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Choosing the Best Contraceptive Method for the Adult With Congenital Heart Disease Candice K. Silversides, MD, MS, FRCPC, Mathew Sermer, MD, FRCPC, and Samuel C. Siu, MD, MS, FRCPC Corresponding author Candice K. Silversides, MD, MS, FRCPC University of Toronto Pregnancy and Heart Disease Research Program, Toronto General Hospital, 585 University Avenue, 5N521 North Wing, Toronto, Ontario M5G 2N2, Canada. E-mail: [email protected] Current Cardiology Reports 2009, 11:298–305 Current Medicine Group LLC ISSN 1523-3782 Copyright © 2009 by Current Medicine Group LLC As women with congenital heart disease (CHD) approach childbearing age, issues such as pregnancy and contraception need to be addressed. Women with CHD represent a heterogeneous population. While some have simple cardiac lesions and thus have contraceptive risks that are no different from those of the general population, others have complex, multisystem disease such that incorrect contraception advice can be associated with serious health consequences. Choosing a contraceptive requires consideration of the risk of pregnancy; the available contraception options; their risks, failure rates, and benefits; and the preferences of the woman. This review focuses on contraceptive selection in women with CHD. Introduction As the population of women with congenital heart disease (CHD) reaching childbearing age continues to grow, issues such as pregnancy and contraception need to be addressed. The current American College of Cardiology/ American Heart Association guidelines for the management of adults with CHD suggest that “it is the duty of the adult CHD specialist to provide or otherwise make available informed advice on contraception, including discussion of risk” [1]. Women with CHD represent a heterogeneous population. While some women have simple cardiac lesions, making their pregnancy and contraceptive risks no different from those of the general population, others have complex, multisystem disease. For them, incorrect pregnancy or contraception advice can result in serious health risks. In this group of women, choice of contraception requires consideration of the following: • Pregnancy risk • Available contraception options and their risks and benefi ts • Failure rates and understanding the consequences of an unplanned pregnancy • Preferences of the woman For women with CHD, these issues can be complex, and input may be needed from a cardiologist who has expertise in CHD and from a gynecologist or obstetrician. In regard to the risks of pregnancy, cardiologists are best suited to assess maternal cardiac risks, address cardiac medication use during pregnancy, and provide information about long-term prognosis when appropriate. Women must be educated about these risks to determine if and when they wish to become pregnant. Risk stratification during pregnancy integrates global and lesion-specific risks and is discussed elsewhere [2–5]. In addition, understanding the risk of pregnancy is important to determine the type of contraception required. For example, in women with prohibitively high pregnancy risks (eg, those with Eisenmenger’s syndrome), permanent forms of contraception may be required. In women at high risk for complications (eg, women with mechanical valves, Fontan circulation, or significant systemic ventricular dysfunction), failure rates are an important consideration [6]. Beyond pregnancy risk assessment, cardiologists are responsible for educating women about safe conceptive options as they relate to their cardiac condition. Complicating matters is our current lack of contraceptive efficacy and safety data in women with CHD. All recommendations are extrapolated from studies in women without CHD, thus highlighting the need for careful individualized assessment. This review focuses on contraceptive selection in women with CHD. Choosing the Best Contraceptive Method for the Adult With CHD Contraceptive Options Contraceptive options include the following: • Combined hormonal contraceptives (COCs; estrogen/progestin formulations) • Progestin-only formulations • Intrauterine devices (IUDs) • Barrier methods • Sterilization/permanent forms of contraception Many women use different forms of contraception during their reproductive years. In a survey from our center, women with CHD reported use of multiple methods of contraception, most commonly condoms, COCs, progestin injections, and rhythm or natural methods. Five percent had undergone a tubal ligation [7•]. Each method has associated benefits and risks that are discussed in detail below. Combined hormonal contraceptives COCs containing estrogens and progestins (synthetic forms of progesterone) inhibit ovulation primarily by suppressing the hypothalamic gonadotropin-releasing factor. COCs vary in the type of progestin and concentrations of estrogen/progestin used. Significant modifications have been made since their introduction; consequently, they are referred to as first-, second-, third-, and fourth-generation COCs. Over time, there have been decreases in the dose of estrogen and changes in the types of progestins used. The current term low-dose COC refers to formulations with 35 μg or less of ethinylestradiol. The most commonly used delivery mode is oral, but other preparations are available and include transdermal patches (ORTHO EVRA; Ortho Women’s Health & Urology, Raritan, NJ) and vaginal rings (NuvaRing; Schering-Plough, Kenilworth, NJ). Nonoral routes are used for 21 days and then removed for 7 days. Failure rates vary from 3% to 8% during the fi rst year of use [6]. Estrogens and progestins can interfere with drug metabolism, and warfarin levels can be affected. Therefore, monitoring of International Normalized Ratio is important, particularly when initiating use of COCs. Estrogen and progestins have several adverse cardiac effects. The most clinically important is that of estrogen, which alters the coagulation profile and results in increased rates of thromboembolic complications. Clinical studies have shown an increased risk of venous thromboembolism in current users compared with nonusers [8,9]. The actual risk of venous thromboembolism from pregnancy itself is much higher. Complications are reported to be highest within the fi rst 6 months of use. The higher doses of estrogen in fi rst-generation COCs were associated with higher rates of thromboembolic disease. Second- and third-generation formulations have been associated with less thromboembolic risk [10,11]. The thromboembolic risks of the newer formulations of COCs and nonoral formulations are not known. COCs have been shown I Silversides et al. I 299 to have a negative impact on the lipid profile (reduction in high-density lipoproteins and increase in low-density lipoproteins/triglycerides) [12] and glucose metabolism [13]. Use of COCs is associated with an increase in blood pressure [14,15]. The risks of arterial thrombotic complications are increased in women taking COCs who have additional vascular risk factors, such as hypertension, diabetes, obesity, smoking, or migraine headaches [16,17]. However, this association between coronary artery events and use of second- and third-generation COCs has not been demonstrated consistently [18,19]. Finally, some data suggest that estrogen may modify the potassium channel, but the clinical significance of this fi nding is unknown. Because of the potential adverse effects of COCs, the World Health Organization (WHO) and American College of Obstetrics and Gynecology (ACOG) have published general guidelines for the use of low-dose COCs (Table 1) [20,21,22••]. Although they are not specific to women with CHD, these guidelines need to be incorporated into decision making for all women. For instance, COCs are contraindicated in women older than 35 years of age who smoke. Other examples that may pertain to women with CHD include contraindications to COCs in women with migraines and focal neurologic symptoms (migraines with aura). Migraines may be more common in some forms of CHD (ie, women with coarctation of the aorta), and this recommendation is important to address [23]. COCs are also contraindicated in women with severe (decompensated) cirrhosis or benign or malignant liver tumors. This may be relevant for women with CHD who have liver disease from a blood transfusion during previous cardiac surgery or in those with a Fontan circulation [24]. There is limited literature addressing contraceptive selection in women with CHD [1,25–27,28••]. The most detailed consensus statement comes from a British working group that developed guidelines for contraceptive use in women with heart disease using the WHO format (Table 2) [27]. These recommendations are discussed below. For women with CHD, the thrombotic risk posed by COCs is the main contraindication to their use. Cardiac lesions classified as WHO class 4 (COC method not to be used) included conditions with increased thrombotic or embolic risks. Lesions with increased thrombotic risks (WHO class 4) include older-style mechanical valves (Starr Edwards, Björk-Shiley), which have higher rates of valve thrombosis; pulmonary hypertension of any cause, which can be associated with pulmonary artery thrombus/pulmonary embolism [29]; and dilated cardiomyopathy and left ventricular dysfunction from any cause (left ventricular ejection fraction < 30%). Women with Fontan operations (particularly the older right atrial to pulmonary artery Fontan operation) are at high risk for atrial thrombus and pulmonary emboli [30] even when they are not exposed to COCs, and some centers advocate prophylactic anticoagulation in all of them. Both the British working group and the American College of Cardiology/American Heart Association adult CHD guidelines have made similar 300 I Congenital Heart Disease Table 1. Recommendations of the American College of Obstetrics and Gynecology for the use of hormonal contraception in women with coexisting medical conditions Recommendations Level of evidence COCs are not recommended for women with a documented history of unexplained venous thromboembolism or venous embolism associated with pregnancy or exogenous estrogen use, unless they are taking anticoagulants. A* COCs should be prescribed with caution, if ever, to women older than age 35 y who are smokers. A* Levonorgestrel IUS is appropriate for women with diabetes without retinopathy, nephropathy, or other vascular complications. A* Women with well-controlled and monitored hypertension who are aged 35 y or younger are appropriate candidates for a trial of combination contraceptives, provided they are otherwise healthy, show no evidence of end-organ vascular disease, and do not smoke. B† Use of COCs by women with diabetes should be limited to those who do not smoke, are younger than age 35 y, and are otherwise healthy with no evidence of hypertension, nephropathy, retinopathy, or other vascular disease. B† Use of COCs may be considered in women with migraine headaches if they do not have focal neurologic signs, do not smoke, are otherwise healthy, and are younger than age 35 y. Although cerebrovascular events rarely occur in women with migraines who use COCs, the impact of a stroke is so devastating that clinicians should consider the use of a progestin-only, intrauterine, or barrier contraceptive in this setting. B† COCs should be used with caution in women older than age 35 y who are obese. B† If COC are continued before major surgery, heparin prophylaxis should be considered. B† Women with controlled dyslipidemia can use low-dose COCs. If the low-density lipoprotein level is > 160 mg/dL, triglyceride level is > 250 mg/dL, or multiple additional risk factors for coronary artery disease are present, alternative contraception should be considered. C‡ Progestin-only contraceptives may be appropriate for women with coronary artery disease, congestive heart failure, or cerebrovascular disease. C‡ *Recommendations and conclusions based on good and consistent scientific evidence. † Recommendations and conclusions based on limited or inconsistent scientific evidence. ‡ Recommendations and conclusions based primarily on consensus and expert opinion. COC—combined hormonal contraceptive; IUS—intrauterine system. (Adapted from American College of Obstetrics and Gynecology Committee on Practice Bulletins-Gynecology [22••].) recommendations to avoid COCs in women with cyanosis related to intracardiac shunts or after Fontan operations (WHO class 4 and class 3, respectively) [1]. With underlying arterial disease, there is a risk for adverse events. Therefore, COCs should not be used in women with ischemic heart disease or previous arteritis involving the coronary arteries (ie, Kawasaki disease). Because of the risk of paradoxical emboli in women with intracardiac shunts or pulmonary arteriovenous malformations, COCs were classified as WHO class 4. Intracardiac shunts, both right to left and left to right (which have the possibility of reversing shunts transiently), were considered contraindications to using COCs. However, an exception was made in the case of intracardiac shunting as a result of a patent foramen ovale, a normal variant in approximately 20% of the population. Some of the WHO class 4 recommendations from the British working group may not be universally accepted. For instance, the consensus from the British working group was that even when treated with warfarin, women with older-style mechanical valves and Fontan circulation should not use COCs. This opinion is not supported by data, and the risk of thrombosis in women who are adequately anticoagulated is not known. Therefore, physician practice may vary when it comes to using COCs in women treated with warfarin. Another example in which physician practice may vary relates to COC use in women with dilated left atrial dimensions greater than 4 cm (WHO class 4, according to recommendations). As no data support this recommendation and many women with CHD will have left atrial enlargement, this type of recommendation needs to be tailored to the individual. WHO class 3 (caution in use) cardiac lesions include newer-generation mechanical heart valves such as bileaflet valves, atrial fibrillation or flutter, prior thromboembolic event and currently on warfarin (ACOG guidelines suggest that COCs are contraindicated in women with prior thromboembolic events), repaired coarctation with aneurysm and/or hypertension, and Marfan’s syndrome with unoperated aortic dilation (Table 2). Although there are many potential adverse effects of COCs, there are also benefits beyond contraception, specifically protection against endometrial and ovarian cancer, reduced risk of benign breast cyst, reduced risk of iron-deficiency anemia, and treatment of acne. These benefits can be important for some women and need to be factored into decision making. Tissue prosthetic valve lacking any of the Atrial septal defect with left-to-right Dilated left atrium > 4 cm features noted in WHO class 3 or class shunt that may reverse with physiologic 4 columns stress (eg, Valsalva maneuver) Mild pulmonary stenosis Pulmonary arteriovenous malformation *Condition with no restriction on use of the contraceptive method. † Condition in which the advantages of the method generally outweigh the theoretical or proven risks. ‡ Condition in which the theoretical or proven risks usually outweigh the advantages of using the method. § Condition that represents an unacceptable health risk if the contraceptive method is used. LVEF—left ventricular ejection fraction; WHO—World Health Organization. (From Thorne et al. [27]; with permission.) Prior left ventricular dysfunction, any cause (eg, dilated cardiomyopathy [LVEF < 30%]) Uncomplicated Marfan’s syndrome Coronary arteritis (eg, Kawasaki disease with coronary involvement) Coronary artery disease Past thromboembolic event (venous or arterial) while not taking warfarin Past cardiomyopathy fully recovered, including peripartum cardiomyopathy Previous thromboembolism Hypertrophic cardiomyopathy lacking any WHO class 3 or class 4 features Fontan heart even while taking warfarin Cyanotic heart disease even while taking warfarin Repaired coarctation with aneurysm and/or hypertension Bileaflet mechanical valves in the mitral Björk-Shiley or Starr Edwards valves even or aortic position while taking warfarin while taking warfarin Simple congenital lesions successfully Small left-to-right shunts not reversible with Marfan’s syndrome with aortic dilation, repaired in childhood and with no physiologic maneuvers (eg, small ventricular unoperated sequelae (atrial or ventricular septal septal defect, small patent ductus arteriosus) defect, patent ductus arteriosus, or total anomalous pulmonary venous drainage) Repaired coarctation with no hyperten- Surgically corrected congenital heart sion or aneurysm disease lacking any of the features noted in WHO class 3 or class 4 columns Uncomplicated mild native mitral and aortic valve disease Bicuspid aortic valve with normal function Atrial fibrillation or flutter if not anticoagulated Most arrhythmias, other than atrial fibrillation Atrial fibrillation or flutter while or flutter taking warfarin WHO class 4 (do not use)§ Mitral valve prolapse with trivial mitral regurgitation WHO class 3 (caution in use)‡ WHO class 2 (broadly usable)† WHO class 1 (always usable)* Table 2. Recommendations for the use of combined hormonal contraceptives (combined oral contraceptives, transdermal patches, and vaginal rings) in women with cardiac disease Choosing the Best Contraceptive Method for the Adult With CHD I Silversides et al. I 301 302 I Congenital Heart Disease Progestin-only contraception Intrauterine devices Oral progestin-only contraceptives (“mini-pills”) act on the cervical mucus to decrease sperm penetration; the injectable form also inhibits ovulation. Norethindrone and norgestrel are the most common progestins (synthetic form of progesterone) used in the oral preparations. Other methods of delivery of progestin-only contraception include intramuscular (depot medroxyprogesterone acetate [Depo-Provera; Pfi zer, New York, NY]), subcutaneous (medroxyprogesterone acetate [Depo-SubQ Provera; Pfi zer, New York, NY]), subdermal implants (etonogestrel [Implanon; Schering-Plough, Kenilworth, NJ]), and levonorgestrel-impregnated intrauterine systems (Mirena; Bayer, Leverkusen, Germany). Because there is no associated increased risk of arterial or venous thrombotic events, progestin-only forms of contraception are often suitable for women with CHD [31–33]. They also have less effect on blood pressure compared with COCs and can be used by smokers. One of the major limitations of the oral preparations is their relatively high failure rates (5% to 10%) within the fi rst year of use; pills also must be taken at the same time each day [6]. The high failure rates limit their use in women at high risk for pregnancy-related complications. Women with Eisenmenger’s syndrome, significant pulmonary arterial hypertension, Marfan’s syndrome with dilated aorta, significant cardiomyopathy, or severe symptomatic aortic stenosis should not use oral progestin-only contraception. Injectable and implantable forms are much longer lasting; depot medroxyprogesterone acetate injections are administered every 3 months, and etonogestrel implants every 3 years. Failure rates are much lower with the injectable and implantable forms of progestin-only contraception and therefore are suitable for women at high risk for adverse pregnancy outcomes. The most common side effect is irregular uterine bleeding, which results in high discontinuation rates. With depot medroxyprogesterone acetate or subdermal etonogestrel implants, women may become amenorrheic over time, but early irregular bleeding remains unacceptable to many. Progestin use can result in fluid retention, which must be considered in some women with clinical heart failure or other conditions adversely affected by volume retention. Although intramuscular injections can result in hematomas in women taking anticoagulants, ACOG guidelines still recommend depot medroxyprogesterone acetate as an appropriate option for this population [22••]. Like the COCs, progestin-only contraception can affect warfarin levels. Although there have been concerns about the use of depot medroxyprogesterone acetate and osteoporosis, the most recent recommendations suggest that in healthy young women and adolescents, the advantages of depot medroxyprogesterone acetate likely outweigh the potential risk of future osteoporosis [22••]. Subdermal etonogestrel implants are considered to be as effective as sterilization. IUDs are inserted into the uterus and prevent fertilization; impede the ability of the sperm to fertilize the ova; and, if fertilization occurs, prevent implantation. There are two commonly used IUDs: the copper IUD and the progestogen-releasing IUD (Mirena). The progestogen-releasing IUD also acts on the cervical mucus to prevent fertilization. IUDs are very effective forms of contraception (failure rates of 0.1% [progestogen-releasing IUD] and 0.8% [copper IUD] during the fi rst year of use). Once inserted, they are effective for many years. The IUDs’ popularity in the United States decreased because of earlier reports of an association with pelvic infections. For women at low risk for sexually transmitted disease, the risk of infection is low, but exposure to sexually transmitted disease is an important factor when considering this form of contraception [34]. There is a risk of infection with IUD insertion that can be eliminated with cautious aseptic technique and antibiotic prophylaxis in high-risk women. The most common side effects after insertion are uterine bleeding, which is reported to be less with the progestogen-releasing IUD. In some instances, expulsion of the IUD can occur. For women with pulmonary hypertension or Fontan circulation, the profound and difficult-to-treat vagal reaction (bradycardia and hypotension) that can occur with device placement makes this form of contraception contraindicated. Otherwise, IUDs are considered a safe and effective alternative in women with CHD. Barrier and chemical forms of contraception Barrier methods of contraception include the male and female condoms, diaphragms, and cervical caps. Spermicides typically contain the surfactant nonoxynol-9 and come in the form of foams, gels, creams, and suppositories. To provide adequate contraception, it is recommended that spermicides be used in combination with barrier methods of contraception. The male latex condom, the most common barrier method, protects against pregnancy and sexually transmitted disease. There are no maternal risks for women with CHD who use barrier methods. However, with typical use, unintended pregnancy within the fi rst year of use occurs in 15% to 32% of women (Table 1). Because of the high failure rates, women at high risk of adverse pregnancy outcomes (discussed previously) should not use this form of contraception. Permanent forms of contraception Sterilization options include vasectomy for the male partner, tubal ligation, and insertion of intratubal stents (Essure). Because of the decreased life span of women with complex CHD and the possibility that men may outlive their spouses and want to father children with a new spouse, couples may not wish to proceed with vasectomy. Tubal ligation and intratubal stents are intended to be permanent. Because of the psychological impact, women should be counseled carefully and Choosing the Best Contraceptive Method for the Adult With CHD educated about the effective alternative options. In some cases, sterilization may be considered if the risk of pregnancy is prohibitive and other options are not acceptable to the women. Tubal ligation has reported failures of 1 in 200 in some series, but others suggest that the failure rates are much lower. Failure rates vary according to the method of surgical approach, the clinical features of the woman, and the experience of the gynecologist [35,36]. Procedures, such as tubal ligation, are associated with increased risks in some women with CHD. General anesthetics, increases in intra-abdominal pressure from abdominal insuffl ation with carbon dioxide, and instrumentation of the cervix resulting in a vasovagal response can be dangerous in patients with Fontan circulation or Eisenmenger’s physiology. There is also a risk of paradoxical emboli from carbon dioxide gas in women with right-to-left shunts. In these patients, laparoscopic complications can be avoided by performing a tubal ligation via a mini laparotomy. Nevertheless, because of the risks, these procedures should be performed by obstetricians or gynecologists with expertise in treating women with CHD. Preliminary data suggest that intratubal stent (Essure) insertion with local anesthesia may be a safer alternative for women with complex forms of CHD [37,38]. As with laparoscopic procedures, these procedures should only be performed at centers with expertise in treating CHD in high-risk women. Specific Recommendations for High-risk Cardiac Lesions Eisenmenger’s syndrome Because of the high risk of pregnancy, women with Eisenmenger’s syndrome should be counseled about the option of permanent contraception [39,40]. However, sterilization procedures are associated with maternal risks, including mortality, and should only be performed at centers with expertise in treating CHD. Mini-laparotomy or intratubal stents may be safer options in this population of women. COCs should not be used due to the risk of thromboembolism. Other safe and effective choices include intramuscular, subcutaneous, and subdermal forms of progesterone-only contraception and IUDs. Mechanical prosthetic heart valves Women with mechanical valves are at high risk for thrombotic complications; therefore, COCs should not be used in those who are not receiving adequate anticoagulation. No data are available on the safety of COCs in women treated with warfarin. Thus, some specialists are comfortable recommending COCs in women appropriately anticoagulated with warfarin. All women treated with anticoagulation must receive preconception counseling about the risks and benefits of various anticoagulation regimens. Progesterone-only forms of contraception, IUDs, and barrier methods are other alternatives. I Silversides et al. I 303 Fontan circulation COCs are not recommended for women who have had a Fontan operation because of the associated risk of right atrial thrombus and/or pulmonary embolus and the potentially disastrous effects of thromboemboli on the Fontan circulation. Because IUD insertion can result in a serious vagal response, it is not recommended in this group of women. Progesterone-only contraception is ideal. Barrier methods can be used, but failure rates need to be considered. Severe systemic ventricular dysfunction Maternal complication rates are high, and in many cases, pregnancy is contraindicated; therefore, barrier methods are not suggested. Because of the potential for thrombus formation, COCs also are not recommended in women who are not being treated with anticoagulation. The recommendation to use COCs in women taking appropriate anticoagulation may vary according to the treating physician. Progesterone-only forms of contraception and IUDs are appropriate options. Emergency Forms of Contraception In the United States, emergency oral contraception (“morning-after” pill) contains the progestin levonorgestrel. Combined forms (ethinyl estradiol and levonorgestrel) are available in other countries. Pills must be taken within 72 hours of intercourse. The predominant side effects are nausea and vomiting, but there is no risk of venous thromboembolism [22••]. In almost all instances, there are no contraindications to emergency contraception use in women with CHD; however, caution is recommended in women with current deep venous thrombosis or ischemic heart disease. The reported failure rate is less than 1%. As with other hormonal contraceptives, warfarin metabolism can be affected, and International Normalized Ratio should be monitored. Contraception Counseling Discussions pertaining to contraception options should begin early and may need to be reassessed as women age or if there are changes in maternal health. A recent study demonstrated that many young women with CHD do not use adequate methods of birth control [41]. Furthermore, several studies have shown that counseling is often done poorly or not at all [7•,42,43]. In a survey of women with CHD in our clinic, we found that only half recalled receiving specific information from a nurse or physician about birth control. Of the 31 women with contraindications to COC use in that series, 45% had used this method of birth control [7•]. In this regard, there is room for improvement. Models include formal contraception counseling programs in the CHD clinic [44], collaborative CHD and contraception clinics [43], and patient educational materials (eg, patient education conferences, websites, or educational pamphlets in CHD clinics). 304 I Congenital Heart Disease Conclusions Contraception counseling is an important aspect of care for women with CHD. It requires understanding of the risks of pregnancy and contraception efficacy and safety. Unfortunately, in many instances, contraception counseling is being done inadequately. As a consequence, some women are exposed to unnecessary risks. Improvements in patient and physician education are necessary. Efficacy and safety studies are not specifically available for women with CHD, and recommendations are based on extrapolations from studies of women without heart disease. In many cases, this extrapolation is adequate, but additional studies are still needed to help us better understand the risk in this unique population. Disclosure No potential confl icts of interest relevant to this article were reported. References and Recommended Reading Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance 1. Warnes CA, Williams RG, Bashore TM, et al.: ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation 2008, 118: e714 –e833. 2. 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