- and T-wave abnormalities. Anterior
precordial Q waves and prolonged PR intervals and QRS duration are
occasionally present.
DIAGNOSTIC CRITERIA
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20. ECHOCARDIOGRAPHY
An echocardiogram should be performed in all patients in whom the
diagnosis of PPCM is being considered. The echocardiogram generally
reveals a global reduction in LV systolic function with LVEF nearly
always dilated. Other possible echocardiographic findings include left
atrial enlargement, LV or left atrial thrombus, dilated right ventricle,
right ventricular hypokinesis, mitral and tricuspid regurgitation, and
rarely small pericardial effusion.
CHEST XRAY
A chest radiograph is not necessary to make a diagnosis of HF or
PPCM, and it exposes the patient to ionizing radiation. Chest
radiograph typically shows enlargement of the cardiac silhouette with
evidence of pulmonary venous congestion and/or interstitial edema,
and, on occasion, pleural effusions 20
PERIPARTUM CARDIOMYOPATHY BY DR. MARYAM IQBAL
21. OTHER
LABS
Viral and
bacterial
cultures
(myocarditis)
Pro BNP
Baselines
CARDIAC
CATHETERIZATI
ON
Invasive cardiac
tests are usually
not used to
diagnose PPCM
CARDIAC
MAGNETIC
RESONANCE
IMAGING (CMR)
CMR is not
generally required
to make the
diagnosis of PPCM,
but it can be
helpful to assess LV
systolic function
and LV volumes,
particularly if
echocardiography
is technically
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PERIPARTUM CARDIOMYOPATHY BY DR. MARYAM IQBAL
22. DIFFERENTIAL DIAGNOSIS PPCM
PPCM is a diagnosis of
exclusion. Some pre-existing
cardiac lesions may manifest
during pregnancy due to
pregnancy-associated
hemodynamic changes.
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23. Pre-existing cardiomyopathy – Any cardiomyopathy may be
unmasked during pregnancy including idiopathic dilated
cardiomyopathy (DCM), familial DCM, or HIV/AIDS
cardiomyopathy (which often presents without ventricular
dilatation)
Pre-existing valvular disease – Either acquired or valvular
disease can be unmasked by pregnancy.
PPCM can also lead to functional valvular disease from
chamber dilation, annular dilation, and leaflet tethering,
which can impair leaflet coaptation. Mitral regurgitation can
result from these changes in PPCM.
Pre-existing undetected congenital heart disease – Aside
from bicuspid valve disease, the most common congenital
lesions that may be first diagnosed during pregnancy are
atrial septal defects, ventricular septal defects, and patent
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25. Treatment of PPCM is largely like treatment for other types of HF. Additional
therapeutic issues for this population may include arrhythmia management,
anticoagulation therapy, mechanical support, and investigational therapies
such as bromocriptine.
1- Heart failure treatment — In women with PPCM and HF, the goals of
medical therapy are similar to those in patients with acute and chronic HF
with reduced ejection fraction due to other causes. These include:
Supplemental oxygen and assisted ventilation as needed
Optimization of preload (Nitrates)
Hemodynamic support with inotropes and vasopressors if required
Relief of symptoms (loop diuretics)
When possible, institute chronic therapies that improve long-term
outcomes 25
PERIPARTUM CARDIOMYOPATHY BY DR. MARYAM IQBAL
26. Mortality benefit drugs: Cardioselective beta blockers. ACE, ARB, ARNI, sodium-glucose luminal
cotransporter-2 (SGLT2) inhibitors, and mineralocorticoid receptor antagonists are to be avoided, as
they are contraindicated in pregnancy.
Anticoagulation: When the ejection fraction (EF) is less than 35%, anticoagulation is recommended due
to a high risk of venous and arterial thrombosis via Warfarin, LMWH,UFH.
Cardiac transplantation and left ventricular (LV) assist devices have been used to treat PPCM. These
should be considered for women with progressive LV dysfunction or deterioration despite medical
therapy.
Arrhythmias management: Women with PPCM with severely reduced LVEF (≤35%) appear to have an
increased risk of ventricular tachyarrhythmias. Wearable cardioverter-defibrillator (WCD) may be
preferred over implantable cardioverter-defibrillator (ICD) in affected women during the first 6 months
after initiation of heart failure therapy.
ROLE OF BROMOCRIPTINEB
Bromocriptine may be considered in patients with severe LV dysfunction. This drug inhibits prolactin
secretion, which may disrupt the ongoing effects of prolactin on the vascular and cardiac
systems.Bromocriptine can be given as 2.5 mg daily for 1 week in uncomplicated cases. Higher doses (2.5
mg bid for 2 weeks, followed by 2.5 mg daily for 6 weeks) are recommended in patients with complicated
course. Treatment with bromocriptine must always be accompanied by anticoagulation.
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27. ROUTE OF DELIVERY
Stabilize patient before delivery
1- Hemodynamically Stable. Vaginal preferred, with
epidural and assisted second stage of labour. (Less
complications, PE)
2- Hemodynamically Unstable. Urgent delivery.
POSTPARTUM
Breastfeeding
2018 ESC guidelines for the management of
cardiovascular diseases during pregnancy, suggest that
prevention of lactation may be considered in patients with
severe HF due to levels of prolactin and high metabolic
demands of lactation and breastfeeding. 27
PERIPARTUM CARDIOMYOPATHY BY DR. MARYAM IQBAL
28. PROGNOSIS
The mortality rate for PPCM varies with region.
A. Mortality rates range from 1.4 to 3.4 percent within 30 days of diagnosis.
B. A systematic review reported all-cause mortality of 8 percent at six months.
C. Twelve-month mortality in this meta-analysis was 9.8 ~Death due to PPCM is usually
caused by ventricular arrhythmias, progressive pump failure, sudden death, or
thromboembolic events.
Adverse Prognostic Factors:
Worse New York Heart Association functional class.
LVEF ≤25 percent.
Being from a Black population
Multiparity (ie, having given birth two or more times)
Age greater than 30 to 35 years
28
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29. LV RECOVERY
In the Investigations of Pregnancy Associated Cardimyopathy (IPAC) study of 100
women in the United States, the majority improved their LV ejection fraction (EF) to
above 50% by 6 months post partum. Predictors that suggest the LV will not recover
include an initial LVEF below 30%, LV dilatation (LV end diastolic diameter [LVEDD] >60
mm) and black race.
RECURRENCE WITH SUBSEQUENT PREGNANCY
In a study of 34 patients (predominantly black women) with subsequent pregnancies
after a diagnosis of PPCM, the risk of relapse was 56%. The risk of recurrence was
higher in those with persistent LV dysfunction than in those with a normalized LVEF.
SUBSEQUENT PREGNANCY
The 2018(ESC) guidelines on the management of cardiovascular diseases and
pregnancy categorize PPCM with persistent LV dysfunction (LVEF < 50%) as modified
World Health Organization level IV (mWHO IV), a category defined as high maternal risk
in which pregnancy should be considered contraindicated 29
PERIPARTUM CARDIOMYOPATHY BY DR. MARYAM IQBAL