Considerations for Heart Failure and Pregnancy

Podcast Transcript

Announcer:

Welcome to Cleveland Clinic Cardiac Consult, brought to you by the Seidel and Arnold, Miller Family Heart, Vascular and Thoracic Institute at Cleveland Clinic.

Karlee Hoffman, DO:

I appreciate your attention. So systolic heart failure in pregnancy, two things we don't really ever want to hear together. I have no disclosures except, I do want to say I do support women who are pregnant and decide to proceed with their pregnancy when their heart failure ... But I, in no way, promote this because like I said, this is an impossible situation. And the only reason I'm up here discussing this is because at Cleveland Clinic we have this true multidisciplinary team that allows us to have successful deliveries with these patients.

In the pregnant patient there's limited data and I'm going to present what we have for you. So one, we're going to discuss one of the cases that keeps me up at night. We're going to set the ground rules for heart failure in pregnancy, discuss where we know the data is, which is peripartum cardiomyopathy. We're going to briefly discuss obstetric considerations, postpartum management and preconception counseling.

So let's start off with this case. It's a 33-year-old African-American female. She has a history of non- ischemic cardiomyopathy, undifferentiated etiology. It was diagnosed in 2015, about two years after she had a delivery. When she was young, didn't have a reason to really present to a doctor during this time. Her EF is now 20% extremely dilated, also has underlying pulmonary hypertension and chronic kidney disease. And she's had one admission for heart failure in 2021.

So she presents to me NYHA Class IV symptoms for an advanced therapy evaluation for heart transplant and an LVAD. She was evaluated at our center and she was actually turned down because she had too many antibodies to proceed with heart transplant. The logistics of her getting a heart transplant were zero because she had 99% antibodies and she had elevated pulmonary hypertension. At that time she did not want to discuss an LVAD.

Like I said before, she had one delivery prior, very uncomplicated pregnancy about 10 years ago. So in August she presented to an ER with abdominal pain and was discharged. Followed up with her OB a month later for missed menses. She was 19 weeks, two days pregnant. And, again, she's on heart failure therapy. So she was admitted for acute heart failure after this ultrasound was found. And then eventually she was transferred to Cleveland Clinic for cardiogenic shock at 22 weeks of pregnancy.

So let's go into the data that we have behind this. So every time I present this, amazing how this trend continues to go up and up and up. So the last data that we have in 2021, when we're looking at pregnancy related mortality in the United States, it continues to increase. 32.9 per 100,000 births in the United States amongst all different races. Again, increase amongst all of these groups.

Specifically why we're talking about heart failure is because heart failure accounts for about 12% of these pregnancy-related deaths in the United States in this timeframe. So, again, going back to the ground rules, let's talk about heart failure. When we look at risk, this is the ultimate risk, the highest risk. WHO risk Class IV? World Health Organization recognizes, if you have an ejection fraction less than 30%, this is an extremely high rate for maternal mortality if you proceed with your pregnancy. And they actually say that if pregnancy occurs, you should terminate. And this should be discussed with the patient.

Why is this the case? Why do these patients struggle during their pregnancy? So what happens during pregnancy, it's an ultimate nine-month stress test. I like to think of it as like a marathon followed by the sprint of labor. So if you look at this through the trimesters ... So we have the first trimester. By the time they get to the second trimester, their cardiac output nearly doubles, their stroke volume increases, their heart rate increases to compensate for this, and their SVR severely decreases.

So you have a hypotensive patient that has almost twice the amount of cardiac output. If you have underlying systolic heart failure, you can see how this does not fare well. So most of our patients with pre-existing heart failure, really the stress of pregnancy comes in the second trimester. If this is a new diagnosis of heart failure, it usually presents in the second to third trimester.

So what causes heart failure in pregnancy? A majority of these patients have what we call peripartum cardiomyopathy and we'll define this a little bit more. But we can't forget about the other causes and these are additional patients we see in our Cardio OB clinic. We have a significant amount of congenital heart disease patients that are living longer, doing better and getting pregnant. We also have undifferentiated non-ischemic cardiomyopathy, hypertrophic cardiomyopathy and then are ischemic and valvular disease.

So for the sake of this talk and the time that we have, we're going to focus on peripartum cardiomyopathy. So let's define it. It's an idiopathic cardiomyopathy that presents with an ejection fraction less than 45%, and it's a diagnosis of exclusion. This is made during the first five months after delivery or the early postpartum period. A lot of times there's a delay in diagnosis because, again, we have young, healthy females, they don't have any reason to see a doctor, they have a lot of other variables going on. After delivery fluid shifts, maybe some gestational hypertension and these symptoms of heart failure really mimic the same symptoms of pregnancy and there's a vast array of severity. So these patients present with preeclampsia, hypertension. Come in with a mildly reduced ejection fraction that can be managed as the outpatient. And some of these patients come in to see us in full-blown cardiogenic shock. The good thing for all these patients is there's a high rate of recovery if this is true-and-true peripartum cardiomyopathy.

All right, so what's the incidence? Depending on what study you look at about one in every 2,500 to 4,000 births in the United States. The thought is, why is this happening? One, females are older when they're getting pregnant, they're also having multi-fetal pregnancies due to IVF and overall just general recognition. Preeclampsia and gestational hypertension account for a large amount of these patients. And also 40% of patients with peripartum cardiomyopathy are African-Americans.

So what happens? Unfortunately, about 50% of these patients present with a catastrophic event, and that's how they're first diagnosed with peripartum cardiomyopathy. That includes cardiopulmonary arrest, pulmonary embolism, other thromboembolism complications, cardiogenic shock requiring mechanical support or death even. And there's a high risk of LV thrombus in these patients. So we'll talk about anticoagulation and what the criteria are for that in a minute.

But again, thromboembolism in these patients anywhere between five and 9% and it's really the perfect storm, right? They have a low ejection fraction, they have a hypercoagulable state, and they're on bedrest around the pregnancy and not really moving around as much as they typically do. So it's a setup for these adverse outcomes in these patients.

So what about the prognosis? Prognosis really depends on where the ejection fraction is at the time of diagnosis. It's really the most reliable predictor of the outcome for the patient. If the EF is less than 30%, there's definitely a lower rate of recovery and increased risk of adverse events. Like I said before, there's definitely a higher rate of recovery in this heart failure group compared to others. And typically we see this in the first three to six months after diagnosis, but there can be a delayed recovery up to two years postpartum.

And a lot of this data came from the IPAC study that showed 72% of postpartum cardiomyopathy patients recovered at 12 months and their ejection fraction was more than 50%, so normal. So what about management? I'm not going to go into this too much, but the main line is you need to know what medications are safe for the fetus during pregnancy and delivery, and also which ones are safe afterwards. A very quick blanket statement, beta blockers are safe for fetal safety. They just monitor them closely with ultrasound throughout. Obviously we have the ACEIs, the ARBs, the MRAs, Ivabradine all are not safe for the fetus, and we avoid these during pregnancy and we have to switch to pregnancy-safe medications such as hydralazine, Isordil, Digoxin.

So what about anticoagulation? I want to point this out because I think we often forget about this. If you look at the guidelines, AHA, if the ejection fraction is less than 30%, they need to be on anticoagulation for at least six to eight weeks postpartum. In the European society guidelines say, if their ejection fraction is less than 35%, they should also be on anticoagulation. We can use Warfarin. It does cross the placentas. We don't use this during pregnancy, but afterwards, completely safe. And then our drug of choice for anticoagulation is low molecular weight heparin because it does not cross the placenta during pregnancy. The DOACs, NOACs are not studied.

So bromocryptine quickly, it's a dopamine agonist that suppresses prolactin. And there have been studies that show that it's associated with an improvement in LV function. So in Europe, there're readily using bromocryptine. Here in the United States, we do not have any data. And we have an ongoing rebirth study as a proof of concept study to show that bromocryptine is successful in improving peripartum cardiomyopathy ejection fractions.

So what about advanced therapies? A lot of these patients present in cardiogenic shock. And the bottom line is, you support them like you support any other patient. You support them with inotropes, you support them with the balloon pump, you support them with ECMO. And if you can save the mom and perfuse the mom, you're perfusing the baby. After delivery, we can think about a durable LVAD. And there are studies that show that women have received LVAD successfully and some of them have recovered and most of them have gone on for a cardiac transplant.

And, again, if you need to be on temporary mechanical support and this is a transplant candidate, you can proceed with transplant in these patients. Of note though, you need to know there's higher rates of graft failure and rejection. Thought is, this is due to higher allograft sensitization and overall much sicker going into transplant than other patients.

So labor and delivery, and I will touch on this just briefly. And, again, this is a situation that none of us want to be in, but requires an immense amount of team and immense amount of planning to prepare for the unpredictable. So obstetric considerations, as long as the mom is not in shock and is stable, they deliver vaginally. And this, again, is not so easy whenever our delivery hospitals are in the community and might not be at the main centers that have the most cardiac support.

But if the patient is not in cardiogenic shock and they're hemodynamically stable, they should deliver vaginally because we know C-sections have a higher risk of infection, higher risk of blood transfusions, surgical complications and, again, the prothrombotic risk.

We need to think about timing also. So stabilizing the mom to get her to viability is very reasonable because we know that complications due to prematurity in these babies are very high. However, if the mom is unstable, that needs to be a reason to promptly deliver. And viability ... Again, what is viability? After 22 weeks is a thought where the fetus can survive outside of the uterus. So the goal of getting them to 22 weeks, 23 weeks, the percentage of survival dramatically increases every week. So at this point, after 22 weeks of viability, every day counts in keeping the mom stable so that you can optimize the outcome for the baby.

So what about postpartum? What do we do? We know these women die from arrhythmias. So if they have a low ejection fraction, it's very reasonable to put a external device on them to treat arrhythmias and not put a permanent ICD in right away. Because our goal is to put them on medical therapy, put them on GDMT and hopefully have a recovery of their ejection fraction. But we know in peripartum cardiomyopathy patients, about 18% of them can have arrhythmias afterwards.

And let's just talk about what none of us as cardiologists really want to talk about but need to talk about. So the key here is avoiding this, right? Avoiding heart failure in pregnancy and it really needs to be an open discussion with the patient. So a lot of us are seeing heart failure patients of childbearing age, and it needs to be a shared decision, it needs to be a conversation, it needs to be documented. And they need to know the risks if they do proceed with pregnancy. One, they need to be off of their medications that can cause fetal risk. And two, they need to know the risk of proceeding with delivering, what that looks like. And I will be the first one to say this is a team approach. I lean heavily on OB and the high-risk maternal fetal medicine doctors for these conversations. And ultimately this is best in a shared group decision.

So what about contraception? This is a question I get asked very frequently, and the bottom line is IUDs are highly effective and least potential for all drug-drug interactions. So, for instance, our transplant patients, they're on immunosuppression, they are pro-thrombotic risk, and IUD is the safest and preferred method for their effectiveness. And this goes for every heart failure patients. The combined typical hormonal contraceptives, we want to avoid them when they have a low EF transplant or an LVAD because of the increased thrombotic risk. Also, the increased risk of hypertension. And the progestin-only pills. Many pills are great, but just not quite as effective.

Also, another topic I think that's important in the preconception counseling portion is that if a female has cardiomyopathy and this is a genetic underlying condition, this can easily be passed on to the baby. So we need to be realistic with them. We need to get them in with the genetic counselors and we need to talk about what testing do we need to do for the offspring if it is a successful pregnancy. And the key thing here, too, is peripartum cardiomyopathy. About 15% of these patients actually have an underlying genetic condition. So please order genetic cardiomyopathy testing on all of your heart failure patients that are younger, looking for underlying etiology.

So what about a subsequent pregnancy? Again, this goes into the preconception counseling. So LVF is the strongest predictor of how the patient's going to do. If their EF is greater than 50%, they're going to have a lower risk proceeding with a subsequent pregnancy. So their risk of recurrent heart failure is only about 30%. If their EF is low, less than 50%, they have a high risk of having complications during pregnancy of about 50%.

And how do we monitor these patients? So despite preconception counseling, despite giving them all the information, it's their right to proceed with pregnancy if they choose to. And, again, like I said before, I support them in doing this as long as they're educated in making their best decision for their underlying health condition.

So how I follow these patients ... One, again, preconception counseling and then I follow them every trimester as long as they're stable and doing well. And then typically we see them about one month prior to delivery to have a whole multidisciplinary delivery plan for them. And I see them close after delivery within two to four weeks afterwards and about one month after that for a repeat echo and repeat lab work.

So back to our case and we're going to wrap this up. So again, two ultrasounds you never want to see together. EF 20% Class IV NYJ class symptoms and currently 22 weeks pregnant. So after many discussions with the patient involving the entire ICU team, ethics, palliative, bioethics, the patient was very hopeful for delivery and viability. And we discussed her options and how the outcome could mean needing a durable LVAD and she was against this completely, but said if it's between death and getting an LVAD, "Of course, I'll proceed with an LVAD."

Her initial hemodynamics, her right atrial pressure was extremely high, cardiac index, extremely low. So we proceeded with an Impella 5.5 to give her full support with hopes to viability at 25 weeks was the ultimate goal. She had initial improvement with the Impella and was doing well until she wasn't. So about one week after Impella's support, she started having sustained runs of VT anywhere between two and five minutes. Cardiac index was dropping and she had end organ malperfusion. So after daily multidisciplinary discussions with the entire team, I can't even tell you how many people, it was a shared decision with the patient to deliver at 23 weeks.

So we delivered her in a cardiothoracic surgery OR with full support and anticipation that we may need more support like ECMO and she was delivered successfully. So post-C-section, she had a complex course, but we continued the Impella support for about two weeks until we could bridge her to a durable HeartMate 3 LVAD that was placed. Baby survived, is doing well, has had a prolonged NICU stay. And with her permission, I have shared this patient who is successfully living, doing great with an LVAD, thriving, coming to visit her baby daily in the NICU, taking care of her eleven-year-old daughter at home. So with that, I appreciate your attention and we'll wrap this up.

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