Peripartum Cardiomyopathy

[Lucille Donnelly]

I was just diagnosed with Peripartum Cardiomyopathy after a recent pregnancy and I would like to know what is the cause of this condition, did it have anything to do with the excessive retension of fluid during pregnancy. What are the chances of this condition returning to normal. I was told that there was a 50% chance of this reurning to normal. If it doesn't return to normal, what are the consequences? My EF was 25% at tha time of diagnosis. One month later, after having another Echo, it was said to be 10% better. What amount of time is needed to fully recover from this? Is there a life expectency attached to Peripartum Cardiomyopathy? If so what is it?
Need some answers to this condition!

[Cleveland Clinic, MD]

Post-Partum (or peripartum) cardiomyopathy is a relatively rare form of heart failure that affects women during or following pregnancy.  Establishing a diagnosis requires 1. The absence of a determinable cause for cardiac failure, 2. Absence of preexisting heart muscle disease, and 3. Time limitations of onset of illness from the last month of pregnancy to the first 5 post-partum months.  Peripartum cardiomyopathy complicates 1 of 1300 to 4000 deliveries in the United States.  This condition may affect women of any race, age, or number of prior deliveries; however, older, multigravida, African American and twin pregnancies are thought to represent predisposing features.  The cause is unknown.  

The treatment is standard heart failure medication (diuretics, digoxin, ACE inhibitors) and in severe cases heart transplant.  Approximately half of patients with this diagnosis will return to normal within 6 months.  Of those who do not return to normal the prognosis is poor with an average survival of 4.7 years after diagnosis (without transplant).  Repeat pregnancies are not recommended for women who have had peripartum cardiomyopathy.  You can find additional information in the following articles.  (your local medical library should be able to help you find these).

Lampert, MB Lang RM. Peripartum cardiomyopathy. Am Heart J 1995; 130:860-870.

Huerta EM, Erice A, Espino RF, et al. Postpartum cardiomyopathy and acute myocarditis. Am Heart J 1985; 110:1079-1081.

[Maria]

Dear doctors,

Surely Lucille, who has already regained some pump function, must have quite a favourable prognosis?
Myself, I wrote to you a year ago, absolutely terrified by the same diagnosis, and I was told that the poor prognosis (average survival 4,7 years) only concerned women who, in spite of medication, had obvious symtoms of heart failure. I have also searched your archives, and several times I have read that of those who does not return to normal, approx. 50 % deteriorates, but 50 % remain stable with mild to moderate symptoms of heart failure!

Dear Lucille,

I wish all the best for you and your little baby, and I hope that you will be able to enjoy your child in spite of your illness. I have had the same diagnosis myself, and now, a year later, I am strong and healthy again. I'll pray that this will happen to you, too.

Love,
Maria

[Cleveland Clinic, MD]

All we can report is what happens to the entire group. AS you suggest for an individual patient it is 100% either way.  We always hope and pray for the best.

[Lucille]

RE; Comment by Maria,

Maria,
If possible, could you contact me in refernce to your comment on my posting. Also terrified by the diagnosis, would like to talk!
Please contact me at
***@****

[AL [e-mail]]

Ok, there is some good news here. I was diagnosed with peripartum cardiomyopathy 7, yes, 7 years ago. I am still here.
I was initially worked up for a transplant, luckily for me
Coreg came out. I take Coreg 25mg BID, Lanoxin 0.125 qd, Zestril 5 BID, and Bumex 2mg HS. I had an ejection fraction of less than
30 and I couldn't do anything. I was basically bedridden for about 2 years.
Things have improved. I don't want to bore you with details of all the tests, major heart centers I have been to, disasters that have resulted, but today, my EF is better, I go to cardiac
rehab 3X week and I have a life back.
I am by no means out of the woods here. I had to get a pacemaker in February, and I have had one problem after another, AND, now that I have found this site, I will be contacting Cleveland Clinic for a consult.
I am 45 years old, I have six kids. The cardiac rehab was, in my mind, the single most important thing, next to the drugs. The support, as well as the exercise and caring people in a similar boat, really keep me up, when its hard to see the forest from the trees.
BUT, as I said before, I AM STILL HERE!!!!
Don't listen to anyone that tells you 4.7 years-you can't worry about it- you could get hit by  a bus tomorrow. Keep the faith.!

[heather]

Dear Lucille,
        My mother is still fighting this illness after five years.  You both have my prayers.  You will live to see your sweet little baby grow up and have some babies.  Keep your head up and always look at the good.  keep a smile on your face!!

[Jessie]

I also was diagnosed with PPC when my first daughter was born.  Told by my cardiologist that I couldn't have more children without endangering my life (that was hard to accept) my OB/GYN told me that the condition wasn't life threatening and that he wanted me to lose weight before he gave me a tubal.  I was distraught.  At my next checkup, a year later, I was pregnant again.  I finally found a doctor who was willing to work with me.  It was a high risk pregnancy, but we both came through it fine, and I had my tubes tied minutes after she was born.  That was two years ago.  I still have a very hard time accepting that I will not have more children, as I was only 23 when my first child was born.  But it helps to have doctors who understand and are willing to work with you.  My condition is stable now, but I still have to be careful.  And with those two precious girls, you can bet I'm careful.  My doctor now wants to put me on the pill to regulate my period, but she's deferring that option to my cardiologist first.  A decision I can agree with.

PPC is a hard condition to accept and fight, because it is so rare.  My OB/GYN has been in practice for 20 years, and I was her first case!  Support groups will help with the emotional upheaval, and if you are lucky to have a supportive family (I am) your life can return to normal -- or close.  I still keep in mind everyday of my life that I have a heart condition and I still remember everyday that I can't have more children (that hurts so much!)but I do have everyday.  My aunt is on a waiting list for a transplant...the doctors think that she had PPC, but her children are 30 and 32 years old.  Who thought 32 years ago that women could have heart failure?  Or that it would be linked to pregnancy.  Fortunately for my husband and children, I am now relatively healthy, thanks to good doctors.

[JO.]

The exact same thing happened to me, gain of fluid during pregnancy and high blood pressure the last few days of pregnancy, CFH 10 days after delivery, and then I lost 50 pounds of fluid.  My EF was 25% 2 months later and now I am "virtually normal" according to my cardiologist.  

Now what I want know about is future pregnancy.  Is it life threatning?  What are the chances of having a normal pregnancy and delivery.  If this occurs again, what are my chances of a similar recovery.  One cardiologist mentioned if a woman has this and then waits 10 years the chances of recurrance is diminished.  Is this true?  I know that each case is different, but after going through several cardiologists in New Orleans, I cannot find anyone who is knowledgable on this topic.  Maybe you could recomend someone in the New Orleans area

[JO.]

The exact same thing happened to me, gain of fluid during pregnancy and high blood pressure the last few days of pregnancy, CFH 10 days after delivery, and then I lost 50 pounds of fluid.  My EF was 25% 2 months later and now I am "virtually normal" according to my cardiologist.  

Now what I want know about is future pregnancy.  Is it life threatning?  What are the chances of having a normal pregnancy and delivery.  If this occurs again, what are my chances of a similar recovery.  One cardiologist mentioned if a woman has this and then waits 10 years the chances of recurrance is diminished.  Is this true?  I know that each case is different, but after going through several cardiologists in New Orleans, I cannot find anyone who is knowledgable on this topic.  Maybe you could recomend someone in the New Orleans area

[VERNESSA]

DEAR LUCILLE, I HAD THE SAME DIAGNOSIS IN APRIL OF 1999 AND I WAS TERRIFIED. A YEAR AND A HALF HAS PASSED AND I AM HAPPY TO SAY THAT I AM NORMAL.

[Rachel]

Well I have a 3 week old daughter and have just been through this so it is new. I had very minimal swelling of my ankles and high blood pressure for 2 weeks then developed shortness of breath. We went to the hospital and were diagnosed with pneumonia. I was admitted and treated for the pneumonia and the plan was for me to go home the next day. That night I went into congestive heart failure. I don't remember much, but they did an emergency c-section and our daughter was pink and crying and just fine, but they had to put a ventilator in me and sent me off to ICU to recover. Spent 4 days there (only 2 on the vent)and lost 32 pounds of fluid while on Lasix. Stayed a few more days on maternity and have been home for 2 weeks. My first trip to the cardiologist was last week and I get my first echo done this Tuesday (10/10/00) - my EF was at 30% when I left the hospital. I am on Lasix, Aldacterone, Linoxin and some potassium and magnesium supplements. I too was crushed to learn that this will probably be our only child. I am 31 and was planning to have 2 or 3, but we feel very blessed to have our daughter (and to have me, I suppose). I had no idea that so many people are affected by this. This is a nice site and it's encouraging to read other's stories.

[kelley]

Good luck to all of you who are having problems.. I too just
had a baby.. She is my third and is now 2months old.. I
started having problems with bradycardia, chest pains and other
symptoms.. Had an echocardiogram done, the results were negative.
Had a Holter monitor and that came back irregular.. I now go
in about a week for a Thallium stress test.. I don't know
what to expect.. This staying in limbo waiting to find out
if something is wrong is a hard place to be..
Take care of yourselves...

[pammelah]

hi, I'm a little affaid right now that the trouble i've been having with my heart is just a sign of things to come, I am 28 and pregnant with my fifth child... did any of you have any warning of peripartum?  Im having eppisodes of pvcs tachycardia, with tightness chest pain and difficulty breathing help.

[Denis Strangman]

I just want to put this information into the record, so that any other people who are researching PPCM, might come across it. My wife Margaret had PPCM in 1986 and was being assessed for a heart transplant but came good. In the process she had a severe episode of potassium deficiency. At the time of the PPCM Marg felt extremely weak and believed she might die at any moment. The following year (1987) she had a cardiac arrest in-hospital 36 hours after a hysterectomy. There was a suspicion that Pethedine might have been responsible. Apart from high blood pressure she has lived a full and useful life. Our last born is now a tall, healthy, 14 year-old. At the time Marg was so weak that she could not even hold him to feed him in hospital. A medical researcher phoned our house some years later and was taken aback when Marg answered the phone - I think he expected her to have died!
Unfortunately, this year (2000) Marg has developed a malignant and aggressive primary brain tumour, known as a gbm iv. Her outlook on life is still prayerful and cheerful. We have a web diary of her progress at: http://www.geocities.com/oldparl/journey.htm
for those who are interested. In her two linked talks she refers to the two cardiac episodes in 1986 and 1987.
Our prayers and best wishes to anyone who has been struck by PPCM. Denis Strangman, Canberra, Australia. ***@****

[Toni Doupe]

Dear Doctors,

      I was wondering, I had Postpartum myocardiomyopathy with CHF in February 1995 and in June of 1995 I was off all medications. What would be the percentage of this returning after 5 years? I don't plan on having anymore children, I was just curious.  Thank You

[Helayna]

I am 32 years old and just had our 5th child. 5 days after she was born I started to have a lot of swelling of the legs,feet, hands and face then 7 days after her birth I woke up unable to breath. The sob was easily resloved at the hospital with lasix and combivent as was my high BP but my pulse has remained at 40-45 bpm. It is now 11 days since her birth and the docters said my echo was inconclusive as to whether this is cardiomyapathy or just simply a fluid overload. My echo sowed an enlarged heart and leaky valves. I know it is difficult to diagnose with this much info. but the doctors are doing sort of a wait and see approach with this so I just wondered about your opinion? I am to weigh myself every am and report any weight gain to my family doctor, then as long as I don't have any more problems they will do another echo in 4-5 months. I am fearful that things will go downhill again with my pulse still so low and then tonight, my 1st night home, my bp was 155/90. That is still lower then when I first went in ( it was 186/90) but much higher then my usual (around 100/60).

[deedle1976]

Simple hormone treatment cures lethal heart condition

08 February 2007

German researchers have discovered that a drug commonly prescribed to new mothers to halt lactation also cures a rare type of heart failure known as postpartum cardiomyopathy (PPCM). The team made the discovery after accidentally designing an animal model that showed them exactly what caused the condition.

Denise Hilfiker-Kleiner from Hannover University and her team designed a genetic knockout mouse to study the function of an important transcription factor called Stat-3 (Signal transducers and activator of transcription 3), a protein that regulates cell proliferation. The modified mouse was missing the gene coding for Stat-3.

  'We bred these mice and noticed that many of them did not survive multiple pregnancies. When we monitored them more closely, we discovered that this knockout was susceptible to this exact type of heart failure [PPCM] so we knew Stat-3 must be involved,' co-author Helmut Drexler told Chemistry World.

The group showed that PPCM is caused when prolactin, a hormone released at birth to stimulate lactation, is converted into a smaller, biologically active peptide known as 16kDa prolactin. This smaller, nastier version of the hormone destroys the endothelial lining of blood vessels and reduces blood flow to the heart. Stat-3 prevents prolactin from being cropped down into this destructive peptide.

The researchers confirmed this by treating the pregnant knockout mice with bromocriptine, a drug that inhibits the release of prolactin. 'In our mouse model bromocriptine completely prevented PPCM,' said Hilfiker-Kleiner.

Stat-3 increases the formation of antioxidant proteins, protecting the heart from oxidative stress. Without these antioxidants, stress on the heart cells leads to increased levels of the enzyme responsible for cleaving prolactin into its damaging counterpart.

PPCM is a very rare condition affecting one in every 1300 to 4000 deliveries in the US but, as Drexler emphasises, it leads to death in around a quarter of the women it affects and many of those who survive require a heart transplant. 'Since this affects a mother shortly after childbirth, it is especially traumatic,' he said.

Fortunately, Bromocriptine is an established drug that has been used for years to halt lactation in new mothers. So it can be used directly to treat women with PPCM or with high risk for PPCM, Hilfker-Kleiner told Chemistry World.

Bromocriptine has already been trialled in a small preliminary study in 12 women with a high risk of PPCM and was shown to prevent PPCM. 'This was a preliminary trial. We need to prove the effectiveness of our therapy in a larger randomised and blinded trial, before we can be sure that it really works.'

[deedle1976]

There has been some very interesting and promising research as to the cause PPCM published this year in the journal 'cell'.

They have discovered that, in mice, PPCM is caused by a faulty prolactin gene. This gene produces a faulty version of the prolactin hormone, which damages the cardiac capillary network. Makes sense really that prolactin is involved when you consider that PPCM develops as the body starts to lactate.
They found that if they gave the mice Bromocriptine(an inhibitor of prolactin) they did not develop PPCM. Bromocriptine is the drug they used to give routinely to non breastfeeding mothers to 'dry up' their milk.
They tested this in a very small sample of women who had PPCM in a previous pregnancy and the ones taking bromocriptine did not develop PPCM the second time whereas most of the ones not taking it did develop PPCM again. They only tried this on small number of women (about 10 I think) so they need to do a trial with more people to prove it however it looks highly possible that they have found the cause and a way of preventing and possibly treating it.



. Here, we show that female mice with a cardiomyocyte-specific deletion of stat3 develop PPCM. In these mice, cardiac cathepsin D (CD) expression and activity is enhanced and associated with the generation of a cleaved antiangiogenic and proapoptotic 16 kDa form of the nursing hormone prolactin. Treatment with bromocriptine, an inhibitor of prolactin secretion, prevents the development of PPCM, whereas forced myocardial generation of 16 kDa prolactin impairs the cardiac capillary network and function, thereby recapitulating the cardiac phenotype of PPCM. Myocardial STAT3 protein levels are reduced and serum levels of activated CD and 16 kDa prolactin are elevated in PPCM patients. Thus, a biologically active derivative of the pregnancy hormone prolactin mediates PPCM, implying that inhibition of prolactin release may represent a novel therapeutic strategy for PPCM.