Peripartum Cardiomyopathy

Adapted from source

 Case report
• Patient , 34 y
• Emergency caesarian section 26.5.07 due to a breach delivery, at 38
weeks, in GA
• G2P1M1
• Slight signs of praeeklampsia with slight oedema, proteinuria and
hypertension up to 160/100 mmHg
• History of hypertension for 8 years, on Ramipril 5 mg, switched to
aldomet 250 mg qid, „heart too big, some investigations done“, mild
hyperlipidemia
• No smoker, no family history for IHD, CMP or sudden cardiac death, no
diabetes mellitus, no congentital heart disease, no rheumatic fever
 Case report
• During pregnancy some shortness of breath
but felt it not particularly unusual, improved
after delivery
• In theatre anesthetist described „stable VT for
about 1 min“, treated with lidocaine
• Admitted to CCU after caesarian for
observation
 Case report
• On examination: Pt. Well, no shortness of breath,
173 cm, 88 kg
• BP 144/70 mm Hg, HR 64/min, RR 14/min
• Minimal pedal oedema, JVP 4 cm
• Chest clear, dual heart sounds (with added S3)
• Troponin negative, TFT normal
• X-ray chest: Cardiomegaly but no heart failure
 Case report
• Informal echocardiogram:
• Dilated left ventricle, 6.7 cm diastolic
• Estimated EF 35-40%
• Mild to moderate mitral regurgitation
• Septum with paradoxic movement due to
LBBB, hyperechogenic
• DD CMP (DCMP, PPCM), IHD
 Case report
• Referred to Dr.
• On 6.6.07 ECG with SR, LBBB
• No clinical changes
• Echocardiogram with marked left ventricular
dilatation and severe global reduction with EF
of 25-30%, mild MR and normal RV size and
function
• Referred to PCH, Heart failure specialist as
inpatient
 Case report
• Referred to Hospital for insertion of an ICD in
view of the low EF making her more
susceptible for VF/VT
• Mirena coil insertion 7/07 as recommended
• At that time in good condition, feeling fine
 Peripartum Cardiomyopathy
Definition
• Disorder of unknown cause
• Initial left ventricular dysfunction and
symptoms of heart failure
• Onset between the last month of pregnancy
and the first 5 months postpartum
 Peripartum Cardiomyopathy
History
• Relationship between Pregnancy and dilated
CMP first reported by Ritchie 1849
• 1870 Virchow and Porak described evidence
of myocardial degeneration at autopsy of
patients who died in the peripartum period
• 1937 Gouley et al: case series of 7 women
developing non-ischemic CMP in late
pregnancy
 Peripartum Cardiomyopathy
History
• 1971 Demakis: PPCM formally defined as
otherwise unexplained LV failure in the last
month of pregnancy or during the first 5
months postpartum, without prior evidence of
heart disease, additionally there must be no
other identifiable cause of heart failure.
 Peripartum Cardiomyopathy
History
• 1997 National Heart, Lung, and Blood institute
(NHLBI) workshop agreeed on a standardized
definition:
• PPCM clinically defined as the onset of cardiac
failure with no identifiable cause in the last
month of pregnancy or within 5 months after
delivery, in the absence of heart disease
before the last month of pregnancy
 PPCM -Definition
• PPCM clinically defined as the onset of cardiac failure with no
identifiable cause (diagnosis of exclusion) in the last month of
pregnancy or within 5 months after delivery, in the absence of
heart disease before the last month of pregnancy
• Additional echocardiographic criteria proposed:
• Ejection fraction of less than 45%, fractional shortening of less
than 30%, or both
• End-diastolic left ventricular dimension of greater than 2.7
cm/m2 body surface-area
 PPCM - Epidemiology
• Incidence: PPCM associated with 1 of every
3000-4000 live births in the US (1000-1300
women annually)
• 1 case per 299 livebirths in Haiti
• 1 case per 1000 livebirths in South Africa
• Reasons for the variation in different
countries unknown. But similar disease
processes in those countries likely.
 PPCM - Epidemiology
• Suggested risk factors: age, gravidity or parity,
African origin, toxaemia or hypertension of
pregnancy, use of tocolytics, twin pregnancy
• But 24-37 % in young primigravid patients.
 PPCM - aetiology
• Cause and mechanism remains unknown
• Nutritional disorders as causes not confirmed
• Autoimmune mechanisms studied, though
some of them have lymphocyte infiltrate,
myocyte oedema, necrosis and fibrosis no
causal link could be established
 PPCM - aetiology
• Viral trigger suggested, viral genomic material found
in biopsies, but no difference to controls
• Microchimerism: fetal cells in maternal blood during
and after pregnancy, increased levels could lead to
initiation of an autoimmune myocarditis.
• Signs of higher inflammatory response: higher levels
of TNF alpha, CRP, Fas/Apo-1 (a marker of apoptosis)
 PPCM - aetiology
• Higher levels of immunoglobulins (class G and
subclasses G1, G2, G3) against cardiac myosin
• Stress activated cytokines ?
• Genetic factors ?
• Excessive prolactin production ?
• So far no cause has been clearly identified
• Aetiology is likely multifactorial
 PPCM – Clinical presentation
• Most common symptoms and signs of systolic heart failure,
most frequently initial presentation is with NYHA III and IV:
• Dysplaced hypodynamic apical impulse (72%)
• Gallop rhythm (92%)
• Functional mitral regurgitation (43%)
• ECG voltage criteria of LV-hypertrophy (66%)
• ST-T wave abnormalities (96%)
 PPCM – Clinical presentation
• Additional symptoms and signs: dependent oedema,
dyspnoea on exertion, orthopnoea, paroxysmal nocturnal
dyspnoea, persistent cough, abdominal discomfort secondary
to passive congestion of liver and other organs, precordial
pain, palpitations.
• In later stages postural hypotension reflecting low cardiac
output and hypotension
• Sudden cardiac arrest
 PPCM – Clinical presentation
• Left ventricular thrombus common in EF < 35%, with possible
peripheral embolism (arterial embolism of limbs, cerebral
embolism, mesenteric artery occlusion with acute abdomen,
acute myocardial infarction due to coronary embolism
• With progression of disease four chamber dilatation may be
seen, with thrombus formationn in left atrium and right
ventricle, then pulmonary embolism is possible as well
 PPCM - Investigation
• To think of PPCM in any peripartum patient
with unexplained disease
• Historytaking
• Clinical examination
• ECG
• Chest X-ray
• Pathology
• Echocardiogram
PPCM-Echocardiogram
PPCM - Echocardiogram
PPCM - Echocardiogram
PPCM - Echocardiogram
 PPCM - Management
• Medical management similar to that for other forms of heart
failure
• Reduce afterload and preload, increase contractility.
• ACE- Inhibitors to reduce afterload by vasodilatation if PPCM
occurs after pregnancy, during pregnancy Hydralazine,
Methyldopa
• Betablockers used since high heart rate, arrhythmias and
sudden death often occur
 PPCM - Management
• Digitalis is safe during pregnancy, may help to maximise contractility and
rate control. Close monitoring as excessive digoxinlevels have been
associated with worse outcome in women.
• Diuretics are safe and are used to reduce preload and relieve symptoms.
• High incidence of thromboembolism in patients with LV EF<35%,
therefore treatment with Heparin followed by Warfarin
• Left ventricular assist devices and heart transplant if necessary
• To check indication for ICD if necessary (EF <35%, secondary prevention of
a serious rhythm event)
 PPMC – experimental therapy
• Immunosuppressive drugs such as Azathioprine and steroids
with limited studies, mixed results
• Use of those agents should be reserved pending further
assessments, perhaps restricted to patients with biopsy-
proven lymphocytic myocarditis in the absence of viral
particles.
• PCR testing for cardiomyotrophic viruses?
• Promising results with pentoxifylline and conventional
therapy ? Significant reduction in the inflammatory marker
TNFα and improved outcome in a study.
 PPCM – follow up
• Depends o response to treatment
• Follow-up echocardiogram in the first several
weeks to confirm improvement of LV systolic
function
• Follow – up with an echocardiogram every 6
months until recovery confirmed or plateau
reached.
 PPCM – follow-up
• Best time to stop ACE- inhibitor or betablocker
is unknown, but at least one of them for at
least 1 year.
 PPCM - Prognosis
• Higher rate of spontaneous recovery of
ventricular function than with other forms of
non-ischemic CMP
• In single prospective studies 15% died and 23-
31% recovered normal left ventricular
function after 6 months.
• Continuing improvement was observed in the
2nd and 3rd year after diagnosis
 PPCM - Prognosis
• Persistence of cardiac dysfunction 6 to 12
months after diagnosis usually indicates an
irreversible problem, but continuing
improvement in cardiac function well beyond
the initial 6-12 months after diagnosis
 PPCM – further pregnancies ?
• Subsequent pregnancies in women with PPCM is associated
with significant decrease in LV-function resulting in clinical
detoriation and even death
• Heart failure symptoms in 21% who entered subsequent
pregnancy with normal LV-function, and in 44% of those with
already abnormal LV-function. All deaths occured in the latter
group.
• Subsequent pregnancy after a diagnosis of PPCM carries
higher risk of relapse if LV systolic function is not fully
recovered first, and even with full recovery some additional
risk of relapse remains.
 PPCM - Literature
• „Peripartum cardiomyopathy“, Lancet, Vol
368, August 19, 2006, p687-693
• „Emergency department evaluation and
management of peripartum cardiomyopathy“,
the journal of emergency medicine, 2007
• „Maternal and fetal outcomes of subsequent
pregnancies in