Question 15

Regarding peri-partum cardiomyopathy (PPCM):

a)    Define peri-partum cardiomyopathy.    (20% marks)

b)    List five differential diagnoses.    (20% marks)

c)    Outline the management.    (60% marks)
 

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College answer

Not available.

Discussion

a) Peripartum cardiomyopathy has some specific defining criteria:

  • Hear failure in the end of pregnancy, or in the following few months
  • No other explanation (diagnosis of exclusion)

This is from the ESC statement from 2010, and is probably enough for a sub-question worth only 20%  of the marks. If one wanted to write a little extra, a previous college answer holds clues to what the college examiners think is a good definition:

  • Onset of heart failure in the last month of pregnancy or within 5 months post-partum
  • Absence of an identifiable cause of heart failure
  • Absence of recognizable heart disease prior to the last month of pregnancy
  • LV systolic dysfunction demonstrated by classical echocardiographic criteria. The latter  may be characterized as an LV ejection fraction < 45%, fractional shortening < 30%, or both, with or without an LV end-diastolic dimension 2.7 cm/m2 body surface area.

This definition was generated by a panel of fourteen experts during a conference workshop (Pearson et al, 2000).

b) Five plausible differentials would include:

  • Massive PE
  • Amniotic fluid embolism
  • Worsening of pre-existing rheumatic MR or TR
  • Pre-eclampsia or eclampsia
  • Pre-existing unrecognized congenital heart disease, unmasked by the volume stress of pregnancy

c) Management is same as you would manage acute heart failure from any cause:

  • Management of preload
    • Diuretics, fluid restriction, venodilators
    • Maintenance of sinus rhythm and atrial systolic contribution
    • Pacing to maintain AV synchrony
  • Management of afterload
    • Left ventricle
      • Vasodilators
      • Beta-blockers
      • ACE-inhibitors are contraindicated in pregnancy
    • Right ventricle
      • Normoxia and normocapnea
      • Avoidance of excessive postive respiratory pressures
      • Pulmonary vasodilators
  • Management of contractility
    • Inotropes
      • Dobutamine
      • Milrinone
      • Levosimendan
    • Cardiac resychronisation
    • Supportive hormones and micronutrients (cortisol, insulin, calcium, glucagon, thyroxine, thiamine etc)
  • Cheating
    • Increase cardiac output by unnatural means:
      • IABP
      • LVAD
      • ECMO
      • Increase the pacemaker rate
  • Planning for delivery and the future:
    • Steroids to help foetal lung maturation, in case something happens
    • Instructions not to breastfeed (prolactin secretion can make PPCM worse)
    • Instructions to avoid getting pregnant again
    • Consider referral for transplant, if LV function fails to improve