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 Questions and Answers with Mary Cannobio, RN, MN, FAAN


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"Questions and Answers with Mary Cannobio, RN, MN, FAAN"

Written by:
Mary Canobio, RN, MN, FAAN
UCLA School of Nursing
Los Angeles, CA

Edited by: Mona Barmash

Posted: September 21, 1998


Gynecologic/Reproductive Issues in Congenital Heart Disease
Q. Can you tell us about the interaction between the menstrual cycle and women's heart symptoms (fatigue, breathlessness, rhythm abnormalities), and how medication fits into this picture?
A. Generally speaking, for women with acyanotic heart disease who are surgically repaired and doing well, there aren't any specific effects that I am aware of. But for some cyanotic females, there seems to be an increase in symptoms such as headache, fatigue, muscle aches premenstrually, but these tend to go away once the period comes. The thought is that these symptoms might be fluid related, but there is no data to support this yet.

Q. Can you briefly address coumadin? I have received contradictory information about whether it makes flow heavier, and do anticoagulants contribute to heavy periods?
A.There are no published data regarding coumadin causing increased menstrual flow. There are reports, however, that say an elevated protime can cause gyn problems such as heavy bleeding. Also, if the woman has some underlying gyn problem like fibroids, there may be increased bleeding.

I should mention that women who are cyanotic may also experience problems with increased menstrual flow (metomenorraghia), painful flow (dymenorrhea), very light flow, or no flow (amenohrrea). If this is a problem, it should probably be checked out by a gynecologist.

Q. I wonder if there is some way to actually measure the stress that pregnancy places on a diseased or damaged heart? It seems nearly every woman with CHD is advised not to get pregnant.
A. There is no one test that measures stress effect of pregnancy on the heart, but if the safety of becoming pregnant in a woman with CHD is at question, there are several ways of determining the "potential " risk to the mom. I should begin by saying that today not every woman with CHD is advised against becoming pregnant. The risk and concern relates more to women with unrepaired and/or complex CHD, and women with pulmonary vascular disease such as Eisenmenger’s Syndrome.

Another category of women who are cautioned about pregnancy are those who have developed clinical symptoms such as arrhythmia’s (irregular heart rate), pulmonary hypertension, or heart failure. Physicians often use the New York Heart Association (NYHA) Functional Classification (I through IV) as one guide to evaluate maternal risk for pregnancy. In general, maternal risk is viewed as low for a woman in Class I, and high for a woman in Class III, IV. The only condition for which pregnancy is definitely contraindicated is in females with pulmonary vascular disease (Eisenmenger’s). Because of the high mortality rate to both fetus and mother, pregnancy in this setting is clearly not advised. Women who remain cyanotic (without PVD) also present a higher rate of risk to both the mom and the fetus. This is another group for which pregnancy is not advised. Women born with complex CHD who have been surgically corrected (such as Fontan, Mustard, Rastelli), are a new group of women. Pregnancy risk is not as clear, but we have some preliminary data that shows that women, for example, after Fontan who are clinically stable, seem to do well through a pregnancy. The verdict is still out on this, but we are encouraged by our first results.

The general rule for any women with CHD wanting to get pregnant is that she should check with her cardiologist when she is ready to become pregnant. If there is any question of risk, the doctor will order a complete physical exam that generally includes a doppler echocardiogram, and electrocardiogram. If these show any irregularity your PMD might then recommend a catheterization.

Q. Is it common for CHD girls to be slow developers and to be small in stature?
A. Yes, but this is generally found in the cyanotic or surgically unrepaired population. In our study, we found that girls with cyanotic CHD were 1) late starters with periods; 2) often didn't regulate until late if at all; 3) and were physically small and underdeveloped. This may have something to do with hormones. What we recommend is that if a female continues to have problems with their periods (light, irregular or no periods) after age 18, that they should see a gynecologist to see if she is ovulating. We should mention that once the defect is surgically repaired and the cyanosis is relieved, the problems of small body stature, underweight, and menstrual irregularities are also reversed.

For females with common or acyanotic CHD, we did not see the same problems. These young women tend to follow menstrual patterns and physical development similar to the general population.

Q. What is the current thinking on hormone replacement therapy (HRT) both perimenopausal and post-menopausal?
A. Surprise, surprise, there is, to my knowledge, no opinion about it. We health professionals tend to forget that patients with CHD are old enough to think about menopause....so I was told by a 58 year old patient of mine. The general rule about use of HRT in the public applies to most menopausal females with CHD if they are in Functional Classes I or II with no cyanosis or pulmonary vascular disease (high pressure in the lungs).

Q. I have major mood swings that are helped by hormones, however, I can't take them due to the fact that I'm on Coumadin. What is your impression of PMS Escape or St. John 's Wort?
A. I have little to no experience with either, but recently I've started to look at St. John’s Wort as an alternative therapy. But I do want to say that as the population of women with CHD ages, problems associated with menopause need to be addressed. Apart from hot flashes or night sweats, we need to think about other problems associated with menopause including osteoporosis, and the risk of coronary heart disease among others. Therefore, those of you reaching your mid-40s need to begin thinking about and discussing Hormone Replacement Therapy with your PMD/GYN. In terms of risk, there is virtually no data with respect to HRT and CHD, but in general, the rules applied to the general public should apply to most women with CHD. More specifically, if the female is acyanotic and falls into functional class I or II, low dose estrogen replacement should not be a problem.

If the woman is cyanotic and/or has pulmonary vascular disease (hypertension), there may be some added concern. However, because the body is no longer producing natural estrogen, the small replacement dose should not present the same risk and concern raised by the higher doses used in birth control pills. Similarly, using over-the-counter therapies such as herbal remedies need to be addressed before you take them....especially women with cyanotic CHD.

Q. At what age do you worry about a well-developed girl with CHD that has not had a real period - just spotting?
A. Menstrual periods and development issues aren't of serious concern for girls between 12 and 17, but once the girl is 18 or older and she's not yet regulated, is just spotting or missing periods, she should be checked out by a gynecologist. Again she may not be ovulating and not ovulating can present as a serious problem. In our studies, this was not common in women with acyanotic CHD, but we found it to be true in a number of women with cyanotic CHD...especially older females or females who were surgically repaired as adults, but remained cyanotic 10 years after menarche.

Q. Does being on regimented aspirin affect the amount of bleeding, or anything else with the bleeding during a menstrual cycle?
A. To my knowledge, aspirin should not affect menstrual periods. If you are cyanotic and you have increased menstrual flow, maybe, but we don't know for sure....it should be checked out by a gynecologist.

Q. I would like to ask about the advisability of taking Hormone Replacement Therapy when having Eisenmenger's syndrome.
A. The concern is the estrogen. Again, we don't have enough experience with the low dose of the estrogen, but there is always concern about using estrogen in women who are cyanotic and who have Pulmonary Hypertension. The concern with estrogen and pulmonary hypertension, or cyanosis has to do with clotting. We know that at higher doses of estrogen, clot formation is a potential risk and even worse in women who smoke, so that's why we don't recommend estrogen based hormones for birth control. The estrogen dose in hormone replacement therapy (HRT) is so much smaller, that the risk may not be as great....but it should always be checked out. As we gain more experience, we are finding that we don't have to be as cautious about certain things such as using estrogen, but as in everything, use of HRT should be discussed with both your cardiologist and gynecologist, and be evaluated on an individual case by case evaluation.

One of the risks associated with use of estrogen has been the risk of clot formation, and over the years, there has been concern about this in all women using birth control for many years. Over the past few years, the estrogen dose has come down quite a bit. In females with CHD, we must be particularly careful in presrcibing estrogen-based birth control, particularly in females with conduits, and shunts. Because of the thickened blood (high hematorcrits) in cyanotic females, the concern is higher, and so we avoid using estrogen-based hormones as contraceptives.

Q. Is it considered safe to have sex during a-fib?
A. I have little experience in this area, but common sense prevails. If you know you are in a rapid atrial fib pattern (you’re having palpitations and shortness of breath, and you know it’s not because of your partner!), then it’s probably a good idea to avoid sexual activity during this time. You could end up with more trouble than it is worth. I’d suggest discussing it with your PMD to see if there’s something to be done to convert you. By the question, I take it that this may be a recurring problem, so it’s hard to answer. But some general rules apply. A sexual relationship with a known partner is generally safe, because it’s comfortable, but new relationships can add to stress (and excitement) which produces more adrenaline, which can irritate an already irritable heart. This is potentially not good. Again, if atrial fib, or for that matter, any heart palpitation or irregularities occur during sexual activity, it is best to discuss it with your physician. He/she may want to do some testing to see what exactly you’re having, and is in a better position to advise you.


References:

Canobbio MM, Mair DD, Rapkin AJ, Perloff JK, George BL Menstrual Patterns in Females after the Fontan Repair.
Am J Cardiol 1990 Jul 15;66(2):238-240.

Canobbio MM, Rapkin AJ, Perloff, JK, Lin A, Child JS Menstrual Patterns in Women With Congenital Heart Disease. Pediatr Cardiol 16:12-15, 1995

Canobbio MM Reproductive issues for the woman with congenital heart disease. Nurs Clin North Am 1994 Jun;29(2):285-297.

Mendelson MA Congenital cardiac disease and pregnancy.Clin Perinatol 1997 Jun;24(2):467-482.


This article was reviewed prior to publication by:

Richard Donner, M.D.
St. Christopher's Hospital for Children
Philadelphia, PA


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