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Congenital Heart Disease and Pregnancy (Drs. Krasuski and Chapa 5/2/14)

Friday, May 2, 2014 - Noon

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It is estimated that one million adults are living with congenital heart defects in the U.S. Progress in treatment has allowed patients with congenital heart disease (CHD) to reach adulthood and live longer. Female CHD patients often wonder if it’s safe for them to get pregnant. Each case must be evaluated individually by a multi-disciplinary team of medical professionals to determine if pregnancy is safe for them. Dr. Krasuski, Director of our Adult Congenital Disease Center and Dr. Jeff Chapa, Head of the Section of Maternal-Fetal Medicine, answer your questions.

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When to seek specialized care

Moderator: Dr. Chapa - The AHA posted a scientific statement this past week regarding diagnosis and treatment of fetal heart disease. When should a mother seek an opinion of a specialized obstetric team vs. a general obstetrician?

Jeff_Chapa,_MD: Good question. It is a good idea to seek a specialized team, including maternal-fetal medicine and pediatric cardiology subspecialists, when a heart defect has been detected in the fetus during pregnancy or if you have a history of a previous child with a congenital heart disease.

pala208: I would also like to add that it's important to seek a specialized team if you are an adult with a CHD!

Richard_Krasuski,_MD: As a cardiologist who specializes in the field of adult congenital heart disease, I could not have said that any better!


Tetralogy of Fallot

misskt22: I am a 38 year old TOF patient that had a pulmonary valve replacement (tissue) in 2012 due to severe regurgitation. Right now I am 10-1/2 weeks pregnant with fraternal twins from donor IVF. This is my second pregnancy. My first was in 2009 (same donor) with a healthy but IURG singleton (born at 37 weeks) before I got my valve replaced. I did not have any cardiac issues during that pregnancy but I did have pregnancy complications (pre-e at 36 weeks, placenta inefficiency, low amniotic fluid,). As a twin pregnancy with the new valve, what potential cardiac or pregnancy concerns should I be looking out for? Also, what potential long term impact to my new valve may occur with a twin pregnancy? Finally, I am considering selective reduction and want to know generally speaking how much "safer" a singleton pregnancy would be versus a twin pregnancy in regards to long term cardiac concerns. I am being seen by a ACHD cardiologist but am seeking a second opinion.

Jeff_Chapa,_MD: Generally, you should be in better shape for handling the stress of pregnancy after valve replacement, so I think you have a good chance to do well. With twins, the physiologic changes and strain on your heart will be a little bit more than with a singleton, but again, if your new valve and heart are working well, you should do well. There are increased risks with a twin pregnancy, including preterm birth, preeclampsia, gestational diabetes, cesarean section, etc. However, outcomes with twins are generally pretty good. The other thing to consider is that there are some risks with selective reduction, which could cause you to lose both fetuses.

Richard_Krasuski,_MD: I would echo those thoughts - in general, we have had excellent success in patients who have undergone valve replacement for Tetralogy of Fallot. We typically assess the heart with an echocardiogram and/or cardiac MRI to get a better sense of how well the right ventricle is doing. If the chamber is small and the heart function is preserved, there should be no problems with pregnancy. If the ventricle is considerably enlarged, or the function reduced, we follow these folks much more closely during pregnancy because of the risk of developing heart failure and/or rhythm disturbances. One other thing to mention is the impact of pregnancy on valve deterioration. There is undoubtedly some extra wear and tear that is placed on the valve by going through pregnancy and may increase the risk of needing further interventions in the future. The good news is that we can now perform many of those interventions without doing open heart surgery, such as implantation of a new heart valve.

nikkie-pa: I am 29 years old and have been taking digoxin for almost two years now. I was born with Tetralogy of Fallot and had surgery to repair it when I was three years old. I am married and would like to get pregnant. Trying to find out information. 1) If I had surgery to repair my TOF, will my pregnancy be safe? 2) Is digoxin safe for pregnancy? 3) What is the likelihood my child will have TOF too?

Richard_Krasuski,_MD: The definitive repair for Tetralogy of Fallot is known as a "complete repair." In this surgery, they patch the ventricular septal defect and they respect the obstructing muscle in the right ventricular outflow tract. Often, the result is considerable leaking of the pulmonic valve. Patients who have severe leaking do better with valve repair or replacement prior to getting pregnant. If the leak is mild or moderate this is not necessary. 2) Digoxin is generally safe in patients who are pregnant. In general, however, we do not utilize much digoxin in patients with TOF as there is no proof that this does much for the right ventricle. In some patients, digoxin can help for rhythm disturbances and may need to be used. 3) The risk of congenital heart disease for TOF patients is slightly higher than the 2.7% I quoted earlier. There are genetic tests which can be done to more accurately determine your risk. We would recommend you see a geneticist before getting pregnant.

Jeff_Chapa,_MD: I agree with Dr. Krasuski. It would also be good to determine what your cardiac function is today, as pregnancy will add a significant strain.


Transposition of the Great Arteries

wendyva22: I was born with transposition of the great arteries, Tetralogy of Fallot. Well my question is how many or what percent of women have been pregnant with this problem? I am 35 and never been pregnant but would really like to have a baby.

Jeff_Chapa,_MD: I don't have a specific number for you, but we are seeing more patients with congenital heart defects surviving into adulthood and having children. I would recommend you have a thorough evaluation of your cardiac function and consult with a cardiologist and maternal-fetal medicine specialist to see if pregnancy would be safe and a good idea for you. We would be happy to see you for an evaluation. If you are not local, we offer an online 2nd opinion service called MyConsult.

Richard_Krasuski,_MD: I am often amazed about how well patients with even the most complex structural heart disease do with pregnancy. Good prenatal assessment is critical to be able to predict or anticipate any problems before they occur.

TayTayla: Hi, I am 35 y/o and was born with Transposition of the Great Vessels. I have a repaired VSD, a mechanical mitral valve, a pacemaker, complete heart block in my ventricles. My EF is 40%. I want to get pregnant very badly. I eat right and exercise every day. My doctor said if I am monitored very closely it may be possible to do this because I am so healthy otherwise. Doctors, what do you think? What is the chance of me surviving this pregnancy? What is the chance of having a healthy baby?

Jeff_Chapa,_MD: Very good questions. The answers are more difficult, however. Your ejection fraction suggests that your heart's function is compromised to begin with. Pregnancy will add a significant strain to your heart. It must pump 50% more blood by the third trimester. Thus, I am not sure your heart will be able to tolerate this added strain, and it could lead to your heart function getting worse and poor pregnancy outcomes. In general, we do not recommend pregnancy in this setting. There are other options to having biological children, like having a surrogate carry the pregnancy for you. You could go see a reproductive endocrinologist to discuss your options further.

Richard_Krasuski,_MD: I tend to be slightly more optimistic. The systemic ventricle in patients with transposition is often affected with ejection fractions that usually range in the 45 - 50% range assuming that what you have is known as congenitally corrected transposition. We recently had a young lady who we followed with cc-TGA and a mechanical systemic atrio-ventricular valve (mechanical tricuspid valve) that we managed through pregnancy with an excellent outcome. This could not have happened without the involvement of our congenital heart team, the vascular medicine team, and of course the terrific high risk OB team. There are important issues with anticoagulation and we generally recommend going on lovenox for the first trimester then converting back to coumadin for the second trimester to late in the third trimester when we convert back to lovenox to facilitate delivery. In terms of your heart function, the most important question is whether you have shortness of breath with exertion now, as I am most concerned by symptoms rather than an absolute number for the ejection fraction.


Pulmonary Valve Disease

michellewehr@yahoo.com: I had my PDA closed and pulmonary valve removed at 12 days old due to pulmonary stenosis. I am currently 38 and have not had the valve replaced. I take insulin for Type II DM and a vitamin. My Echo (April 2014) had the following result: Compared to prior, the RV has enlarged slightly. There is borderline concentric LV hypertrophy. EF = >65%.The transmitral spectral Doppler flow pattern is normal for age. The RV is moderately dilated. The RA is mild to mod dilated. Status-post pulmonary valvectomy. There is residual pulmonic valve tissue at the annulus. There is mild valvular pulmonic stenosis. Severe pulmonic valvular regurgitation. Moderate pulmonary artery dilation. I am told my defect/repair is unusual, so doctors do not know what to tell me regarding the safety of a pregnancy. Have you had any patients who did/did not have a successful pregnancy with this type of defect? Do you think it would be safe or best to avoid? Is there other information that should be gathered to determine the safety?

Richard_Krasuski,_MD: This defect is in fact not so unusual. The main issue that you currently have is a very leaky pulmonary valve, which has led to right heart enlargement and dysfunction. I would recommend that a new valve be implanted either surgically or through a catheter procedure (if you are eligible) before you get pregnant. In general, this is safer and we have good experience with patients who have gone through this. Though, patients with your condition can go through pregnancy safely - the risks are certainly higher if it is done before surgical or catheter repair.

Jeff_Chapa,_MD: As a rule of thumb, it is best to optimize your health, prior to conceiving.


Mitral and Aortic Valve Disease

Moderator: We have had questions from patients who have mitral valve prolapse or other forms of valve disease and are on beta blockers to help with palpitations. Are there issues with medications and pregnancy that they should be aware of?

Jeff_Chapa,_MD: Beta blockers are generally safe in pregnancy. There is some risk for fetal growth restriction with beta blockers, so I would recommend following fetal growth during the third trimester. Generally, this risk is small and the benefits of treatment justify the use of the medication.

jada33: I am 32 years old. I have a bicuspid aortic valve which has always been mild. I am now 15 weeks pregnant. My recent echo said mild - moderate regurgitation. I am wondering if this will be a problem with pregnancy. Will this be a problem with labor? My doctor said I should be fine and come back in a year - but is that ok?

Richard_Krasuski,_MD: In general, leaky valves are very well tolerated during pregnancy while narrowed valves are not. The exception is patients with severely leaky valves where there is evidence of heart failure before pregnancy. Given the mild degree of disease prior to your pregnancy, you should do very well with this. In terms of follow up, I usually see my patients who are pregnant, at least once at approximately 20 weeks, as by this point the volume status is maximal and we can get a good idea of how the heart will do through the rest of the pregnancy. I then see patients (as long as they are doing very well) approximately three months after delivery.

Jeff_Chapa,_MD: I agree with Dr. Krasuski. I would also recommend that you have a follow-up echo later in gestation when the physiologic changes of pregnancy have peaked.


Irregular Heartbeats, Pacemakers and Pregnancy

KathyB24: I have had SVT with two ablations since I was 22. I am thinking of AV node ablation with pacemaker. I am now 24, getting married and wonder your thoughts on AV Node ablation w/pacemaker in patients with SVT and pregnancy?

Richard_Krasuski,_MD: This is certainly one way of approaching the problem of atrial arrhythmias. In a young patient we try to preserve the synchrony between atrial and ventricular chambers as much as possible. There are several new techniques of ablation that may allow you freedom from a pacemaker. So it is always helpful to get a second opinion from an electrophysiologist about these options before implanting a device.

Managing a pacemaker through pregnancy is not that difficult but does require regular follow up.

MadelineL: I am a 32 year old woman in my 3rd month of pregnancy I now have complete heart block and going in for a pacemaker. Is the heart block caused by pregnancy? Are there issues with me getting a pacemaker during pregnancy?

Jeff_Chapa,_MD: No, the heart block is not caused by pregnancy. It can however cause significant problems for you and your baby during pregnancy. Putting in a pacemaker is generally safe during pregnancy; there is some radiation exposure with this procedure, but it is minimal and generally has no effect on the baby. Also, the benefits of getting the pacemaker outweigh any risks to the pregnancy.

Richard_Krasuski,_MD: I agree with Dr. Chapa - it is unusual for women to develop complete heart block during pregnancy. This should prompt further investigation including an echocardiogram and such blood work as lyme serologies. It is probably best to have the pacemaker implanted as appropriate heart rate adjustments during pregnancies are critical to the growth and development to the baby.

riah: I started having heart palpitations when I was four months pregnant last year and the cardiologist prescribed me medication but I did not take it because it started to slow down and become less frequent. I'm still getting them and my baby is seven months old. He prescribed me digoxin in October because I was nursing but that didn't work. So the cardiologist prescribed me metoprolol last week for two weeks two times a day as needed. How long should the palpitations be going on and am I on the right track. The EKG is normal every time and my blood pressure is normal. When they did an echo while I was pregnant it was normal too. The doctor says it waxes and wanes is this true? Thanks in advance. -Whurriah.

Richard_Krasuski,_MD: Heart palpitations are very common during pregnancy, possibly related to all the changes in hormones that occur during this delicate process. Usually these are due to either premature atrial contractions or premature ventricular contractions and usually these resolve after delivery. In some cases, palpitations can continue and in those cases it would be wise to wear a holter monitor or an event monitor to help determine what the cause is.

Jeff_Chapa,_MD: I would agree with this completely. Beta blockers can help with your symptoms and are generally safe during pregnancy. There is some small risk for growth restriction in the baby with beta blockers, and I would recommend you have ultrasounds to follow the growth of the baby.

Jenny28: I am 28 years old and pregnant eight weeks. Last week I started having rapid heart rates and my blood pressure was going up. I am currently wearing a monitor to check this out. But if your heart is going up - it feels like in the 200s will that affect the baby? I am worried what this can be. What questions should I be asking. This is so very stressful.

Richard_Krasuski,_MD: Sometimes when the heart races like this it can cause the blood pressure to drop and this is when we become concerned. In your case, it appears that the blood pressure is good or even elevated. In such cases, it is unlikely that this is causing any harm to your baby. There are certainly medications that can be given to treat the condition but a monitor is the best way to figure out what the problem is before starting treatment. It is important to recognize that stress itself can cause release of hormones which can provoke rhythm disturbances and thus over-worrying may actually increase risk of having further problems. So one suggestion would be to try to get your mind off of this problem and just try to take a deep breath and relax every now and then.

Jeff_Chapa,_MD: Sometimes, the hormones from pregnancy can cause women to feel this way. It often resolves on its own. If your blood pressure is truly high, then treatment may be needed. I would definitely follow-up with your doctor about this.


Ventricular Septal Defects

Maria221: I have a small VSD that was not large enough for fixing. I am now pregnant and worried that this may be a problem during my pregnancy. I am very worried. What can I expect?

Jeff_Chapa,_MD: If you have been doing well prior to pregnancy with no symptoms, you will probably be fine. I would recommend getting an echocardiogram during pregnancy to make sure that you don't have any significant problems with your cardiac function, specifically like pulmonary hypertension.

Richard_Krasuski,_MD: There are a variety of different ventricular septal defects (VSD) - peri-membranous; muscular; and supracrystal. Each type can have its own problems. In general, if the defect is small, and the heart is normal sized, without pulmonary hypertension, pregnancy should be safe.

Hetal: Good Evening, I am from NJ, USA. My elder sister is suffering from VSD (Ventricular septal defect). Because of her prolong defect of VSD she has PAH (Pulmonary Arterial hypertension). Well she turns 36 today. And we pray to God for many more awesome years ahead as her birthday today. Is there any medical treatment that she can pass her life as normal person. Right from birth she has VSD but, that time it was so costly for my parents to do surgery. We don't want to lose her we love her so much. She is married and her husband knew from the beginning that she couldn't conceive baby or she doesn't have normal life. As the time goes she had many complications sometimes she feels okay and sometimes she feels so tired. if you need to see her reports I can email you. My gynecologist told me about this institution which has best heart surgeon. I need your suggestion.

Jeff_Chapa,_MD: They have told you the right thing. She should not get pregnant.

Richard_Krasuski,_MD: A VSD is a communication between the two pumping chambers (ventricles) of the heart. If the defect is large and goes unrepaired, pulmonary hypertension (high blood pressure in the lungs) can result. In such cases, the blood starts flowing in the opposite direction across the defect (right to left) and results in what is known as Eisenmenger's Syndrome. We used to think that there were no medical therapies for this available - now we realize that some of the medications used for treatment of pulmonary hypertension can have dramatic benefits for these patients. Thus, improving their qualities of life. I agree with Dr. Chapa that pregnancy should be contraindicated in Eisenmenger patients because the risk of death during pregnancy may be as high as 50 percent for mothers.


Hypertrophic Cardiomyopathy (HCM) and Pregnancy

Karen0223: My daughter is 26 and 12 weeks pregnant. She has HCM, with mild symptoms. Her pregnancy was not planned and I am very worried whether she should even be pregnant, given her heart condition. Should I be concerned?

Jeff_Chapa,_MD: Yes - she should be evaluated by a cardiologist and maternal fetal medicine specialist in the very near future. There is increased stress on the heart with pregnancy and we need to know if she will be able to tolerate this.

Richard_Krasuski,_MD: Hypertrophic cardiomyopathy (HCM) is an abnormally thick heart muscle which prevents the heart from pushing out as much blood as it needs to. It can vary from very mild to quite severe. The times during pregnancy when a patient is at greatest risk is early when problems such as nausea and vomiting can lead to volume depletion and greater obstruction. It is critically important that she remain well hydrated during pregnancy. Also delivery is challenging because loss of blood can lead to greater obstruction as well. The coordination between OB and Cardiology is critical here!


Congenital Heart Disease and Genetics

CuriousMom: Are congenital heart defects genetic? If a mother is born with a heart defect, what are the chances that her children will be born with some type of heart defect as well?

Jeff_Chapa,_MD: Congenital heart defects are multifactorial, and certainly, there is a genetic component. Women with congenital heart defects are at increased risk to have children with congenital heart defects. The risks vary depending on the type of heart defect the woman has, but in general the risk is less than 5%.

Richard_Krasuski,_MD: It is definitely dependent on the type of defect. The risk of congenital heart defects in the general population is 0.8%. If a first degree relative has congenital heart disease it is 2.7%.


Fetal Heart Disease

Saraji86: I am 18 weeks pregnant. My doctor told me that the fetus has an enlarge heart and this could be bad for development. Is this something that can be treated during pregnancy - or something we wait until after the baby is born?

Jeff_Chapa,_MD: You should follow up with a pediatric cardiologist who specializes in fetal cardiology. Depending on the underlying cause, there could be potential treatments that could be started or performed during pregnancy.

Richard_Krasuski,_MD: It is amazing how quickly pediatric cardiology has advanced. Some of the catheter procedures can even be performed before the baby is born (such as dilating a narrowed aortic valve).


In Closing

Richard_Krasuski,_MD: It is so great to be involved with this web chat as this is such a critically important issue. One to four percent of all pregnancies are complicated by heart disease. Congenital heart disease is now the most common problem from a cardiovascular standpoint that we encounter during pregnancy. We are certainly pushing the envelope of who can safely be monitored and aided through pregnancy. We are gaining more and more data in a variety of areas in this field and I would stay tuned, as a lot of our recommendations are changing.

Jeff_Chapa,_MD: Cardiovascular disease is becoming the leading cause of maternal mortality in the United States and other developed countries. This is in part due to advances in care for women with congenital heart disease. Pregnancy poses a significant strain on the heart and therefore is important for women with heart disease to have a complete evaluation of their cardiac function prior to conceiving. The key to successful outcomes for these patients is collaborative care across multiple specialties.


To schedule an appointment with Jeff Chapa, MD or with any of the specialists in our Women’s Health Institute at Cleveland Clinic, please call 216.444.6601 or call toll-free at 800.223.2273, ext. 46601. You can also visit us online at clevelandclinic.org/obgyn.

To make an appointment with Richard Krasuski, MD or any of our cardiologists in the Miller Family Heart and Vascular Institute in Cleveland, please call 216.444.6697 or toll-free 866.289.6911. You can also visit us online at clevelandclinic.org/heart.

Reviewed: 05/14

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