March 19, 2025 • 25 mins
Join our expert as they delve into the critical aspects of recognizing and diagnosing peripartum cardiomyopathy (PPCM), a serious heart condition affecting pregnant and postpartum women. This episode will cover guideline-directed medical therapy tailored for PPCM during pregnancy and the postpartum period. Additionally, postpartum care strategies will be explored along with a patient's personal journey to provide a comprehensive understanding of the condition.
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Episode Transcript
Available transcripts are automatically generated. Complete accuracy is not guaranteed.
(00:15):
Hi. Thanks for
tuning in to this episodeentitled Peripartum Cardiomyopathy:
Recognition, Management,and Postpartum Care.
I'm Doctor Michael Honigberg.
I'm a cardiologist at MassachusettsGeneral Hospital in Boston.
Hi, my name is Renee Quinn,a patient of Doctor Honigberg’s.
The recommendations and opinions presentedmay not represent the official position
of the American Heart Association.
(00:36):
The materials are for educationalpurposes only
and do not constitute an endorsementor instruction by AHA.
The AHA does not endorse any productsor devices.
My disclosures are noted on this slide.
ReneeQuinn has no disclosures at this time.
In this episode,we will cover the evaluation, diagnosis,
and medical management of PeripartumCardiomyopathy and its complications.
(01:00):
You will also hear a first hand accountfrom a patient of mine
as she shares her story.
To bring more awareness to this rarebut serious disease
of Peripartum Cardiomyopathy.
After today'sepisode, you should feel comfortable
recognizing and diagnosing PeripartumCardiomyopathy and its complications.
Adapting guideline directedmedical therapy for cardiomyopathy
(01:22):
to pregnancy in the postpartum setting,and providing counseling regarding
appropriate contraception in individualswith Peripartum Cardiomyopathy.
All right.
Well Renee,thank you so much for joining us today.
I was wondering
if you could sort of tell our listenersa little bit about yourself.
Sure. Thank you, Doctor Honigberg.
I'm excitedto be here to have this conversation.
(01:43):
My name is Renee Quinn,and I became pregnant
with my twins back in 2019.
Having, you know, met my husband up here.
We moved to Boston,and we try to keep, life
sane as much as possible around here.
I can't believe it's been five years.
Maybe you could take our listeners backto, you know, when we first met
(02:05):
and maybe describethe really surprising series of events.
The pregnancy was fantastic,but I noticed, like,
towards the end of my pregnancy,that my heart rate was elevated
and like much more than usual, but nothingthat was like slowing me down in my day
to day.
Fast forwardthen, you know, through 38.5 weeks,
I delivered the twins, via C-section.
(02:27):
And then right after that, it probably,I don't know, maybe six,
eight hoursafter I was having difficulty breathing,
I would just lay down like,you know, in my bed with the twins,
one of them,or I would be doing something.
And I just noticedit was really hard to breathe.
And I thought that, you know, I'd neverhad a C-section before, so I didn't know
(02:48):
if this was a traditional, like C-sectionsymptom, you know, post having the twins.
And so I finally told my nurse after maybea day, they looked at my blood pressure.
Everything was great.
They were looking at my heart ratelike things looked normal.
But, I wasn't feeling normal.
I had a horrible night's sleep at home,I didn't sleep, and I couldn't walk
from, like, you know, here to, like, 6or 7ft away without getting out of breath.
(03:11):
So I just mentioned to my pediatricianwhen I was at their one week appointment,
I said, I think I'm,
I don't know, like I'm having difficultybreathing is is normal.
And she's like, no, I would call your O.B.and just like mention that to her.
So I did.
We instantlydid an EKG, and the EKG came back
with some alarming signals thatI didn't know what they were at the time.
But she said, listen,I think you need to go to the E.R.
and then that's when, you know,everything started to unfold.
(03:35):
I don't know how much moreyou want me to go on from there,
but that's, you know, reallywhat is unfolding.
And I think it's really striking, like youstarted to experience this immediately.
You were in a hospital, and I think,you know, still not recognized.
Sort of in a hospital is just sort of,I think a striking aspect of the story.
Do you remember what happened nextafter you went back
to the emergency room and they startedrunning more and more diagnostic tests?
(03:58):
I seemed healthy,and all my things were looking,
like somewhat healthier,other than how I was feeling.
But, so then I get to the E.R.,and I had a ton of fluid.
They did another EKGjust to do, like comparing
to, like, that quoteunquote baseline from the other day.
And they instantly just
recognized the leftbundle branch blockage.
(04:19):
And then they noticed that my ejectionfraction was below average
from where it should be.
We ended upputting you on metoprolol and Enalapril.
Yeah.
Well, because I was breastfeedingand like there was just like
a number of thingslike we had to deal with at that point.
And as soon as they recognizedand they came in and said,
we think this is a PeripartumCardiomyopathy case.
And they described to me what it was,and I was in the E.R.
(04:41):
for two, almost three days.
They were really monitoring,monitoring my like every movement,
like when I would go upto go to the bathroom or,
you know, get something to eat,
they would make sure someone was in therebecause my heart rate would spike really
high, like it would get into the 120, 130,like even just walking
across the room to the bathroom.
And so as a fluid started to come outas the medicine,
(05:03):
like we started to work that in thingsstarted to show like some improvements.
My ejectionfraction was still what it was.
And then at that point I was discharged.
But then saw you like immediatelyafter that.
What did it feel like going home?
I guess feeling better in some waysafter diarrhea, a whole bunch of fluid,
but sort of receivingthis really surprising diagnosis
(05:24):
with infant newborn twins at home.
What was that early post-discharge periodlike for you?
I'd always been like a very healthyperson, just generally like on paper.
And then I have done a number of marathonsand was physically active.
And so I think it was just the first timein my life where
I was diagnosed with somethingvery serious on top of like
just giving birth to twins and having to,you know, take care of them.
(05:48):
And it was a really difficult time.
And it was really just this ejectionfraction piece in that love
bundle branch box that we didn't knowif I had that forever or it
because that was my first EKG,or if it came about
because of the Peripartum Cardiomyopathy.
When January came, you know, we didour checkup like things were still like on
the up and up.
We're all doing really great.
(06:09):
But it was a very challenging,like four months
after that, just to get all of uslike into a normal rhythm.
It's unusualthat we get such an early sneak peak at,
at the heart's functionafter a diagnosis.
You know, we got that sort of early sneakpeak for you
at like about two weeksafter we first met, or even less.
(06:30):
And already your heart had responded
to the medications,which was really wonderful.
Tell us a little bit.
You were very active before all this,and you became very active after this.
What was the Rhodes Marathon like foryou after Peripartum Cardiomyopathy?
Yeah.
So, I just remember, again, I'm tryingnot to get emotional about it,
but I just remember, like, runningwas like a form of, like, wellness for me.
(06:53):
Like just physical wellness,but also the mental wellness.
I, was so nervous
because I lay there, I was like,I don't even care if I can't run again.
I just want to be able to, like, walk.
And, I just kept sayingthat over and over and believe it or not,
you know, like week by week, monthby month, I think I started walking more
like in that November
December timeframe, just like getting outand doing like one mile walks and,
(07:14):
and then, you know,
obviously consulted with youand was able to start running again.
Maybe it was like a year and a half.
I completed another marathon and,have since done three more
since you and I have connected.
And you're sort of like healthy,low resting heart rate and, you know,
untreated blood pressure.
Actually, we're a little bit of a barrier
to your PeripartumCardiomyopathy treatment.
And you were very little medication.
(07:36):
And then we sort of decided togetherto just stop everything and monitor.
Yeah.
And I will add to thatsomething like some great advice
you gave me that I use tothis day is really like basing off of your
that's the one thing for womenin particular, is just to be very cautious
of like how you're feeling.
And I think that's like the messagethat we all share.
You know, it's just like making sureyou're communicating.
I just always go back to what you said.
(07:57):
It's like, how are you feeling?
And, if I'm feeling greatand I can breathe and I'm running
and everything, then that was like,that's reassurance for me.
Yeah.
Listening to your body, what messagewould you want to convey to either
health care providers or other folks,learning about Peripartum Cardiomyopathy?
Call your doctor if you ever haveany questions or concerns, like if you're
(08:20):
even if it takes upany room in your mind like that
higher heart rate that I had like beforeI had the twins like
it was like a little bit of a question,but not really because I was feeling fine.
Just call your doctor.
And then in terms of like the recoveryprocess, I will say, because Peripartum is
so intertwined with like postpartum, justgeneral hormones and feelings in general,
(08:41):
is that period of timeas your recovery is just very dynamic.
And again, like to make sure you take caregood care of yourself and to rest.
Sleepwas really important to me, to recover.
And so I made sure that I was gettingeight hours of sleep a day.
However, I had to manage that.
And so and it was really greatto have the support system
around me to be able to do that,because I truly believe,
(09:03):
like based on my prior fitnessand then, you know, the rest in recovery,
I was able to get in that postpartumphase really helped me
recover a lot sooner than, say,maybe if I didn't have that opportunity.
So those are the things I would share.
And to obviously for anyone watching thisthat is not pregnant,
but thinking about getting pregnant,I do think like physical fitness
(09:23):
and like your heart strength
is as obviously we all knowthat's really important in general.
But to be able to make sure that,you know, you're healthy.
Those are all such goodand important messages.
Renee,thank you so much for sharing your story.
And for your time. I really appreciate it.
See your next follow up.
Now, let's take a deeper diveinto Peripartum Cardiomyopathy.
(09:44):
We'll start with a patient case.
This is a 32 year old
woman who's healthy, who's pregnantfor the first time with twins.
She's been on low dose aspirinstarting at 12 weeks gestation
for the prevention of pre-eclampsiain the setting of twin pregnancy.
And her pregnancy has been uncomplicated,including no
hypertensive disorders of pregnancyor gestational diabetes.
(10:06):
She is admitted for induction of laborat 38 weeks.
Induction is unsuccessful in her labor.
Labor fails to progress,and so she undergoes cesarean section
and delivers healthy twin infants.
On postpartum day two,
she develops orthopnea and worseninglower extremity edema.
(10:26):
She's discharged homewhere she experiences
progressive dyspnea on exertionand supine cough, interfering with sleep.
On postpartum day six,she represented to her local hospital.
She denied chest pain or pressureat any points
upon evaluation at her local hospital,
her NT-proBNP was elevated at 2400.
(10:50):
Her high sensitivity troponin T
was mildly elevated but dynamic,
and she underwent a chest X-raywhich showed bilateral pulmonary edema.
This is her EKG.
She had notpreviously had an EKG for comparison,
but it shows, as you can see, sinus rhythmwith the left bundle branch block.
(11:10):
What is your next diagnostic step?
In this case,
if you select a transthoracicechocardiogram you are correct.
And this is this patient'stransthoracic echocardiogram, which shows
mildly reduced the left ventricularejection fraction at approximately 45%.
You can also see an abnormal patternof septal activation
(11:31):
in the setting of her left bundlebranch block.
So this is Peripartum Cardiomyopathy.
How do we define PeripartumCardiomyopathy?
PPCM is defined as new onsetcardiomyopathy, presenting toward
the end of pregnancy, or more commonly,in the early postpartum period.
It's defined typically as it leftventricular ejection fraction less than
(11:51):
45%. And in the absence of any preexistingstructural heart disease
or an alternative diagnosis, such as,for example, coronary artery disease.
The global
prevalence of PPCM varies widely acrosscountries, with the highest prevalence
reported in Nigeriaand nearly 1 in 100 live births
(12:12):
in Haiti,at approximately 1 in 300 births.
On the other end of the spectrum,prevalence is closer to 1
in 10,000 in Denmarkand 1 in 20,000 in Japan and in the US,
prevalence is reportedto be 1 in 1000 births.
There are several reported
risk factors for Peripartum Cardiomyopathyin the literature, and key
(12:34):
risk factors include older maternal age, black maternal race,
and preexisting hypertensionor other cardiometabolic conditions.
In addition, multiple gestation,which is to say twin
pregnancy, is also a strong riskfactor for PPCM
And the more risk factorsyou have, the higher your risk of PPCM.
(12:55):
So the pathophysiology of Peripartum
Cardiomyopathy is interestingand remains only partially understood.
This plot here, the bar graphs show
the frequency of incidence of PPCM
versus weeks of gestationor weeks postpartum.
And so you can see from the solidblue bar graphs that the peak incidence
(13:17):
of PPCM occursin the first 1 to 4 weeks postpartum.
And within that 1 to 4 week time window,the first postpartum week
is the single most, most common timing of PPCM onset.
This stands in contrast to changesin maternal hemodynamics across pregnancy,
which is to say, we don't believethat the hemodynamic changes in pregnancy
(13:41):
are directly related to the conditionor the development of PPCM.
We see in the black linethat cardiac output increases
progressively across gestation,beginning in the first trimester,
and largely levels offand sort of the late second trimester.
In the third trimester, by contrast,
other changes in pregnancy,such as circulating
(14:03):
levels of placental hormones,peak much closer to the time of delivery
and closer to the time of PeripartumCardiomyopathy onset.
And what's shown in red here is, you know,the trend in one of those placental
derived proteins as folate one or solubleFMS like tyrosine kinase receptor one,
which is a keyantiangiogenic biomarker of pre-eclampsia
(14:23):
that we also think has a rolein Peripartum Cardiomyopathy onset.
In fact, to that point, pre-eclampsia is
strongly associatedwith Peripartum Cardiomyopathy.
You can see in the, the red and greenpie charts in the middle.
On top is the proportion of all deliveriesat one tertiary care center
affected by pre-eclampsia. In red.
(14:43):
And on the bottom are the proportion
of Peripartum Cardiomyopathy casesthat also were affected by pre-eclampsia.
And you can see, in short,pre-eclampsia is much more common
among Peripartum Cardiomyopathy casesthan the general postpartum population.
And that, split molecule
that we know is a key pre-eclampsiabiomarker.
Even in women without PPCM, there's athere's a strong association
(15:07):
between circulating split levelsand subclinical measures of left
ventricular systolic dysfunction,such as global longitudinal strain.
So folate one and other placentalbiomarkers are not the full story.
So what else is going on in PPCM.
pathophysiology.
Well we've come to appreciate thatthere is a strong role of preexisting
(15:29):
maternal predisposition to cardiomyopathy,including, you know, other gene,
a genetic predispositionto dilated cardiomyopathy in general.
So this plot here is showing the relative
frequency of different,rare genetic variants in key
cardiomyopathy genesbetween dilated cardiomyopathy shown
(15:50):
on the horizontal axis and PeripartumCardiomyopathy, seen on the vertical axis.
And you can see that there's a prettytight correlation in the frequency
of these deleterious variantsbetween the two conditions,
which another way of sayingmight be Peripartum Cardiomyopathy
looks a lot like dilated cardiomyopathy,but unmasked in the setting of pregnancy.
So toput it all together, the current proposed
(16:12):
PPCM pathophysiologyis something of a two hit model
where underlying maternal susceptibilityto cardiomyopathy intersects
with the anti vascular effectsof pregnancy hormones to yield
the diagnosis of PeripartumCardiomyopathy.
So Peripartum Cardiomyopathymost commonly present is decompensated
heart failure with volume overload againtoward the end of pregnancy,
(16:34):
or most commonlyin the early postpartum period.
Some other, much less common presentationsinclude cardiogenic shock, unstable
arrhythmias, and arterial thromboembolismfrom left ventricular thrombus.
Echocardiogramis the diagnostic modality of choice
to make the diagnosis of PeripartumCardiomyopathy.
Cardiac CT may be usefulto rule out other conditions
(16:56):
such as PeripartumSpontaneous Coronary Artery Dissection
data are somewhat inconsistenton cardiac MRI
findings and Peripartum Cardiomyopathy,but MRI might be useful
if echocardiographyis technically limited.
Remember that gadoliniumis not used in the context of pregnancy.
Potential complications of
PPCM include cardiogenic shock,arrhythmia, including ventricular
(17:20):
arrhythmias, and arterialor venous thromboembolism.
How do we knowwho's most likely to experience
adverse outcomes after PeripartumCardiomyopathy versus recover?
Well, one very strong risk
factor is the left ventricular ejectionfraction at presentation.
And here I'm showing data from the NorthAmerican iPAC registry of PPCM.
(17:42):
And in the iPAC registry.
The vast majority of adverse eventsduring follow up occurred in women
whose left ventricular ejection fractionwas less than 30% at presentation,
and those who had a EF greater than 30%had a very high rate
of recovering to a normal rangeF at follow up, as shown on the right.
(18:02):
With 86% of women in this group achieving
an F greaterthan or equal to 50% during follow up,
it's worth emphasizing
some important racial disparitiesand Peripartum Cardiomyopathy as well.
These are datafrom the University of Pennsylvania
and a cohort of PPCM cases followed there.
And in short, women with black racecompared to other women
(18:25):
presented later at the time of PeripartumCardiomyopathy diagnosis,
they were about half as likelyto recover to a normal range
ejection fraction,and when they did, they did so much later
took more than twice as long to recoverto a normal range ejection fraction.
We don't currently knowwhether this is due to, you know,
social factors driving these disparitiesand outcomes or whether there are sort
(18:49):
of inherent genetic differences between, black individuals and other individuals.
And this is an importanttopic for future research.
So what medications are appropriatefor our patient?
Well, loop diuretics are important fordecongestion and removing excess volume.
And then beyond, volume managementadapting guideline
(19:09):
directed medical therapy for heifer offto pregnant or postpartum patients.
So medication classes like beta blockers
are compatiblewith both pregnancy and breastfeeding.
We don't use Ace inhibitors, angiotensinreceptor blockers, or sacubitril valsartan
during pregnancy.
But Enalapril and several otherAce inhibitors are compatible
with breastfeeding.
(19:30):
Mineralocorticoid receptor antagonistslike spironolactone should not be used
in pregnancy, but spironolactoneis compatible with breastfeeding
owing to both animal datashowing some concerning signals.
A lack of human data.
We don't use SGLT2 inhibitorsduring pregnancy or breastfeeding.
Importantly, hydralazine and nitratesare compatible with both pregnancy
and breastfeeding,so multiple medication options available.
(19:55):
What about bromocriptine.
So bromocriptine is a dopamine agonist.
There is one very small 20 personrandomized trial conducted in South Africa
that suggested potential benefits
of bromocriptine in patientswith Peripartum Cardiomyopathy.
And there'ssome additional supportive data
from an observational German registry.
However, there's a little bitof a difference of opinion between North
(20:16):
America and Europe with respectto the role of remote Krypton.
Most participants in the iPAC registryrecovered
to normal range ejection fractionwithout using bromocriptine.
bromocriptine is associatedwith thrombotic complications.
Bromocriptineis incompatible with breastfeeding.
And we know breastfeeding hasother benefits to both infant and mother.
So there is current wide practicevariation around the use of bromocriptine.
(20:39):
Although it's generally not used in NorthAmerica, it's reasonable to consider
in patients who present with very severe
cardiomyopathy, with severely reducedejection fraction or cardiogenic shock.
There is an ongoing NIH supportedrandomized trial called Rebirth,
which is testing bromocriptineversus placebo in patients with Peripartum
Cardiomyopathy,And we await those results.
(20:59):
Other aspectsof Peripartum Cardiomyopathy management
anticoagulation should be consideredin women presenting with very low ejection
fraction, as recommended by boththe European Society of Cardiology
and the AHA noting that low
molecular weight heparin is compatiblewith both pregnancy and breastfeeding,
and then in other forms of cardiomyopathy,we consider implanted defibrillators
(21:22):
for sudden arrhythmic death preventionwhen the ejection fraction is very low.
Because we because many women
with PPCM will recoverover several months,
it may be premature to implant, a defibrillator in this population.
The ESC and AHA both advise considerationof a wearable cardioverter defibrillator.
If ejection fractions below 35%as a bridge to left ventricular recovery,
(21:47):
or if systolic function remains very poor
to ICD after a period of 3 to 6 monthsof guideline directed medical therapy.
How should this
patient be counseledregarding repeat pregnancy?
In general, repeat pregnancyafter PPCM is discouraged,
especially if the LV EF is persistently,less than 50% as these
(22:10):
patients are at high risk for adverseoutcomes with subsequent pregnancy.
In women who recover their ejectionfraction to normal range,
there's still higher risk than averagein this population
with 20% risk, of deteriorationin a subsequent pregnancy
and many patientswho recover their ejection fraction.
Shared decision making is importantas additional pregnancy is very important
(22:31):
to our patients.
In a portion of cases and we want tosupport them in their reproductive goals.
To that end, when our patients electto proceed to proceed with repeat
pregnancy, frequent clinicaland echocardiographic, monitoring
is generally advised, as depictedin the in the panel at the bottom.
(22:52):
Unintended pregnancy is very importantto prevent
in this population for the reasonsmentioned on the previous slide.
And so it's importantthat we have a discussion
with our patients about contraceptionand after a diagnosis of PPCM,
fortunately there are many contraceptive options available to to our
to patients with PPCM,with the CDC medical eligibility criteria
(23:13):
for contraceptive use indicatingthat benefits outweigh risks for,
options like copperand hormonal intrauterine devices or IUDs,
progestin secreting implants,Depot progestin, and progestin
only oral contraceptive pills combinesestrogen and progestin.
Oral contraceptives are not recommendedin PPCM patients, primarily due to issues
(23:36):
of volume retention and potentialincreased risk of arrhythmia.
In addition, barriermethods such as condoms
can be very important,but when they're used on their own, have
an unacceptably high rate of failureand so may result in unintended pregnancy.
And so it's it'sgenerally advised that other
more effective methodsare used in addition to barrier methods.
(23:58):
So coming
back to our patient, fortunatelythis patient had very good outcome.
Her ejection fraction recovered to 55%at three months postpartum.
She was treated with both metoprololsuccinate and enalapril
at low doses due to low baseline bloodpressure, her enalapril was discontinued
after three months when her echocardiogramshowed improvements.
(24:20):
Her metoprolol was later discontinuedafter shared decision making
and on serial monitoring,her ejection fraction remained stable,
although with a persistent leftbundle branch block.
She sees me in follow up once per yearwith the 12 lead EKG at each visit.
So to summarize today's episode,Peripartum Cardiomyopathy is an idiopathic
cardiomyopathy occurring in late pregnancyor the early postpartum period.
(24:45):
Risk factors include,
increasing maternal age and preexistingcardiometabolic risk factors,
black material race, pre-eclampsia,and multiple gestation.
Echocardiography is generallythe diagnostic modality of choice
in most patients.
Cardiomyopathy therapy canand should be adapted to pregnancy
and breastfeeding, and effectivecontraception is crucial after PPCM.
(25:06):
For more information,please visit the AHA’s web page
on the role of cardiovascular healthin maternal health.
Thanks very much for your attention.
Hi. Thanks for
tuning in to this episodeentitled Peripartum Cardiomyopathy:
Recognition, Management,and Postpartum Care.
I'm Doctor Michael Honigberg.
I'm a cardiologist at MassachusettsGeneral Hospital in Boston.
Hi, my name is Renee Quinn,a patient of Doctor Honigberg’s.
The recommendations and opinions presentedmay not represent the official position
of the American Heart Association.
(00:36):
The materials are for educationalpurposes only
and do not constitute an endorsementor instruction by AHA.
The AHA does not endorse any productsor devices.
My disclosures are noted on this slide.
ReneeQuinn has no disclosures at this time.
In this episode,we will cover the evaluation, diagnosis,
and medical management of PeripartumCardiomyopathy and its complications.
(01:00):
You will also hear a first hand accountfrom a patient of mine
as she shares her story.
To bring more awareness to this rarebut serious disease
of Peripartum Cardiomyopathy.
After today'sepisode, you should feel comfortable
recognizing and diagnosing PeripartumCardiomyopathy and its complications.
Adapting guideline directedmedical therapy for cardiomyopathy
(01:22):
to pregnancy in the postpartum setting,and providing counseling regarding
appropriate contraception in individualswith Peripartum Cardiomyopathy.
All right.
Well Renee,thank you so much for joining us today.
I was wondering
if you could sort of tell our listenersa little bit about yourself.
Sure. Thank you, Doctor Honigberg.
I'm excitedto be here to have this conversation.
(01:43):
My name is Renee Quinn,and I became pregnant
with my twins back in 2019.
Having, you know, met my husband up here.
We moved to Boston,and we try to keep, life
sane as much as possible around here.
I can't believe it's been five years.
Maybe you could take our listeners backto, you know, when we first met
(02:05):
and maybe describethe really surprising series of events.
The pregnancy was fantastic,but I noticed, like,
towards the end of my pregnancy,that my heart rate was elevated
and like much more than usual, but nothingthat was like slowing me down in my day
to day.
Fast forwardthen, you know, through 38.5 weeks,
I delivered the twins, via C-section.
(02:27):
And then right after that, it probably,I don't know, maybe six,
eight hoursafter I was having difficulty breathing,
I would just lay down like,you know, in my bed with the twins,
one of them,or I would be doing something.
And I just noticedit was really hard to breathe.
And I thought that, you know, I'd neverhad a C-section before, so I didn't know
(02:48):
if this was a traditional, like C-sectionsymptom, you know, post having the twins.
And so I finally told my nurse after maybea day, they looked at my blood pressure.
Everything was great.
They were looking at my heart ratelike things looked normal.
But, I wasn't feeling normal.
I had a horrible night's sleep at home,I didn't sleep, and I couldn't walk
from, like, you know, here to, like, 6or 7ft away without getting out of breath.
(03:11):
So I just mentioned to my pediatricianwhen I was at their one week appointment,
I said, I think I'm,
I don't know, like I'm having difficultybreathing is is normal.
And she's like, no, I would call your O.B.and just like mention that to her.
So I did.
We instantlydid an EKG, and the EKG came back
with some alarming signals thatI didn't know what they were at the time.
But she said, listen,I think you need to go to the E.R.
and then that's when, you know,everything started to unfold.
(03:35):
I don't know how much moreyou want me to go on from there,
but that's, you know, reallywhat is unfolding.
And I think it's really striking, like youstarted to experience this immediately.
You were in a hospital, and I think,you know, still not recognized.
Sort of in a hospital is just sort of,I think a striking aspect of the story.
Do you remember what happened nextafter you went back
to the emergency room and they startedrunning more and more diagnostic tests?
(03:58):
I seemed healthy,and all my things were looking,
like somewhat healthier,other than how I was feeling.
But, so then I get to the E.R.,and I had a ton of fluid.
They did another EKGjust to do, like comparing
to, like, that quoteunquote baseline from the other day.
And they instantly just
recognized the leftbundle branch blockage.
(04:19):
And then they noticed that my ejectionfraction was below average
from where it should be.
We ended upputting you on metoprolol and Enalapril.
Yeah.
Well, because I was breastfeedingand like there was just like
a number of thingslike we had to deal with at that point.
And as soon as they recognizedand they came in and said,
we think this is a PeripartumCardiomyopathy case.
And they described to me what it was,and I was in the E.R.
(04:41):
for two, almost three days.
They were really monitoring,monitoring my like every movement,
like when I would go upto go to the bathroom or,
you know, get something to eat,
they would make sure someone was in therebecause my heart rate would spike really
high, like it would get into the 120, 130,like even just walking
across the room to the bathroom.
And so as a fluid started to come outas the medicine,
(05:03):
like we started to work that in thingsstarted to show like some improvements.
My ejectionfraction was still what it was.
And then at that point I was discharged.
But then saw you like immediatelyafter that.
What did it feel like going home?
I guess feeling better in some waysafter diarrhea, a whole bunch of fluid,
but sort of receivingthis really surprising diagnosis
(05:24):
with infant newborn twins at home.
What was that early post-discharge periodlike for you?
I'd always been like a very healthyperson, just generally like on paper.
And then I have done a number of marathonsand was physically active.
And so I think it was just the first timein my life where
I was diagnosed with somethingvery serious on top of like
just giving birth to twins and having to,you know, take care of them.
(05:48):
And it was a really difficult time.
And it was really just this ejectionfraction piece in that love
bundle branch box that we didn't knowif I had that forever or it
because that was my first EKG,or if it came about
because of the Peripartum Cardiomyopathy.
When January came, you know, we didour checkup like things were still like on
the up and up.
We're all doing really great.
(06:09):
But it was a very challenging,like four months
after that, just to get all of uslike into a normal rhythm.
It's unusualthat we get such an early sneak peak at,
at the heart's functionafter a diagnosis.
You know, we got that sort of early sneakpeak for you
at like about two weeksafter we first met, or even less.
(06:30):
And already your heart had responded
to the medications,which was really wonderful.
Tell us a little bit.
You were very active before all this,and you became very active after this.
What was the Rhodes Marathon like foryou after Peripartum Cardiomyopathy?
Yeah.
So, I just remember, again, I'm tryingnot to get emotional about it,
but I just remember, like, runningwas like a form of, like, wellness for me.
(06:53):
Like just physical wellness,but also the mental wellness.
I, was so nervous
because I lay there, I was like,I don't even care if I can't run again.
I just want to be able to, like, walk.
And, I just kept sayingthat over and over and believe it or not,
you know, like week by week, monthby month, I think I started walking more
like in that November
December timeframe, just like getting outand doing like one mile walks and,
(07:14):
and then, you know,
obviously consulted with youand was able to start running again.
Maybe it was like a year and a half.
I completed another marathon and,have since done three more
since you and I have connected.
And you're sort of like healthy,low resting heart rate and, you know,
untreated blood pressure.
Actually, we're a little bit of a barrier
to your PeripartumCardiomyopathy treatment.
And you were very little medication.
(07:36):
And then we sort of decided togetherto just stop everything and monitor.
Yeah.
And I will add to thatsomething like some great advice
you gave me that I use tothis day is really like basing off of your
that's the one thing for womenin particular, is just to be very cautious
of like how you're feeling.
And I think that's like the messagethat we all share.
You know, it's just like making sureyou're communicating.
I just always go back to what you said.
(07:57):
It's like, how are you feeling?
And, if I'm feeling greatand I can breathe and I'm running
and everything, then that was like,that's reassurance for me.
Yeah.
Listening to your body, what messagewould you want to convey to either
health care providers or other folks,learning about Peripartum Cardiomyopathy?
Call your doctor if you ever haveany questions or concerns, like if you're
(08:20):
even if it takes upany room in your mind like that
higher heart rate that I had like beforeI had the twins like
it was like a little bit of a question,but not really because I was feeling fine.
Just call your doctor.
And then in terms of like the recoveryprocess, I will say, because Peripartum is
so intertwined with like postpartum, justgeneral hormones and feelings in general,
(08:41):
is that period of timeas your recovery is just very dynamic.
And again, like to make sure you take caregood care of yourself and to rest.
Sleepwas really important to me, to recover.
And so I made sure that I was gettingeight hours of sleep a day.
However, I had to manage that.
And so and it was really greatto have the support system
around me to be able to do that,because I truly believe,
(09:03):
like based on my prior fitnessand then, you know, the rest in recovery,
I was able to get in that postpartumphase really helped me
recover a lot sooner than, say,maybe if I didn't have that opportunity.
So those are the things I would share.
And to obviously for anyone watching thisthat is not pregnant,
but thinking about getting pregnant,I do think like physical fitness
(09:23):
and like your heart strength
is as obviously we all knowthat's really important in general.
But to be able to make sure that,you know, you're healthy.
Those are all such goodand important messages.
Renee,thank you so much for sharing your story.
And for your time. I really appreciate it.
See your next follow up.
Now, let's take a deeper diveinto Peripartum Cardiomyopathy.
(09:44):
We'll start with a patient case.
This is a 32 year old
woman who's healthy, who's pregnantfor the first time with twins.
She's been on low dose aspirinstarting at 12 weeks gestation
for the prevention of pre-eclampsiain the setting of twin pregnancy.
And her pregnancy has been uncomplicated,including no
hypertensive disorders of pregnancyor gestational diabetes.
(10:06):
She is admitted for induction of laborat 38 weeks.
Induction is unsuccessful in her labor.
Labor fails to progress,and so she undergoes cesarean section
and delivers healthy twin infants.
On postpartum day two,
she develops orthopnea and worseninglower extremity edema.
(10:26):
She's discharged homewhere she experiences
progressive dyspnea on exertionand supine cough, interfering with sleep.
On postpartum day six,she represented to her local hospital.
She denied chest pain or pressureat any points
upon evaluation at her local hospital,
her NT-proBNP was elevated at 2400.
(10:50):
Her high sensitivity troponin T
was mildly elevated but dynamic,
and she underwent a chest X-raywhich showed bilateral pulmonary edema.
This is her EKG.
She had notpreviously had an EKG for comparison,
but it shows, as you can see, sinus rhythmwith the left bundle branch block.
(11:10):
What is your next diagnostic step?
In this case,
if you select a transthoracicechocardiogram you are correct.
And this is this patient'stransthoracic echocardiogram, which shows
mildly reduced the left ventricularejection fraction at approximately 45%.
You can also see an abnormal patternof septal activation
(11:31):
in the setting of her left bundlebranch block.
So this is Peripartum Cardiomyopathy.
How do we define PeripartumCardiomyopathy?
PPCM is defined as new onsetcardiomyopathy, presenting toward
the end of pregnancy, or more commonly,in the early postpartum period.
It's defined typically as it leftventricular ejection fraction less than
(11:51):
45%. And in the absence of any preexistingstructural heart disease
or an alternative diagnosis, such as,for example, coronary artery disease.
The global
prevalence of PPCM varies widely acrosscountries, with the highest prevalence
reported in Nigeriaand nearly 1 in 100 live births
(12:12):
in Haiti,at approximately 1 in 300 births.
On the other end of the spectrum,prevalence is closer to 1
in 10,000 in Denmarkand 1 in 20,000 in Japan and in the US,
prevalence is reportedto be 1 in 1000 births.
There are several reported
risk factors for Peripartum Cardiomyopathyin the literature, and key
(12:34):
risk factors include older maternal age, black maternal race,
and preexisting hypertensionor other cardiometabolic conditions.
In addition, multiple gestation,which is to say twin
pregnancy, is also a strong riskfactor for PPCM
And the more risk factorsyou have, the higher your risk of PPCM.
(12:55):
So the pathophysiology of Peripartum
Cardiomyopathy is interestingand remains only partially understood.
This plot here, the bar graphs show
the frequency of incidence of PPCM
versus weeks of gestationor weeks postpartum.
And so you can see from the solidblue bar graphs that the peak incidence
(13:17):
of PPCM occursin the first 1 to 4 weeks postpartum.
And within that 1 to 4 week time window,the first postpartum week
is the single most, most common timing of PPCM onset.
This stands in contrast to changesin maternal hemodynamics across pregnancy,
which is to say, we don't believethat the hemodynamic changes in pregnancy
(13:41):
are directly related to the conditionor the development of PPCM.
We see in the black linethat cardiac output increases
progressively across gestation,beginning in the first trimester,
and largely levels offand sort of the late second trimester.
In the third trimester, by contrast,
other changes in pregnancy,such as circulating
(14:03):
levels of placental hormones,peak much closer to the time of delivery
and closer to the time of PeripartumCardiomyopathy onset.
And what's shown in red here is, you know,the trend in one of those placental
derived proteins as folate one or solubleFMS like tyrosine kinase receptor one,
which is a keyantiangiogenic biomarker of pre-eclampsia
(14:23):
that we also think has a rolein Peripartum Cardiomyopathy onset.
In fact, to that point, pre-eclampsia is
strongly associatedwith Peripartum Cardiomyopathy.
You can see in the, the red and greenpie charts in the middle.
On top is the proportion of all deliveriesat one tertiary care center
affected by pre-eclampsia. In red.
(14:43):
And on the bottom are the proportion
of Peripartum Cardiomyopathy casesthat also were affected by pre-eclampsia.
And you can see, in short,pre-eclampsia is much more common
among Peripartum Cardiomyopathy casesthan the general postpartum population.
And that, split molecule
that we know is a key pre-eclampsiabiomarker.
Even in women without PPCM, there's athere's a strong association
(15:07):
between circulating split levelsand subclinical measures of left
ventricular systolic dysfunction,such as global longitudinal strain.
So folate one and other placentalbiomarkers are not the full story.
So what else is going on in PPCM.
pathophysiology.
Well we've come to appreciate thatthere is a strong role of preexisting
(15:29):
maternal predisposition to cardiomyopathy,including, you know, other gene,
a genetic predispositionto dilated cardiomyopathy in general.
So this plot here is showing the relative
frequency of different,rare genetic variants in key
cardiomyopathy genesbetween dilated cardiomyopathy shown
(15:50):
on the horizontal axis and PeripartumCardiomyopathy, seen on the vertical axis.
And you can see that there's a prettytight correlation in the frequency
of these deleterious variantsbetween the two conditions,
which another way of sayingmight be Peripartum Cardiomyopathy
looks a lot like dilated cardiomyopathy,but unmasked in the setting of pregnancy.
So toput it all together, the current proposed
(16:12):
PPCM pathophysiologyis something of a two hit model
where underlying maternal susceptibilityto cardiomyopathy intersects
with the anti vascular effectsof pregnancy hormones to yield
the diagnosis of PeripartumCardiomyopathy.
So Peripartum Cardiomyopathymost commonly present is decompensated
heart failure with volume overload againtoward the end of pregnancy,
(16:34):
or most commonlyin the early postpartum period.
Some other, much less common presentationsinclude cardiogenic shock, unstable
arrhythmias, and arterial thromboembolismfrom left ventricular thrombus.
Echocardiogramis the diagnostic modality of choice
to make the diagnosis of PeripartumCardiomyopathy.
Cardiac CT may be usefulto rule out other conditions
(16:56):
such as PeripartumSpontaneous Coronary Artery Dissection
data are somewhat inconsistenton cardiac MRI
findings and Peripartum Cardiomyopathy,but MRI might be useful
if echocardiographyis technically limited.
Remember that gadoliniumis not used in the context of pregnancy.
Potential complications of
PPCM include cardiogenic shock,arrhythmia, including ventricular
(17:20):
arrhythmias, and arterialor venous thromboembolism.
How do we knowwho's most likely to experience
adverse outcomes after PeripartumCardiomyopathy versus recover?
Well, one very strong risk
factor is the left ventricular ejectionfraction at presentation.
And here I'm showing data from the NorthAmerican iPAC registry of PPCM.
(17:42):
And in the iPAC registry.
The vast majority of adverse eventsduring follow up occurred in women
whose left ventricular ejection fractionwas less than 30% at presentation,
and those who had a EF greater than 30%had a very high rate
of recovering to a normal rangeF at follow up, as shown on the right.
(18:02):
With 86% of women in this group achieving
an F greaterthan or equal to 50% during follow up,
it's worth emphasizing
some important racial disparitiesand Peripartum Cardiomyopathy as well.
These are datafrom the University of Pennsylvania
and a cohort of PPCM cases followed there.
And in short, women with black racecompared to other women
(18:25):
presented later at the time of PeripartumCardiomyopathy diagnosis,
they were about half as likelyto recover to a normal range
ejection fraction,and when they did, they did so much later
took more than twice as long to recoverto a normal range ejection fraction.
We don't currently knowwhether this is due to, you know,
social factors driving these disparitiesand outcomes or whether there are sort
(18:49):
of inherent genetic differences between, black individuals and other individuals.
And this is an importanttopic for future research.
So what medications are appropriatefor our patient?
Well, loop diuretics are important fordecongestion and removing excess volume.
And then beyond, volume managementadapting guideline
(19:09):
directed medical therapy for heifer offto pregnant or postpartum patients.
So medication classes like beta blockers
are compatiblewith both pregnancy and breastfeeding.
We don't use Ace inhibitors, angiotensinreceptor blockers, or sacubitril valsartan
during pregnancy.
But Enalapril and several otherAce inhibitors are compatible
with breastfeeding.
(19:30):
Mineralocorticoid receptor antagonistslike spironolactone should not be used
in pregnancy, but spironolactoneis compatible with breastfeeding
owing to both animal datashowing some concerning signals.
A lack of human data.
We don't use SGLT2 inhibitorsduring pregnancy or breastfeeding.
Importantly, hydralazine and nitratesare compatible with both pregnancy
and breastfeeding,so multiple medication options available.
(19:55):
What about bromocriptine.
So bromocriptine is a dopamine agonist.
There is one very small 20 personrandomized trial conducted in South Africa
that suggested potential benefits
of bromocriptine in patientswith Peripartum Cardiomyopathy.
And there'ssome additional supportive data
from an observational German registry.
However, there's a little bitof a difference of opinion between North
(20:16):
America and Europe with respectto the role of remote Krypton.
Most participants in the iPAC registryrecovered
to normal range ejection fractionwithout using bromocriptine.
bromocriptine is associatedwith thrombotic complications.
Bromocriptineis incompatible with breastfeeding.
And we know breastfeeding hasother benefits to both infant and mother.
So there is current wide practicevariation around the use of bromocriptine.
(20:39):
Although it's generally not used in NorthAmerica, it's reasonable to consider
in patients who present with very severe
cardiomyopathy, with severely reducedejection fraction or cardiogenic shock.
There is an ongoing NIH supportedrandomized trial called Rebirth,
which is testing bromocriptineversus placebo in patients with Peripartum
Cardiomyopathy,And we await those results.
(20:59):
Other aspectsof Peripartum Cardiomyopathy management
anticoagulation should be consideredin women presenting with very low ejection
fraction, as recommended by boththe European Society of Cardiology
and the AHA noting that low
molecular weight heparin is compatiblewith both pregnancy and breastfeeding,
and then in other forms of cardiomyopathy,we consider implanted defibrillators
(21:22):
for sudden arrhythmic death preventionwhen the ejection fraction is very low.
Because we because many women
with PPCM will recoverover several months,
it may be premature to implant, a defibrillator in this population.
The ESC and AHA both advise considerationof a wearable cardioverter defibrillator.
If ejection fractions below 35%as a bridge to left ventricular recovery,
(21:47):
or if systolic function remains very poor
to ICD after a period of 3 to 6 monthsof guideline directed medical therapy.
How should this
patient be counseledregarding repeat pregnancy?
In general, repeat pregnancyafter PPCM is discouraged,
especially if the LV EF is persistently,less than 50% as these
(22:10):
patients are at high risk for adverseoutcomes with subsequent pregnancy.
In women who recover their ejectionfraction to normal range,
there's still higher risk than averagein this population
with 20% risk, of deteriorationin a subsequent pregnancy
and many patientswho recover their ejection fraction.
Shared decision making is importantas additional pregnancy is very important
(22:31):
to our patients.
In a portion of cases and we want tosupport them in their reproductive goals.
To that end, when our patients electto proceed to proceed with repeat
pregnancy, frequent clinicaland echocardiographic, monitoring
is generally advised, as depictedin the in the panel at the bottom.
(22:52):
Unintended pregnancy is very importantto prevent
in this population for the reasonsmentioned on the previous slide.
And so it's importantthat we have a discussion
with our patients about contraceptionand after a diagnosis of PPCM,
fortunately there are many contraceptive options available to to our
to patients with PPCM,with the CDC medical eligibility criteria
(23:13):
for contraceptive use indicatingthat benefits outweigh risks for,
options like copperand hormonal intrauterine devices or IUDs,
progestin secreting implants,Depot progestin, and progestin
only oral contraceptive pills combinesestrogen and progestin.
Oral contraceptives are not recommendedin PPCM patients, primarily due to issues
(23:36):
of volume retention and potentialincreased risk of arrhythmia.
In addition, barriermethods such as condoms
can be very important,but when they're used on their own, have
an unacceptably high rate of failureand so may result in unintended pregnancy.
And so it's it'sgenerally advised that other
more effective methodsare used in addition to barrier methods.
(23:58):
So coming
back to our patient, fortunatelythis patient had very good outcome.
Her ejection fraction recovered to 55%at three months postpartum.
She was treated with both metoprololsuccinate and enalapril
at low doses due to low baseline bloodpressure, her enalapril was discontinued
after three months when her echocardiogramshowed improvements.
(24:20):
Her metoprolol was later discontinuedafter shared decision making
and on serial monitoring,her ejection fraction remained stable,
although with a persistent leftbundle branch block.
She sees me in follow up once per yearwith the 12 lead EKG at each visit.
So to summarize today's episode,Peripartum Cardiomyopathy is an idiopathic
cardiomyopathy occurring in late pregnancyor the early postpartum period.
(24:45):
Risk factors include,
increasing maternal age and preexistingcardiometabolic risk factors,
black material race, pre-eclampsia,and multiple gestation.
Echocardiography is generallythe diagnostic modality of choice
in most patients.
Cardiomyopathy therapy canand should be adapted to pregnancy
and breastfeeding, and effectivecontraception is crucial after PPCM.
(25:06):
For more information,please visit the AHA’s web page
on the role of cardiovascular healthin maternal health.
Thanks very much for your attention.
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