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Case Report

Peripartum Cardiomyopathy
Presented by :
Dede Achmad Basofi, S. Ked
I 111 12 011

Supervisor :
Letkol CKM dr. Prihati Pujowaskito, Sp. JP (K), MMRS

Department of Cardiology
Dustira District Hospital, Cimahi
Faculty of Medicine
Tanjungpura University
2018
Case Delivery
Autoanamnesis
• Patient Identity
• Name : Mrs. NA
• Sex : Female
• Age : 35 years old
• Religion : Islam
• Address : Hegarmanah Street, No. 89
• Admission Time : March 15th 2018, 09.08 PM
• Interview
• Chief Complain : Shortness of breath
• A women 35 years old came to Emergency Department
with shortness of breath, this complain felt from 2 weeks
ago
• Shortness of breath felt when she has activity
• Sleeping with 3-4 pillows
• Cough from 1 week ago
• She complained about leg swelling from 2 weeks ago
Case Delivery
Autoanamnesis (1)
• History of past illness
• Diabetes Melitus from 3 years ago (+)
• Hypercholestrol (+)
• Hypertension from 4 years ago (+)
• Angina (-)
• Alergic (-)
• Heart failure (-)
• Post SC third children in the age of 35 years old
• Preeklampsia (+)
Case Delivery
Physical Examination
BP = 150/80 mmHg PR = 88 bpm, reguler RR = 32 tpm, T = 36.8 celcius
takipneu

General appearance looked moderately ill GCS E4 V5 M6


Eye Anemic (-) Icteric (-)
Neck JVP 5+4 cmH2O
Thorax Retraction (-), Ictus Cordis not seen
Cor S1 S2 single, murmur (+), gallop (-)

Pulmo Simetric, Vesicular D = S Rh +/+ Wh - /-

Abdomen Soefl, Hepar : Liver span 11 cm, spleen traube space tympani.
Tenderness (-)
Extremities Oedema ( +/+), CRT < 2”
Case Delivery
15th March 2018
09.22 PM Electrocardiography
Case Delivery
Electrocardiography (1)

Rythm : Sinus
Freq : 68 bpm, regular
Axis : LAD
P Wave : 0,28 s
P-R Interval : 0,36 s
QRS Wave : 0,10 s
Abnormality : P Mitral, T inverted in all leads
Conclussion : Sinus Rhytm, normocardia,
LAD, Left Atrium Enlargement, Ischemia
Extensive Anterior + Posterior
Case Delivery
16th March 2018
11.15 AM Echocardiography
Case Delivery
Echocardiography
• Findings
• ECG rhytm : Sinus rhytm
• Left Ventricle : mildly enlarge, severe hypokinesis
of LV contractility, LV systolic function severely
impaired, EF : 20-25%
• Left Atrium : normal size
• Right Ventricle : mildly enlarge, 3,4-3,7 cm
• Right Atrium : mildly enlarge
• Aortic Valve : structurally normal
• Mitral Valve : moderate regurgitation
• Tricuspid Valve : moderate regurgitation
• Pulmonic Valve : not well visualized
Case Delivery
Echocardiography
• Conclusions
• Mildly dilated LV, RA, RV
• Poor LV systolic function, EF 20-25%, global
severe hypokinetic
• Diastolic dysfunction grade II
• Reduce RV contractility
• Summary
• PPCM
• CHF
• Pulmonary Hypertension
• Moderate MR
• Moderate TR
Case Delivery
Laboratory Findings
15th March 2018

Hemoglobine : 14,3 g/dl


Eritrosite : 4,8 x 106/µl
Leukocyte : 6.100/µl
Hematocrtye : 42.2%
Trombosite : 273.000/µl
MCV : 88,3 fl
MCH : 29,9 pg
MCHC : 33,9 g/dl
RDW : 14,6%
Ba/Eo/Sg/Li/M : 0,5/1,0/48,9/44,4/5,2

Interpretation : Normal findings


Case Delivery
Diagnose
• Clinical Diagnose
• Congestive Heart Failure NYHA Class III
• Anatomic Diagnose
• Left Ventricle dilated
• Cardiomegaly
• Etiology Diagnose
• Pregnancies
Case Delivery
Therapy
• Non Medicamentose
• Semi fowler
• O2 2-4 Liter via Nasal Cannule
• Low salt intake
• Fluid Restriction
• Echocardiography examination every 6
months
• Medicamentose
• Control Risk Factor
• PO. Furosemide 1x40 mg
• PO. Ramipril 1x25 mg
Case Delivery
Prognosis
• Quo ad vitam : Dubia ad Malam
• Quo ad sanactionam : Dubia ad Malam
• Quo ad functionam : Dubia ad Malam
Thank You!
Discussion
• Diagnose PPCM?
• From anamnesis and physical examination and supportive
examination (Echocardiography)
Anamnesis Physical Examination
Presenting towards the end of Signs and symptomps of HF 
pregnancies or first week until 6 months Ortopnea, Elevated JVP, peripheral
after --> 2 months after SC third child edema

Multipara  >3 times pregnant Cor  Murmur Echocardiography


LV dilatations
LV systolic dysf.
Risk Factors such as Hipertension, Mitral regurgitation
Diabetes Melitus, Smoking  Diabetes Pulmonary hipertension
Melitus, Hipertension

Preeclampsia

From the position statement of ESC HFA working group on peripartum cardiomyopathy,
Eur J Heart Fail 2010 12, 767-778
Discussion
• Therapy?
• PO. Ramipril 1x2,5 mg • PO. Bisoprolol 1x5 mg
• ACE Inhibitor  • Beta Blocker 
• Potentially all patients • Beta blocker
with HF and LVEF <40% cardioselective
• First line treatment in • High affinity to
patients with HF NYHA adrenoreceptor
Class II-IV • Slows down heart rate
• Benefit in patients with (negative kronotropic)
asymptomatic LV systolic • Inotropic negative
dysfunction (miocard contractility)
• Start with low dose • Reducing cardiac output,
reducing peripheral
resistence  Blood
pressure reducing

2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure
Discussion
• Therapy?
• PO. Furosemide 1x40 mg
• Loop Diuretics
• To relieve breathlessness and
oedema in patients with
symptomps and signs of
congestion
• Reducing preload
• Reduvcing atrial natriuretic peptide
(ANP)  cardiac output reducing
• Combination with ACE-Inhibitor 
prevent resistence furosemid

2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure
Resume
• Peripartum Cardiomyopathy presenting with Heart Failure to
secondary to Left Ventricle dysfunction (EF <45%) or LV may not be
dilated
• Appears at the end of pregnancies or in the months following
delivery
• Causes are probably genetics or risk factors such as hipertension,
diabetes melitus, smoking etc.
• Early signs and symptomps are : peripheral edema, dyspnoe on
exertion, orthopnoe, paroxysmal nocturnal dyspnoe, and
persistent cough  Heart Failure
• Management are  If heart failure symptoms begin after delivery  ESC
HFA guidelines apply
• Supportive examination such as echocardiography and MRI
• Prognose  show mortality rates of 10% at 6 months and 28% at
2 years
Introduction

• PPCM is usually presenting with HF secondary to LV


systolic dysfunction (EF less than 45%, LV may not be
dilated)
• Appears at the end of pergnancy or in the months following
delivery
• Diagnosis of exclusion
Incidence of Peripartum Cardiomyopathy

Gunderson et al., 2011, USA 1995 – 2004 110 1 : 2066 Population based
Hasan et al., 2010, Pakistan 2003 – 2007 32 1 : 837 Case series (single institution)

PPCM defined as: HF symptoms that appear one month before to 5 months after delivery. National Heart Lung and Blood Institute (2000)
Pathophysiology of PPCM

1. Cardiac tissue
microenvironment:
• impaired metabolism of
cardiomyocytes
• disrupted
microcirculation

2. SYSTEMIC
FINDINGS:
• Inflammation –
elevated levels
of sFas/Apo-1, C-reactive
protein, interferon gamma
(INFγ) and IL-6
• Autoimmune processes
Pathophysiological basis for cardiomyocyte malfunction in
PPCM: oxidative stress and prolactin

Oxidative stress activation of


cathepsin D (aCD) in cardiomyocytes
cleavege of prolactin via cathepsin D
creation of 16 kDa subform of
prolactin

16 kDa subform of prolactin is anti-


angiogenic, pro-apoptotic and pro-
inflammatory

Yamac H et al., Prolactin: a new therapeutic target in peripartum cardiomyopathy.


Heart. 2010 Sep;96(17):1352-7. Epub 2010 Jul 23.
Risk factors for development of
peripartum CMP

Blauwet LA, Cooper LT. Diagnosis and management of peripartum


cardiomyopathy.
Recent scientific evidence for genetic
susceptibility to peripartum CMP

• A subset of PPCM is an initial manifestation


of familial DCM (van Spaendonck-Zwarts KY,
Circulation 2010)
– mutation in the gene encoding cardiac
troponin C (TNNC1)

• African Americans have higher risk of


PPCM, and
Hispanics have lower risk than average
population

Position statement of HFA working group on


PPCM –
“general genetic
From the position testing
statement of ESC HFAis notgroup
working recomended as a
on peripartum cardiomyopathy,
routine but is currently being done as part of Eur J Heart Fail 2010 12, 767-778
Early signs and symptoms

• Peripheral edema
• Dyspnoea on exertion
• Orthopnoea
• Paroxysmal nocturnal
dyspnoea
• Persistant cough

these symptoms mimic


normal physiological
findings of pregnancy

From the position statement of ESC HFA working group on peripartum cardiomyopathy,
Eur J Heart Fail 2010 12, 767-778
Onset of symptoms

• The majority of patients (78%) experiences


signs of heart failure 4 months after delivery
• 9% of patients present in the last month of
pregnancy
• 13% present either prior to 1 month before
delivery, or more than 4 months postpartum

Complications
1. Left ventricular thrombosis in
patients with severely impaired
systolic function (EF<35%)
2. Peripheral embolic episodes –
cerebral, coronary, mesenteric,
pulmonary
Peripartum Cardiomyopathy with left ventricular thrombus. Courtesy of K. Sliwa, Soweto Cardiovascular Research
Early diagnostic algorithm

Breathless woman towards


the end of pregnancy/early
post partum

ECG OR natriuretic peptides


ECG abnormalities – ST and AND echocardiography
T wave abnormalities in
96%, voltage criteria
consistent with LV Any abnormalities All normal
hypertrophy in 66%.

B-type natriuretic peptide –


increased plasma levels in Further Consider non-
virtually all patients cardiology
diagnostic cardiovascul
s ar causes

From the position statement of ESC HFA working group on peripartum cardiomyopathy,
Eur J Heart Fail 2010 12, 767-778
Cardiac imaging in PPCM patients

• Echocardiography :
– diagnosis: LV dilatation, diminished systolic function
(LVEF <45%)
– prognosis: LVEDD >60mm and/or LVEF <30%
predicts poor recovery of LV function
– rulling out LV thrombus

• MRI: more accurate measurements of LV


volumes and function, higher sensitivity in
diagnosing LV thrombus; measurement of late
enhancement following administration of
gadolinium to exclude myocardial inflammation

 Echocardiography repeated at discharge, 6


weeks, 6 months, and anually to evaluate the
efficacy of medical treatment
Therapy options for acute heart failure in
peripartum cardiomyopathy

1. Emergency medication:
administration of oxygen + i.v.
diuretics if congestion is present
+ i.v. nitrate if SBP >110mmHg
2. i.v. inotropics and/or intra-aortic
balloon pump counterpulsation
3. Implantation of left ventricular
assist device, at least as “bridge to
transplantation”
4. Up to 11% of patients with PPCM
undergo heart transplantation

From the position statement of ESC HFA working group on peripartum cardiomyopathy,
Eur J Heart Fail 2010 12, 767-778
Medical therapy for stable heart failure
in peripartum cardiomyopathy

1. If heart failure symptoms begin after delivery  ESC


HFA guidelines apply
2. If heart failure onsets prior to delivery, following restrictions
apply:
a. ACE inhibitors and ARBs are contraindicated, instead
hydralazine and long-acting nitrates should be used
b. Beta-1 selective blockers are preferred (generally
beta-blockers are not contraindicated)
c. Warfarin is contraindicated, should be replaced by
unfractioned or low-molecular weight heparin
d. Diurectics should be used with caution,
spirinolactone and eplerenone should be avoided

From the position statement of ESC HFA working group on peripartum cardiomyopathy,
Eur J Heart Fail 2010 12, 767-778
Indications for cardiac resynchronization therapy (CRT)
and implantation of defibrillators (ICD)

 In PPCM patients with severely


impaired systolic function 6
months after onset of
symptoms, despite optimal
therapy, ICD is advisable (along
with CRT if patient has NYHA III
or IV and QRS duration >
120ms)

From the position statement of ESC HFA working group on peripartum cardiomyopathy,
Eur J Heart Fail 2010 12, 767-778
Novel therapies : PPCM targeted at its
fundaments
Effects of biologically
active form of prolactin Oxidativ
e stress
(16 kDa) in the
cardiac
microenvironment is
found to be associated
with cardiomyocyte
malfunction Cathepsin
D

Bromocriptine inhibits Biologically


secretion of prolactine and
detrimental 16 kDa active
subform is not generated form of
prolactin

Lok SI et al. Peripartum cardiomyopathy: the need for a national


database.
Bromocriptin in PPCM: Clinical Results

• Dosing : 2.5mg twice daily for 2 weeks,


than 2.5mg daily for 6 weeks
• Systolic function: change in LVEF in the
bromocriptine group from 27% to 58% at
6 months vs. 27% to 36% in the control
group
• Thrombo-embolic complications (occurence
of MI has been reported) in patients taking
bromocriptine anti-coagulation therapy is
required
Delivery, breastfeeding and subsequent pregnancies
1. Timing:
 Urgent delivery is recomended if haemodynamic instability
is persistent
2. Mode:
 If mechanical support or i.v. inotropics are required
Caesarean section is preferred
 Continuous spinal anaesthesia, and combined spinal and
epidural anaesthesia are recommended

3. Breastfeeding – not recommended in PPCM patients due to


described detrimental effects of prolactin

4. Subsequent pregnancies - contraindicated, if LVEF <25%


at diagnosis, or subsequently not normalized
From the position statement of ESC HFA working group on peripartum cardiomyopathy,
Eur J Heart Fail 2010 12, 767-778
Prognosis of PPCM varies geographically ?

Single center study, mortality


Turke rate 30% at 4 years
y

Sout
h
Single center studies, Afric
a
mortality rate 14-16% at 6
months
Population-based study from South
Africa show mortality rates of 10%
at 6 months and 28% at 2 years

Possible independent mortality predictors: NYHA class, LVEF,


QRS duration, late onset of symptoms
Thank You!
ONCE AGAIN!

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