peripartum cardiomyopathy edited

8/8/2019 Peripartum Cardiomyopathy Edited

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Peripartum Cardiomyopathy

Adapted from source


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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, nodiabetes mellitus, no congentital heart disease, no rheumatic fever


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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


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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


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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


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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 EFof 25-30%, mild MR and normal RV size and

function Referred to PCH, Heart failure specialist as

inpatient


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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


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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


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History

Relationship between Pregnancy and dilated

CMP first reported by Ritchie 1849

1870 Virchow and Porak described evidenceof 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


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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.


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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 diseasebefore the last month of pregnancy


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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


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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 differentcountries unknown. But similar disease

processes in those countries likely.


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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.


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PPCM - aetiology

Cause and mechanism remains unknown

Nutritional disorders as causes not confirmed

Autoimmune mechanisms studied, thoughsome of them have lymphocyte infiltrate,

myocyte oedema, necrosis and fibrosis no

causal link could be established


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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


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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%)


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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


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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


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PPCM - Investigation

To think of PPCM in any peripartum patientwith unexplained disease

Historytaking

Clinical examination

ECG

Chest X-ray

Pathology

Echocardiogram


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PPCM-Echocardiogram


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PPCM - Echocardiogram


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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 PPCMoccurs after pregnancy, during pregnancy Hydralazine,

Methyldopa

Betablockers used since high heart rate, arrhythmias and

sudden death often occur


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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


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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

TNF and improved outcome in a study.


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PPCM follow up

Depends o response to treatment

Follow-up echocardiogram in the first several

weeks to confirm improvement of LV systolicfunction

Follow up with an echocardiogram every 6

months until recovery confirmed or plateau

reached.


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PPCM follow-up

Best time to stop ACE- inhibitor or betablocker

is unknown, but at least one of them for at

least 1 year.


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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


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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


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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 fullyrecovered first, and even with full recovery some additional

risk of relapse remains.


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PPCM - Literature

Peripartum cardiomyopathy, Lancet, Vol

368, August 19, 2006, p687-693

Emergency department evaluation andmanagement of peripartum cardiomyopathy,

the journal of emergency medicine, 2007

Maternal and fetal outcomes of subsequent

pregnancies in

peripartum cardiomyopathy edited

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