GAGAL JANTUNG AKIBAT KEHAMILAN

( PERIPARTUM CARDIOMYOPATHY )

dr. Stefan Hendyanto, Sp.JP - FIHA

RS Dirgahayu, Samarinda

differentiated from PPCM. Cases of acute Takotsubo syndrom

during the final trimester or following emergency delivery hav
been reported, and these require careful assessment to differen
6,7
tiate from PPCM.
In 2010, Study Group on peripartum cardiomyopathy of
the Heart Failure Association (HFA) of the European
Society of Cardiology (ESC)
Definition of peripartum cardiomyopathy
1. Heart failure secondary to left ventricular systolic
dysfuntion with a LVEF < 45%
2. Occurrence towards the end of pregnancy or in the
months following delivery (mostly in the month following
delivery)
3. No other identifiable cause of heart failure
◼Fetalgrowth is dependent on good blood flow
to the uterus and placent

◼An insufficient blood flow means decreased

oxygenation resulting in slowed growt

◼ Thisshould prompt further investigation to

discover heart disease
a

◼The incidence in the west ranges from 1 in

4000 deliverie
◼Sixty percent present within the first 2 months
postpartu
◼Up to 7% may present in the last trimester of
pregnanc
◼Geographic variations exist with a higher
incidence reported in areas of Africa because of
malnutrition and local customs in the
puerperium
y

◼Japanese cohort = 1: 20 00

◼In South Korea = 1:174

◼Indonesia??—> Data in Dr. Soetomo Hospital

from January 2014 to December 2016 = 25
1

◼maternal age >30 yr

◼multiparity and multiple pregnancies
◼pre-eclampsia
◼racial origin (black)
◼hypertension, diabetes, smokin
◼malnutritio
◼prolonged use of tocolytic beta-agonists
◼family history but in majority of cases there is
no family history
n

Uncertai
◼ genetic pre-dispositio
◼ low selenium level
◼ viral infection
◼ stress-activated cytokine
◼ inflammatio
◼ autoimmune reactio
◼ pathological response to haemodynamic stres
◼ unbalanced oxidative stres
◼ induction of antiangiogenic factors
n

Symptoms of worsening cardiac failure like:

▪dyspnoea on exertion
▪fatigue
▪ankle oedema
▪embolic phenomena
▪atypical chest pains and
▪haemoptysis.
▪Many of above symptoms may occur even in
normal pregnancy and can be mistaken for a
diseased state

◼evidence of a raised CVP

◼tachycardi
◼cardiomegaly with a gallop rhythm (S3
◼mitral regurgitatio
◼pulmonary crackles and
◼peripheral oedema
a

 Chest radiograph:
▪cardiomegaly with pulmonary oedema
▪pulmonary venous congestion

The ElectroCardioGram:
◼no specific ST and T wave change
◼atrial or ventricular arrhythmias and
◼conduction defects
n­

◼may reveal enlargement of all four chambers

with marked reduction in left ventricular systolic
functio
◼small to moderate pericardial effusion and
◼mitral, tricuspid and pulmonary regurgitation
◼Ventricular wall motion, ejection fraction and
cardiac output are decreased and
◼pulmonary wedge pressure is increased
n

Table 1 Differential diagnoses of peripartum cardiomyopathy
History
.........................................................................................................................................................................................
PPCM No known cardiac disease, no
HF signs and/or symptoms
prior pregnancy
Myocarditis Prior viral infection (e.g.
respiratory)
Pre-existing idiopathic/ HF signs and/or symptoms
familial dilated or and/or known heart disease
acquired prior pregnancy
cardiomyopathy
Takotsubo syndrome Chest pain, very stressful
delivery or emergency due to
foetal complications
Pregnancy-associated Chest pain, epigastric pain
myocardial infarction
Pulmonary embolism Chest pain, unilateral leg
swelling, acute dyspnoea
Onset
Towards the end of pregnancy
and the months following
delivery
Acute or subacute onset after
viral infection
During second trimester of
pregnancy
Acute onset, during delivery or
immediately after delivery
Acute onset, during pregnancy
or immediately after delivery
Acute onset during pregnancy or Elevated natriuretic peptides
after delivery
Biomarkers
Elevated natriuretic peptides
Elevated troponin, elevated
CRP
Elevated natriuretic peptides
Elevated natriuretic peptides
Elevated troponin
and/or troponin, elevated
D-dimer
Echocardiography/ Differentiation
cardiac MRI from PPCM
Reduced systolic LV function, –
LVEF <45%
Normal or reduced systolic LV Cardiac MRI (LE pattern),
function, typical myocardial myocardial biopsy
late gadolinium enhancement
pattern, pericardial effusion
Reduced systolic LV function, History, echocardiography,
RV dysfunction possible, cardiac MRI (LE pattern)
typical myocardial LE pattern
(DCM)
Regional wall motion History, echocardiography
abnormalities with typical
anatomical patterns
Regional wall motion History, ECG, coronary
abnormalities, ischaemic angiography, cardiac MRI
myocardial scar (LE pattern)
RV dysfunction, RV dilatation, Computed tomography, VQ
LV function usually normal scan
Amniotic fluid embolism Chest pain during/immediately Acute onset during delivery or Elevated natriuretic peptides Reduced RV systolic function, History, echocardiography
after delivery, acute dyspnoea immediately after delivery possible RV dilatation
Hypertensive heart Pre-existing or new-onset During second trimester of Elevated natriuretic peptides LV hypertrophy, diastolic History, echocardiography
disease/severe hypertension, proteinuria pregnancy dysfunction, transient LV
pre-eclampsia dysfunction
Hypertrophic Familial predisposition During second trimester of Elevated natriuretic peptides LV hypertrophy, typical History, echocardiography,
cardiomyopathy pregnancy myocardial late enhancement cardiac MRI (LE pattern)
pattern, LVOTO (HOCM)
HIV/AIDS HIV infection, AIDS During second trimester of Elevated natriuretic peptides Reduced systolic LV function, HIV serology/test
cardiomyopathy pregnancy LV/RV often not dilated
Pre-existing (unknown) HF signs and/or symptoms prior During second trimester of Elevated natriuretic peptides (Corrected) congenital heart History, echocardiography
congenital heart pregnancy, known heart pregnancy defects, cardiac shunts
disease disease, prior cardiac surgery
Pre-existing valvular HF signs and/or symptoms prior During second trimester of Elevated natriuretic peptides Valvular stenosis or History, echocardiography
heart disease pregnancy, known heart pregnancy regurgitation, prosthetic
disease heart valves

AIDS, acquired immunodeficiency syndrome; CRP, C-reactive protein; DCM, dilated cardiomyopathy; ECG, electrocardiogram; HOCM, hypertrophic obstructive cardiomyopathy; HF, heart failure; HIV, human immunodeficiency virus;
LE, late enhancement; LV, left ventricular; LVEF, left ventricular ejection fraction; LVOTO, left ventricular outflow tract obstruction; MRI, magnetic resonance imaging; PPCM, peripartum cardiomyopathy; RV, right ventricular; VQ,
ventilation–perfusion.
 * Symptoms during end of pregnancy or months following delivery:

Suspected acute PPCM* dyspnoea, orthopnoea, peripheral oedema, chest pain, dizziness,
palpitations, fatigue, depression, cough

** Cut-off for acute HF: NT-proBNP >300 pg/ml, BNP >100 pg/ml

Natriuretic peptides, ECG,

chest X-ray, and echocardiography

Natriuretic peptides↑** Natriuretic peptides↑ Natriuretic peptides

and LVEF <45% and LVEF ≥45% normal and LVEF ≥45%

Acute PPCM likely

Exclude overt pre-existing Consider other cardiac and Consider extracardiac

heart disease extracardiac origin
(e.g. chemotherapy-induced of symptoms
cardiomyopathy, congenital or (e.g. pulmonary embolism, amniotic
fluid embolism, isolated RV dysfunction,
valvular heart disease,
hypertensive disorders of pregnancy,
hypertrophic cardiomyopathy)
eclampsia, sepsis)
origin of symptoms
(e.g. anaemia, pneumonia, renal
disease, hypertensive disorders
of pregnancy, eclampsia, depression,
physiological changes)

Figure 1 Diagnostic pathway in patients with suspected peripartum cardiomyopathy (PPCM). BNP, B-type natriuretic peptide;
ECG, electrocardiogram; HF, heart failure; LVEF, left ventricular ejection fraction; NT-proBNP, N-terminal pro-B-type natriuretic peptide;
RV, right ventricular.
Table 2 Diagnostic tests that are recommended for the diagnosis of peripartum cardiomyopathy at initial diagnosis
and at follow-up visits

Clinical ECG Natriuretic Echocardiography Chest Cardiac CT Coronary

examination peptides X-ray MRI scan angiography
...........................................................................................................................................
Diagnosis of PPCM X X X X X (X)b (X)b (X)b
4-6 weeks after diagnosis X X X X
3 months after diagnosis X X Xa X
6 months after diagnosis X X Xa X (X)b
12months after diagnosis X X Xa X
18months after diagnosis X X Xa X
Annually for at least 5 years X X Xa X
after diagnosis (especially if
not fully recovered)

Generally, an individual approach is recommended depending on the severity of the disease and/or potential differential diagnoses.
CT, computed tomography; ECG, electrocardiogram; MRI, magnetic resonance imaging; PPCM, peripartum cardiomyopathy.
a May be considered depending on costs and local availability.
b May be considered depending on the clinical presentation and/or differential diagnoses.
 Mild PPCM Moderate PPCM Severe PPCM

Clinical Subacute heart failure Acute heart failure Cardiogenic shock

presentation Haemodynamic stability Haemodynamic instability
Haemodynamic stability
Respiratory insufficiency Respiratory insufficiency

ECG No specific changes No specific changes, often tachycardia No specific changes, often tachycardia

Pulmonary congestion, Pulmonary congestion, Pulmonary congestion, enlarged cardiac

Chest X-ray
may also be normal enlarged cardiac silhouette silhouette, pleural effusion

Natriuretic
peptides ↑ ↑↑ ↑↑↑

LVEF <25%, RV dysfunction

Echocardiography LVEF 30-45% LVEF 20-35%
and dilatation possible

Normal ward, ambulatory treatment

Intermediate care (IMC), HF unit (HFU) Intensive care unit (ICU)
in selected patients possible

– Oral HF drugs – Diuretics i.v. – Diuretics i.v.

– Oral diuretics in case of fluid overload
– Consider bromocriptine for 1 weeka
Therapy
– Consider vasorelaxants if SBP >110 mmHg
– Supplemental O2, non-invasive ventilation
if necessary
– Avoid inotropes/catecholamines
– Consider bromocriptine for 8 weeksa if
LVEF <25%
– Oral HF drugs
– Inotropes/catecholamines if needed
– Invasive ventilation
– Mechanical circulatory support (Impella
and/or ECMO)
– Consider bromocriptine for 8 weeksa,
uptitration depending on prolactin levels
– Oral HF drugs after stabilization

Figure 2 Overview of different clinical scenarios in patients with peripartum cardiomyopathy (PPCM). Typical results from diagnostic tests
and recommended monitoring/treatment options are depicted according to disease severity. ECG, electrocardiogram; ECMO, extracorporeal
membrane oxygenation; HF, heart failure; HFU, heart failure unit; ICU, intensive care unit; IMC, intermediate care unit; LVEF, left ventricular
ejection fraction; RV, right ventricular; SBP, systolic blood pressure. a Bromocriptine may be considered in PPCM patients (class IIb
recommendation) and should be accompanied by at least prophylactic anticoagulation.
50-60% patients show complete or near complete
recovery within the first 6 months postpartu

In others, either continued clinical deterioration leading

to early death or persistent left ventricular dysfunction
and chronic heart failure results

There is an initial high risk period with mortality of

25-50% in the first 3 months postpartum

Patients with persistent cardiomegaly at 6 months have a

reported mortality of 85% at 5 years

◼Subsequent pregnancies in women with PPCM

are often associated with relapses and high risk
for maternal morbidity and mortality

◼shouldbe discouraged in women with PPCM

who have persistent cardiac dysfunctio

◼Reported mortality rates = 2% in Germany -

12.6% in from South Africa

◼The out come is highly variabl

◼Some develop persistent disease while some

return to normal state slowl

◼These patients has a better survival rate than

other types of cardiomyopathy
y

Table 3 Modified World Health Organization classification of maternal cardiovascular risk

mWHO I mWHO II mWHO II–III mWHO III mWHO IV

Diagnosis (if other- Small or mild Unoperated atrial or Mild left ventricular impair- Moderate left ventricular Pulmonary arterial
wise well and – pulmonary stenosis ventricular septal ment (EF >45%) impairment (EF 30–45%) hypertension
uncomplicated) – patent ductus defect
Hypertrophic Previous peripartum cardio- Severe systemic ventricu-
arteriosus
Repaired tetralogy of cardiomyopathy myopathy without any resid- lar dysfunction (EF <30%
– mitral valve prolapse
Fallot ual left ventricular impairment or NYHA class III–IV)
Successfully repaired Native or tissue valve dis-
simple lesions (atrial or Most arrhythmias ease not considered WHO Mechanical valve Previous peripartum car-
ventricular septal defect, (supraventricular I or IV (mild mitral stenosis, diomyopathy with any
Systemic right ventricle with
patent ductus arteriosus, arrhythmias) moderate aortic stenosis) residual left ventricular
good or mildly decreased
anomalous pulmonary impairment
Turner syndrome Marfan or other HTAD ventricular function
venous drainage)
without aortic syndrome without aortic Severe mitral stenosis
Fontan circulation.
Atrial or ventricular dilatation dilatation
If otherwise the patient is well Severe symptomatic
ectopic beats, isolated
Aorta <45 mm in bicuspid and the cardiac condition aortic stenosis
aortic valve pathology uncomplicated
Systemic right ventricle
Repaired coarctation Unrepaired cyanotic heart with moderate or
disease severely decreased ven-
Atrioventricular septal
tricular function
defect Other complex heart disease
Severe aortic dilatation
Moderate mitral stenosis
(>45 mm in Marfan syn-
Severe asymptomatic aortic drome or other HTAD,
stenosis >50 mm in bicuspid
aortic valve, Turner syn-
Moderate aortic dilatation
drome ASI >25 mm/m2,
(40–45 mm in Marfan syn-
tetralogy of Fallot >50
drome or other HTAD;
mm)
45–50 mm in bicuspid aortic
valve, Turner syndrome ASI Vascular Ehlers–Danlos
20–25 mm/m2, tetralogy of
Severe (re)coarctation
Fallot <50 mm)
Fontan with any
Ventricular tachycardia
complication

Risk No detectable increased Small increased risk of Intermediate increased risk Significantly increased risk of Extremely high risk of
risk of maternal mortality maternal mortality or of maternal mortality or maternal mortality or severe maternal mortality or
and no/mild increased moderate increase in moderate to severe morbidity severe morbidity
risk in morbidity morbidity increase in morbidity

Maternal cardiac 2.5–5% 5.7–10.5% 10–19% 19–27% 40–100%

event rate

Counselling Yes Yes Yes Yes: expert counselling Yes: pregnancy contrain-
required dicated: if pregnancy
occurs, termination
should be discussed

Care during Local hospital Local hospital Referral hospital Expert centre for pregnancy Expert centre for preg-
pregnancy and cardiac disease nancy and cardiac
disease

Minimal follow-up Once or twice Once per trimester Bimonthly Monthly or bimonthly Monthly
visits during
pregnancy

Location of delivery Local hospital Local hospital Referral hospital Expert centre for pregnancy Expert centre for preg-
and cardiac disease nancy and cardiac
disease

ASI = aortic size index; EF = ejection fraction; HTAD = heritable thoracic aortic disease; mWHO = modified World Health Organization classification; NYHA = New York
Heart Association; WHO = World Health Organization.
◼PPCM mimics changes occurring in normal
pregnanc

◼Fetalgrowth retardation may point towards this

conditio

◼Treat like any other cardiac failure along with

anti coagulant therap

◼Advice against subsequent pregnancies

n

 BOARD scheme

Bromocriptine Oral HF drugs Anticoagulation Relaxants Diuretics

(dose according (beta-blocker, (at least in (intravenous (in case of fluid
to severity of ACE inhibitor/ prophylactic vasodilators if overload)
the disease) ARB, MRA) dose) SBP >110 mmHg)

LVEF ≥ 25%,
Bromocriptine 2.5 mg o.d. for 7 days,
no cardiogenic shock,
at least prophylactic anticoagulation
no ICU treatment

LVEF <25%, Bromocriptine 2.5 mg b.i.d. for 14 days

and/or RV dysfunction, followed by bromocriptine 2.5 mg o.d.
and/or cardiogenic shock, for another 42 days, at least
and/or ICU treatment prophylactic anticoagulation

Start with bromocriptine 2.5 mg b.i.d.,

ICU treatment, uptitrate to a maximum of 10-20 mg
cardiogenic shock daily depending on serum prolactin
with ventilation and/or MCS levels until successful suppression,
at least prophylactic anticoagulation

Figure 3 BOARD scheme for the therapy of patients with acute peripartum cardiomyopathy (PPCM). Of note, this scheme addresses
patients after delivery who do not breastfeed. If bromocriptine treatment is considered (class IIb recommendation), different regimens
are recommended according to disease severity. ACE, angiotensin-converting enzyme; ARB, angiotensin receptor blocker; b.i.d., twice daily;
HF, heart failure; ICU, intensive care unit; LVEF, left ventricular ejection fraction; MCS, mechanical circulatory support; MRA, mineralocorticoid
receptor antagonist; o.d., once daily; RV, right ventricular; SBP, systolic blood pressure.
Thank you