This Keynote address by Dr. Carole Warnes discusses the risks associated with pregnancy in patients with cardiovascular disease and suggests a recommended management plan.
Thanks very much, Deepak. It's a delight to be here and congratulations on everything you've accomplished in this fantastic meeting. Um So I called it, what provider should know about pregnancy because you may wonder and here are my learning objectives. I'd like you to recognize the risks that are associated with pregnancy and heart disease and try and recommend a management plan for a pregnant patient. Um Italians know a lot about love and one of my Italian colleagues gave me this poem. At least they talk about it a great deal. Even if they don't know much about it. If you have heart disease, don't fall in love. If you fall in love, don't make love. If you make love, don't get pregnant and if you get pregnant, don't have a child. So in my next 30 minutes, I'm gonna see whether this poem is actually true and good advice. 2% of pregnancies involve maternal cardiac disease and the good news is is that most women with heart disease can have a pregnancy with proper care. But for most, there's an increased risk to both mother and baby. And the key is the pre pregnancy evaluation. If you have a chance to get there. And I got into this because I do congenital heart disease. And in fact, now with the declining rheumatic heart disease, most maternal heart disease is congenital in origin. And why do you need to know about it? Because in the US, we're not doing so well. Here you can see causes of pregnancy related death and cardiovascular disease right at the top of the list. So we need to know about it. And in fact, in 2022 40% of pregnancy related deaths were attributable to heart disease. More than three quarters were preventable even in the US. So we're not doing so well. And women have different attitudes to pregnancy. Some will say not gonna do it for all kinds of reasons, but there are others and I think this applies to most women. Perhaps some of you in the room will do anything and everything to have a baby. More than 1600 shots, four years of therapy to get this bundle of joy. So it's a very different paradigm with attitudes. We have to respect obviously the attitude of the mother and promote her autonomy and try and help her get safely through if that's what she chooses. But there are problems as we're counseling and examining these patients because of course the symptoms and signs of a normal pregnancy. I saw one of you in the restroom short of breath can suggest the presence of cardiac disease and sometimes heart disease will present for the first time in pregnancy. When you have a pregnancy, it exacerbates any heart disease you may have. And of course, maternal cardiac disease increases the baby's risk. So there are many problems and fortunately, now we have some guidelines to help us with management. I was happy to be involved in the ESC guidelines. And Nishimura guidelines on valvular heart disease also has a lot about pregnancy and valve disease. So we do have a lot more data now on which to help guide management. But the key as I mentioned is not getting to the church on time, but getting for pre pregnancy counseling doesn't always happen. But this is what our patients with heart disease need to know. Please come and see somebody who's expert before you consider a pregnancy. And at that time of pre pregnancy counseling, which can take a long time. Sometimes a two hour consult, they really need to go to a center where they either know about congenital heart disease or valve disease, whichever one you're dealing with and high risk pregnancy care. You really need a good history and a physical exam for the fellows. Your ECG and chest X ray echo of course, is very helpful in assessing all the hemodynamics, ventricular function and so on. And if there's any doubt about pulmonary pressure, I'd encourage you to do a catheterization because pulmonary hypertension is a big bugaboo for patients considering pregnancy and exercise testing. Many patients, for example, with congenital heart disease, think they're normal, but you put them on a treadmill and it's a whole different story. They simply don't know, have never known what normal is. Exercise testing. Very helpful to guide you. And at that time, you need to be looking at what medications the patients may be on. Anticoagulation is part of that. You might need to consider genetic testing and counseling and you need to tell them what the plan would be if you get pregnant. Do you call me after your mother, your partner? Am I the next one on the phone or do you call me when you're about 14 weeks pregnant and we'll work it through. You need to outline the whole plan, both CV and obstetric and get them involved with your high risk ob and talk about. Are you gonna come and see me? Are you gonna deliver in Rochester, Minnesota? Can you deliver in Poughkeepsie wherever you are, all that needs to be outlined at the beginning at that time of counseling. So let's look at some practical things. I just remind you perhaps what you learned in medical school about the volume changes of pregnancy that that begins really early on in the first trimester, the plasma volume goes up followed by a lesser rise in red cell mass. That's the anemia of pregnancy which makes all the heart stuff worse. But you can see that around the time of the middle of the second trimester. Um Here, it's about 50% above baseline. So that person with those hemodynamics is gonna have to withstand a 50% increase in volume load a lot for the heart to deal with. And the heart rate goes up, peripheral resistance goes down as placental flow increases, blood pressure tends to stay the same or fall and venous pressure in the legs goes up, which is why 80% of healthy women get pedal edema. But if you think about these changes, the fall in afterload and the increased cardiac output, the after load is helpful for regurgitant lesions. But for stenotic lesions, it makes everything worse. If you've got aortic stenosis and you drop your afterload, you're in serious trouble potentially. So in general, a rough guideline is regurgitant lesions are better tolerated than stenotic lesions. One thing to keep in mind at the time of prepregnancy counseling, the guidelines say risk assessment is absolutely necessary. A class one indication and this is the classification that you should use like NYH A 1 to 4 and at risk one very little increase in mortality or morbidity. But by the time you go up the scale to number four, these are the ones that we really worry about. These are really high risk of death or morbidity. Pregnancy is contra indicated. And if they do get pregnant, you have the unfortunate situation of discussing potential termination. So I'm gonna focus my attention I'm gonna do some cases. I was told I have to pimp dak because he knows everything about everything. So fellows take a breath. Um I'll be nice. Um But so let's focus on these particular class four worrisome lesions and these are they pulmonary hypertension of any cause. Again, with that 50% volume load. If your ef is less than 40 particularly less than 30% your patient's gonna get into trouble probably around the second trimester, peripartum cardiomyopathy, stenotic lesions, mitral aortic stenosis and the bad aorta will touch on a little bit. The number one is this pulmonary hypertension, whatever the cause is, and we'll focus on this E manga syndrome. Those patients who had a big shunt who developed pulmonary vascular disease, whether it's idiopathic, they have a more that's over 30% often in the peripartum period. You deliver a baby and the mother crashes within 24 48 hours after delivery. So these are patients that we really have to counsel. Please don't get pregnant. Peripartum cardiomyopathy. You've perhaps seen some of these cases. We do have some more data about these cases. This was a an international pregnancy associated cardiomyopathy study published a few years ago, but it looked at some good data to help. Tell us are there echo markers as to how women will do when they present with peripartum cardiomyopathy. About three quarters of women will recover ie and ef more than 50% a year later. Some will have continued bad events or reduced ef permanently. For some reason, African American women seem to do worse both at presentation and follow up. But these echo markers I think are interesting and can help guide us. Now, on the horizontal axis, you see ejection fraction of presentation divided into two groups on the left, less than 30% on the right, better than 30%. And in that pale blue lavender color is the final status of the ejection fraction. And look at the comparison as to how many recovered based on the initial ejection fraction. If you presented on the right with an ef better than 30% 86% of women recovered. But if your EF was worse than that, only 37% of women recovered. So that EF presentation will help guide whether the woman is going to do well at one year. The other thing they looked at is the size of the LV. If the diastolic dimensions bigger than six on the right, they'll do a lot worse in terms of recovery than if the LV is smaller, the cut off being six centimeters. And if you had the combination of a bad ef less than 30 a big LV, none recovered at one year. So these are some markers we can use to help guide us. And one of the questions you may be asked in those women who've recovered happily with their ejection fraction is, should they have a second pregnancy. And the problem is that they may have, even if the LV is now completely normal with a second pregnancy, they can still have clinical deterioration. The ef can go down the second time and the second time it stays down and doesn't recover. There's a low risk of death but the ef may be damaged. So, in general, our vices don't have a second pregnancy. Let's focus on stenotic lesions and maybe I'll ask for some audience participation. We'll do low tech and I'll ask you to see if you're caffeinated and raise a hand. So here's a 30 year old who's 32 weeks pregnant and she's in your emergency department. Fellows. Maybe you're called to see what's going on and what you should do. She's dis Nick, she's tachycardic. The JVP is elevated. You're doing your physical exam. She's got a loud one, a normal S two, but she's got an opening snap and a diastolic murmur at the left sternal border and you hear crackles in the lungs. She's got edema and she's already starting on diuretics. So, you've made the diagnosis, fellows, right? We're all looking uncomfortable. So she should have mitral stenosis and be in deep doo do, right? Ok. So here's the echocardiogram. We're looking at the mitral valve and you can see the turbulent flow and there's the gradient 15 millimeters of mercury. She's 32 weeks pregnant. And what to do. Fellows. Where are the balloonatics? What would you like to do with this lady? She's in heart failure. She's in deep trouble. He's got this bad mitral valve. What would you like to do? Someone has an answer before he gets this one. Pointing to your colleagues. You're getting called on. I was gonna call structural structural. Ok. So you're thinking, help me, help me. So you want a ball lunatic, I think, right. So you're worried about this lady, obviously. And of course, we're also worried about the baby because anytime somebody's sick, we've got a 200% mortality. So anybody here like to balloon. This valve. Come on, speak to me. Some people would. Ok. Well, you fell into my trap. What to do because what you actually have to do is slow the heart rate down and this is what we did. You give them beta blockers, slow the heart rate down to 60 the gradient now is four millimeters and you can get it to 38 weeks and deliver the baby. So you only do balloon intervention when you've slowed the heart rate down. So there's more time for diastolic filling, get her out of heart failure, filler full of beta blockers. Don't worry about the baby. The beta blockers will be good and you'll get them safely through pregnancy. You rarely need to intervene with the balloon only if it's refractory to all your medical therapy. Beta blockers are the mainstay. Let's talk about this common problem. The bic cuspid valve. And of course, you know, there are aortic medial changes that are associated with it. These are the contraindications to pregnancy for aortic disease. The bic cuspid valve is vulnerable to degeneration dissection. Of course, there's an increased cardiac output with pregnancy. But while the hormones soften the pelvis, they also soften the aorta. And so there's an increased risk of fragility during a pregnancy for Marfan, it's even worse. So the cut off to contra indicate pregnancy is 45 millimeters in size or less. If there's a family history of dissection or sudden death. For Turners, patients who can be made pregnant with reproductive endocrinology, we index the aortic size because they're short. So 25 millimeters per meter squared and for the awful lowest deeds, they have terrible fragility and the, the cut off is 45 millimeters again. So they, your guidelines about the a Autopay for the valve itself, mild or moderate A s. If you're asymptomatic, they'll usually do ok and moderate A s will be ok. As long as they have no symptoms, the LV is happy and they do a good exercise test. But if you have severe aortic stenosis, as I mentioned that falling after load is gonna be bad news. So you really need to intervene before they get pregnant but doesn't always happen. Let's take this case who gave me chest pain. A 47 year old Amish lady who lives in her community, she's in the 22nd week of her 11th pregnancy. And she, as she walks around her community has been getting short of breath and chest pain for two years even before she got pregnant. But of course she's never seen doctor now. She's pre sinkable. She's got much worse during the pregnancy. She was in my office getting chest pain, walking a few feet to the bed, climbing on the couch and getting chest pain. As was I and on exam, she had a loud systolic murmur. All the features you just heard about for severe aortic stenosis. And here's a echocardiogram. And I think you can see she has a happy left ventricle, albeit a bit hypertrophy. But look at this awful lumpy calcified aortic valve with not good motion. And here we try and zoom in on it. Pregnant ladies have more challenge with their echoes, but it's a B cuspid valve with lumps on it. And here's the gradient, 100 and 29 millimeters of mercury. Now, what to do? These are our options, bed rest. You can buy a bit of time sometimes with beta blockade, you can deliver the baby early. But remember the fetus isn't going to be viable until at least probably 28 weeks. Would you consider getting your friendly ball lunatic and doing a valvotomy? Would you do a valve replacement or a tavy? Think about what to do. Um In this situation, she was having angina pretty much with minimal exercise you can't buy any time, you have to do something. And fairly soon the valve was very lumpy, calcified, not pliable. We didn't think a good candidate for a balloon. So our only option was either to do some sort of intervention or a valve replacement surgery during pregnancy is not ideal, but we've learned a lot about how to do this in the last 20 years or so and some certain things can make it safer, try and do it between 24 and 28 weeks pregnant if that's an option, because you're likely to get fetal viability. Don't use hypothermia, use normothermia for the baby and your fastest best most experienced surgeon and that's what we did. So at 25 weeks, discuss valve options, she had a tissue valve bypass time was 31 minutes with Hartzel Shaf, our fastest surgeon normothermia. She made an uneventful recovery. Thank God. And at 32 weeks though, the baby started to deteriorate with growth retardation, fetal tones were going. Of course, it was a breach and we had to do an urgent c section. But in fact, both mother and baby, thank God did did well. So it shows you how they can present with really scary times but urgent surgery should never be emergent. Best surgeon, normothermia, short bypass and the risk to mother. Now, as long as it's done properly in a good center is very low. Certainly some risk to the fetus. But if you can get them to an age where fetus is viable, do it with ob standby so that the ob people can take the baby out quickly if necessary. Lastly, let's touch on this problem is when you're selecting your valves for these patients, do you choose in the young person, a tissue valve with obvious deterioration and as you know, the younger the patient, the faster a tissue valve will degenerate. Um and you, of course, she's gonna be subject to reoperation. So if you choose that in a 22 year old, at some point, maybe when she's 28 or 30 she's gonna need another valve. Or do you have a mechanical valve with all the concerns about anticoagulation and whether the valve is going to clot off and she's gonna stroke. So the guidelines say, get the pregnancy heart team involved, particularly if there's a mechanical valve and I'll share a case with you and consider a bioprosthesis in young women contemplating pregnancy much safer to manage. Now, when we talk about the mechanical valves, you all in this room probably manage many patients with mechanical valves through their hip surgeries, their prostate. You're used to bridging them all of that. And you may say, oh, we can, we can do this, but I can tell you with pregnancy, it is not at all the same story. Pregnancy is the time when the blood is the stickiest and if anything is going to happen, it's when they're pregnant, particularly with the mitral tilting disc valve. It is not the same as your patients having non cardiac surgery. So here's a case, a 31 year old nurse, a nurse who worked in an or with a cardiac surgeon. She was his favorite assistant, Saint Jude Mechanical mitral valve. 10 years before she sought advice before she had her pregnancy and the exam and the echo are good. She's got a gradient of five. She's on Warfarin 3 mg a day. And if she wants a pregnancy and she's visiting you, I would ask you which anticoagulant treatment is safest for her. So let's see, a show of hands. Would you use low molecular weight heparin weight adjusted in the first trimester to avoid Coumadin, embryopathy then switch to Coumadin and unfractionated heparin around the time of delivery. Please vote. Nobody caffeinated here. Speak to me. No votes, one vote from DC. OK. Low molecular weight heparin throughout a few votes and Coumadin throughout one or two and everybody else is asleep. Oh dear. I'm not doing my job. OK. So I'm going to ask you another question and please, let's see more hands here when you're counseling her. What percent chance of successful outcome for both mother and baby? Would you quote whatever strategy you choose? What would you tell her more than 90% success? More than 80% D pack and a few colleagues. More than 60% not so good. Uh few and again, lots of people asleep. OK. So let's look at some data. This is actually what happened. She asked advice about anticoagulation. And a cardiologist said, don't worry, we'll just change it to. Heparin started her on Lovenox 75 B ID at six weeks of pregnancy. Very fast. Worried about Coumadin, embryopathy question from patients. Should anticoagulation be monitored? Not necessary is the answer at 11 weeks of pregnancy, she's getting a bit short of breath. She asked, can I have an echo? She working in the hospital, the mitral valve gradient is now 14. And the cardiologist says just your pregnancy three days later, she's in the or she drops the scalpel and she strokes can't move the right arm. So I get the call from the surgeon, not the cardiologist. Can you please see my patient? And here's her echocardiogram. And as you look at that mitral valve, I think you can see that there's a lump on there that is quite large and that one disk over on this side is not moving, consistent with her exam and echo. So what to do? Now, there's also the 3d, the disc is stuck. There's a clot gradient is 14. What do we do? We sent for certain. So again, a redo now, so 46 minute bypass, but there you can see the pander and the thrombus on her valve and unfortunately, she did well. So let's just look at this data in the remaining few minutes about what to do. Managing these patients with these mechanical valves. Warfarin is a much better anticoagulant during pregnancy. It's much safer for the mother. Yes, it crosses the placenta. We worry about the embryopathy in the first trimester, but there's no ideal anticoagulant. We worry about the fetus, brain bleeding, hemorrhage, placenta. And of course, there are these cns abnormalities, blindness in the baby that can be tragic. Heparin. On the other hand, is a much worse anticoagulant. And any time you use it in pregnancy, you more than double the risk of valve thrombosis. It doesn't cross the placenta, but you double the risk of valve thrombosis. And these are the strategies that we usually use for these patients, either Warfarin heparin in the first trimester, avoiding embryopathy and then Warfarin and then around the time of delivery, unfractionated heparin or Heparin throughout. And you voted on these and DPA I think you voted for number two. Is that right? Ok. So here's the data. Um Yes, we worry about the embryopathy and the CNS problems. But what we've learned is that the risk to the baby with warfarin is dose dependent and if mothers on 5 mg or less of Coumadin, that risk of fetal embryopathy is around less than 5%. So it's quite low, but it's much better for mother because it's a better anticoagulant. So the guidelines, both the ESC and AC C guidelines recommend that if mothers on 5 mg or less of Coumadin you continue it in the first trimester, shared decision making much discussion. If she's on more than 5 mg of Coumadin, consider low molecular weight heparin shared decision making. Then back to Coumadin. And in the third trimester, you can't be delivering a baby if mother's on Coumadin because baby is anticoagulated. You can't do a vaginal delivery, baby will bleed in the head. So you need to change to unfractionated around the time of delivery. If you stop vitamin K antagonists and you elect to use low molecular weight heparin, you have to monitor anti 10 A levels weekly and you need to monitor it very carefully and you need to do it in hospital. You never just use weight based heparin because they clot. And here's some data from a recent meta analysis, 800 pregnancies looking at maternal outcomes. And you can see that with vitamin K antagonists, the outcome for mother was considerably better than any time. You use low molecular weight heparin. But for baby, the fetal outcome not so good. And this is the trade off. Look at the problem with vitamin K antagonists, problems with spontaneous abortion and fetal death. So it's this awful trade off. Low dose Coumadin is better again, but not without its problem. And this is a nice composite that these authors put together showing you what the composite outcome is of a fetal event or a maternal event. And the very best outcomes occur with low dose warfarin and remember our patient was on three or 4 mg a day. So that has the best outcome in terms of composite. And these actually don't include things like fetal hemorrhage and so on. So this is a nice thing to share with your patients. And this recent registry perhaps that's more reflective of real world is this study looking at um mechanical heart valve outcomes, half of valve thrombosis occurred in the first trimester, all were on heparin. So this is not a good option. Hemorrhage occurred in a quarter. And here again, the problem with fetal mortality. Anytime you use vitamin K antagonist, the miscarriage rate you see there in yellow is high 29%. So it's this trade off, higher rate of fetal loss versus higher risk of valve thrombosis. The outcomes data D pack, you did get one wrong. 58% of patients were free of serious adverse events a lot higher than we thought. So in summary pre pregnancy counseling, we really need to get there. Stenotic lesions are problematic. The aorta is bad in BSD valve, severe A s contraindicated mitral stenosis, use your beta blockers and mechanical valves. There's really no easy solution. Warfarin in low dose is best for the mother. And if you use low molecular heparin, see your patients every week for anti 10 A monitoring. So I'll leave you with this picture that to me is a woman and her partner. Looking at the future, wondering with a heart disease. Can she have a baby? Um And the good news is, as I mentioned, is most of them can. And hopefully for her, if you look at the picture, you might see a baby, a fetus in the future. Here's the feet and here's the head if we take proper care of them. Thanks very much.