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Peripartum cardiomyopathy

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Title: Peripartum cardiomyopathy


1
Peripartum cardiomyopathy
  • Suhail Allaqaband, M.D
  • Sinai Samaritan Medical Center
  • Milwaukee, WI

2
EPIDEMIOLOGY
  • Peripartum cardiomyopathy (PPCM) is a form of CHF
    that affects women late in pregnancy or in the
    early puerperium
  • True incidence of PPCM is unknown estimates
    proposed over the last several decades range from
    1 per 1300 to 1 per 15,000 live births
  • In some regions of Nigeria, the incidence has
    been reported to be as high as 1100

3
ETIOLOGY
  • A distinct etiology of PPCM remains unknown
  • Nutritional deficiences
  • Myocarditis
  • Infections
  • Autoimmune
  • Idiopathic

4
Myocarditis
  • Peripartum heart disease an endomyocardial
    biopsy study.Sanderson JE Olsen EG Gatei D
  • Br Heart J 1986 Sep56(3)285-91
  • Endomyocardial biopsies were performed in 11
    African women in Nairobi who presented with the
    peripartum cardiomyopathy.
  • 5 patients had evidence of a myocarditis
  • Of the 9 patients who were followed up, 3 out of
    4 with myocarditis had persistent heart failure
    and 4 out of 5 without myocarditis improved

5
Myocarditis
  • Peripartum myocarditis and cardiomyopathy
  • Circulation 1990 Mar81(3)922-8
  • 18 consecutive patients with peripartum
    cardiomyopathy
  • 14 of the 18 patients (78) had evidence of
    myocarditis
  • Of these, 10 were treated with immunosuppressive
    therapy
  • Nine of the 10 treated patients had subjective
    and objective improvement
  • Follow-up biopsies in these patients showed
    resolution or substantial improvement in
    myocarditis
  • Four patients with myocarditis not treated with
    immunosuppressives, also improved

6
Myocarditis
  • Peripartum cardiomyopathy clinical, hemo-
    dynamic, histologic and prognostic
    characteristics J Am Coll Cardiol 1986
    Jul8(1)52-6
  • 84 patients with different types of
    cardiomyopathy
  • 14 patients with peripartum cardiomyopathy
  • Myocarditis was identified on endomyocardial
    biopsies in 29 percent of the women with PPCM
    compared to 9 percent of the remaining patients
    with an idiopathic cardiomyopathy

7
Myocarditis
  • Incidence of myocarditis in peripartum
    cardiomyopathy.
  • Am J Cardiol 1994 Sep 174(5)474-7
  • Retrospective review of endomyocardial biopsy
    specimens from 34 patients fulfilling the
    criteria for a diagnosis of PPCM
  • Incidence of myocarditis was 8.8 (3 of 34)
  • Comparable to that found in an age- and
    sex-matched control population undergoing
    transplantation for IDC (9.1)

8
ETIOLOGY
  • Maternal immunologic response to a fetal antigen
    has been proposed as potential etiology of PPCM
  • A study of humoral immunity in 39 Nigerian women
    with PPCM revealed no differences between
    subjects and controls
  • Familiar clustering of PPCM has been observed
    indicating a genetic etiology

9
Risk factors
  • Age greater than 30 years
  • Multiparity
  • Women of African descent
  • Pregnancy with multiple fetuses
  • A history of preeclampsia, eclampsia, or
    postpartum hypertension
  • Maternal cocaine abuse or selenium deficiency
  • Long term (gt4 weeks) oral tocolytic therapy with
    beta adrenergic agonists

10
CLINICAL PRESENTATION
  • PPCM is rarely seen before 36 weeks of gestation
  • Affected patients usually present during the
    first four to five months postpartum
  • Signs and symptoms similar to patients with
    systolic LV dysfunction

11
DIAGNOSIS
  • The development of heart failure in the last
    month of pregnancy or within five months after
    delivery
  • Absence of a determinable cause for the heart
    failure
  • Absence of demonstrable heart disease before the
    last month of pregnancy
  • Presence of echocardiographically demonstrable
    decrease in left ventricular systolic function

12
DIAGNOSIS
  • ECG findings include
  • sinus tachycardia
  • nonspecific ST and T wave abnormalities
  • voltage abnormalities
  • Q waves are occasionally present in the anterior
    precordium
  • PR and QRS intervals may also be prolonged

13
Echocardiography
14
Endomyocardial biopsy
  • Role of endomyocardial biopsy remains unclear
  • Until the cause(s) of PPCM are firmly
    established, the collection of biopsy data should
    not be discouraged

15
DIAGNOSIS
  • Viral and bacterial cultures, as well as selected
    viral titers (eg, Coxsackie B) should also be
    considered

16
TREATMENT
  • Non-pharmacological
  • Salt restriction (4gm/d)
  • Water restriction (2 L/D)
  • Pharmacological
  • Pre-load reduction (diuretics, nitrates)
  • After-load reduction (hydralazine, nitrates,
    amlodipine)
  • ACE-I contraindicated during pregnancy
  • ionotropes (digoxin, dopamine, dobutamine)
  • Beta-blockers

17
TREATMENT
  • Anticoagulation
  • Patients with PPCM are highly predisposed to
    thromboembolic phenomena due to
  • hypercoagulable state of pregnancy
  • stasis of blood in the left ventricle
  • Patients with markedly dilated, very poorly
    contractile ventricles should generally be
    anticoagulated

18
TREATMENT
  • Immunosuppressive agents
  • May be initiated in patients with PPCM and
    biopsy-proven myocarditis, but efficacy is
    unclear
  • Empiric immunosuppression, in the absence of
    evidence of myocarditis, is not currently
    recommended

19
TREATMENT
  • Cardiac transplantation
  • Only acceptable treatment for women in whom more
    conventional therapy is not successful
  • Aggressive life support measures such as
    ventricular assist devices may be used as a
    bridge to transplantation

20
Long-term outcome after heart transplantation for
peripartum cardiomyopathy.Am Heart J 1994
May127(5)1318-23
  • Compared the courses of eight consecutive
    patients undergoing transplantation for PPCM with
    those of nine female age-matched control subjects
    undergoing transplantation for IDCM
  • Survival rates were 75 and 78 at 1 year and 60
    and 78 at 5 years in PPCM and IDCM patients,
    respectively
  • Among patients surviving gt6 months after
    transplantation, after a mean follow-up period of
    4.5y for those with PPCM and 7.8y for those with
    IDCM, 83 and 100, respectively, were
    rehabilitated

21
PROGNOSIS
  • Peripartum cardiomyopathy prognostic variables
    at initial evaluation
    Int J Cardiol 1991
    Sep32(3)377-80
  • At initial evaluation, 16 patients were in NYHA
    Class IV and 4 in Class III
  • During mean follow up of 6 months
  • 12 patients improved to Class I
  • 7 patients either failed to improve or
    deteriorated
  • one patient died

22
  • The patients who deteriorated, as compared to
    those who improved, were
  • Older (30 vs 24 years)
  • Of higher parity (3 pregnancies vs 1.5
    pregnancies)
  • Had later onset of symptoms after delivery (7.6
    vs 3 weeks)
  • Had higher echocardiographic left ventricular end
    diastolic dimensions (7.0 cm vs 3.0 cm)
  • Had higher mean pulmonary arterial (38 vs 28
    mmHg) and pulmonary wedge pressures (24 vs 20
    mmHg)
  • Conduction defects were present on the surface
    electrocardiogram in all the patients who
    deteriorated, as compared to 4 patients who
    improved

23
Future pregnancy
  • Patients with PPCM and persistent left
    ventricular dysfunction are at extremely high
    risk of complications and death with subsequent
    pregnancies
  • These patients should be advised to avoid
    pregnancy unless, successful cardiac
    transplantation is performed

24
Future pregnancy
  • In patients who recover ventricular function the
    true risk of pregnancy remains uncertain
  • These women should be counseled about the
    potential risks prior to pregnancy
  • They may suffer a deterioration in cardiac
    function when subjected to the hemodynamic
    stresses associated with pregnancy
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