Understanding PPCM

[JAMESFETT]

Peripartum Cardiomyopathy (PPCM)

DEFINITION:
1)Onset of heart failure during the last month of pregnancy up to 5 months postpartum (in some the onset may be a month or so earlier or later).
2)No other causes for heart failure.
3)No previous history of heart disease.
4)Echocardiographic evidence of decreased systolic heart function

FREQUENCY:
In the United States, the estimated incidence is between 1 in 2000 to 4000 live births. No precise figure is available because there are no population-based PPCM registries in the USA. In some countries the incidence seems to be higher, such as South African Bantus with an estimated 1 case per 1000 live births, and Haiti with a calculated incidence of 1 case per 350 live births.

CAUSE:
The exact cause of PPCM remains unknown. Leading suspects include viral infections, autoimmune or immune system dysfunction, pregnancy-associated hormones, and genetic factors. Pregnancy-altered hormones, increased heart workload, and immune system changes during pregnancy appear to play a role. However, PPCM can occur in any pregnancy, to mothers of all ages, and in all races.

SYMPTOMS:
Fatigue. Some fatigue is normal with pregnancy and while caring for a new baby, so it's important to know your body and what is out of the norm.
Difficulty breathing. This unusual (for you) shortness of breath may occur with activity or exercise or when lying down.
Swelling. Ankles, feet, hands, and face are all places fluid retention can cause swelling. In severe cases, abdominal swelling may also be present.
Palpitations, or feeling like your heart is racing, stopping, skipping beats, or fluttering.
Unexplained cough. An irritable cough that won’t go away, and not connected with a cold or upper respiratory infection, may be a clue about fluid retention and decreased heart function.
Frequent night time urination. This is from extra fluid accumulating in the legs moving into the circulation while lying down.
Chest Pain. Although not frequent, it is always possible to have chest pain, very mild or more severe. Always seek immediate care for chest pains.

DIAGNOSIS:
Following medical history and physical exam, the most important test is an echocardiogram or ultrasound exam of the heart. It will quickly indicate if there is decreased pumping action (reduced ejection fraction) of the left ventricle and if there is enlargement of the left ventricle, which are the two findings most important in the diagnosis of PPCM. An echocardiogram is absolutely essential if PPCM is suspected.
Other tests, such as electrocardiogram (ECG), chest x-ray, blood tests of cardiac troponin and C-Reactive Protein may be abnormal or normal, and are not diagnostic. Measuring blood B-type Natriuretic Peptide (BNP) is helpful because an elevation indicates stress on the left ventricle and can be used as an indicator of progress in treatment of heart failure.

TREATMENT:
The medications most useful in the treatment of heart failure from PPCM are:
1)Diuretics as needed to decrease excess fluid in the circulation and body tissues, 2)Angiotensin Converting Enzyme-inhibitors (ACE-I) to lower the load on the heart’s pumping action, and 3)Beta-blockade or beta-adrenergic blockers (B-B) to lower afterload and protect against undesirable arrhythmias. The latter two require careful dosage adjustments, starting low to avoid too much lowering of the blood pressure, and very gradually increasing the dosage as tolerated and as needed to help improve the pumping action of the heart. [EXAMPLE (CONSULT YOUR DOCTOR FOR ACTUAL RX & DOSAGE): Start carvedilol (Coreg) at 3.125 mg two times per day; If tolerated by blood pressure not falling and if needed to improve symptoms and heart function (EF determined by echo) increase by steps every two weeks to 6.25 mg, then 12.5 mg, then 15.625 mg, then 18.75 mg, then 21.875 mg, then 25 mg two times per day. When increasing the dose take the first increased level at bedtime in order to minimize any effect on lowered B.P.] Other medications are sometimes needed, such as anticoagulants to avoid blood clots if the left ventricular ejection fraction is below 30 %, spironolactone to help control levels of blood potassium, and digoxin if not responding to the first line medications.

PROGNOSIS:
Almost everyone responds to treatment. Many PPCM patients improve quickly, regaining normal heart function within the first few weeks and months after diagnosis. Others continue to improve over the next several months and years. Even those who do not return to completely normal heart function can improve a great deal with treatment, and live a long, comfortable life. REMEMBER: Healing can continue well beyond the first 6 months after starting treatment, even into the 4th and 5th years; that all dependnds upon how many damaged but salvageable heart muscle cells exist.

Newer, effective treatments have progressively improved survival rates to over 95% and lead to improvement in almost everyone with full recovery of heart function in over 50 % of PPCM patients. Additional evaluations such as cardiac MRI, heart catheterization and endomyocardial biopsy may be necessary for the few patients who are not responding to usual treatment. In a very, very few it may be necessary to consider an implantable cardioverter-defibrillator (ICD) and eventually heart transplant; but that is unlikely to be required.

FUTURE PREGNANCY:
Upon making a full recovery, some women consider the possibility of having another child, and many have experienced a successful subsequent pregnancy. It is important to understand the risks for relapse of heart failure. To learn more about the risks should you consider a subsequent pregnancy, please view the algorithm in the Post-PPCM Baby forum. Also remember that careful monitoring of the subsequent pregnancy is very important in order to diagnose any relapse early and start effective treatment available. We are learning that the lowest risk of relapse of heart failure is associated with PPCM moms who have regained heart function to the level of LVEF 55 % or more and who have adequate contractile reserve on exercise stress echo. Even so, there is never any guarantee that relapse will not occur.

NEW INVESTIGATION: Every new PPCM patient is a candidate to have viral antibodies measured in a blood sample drawn from an arm vein. There are two types of testing to do: 1)IgM (recent infection) and IgG (previous infection) antibodies to Adenovirus, Coxsackievirus, E-B virus, Human herpesvirus 6, Cytomegalovirus, Hepatitis C virus, Parvovirus B19; these are the more common "cardiotropic" viruses. 2)If the IgM antibody test is positive, the Polymerase Chain Reaction (PCR) test for viral particles should be done for that virus.

[4girlsmama]

a cardioverter-defibrillator is that a pacemaker?

[tabs]

No. A pacemaker is a small device that stimulates the heart to contract (beat) at a certain rate.

A cardiac defibrillator (ICD) is a machine that monitors the rythm of the heart (how it is contracting) and applies a therapy (either pacing or a shock) to put the heart back into a normal rhythm.

An ICD can be used as a pacemaker, but its main function is to correct a heart from a fatal rhythm. Think of the external paddles they put on a chest during a cardiac arrest only this machine delivers the shock right to the heart.

Hope that helps,
Twilah

[4girlsmama]

Thanks Twilah,
My docs have mentioned both but I wasn't sure what the difference was. that clears things up for me:p

[angela]

I have had a pacer for 8 years now and my heart was getting better but started having breathing problems and they did an echo and found out I was back to EF 14% and now I need a defibrillator. The only thing that I am really nervous about the procedure (I am having it put in tomorrow) is that they told me they have to put me into cardiac arrest to see if it works. I hope it (defibrillator) works because if it doesn't they said I will need to be put onto the transplant list.

I love this site my husband found it for me and I come on here just about everyday to see what you all write about.

Angela

[Elliesmom]

Hope your surgery went well, let us know how it went when you get a chance.

I grew up in Menifee... singing "it's a small world after all:p "

[angela]

Thank you so much for your concern. I got out of the hospital yesterday and I was home by 4pm. I am just sore at the site they put the defibrillator on I am not sore where they took out the pacemaker. The first thing I did when I came out of the anestesia(sp) was start crying that I was still there. I was worried and so was everyone else. Thank you so much for your support.

Angela :-)

[4girlsmama]

So glad that you are doing well! Rest lots and feel better soon:)

[pistons22girl]

Angela, I am glad you are ok. Hope this is what it takes to get you better.. Rest and hope you are feeling better soon.

[JAMESFETT]

Did you know?:

Current conventional heart failure therapy of PPCM with diuretics, ACE-inhibitors (or hydralazine/nitrates if still pregnant), and beta-blockers results in an unprecedented survival of almost every PPCM patient.

Current therapy of PPCM results in improvement in almost every PPCM patient and complete recovery of heart function in almost two-thirds of patients.

Successful subsequent pregnancy is possible in most PPCM patients who have fully recovered heart function, although careful monitoring for relapse is necessary. There is effective treatment available for those who relapse.

IF you have PPCM and IF you are slow to recover (left ventricular EF below 20 per cent at two months after diagnosis or below 40 percent at 6 months after diagnosis it is important to be considered for additional evaluation, including cardiac magnetic resonance imaging, cardiac catheterization, and endomyocardial biopsy BECAUSE you could be a candidate for non-conventional treatment that may give a boost to recovery.

Never lose hope.

JD

[sbdoyle]

Dear Doctor and Members:
Hours after delivery I had all those symptoms and medication never helped me recover. I had two VAD and was in critical condition and never left the hospital until I received a transplant. I am almost 2 years out. Could this have been prevented prior to delivery? I was retained so much fluid and no one listened. What are your feelings on future pregnancies? Does anyone know of anyone in my situation, HELP!

[JAMESFETT]

Thank you for letting us know about your situation. I am sorry you had such a difficult time, but glad you are doing well with your transplanted heart. There is an entire medical literature about pregnancy in heart transplant women, and it would be good to be in close touch with cardiologists in your transplant team for the best advice. One can never go back; but in this new day, effective treatment and management assure that almost everyone with PPCM gets better, well over half of PPCM patients fully recover, and transplant is required by the very, very few.

JD

[JAMESFETT]

update, that's all.

[JAMESFETT]

here it is....

[JAMESFETT]

Here it is....

[JAMESFETT]

here it is

[JAMESFETT]

Did you know?:

Current conventional heart failure therapy of PPCM with diuretics, ACE-inhibitors (or hydralazine/nitrates if still pregnant), and beta-blockers results in an unprecedented survival of almost every PPCM patient.

Current therapy of PPCM as outlined above results in improvement in almost every PPCM patient and complete recovery of heart function in up to two-thirds of patients.

Successful subsequent pregnancy is possible in most PPCM patients who have fully recovered heart function, although careful monitoring for relapse is necessary. There is effective treatment available for those who relapse.

IF you have PPCM and IF you are slow to recover (left ventricular EF below 20 per cent at two months after diagnosis or below 40 percent at 6 months after diagnosis it is important to be considered for additional evaluation, including cardiac magnetic resonance imaging, cardiac catheterization, and endomyocardial biopsy BECAUSE you could be a candidate for non-conventional treatment that may give a boost to recovery.

Never lose hope.

JD

[momof3]

Dear Dr. Fett,
After my third child, I became sick. My EF was 5-10% at DX. I was transferred the next day to a different hospital the next day-Hospital of University of Pennsylvania-because they were better equipped to handle my problem. My EF at Penn was 10-15%. Six weeks later it went up to 20%. I recovered normal function(was mild to moderately dillated) in my right ventricle and my LV went from severely dillated to mild to moderately. My doctor is very optomistic that I will recover. I feel pretty good although I need to rest often. I will not be getting another echo for about 4 months. I am teased that I am a "numbers watcher" and they want me to focus on how I feel. I am on maximum doses of Coreg-25 2x a day, beta blockers, ACE inhibitors, postassium, blood thinners, and diuretics (every other day). Why does my doctor not want to repeat my echo sooner? You also said in a thread that if EF was still low after 2 months, that further treatment should be looked into. Should I be pushing for this? I think my doctor is wonderful and knowledgeable. I question though, how does she just "know" that I will recover, when I don't always have faith in it myself? Is it a doctor's sixth sense?

[JAMESFETT]

That's progress, and you are on good treatment. You are certainly headed in the right direction, and I am glad for your good working relationship with your physician. If she would like to discuss any aspect with me, please let me know. I have summarized guidelines to follow: [Am I a candidate for heart catheterization? Sometimes heart catheterization is needed to rule out other kinds of heart disease that could lead to heart failure, such as coronary artery disease or other causes of dilated cardiiomyopathy. Some women with PPCM whose EF has not increased to greater than 20 % by 2 months post-diagnosis or over 40 % by 6 months post-diagnosis may be candidates for heart catheterization with endomyocardial biopsy, preceded by cardiac MRI scan. The reason for these additional studies is to determine if alternative treatments are indicated, and what type.] Best wishes in 2009.

JD

[JAMESFETT]

I am urging physicians who care for PPCM patients to remember that the beta-1-adrenoreceptor antibodes, seemingly present in all PPCM patients, are agonistic, meaning they are active to influence the heart to work harder. Their effect can be at least partially negated by beta-blockers, so in treatment, carvedilol or metoprolol or bisoprolol are treatments of choice.

JD

[JAMESFETT]

Consider a stress echo if you are thinking of any future pregnancy and you have returned to normal LVEF (55 % or more).

JD

[Dani & Jaden]

Hi, my name is Danielle and I was diagnosed 8/9/08. I'm still wondering (actually panicing) about prognosis, mortality, etc. I've been on disability since last April and am wondering if any of you have been successful with an appeal. I read all these great stories of all you strong, strong woman and wonder how in the heck you are all managing with kids, husbands, your condition/syptoms and still working a 40 hour work week.. I'm still out of breath taking a shower, changing my 17 month old's diaper, walking up & down stairs.. How do you all do it???? my EF is 40%, however i had a stress echo this past tuesday and it went up to approx. 55% (which is confusing as well)...Any replies would be greatly appreciated!!! Thank you!!!

[JAMESFETT]

Best wishes for continuing improvement. What medications are you taking? I suggest you also post on the message forum, "General Support" and you are likely to have more of your "sisters" see it and respond.

JD

[LovinLife]

I see you sister :) I think things get easier with time and with the rise of your EF. I have always stayed home with my kids and can rest when needed. These other warriors will have to speak for themselves. I don't see how they do it either! It sure does make me proud!

[4girlsmama]

It is difficult when you are chasing a little one. I have always had to work full time and my EF is hanging out around 45-47% these days. I find it key to know your limits and rest when you are starting to get fatigued. LovinLife is right, it does get easier in time. Hang in there and make sure you get plenty of life.

[Dani & Jaden]

Thanks for all your kind words of support. It's comforting to know that there are other women out there who understand what my family is going through

[JAMESFETT]

Thank you for posting your medications. It appears that you are not yet 2 years out from diagnosis; so you still have time for improvement. Working with your cardiologist, you have the possibility of helping reach better heart function by:
1)considering increasing your carvedilol (Coreg) since you are on a very low dosage, 1/4 of maximum, if tolerated and needed.
2)considering going to a longer-acting ACE-I (such as lisinopril) since the captopril is one of the earliest ACE-I, requires multiple daily dosages, and your current dosage is also very low.

In the message forum, "PPCM for patients and health professionals," I have posted a message at the top about what one could do if there is recovery delay:

What Might Be Done to Improve Systolic Heart Function in Delayed Recovery* ("Slow Responder") of Peripartum Cardiomyopathy?

*Definition of “delayed recovery" or "Slow Responder": Left ventricular ejection fraction (LVEF) <25-30 % at 2months post-diagnosis or <35 to 40 % at 4 months post-diagnosis and/or LVEF <45 % at 6 months post-diagnosis.

First, review data to confirm that all criteria are met for a diagnosis of peripartum cardiomyopathy (PPCM) and to be sure that other causes of heart failure have been excluded.

Treatment regimen should include both ACE-Inhibitors (ACE-I) and beta-blockers (BB) at maximum tolerated dosages. If that dosage has not yet been reached, very slow increases, one medicine at a time and at approximately two-week intervals, should be attempted, until maximum dosage or limit of tolerance of side-effects has been reached.

Some laboratory testing or scans may indicate ongoing activity of disease:
a)Plasma high sensivity-C-Reactive Protein (hsCRP), in excess of 5 mg/liter may indicate ongoing inflammatory process.
b)Plasma B-type Natriuretic Peptide (BNP) above established “cut-off” level may indicate continuing stress on left ventricle.
c)Cardiac magnetic resonance imaging (CMR), with gadolinium enhancement, may indicate inflammatory process and/or fibrosis.

If these tests indicate evidence of ongoing activity of disease, consideration can be given to endomyocardial biopsy (EMB). EMB tissue should be preserved both in formalin and with liquid nitrogen “quick-freeze” so as to be available for H & E staining, immunohistochemical staining, and polymerase chain reaction testing for DNA/RNA viral genômes.

Because of the number of women who still fail to reach recovery levels, it is important to give consideration on a research basis protocol to alternative methods of treatment:
a)Intravenous immunoglobulin therapy (IVIG) in the acute fulminant and deteriorating situation (earlier studies = no benefit).
b)Immunoabsorption, excellent potential.
c)Anti-viral treatment when tissue viral PCR-positive.
d)Immune suppression when tissue viral PCR-negative.
e)Prolactin inhibition when elevated plasma Cathepsin-D, abnormally elevated plasma Prolactin 23-kDa, and/or presence of plasma Prolactin-16 kDa (basic foundational research still required regarding the latter three, but so far encouraging prospects for some cases of PPCM).

----James D. Fett, MD, 1 August 2009

[Dani & Jaden]

Dear Dr. Fett,
Thank you so much for your message and your concern, I feel as though I have a new guardian angel:) As I mentioned on my message, I had an appt with Dr. Uri Elkayam with USC Internal Medicine for this morning 1/25, but they cancelled my appt and said that he wasn't seeing patients. My current Cardiologist, Dr. M. Leila Rasouli was anxious to hear what he would say re: my condition/test results and to see if increasing my meds would be ok at this time, also considering adding Digoxin as well, as another Cardiologist had suggested. I did a stress echo last Tuesday, 1/19/10 and my resting EF was still around the 40% mark and treadmill EF was close to 55%, which she said was encouraging. I will pass your informative article onto her this week. Dr. Rasouli also wanted to up my Coreg but only wants to increase my meds at small increments and only one at a time to see how each one effects me (Due to my anxiety, I started Wellbutrin last month and only wanted to change one med at a time). I see her again mid February. Thank you again for your post, I really, really appreciate the info and concern....Dani

[JAMESFETT]

Thank you for the follow-up. I wish you could have seen Dr. Elkayam because I have great confidence in his opinions; however, I am sure the other cardiologist(s) will be just fine.

When the LVEF is above 30 % there is little extra benefit from digoxin, and it carries its own risk of adverse effects, especially upon increasing possibility of myocardial irritability and ventricular tachyarrhythmias. In any event, should you ever go on it, in women there may be higher risk of side effects than in men, and one is prudent to stay on the low-dose side, which is 0.125 mg per day if normal kidney function. However, it is not much used when the LVEF is above 30 %.

Captopril, as an ACE-I is one of the first (oldest) in that class if not the first, and it may also have a higher profile of adverse effects; so one of the longer acting (newer) ACE-I may be easier to take and even more effective.

Your projected increase of carvedilol sounds good to me.

I think you have very good potential to experience additional healing if you can move to the higher dosages of ACE-I and BB (of course only one at a time, and as you say, in slow increments). Best wishes.

JD