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1 Introduction

With improvements in medical and surgical management, most women with congenital or acquired heart disease will reach reproductive age and become sexually active [1, 2]. As for healthy women, contraception is important for women with heart disease for various reasons. Besides preventing unintended pregnancies, it can be used for cycle control, treatment of hyperandrogenism and prevention of sexually transmittable diseases [3–5]. However, some issues, like drug interactions with cardiac medication and effects on haemodynamics, are very specific to women with cardiac disease and deserve particular attention.

Women with simple cardiac lesions often have similar pregnancy and contraceptive-related risks as healthy women, but these risks are substantially higher in women with complex heart disease [1, 2, 6]. Adequate contraception prevents unintended pregnancies and thereby the pregnancy associated risk of that particular cardiac condition. If pregnancy is undesired, contraception prevents termination of pregnancy, which, beside the emotional burden, also carries added medical risks in this population.

Contraception can also help to carefully plan and prepare women for pregnancy, which helps achieving better pregnancy outcomes. Some cardiac conditions imply an absolute contraindication for pregnancy, making effective contraception an essential part of disease management [2].

Most women with heart disease are not or, perhaps even worse, inappropriately advised on the use of contraceptives [7]. Oestrogen-containing formulations with their inherent increased risk of thromboembolic disease are still widely prescribed, while safer alternatives with better contraceptive efficacy are available [8, 9]. Each woman deserves personalised contraceptive advice, which takes her specific medical problems and personal preference into account. A team, consisting of general practitioners, cardiologists and gynaecologists, each with their particular area of expertise on the subject, is best paced to provide contraceptive advice to women with cardiac disease. In young girls with heart disease, it is important to address contraception from the menarche, which often starts around the age of 12–13; while this might seem early, up to 30 % will have sexual intercourse before the age of 15 and up to 50 % by the age of 17 [7, 8]. Usually, this event is not planned and without prior discussion with their parents or healthcare providers.

Most contraceptive advice is based on data from women without heart disease. The first consideration is the efficacy of the contraceptive method. Large studies have determined efficacy of each method, expressing efficacy as the incidence of unplanned pregnancy over a year of theoretical contraceptive use (correct use) or typical use (use in real live) as the main outcome measure [10]. The efficacy of a contraceptive method is based on its mechanism of action and is dependent on correct use. The discrepancy between theoretical and typical use is more pronounced when the method requires a substantial amount of compliance or in case of a smaller safety window (time frame in which contraceptive efficacy persists). Equally, the chances of the patient continuing to use a given contraceptive method are higher if she feels well while using that particular method [6]. Creating realistic expectations during counselling leads to higher satisfaction and helps accepting side effects that may be undesirable, as is the case with abnormal bleeding patterns [11–13]. These factors are important when striving for optimal patient adherence and long-term results.

The modified WHO classification of maternal cardiovascular risk assesses risks and consequences of pregnancy, and WHO medical eligibility criteria (WHO-MEC) for contraceptive use can be used as a guideline in women with specific conditions [14, 15]. Recommendations are made using four categories for each contraceptive method and medical condition including heart disease. Categories range from 1, where there is no restriction on the use of the contraceptive method, to category 4 where the condition represents an unacceptable health risk if the contraceptive method is used (Table 2.1) [15, 16]. These guidelines are regularly updated based on available evidence or on expert opinion if the evidence is lacking.

Table 2.1 MEC categories for contraceptive eligibility [15]
Full size table

2 Short-Acting Contraceptives

Barrier contraceptives (male condoms, female condoms, diaphragms), coitus interruptus and fertility awareness-based methods (calendar methods, temperature curves) are considered insufficient as failure rates are substantial [10]. While 1 year of typical use of male condom as a sole form of contraception is associated with pregnancy rates up to 18 %, this method still has its value in protection against sexually transmitted diseases (STDs) or as an additional method of contraception when necessary.

Combined oestrogen and progesterone contraceptives are successful in preventing pregnancies, but their efficacy is highly dependent on correct use [10, 17].

They consist of different combinations and doses of either ethinylestradiol or estradiol along with a progestogen. The progestogen component varies and is classified by generation (1–4), each with specific characteristics. They are usually delivered in the form of a daily oral tablet but can also be administered transdermally (weekly patch) or through the vaginal mucosa (3-week vaginal ring), thereby avoiding the hepatic first-pass effect. This method acts on three different levels. Ovulation is inhibited; cervical mucus is thickened, preventing sperm penetration; and endometrial receptivity is altered, preventing implantation.

An advantage of the combined oral contraceptives (COC) is improved cycle control, with lighter, less painful and more regular periods. Traditionally, they are used for 3 weeks, after which a withdrawal bleed is induced, mimicking the natural cycle. However, the frequency of withdrawal bleeds can easily be reduced if desired by prolonged or continuous use [3, 4, 18]. Combined oral contraceptives can help in managing ovarian cysts, PCOS (polycystic ovary syndrome), or relieve symptoms of hyperandrogenism [5].

Of concern is the two- to sevenfold increased risk of venous thrombosis associated with any type of COC. This is mainly induced by the oestrogen component which elevates the level of circulating vitamin K-depending clotting factors, plasminogen and platelet adhesion and reduces the anti-thrombin levels [16]. While this increase in risk is substantial and consequences are important, one should bear in mind that the absolute risk remains low in the range of 8–10/10,000 women-years exposure.

COC also increase the risk of arterial thrombosis and dyslipidaemia and may induce hypertension through an increase in the circulating blood volume [16, 19, 20].

Therefore, COC are not recommended or are contraindicated when the cardiac condition increases the risk of hypertensive, ischaemic or thrombogenic complications or when the consequences of such complications are more severe [9, 15]. As such, COC are not suitable in women with (a history of) ischaemic heart disease, hypertension and additional thrombogenic factors or women with atrial flutter or fibrillation [9, 21–23]. In women with potential right to left shunts (cyanotic heart disease, unoperated ASD), venous thrombosis might result in paradoxical embolism and stroke.

In women with complicated valvular disease or Fontan circulation, the risks and consequences of thrombogenic complications (mechanical valve thrombosis, pulmonary embolus) are such that this form of contraception is also contraindicated.

While some controversy exists on whether the increased thrombogenic risk of COC persists when anticoagulant drugs are used, most guidelines still recommend against the use of COC in these cases. An exception might be made in women on oral anticoagulants in whom ovulation bleeding leads to a massive life-threatening haemoperitoneum. In these rare cases, COC is the method of contraception that most effectively suppresses ovulation.

Also one should be aware of the potential influence of both progestogens and oestrogens on the metabolism of warfarin, requiring more frequent INR monitoring when COC are initiated in women on established oral anticoagulation therapy [24]. Alternatively, some drugs used in certain cardiac conditions may reduce the efficacy of combined oral contraceptives. Bosentan, which is used in management of pulmonary hypertension, accelerates the metabolism of contraceptive steroids, requiring additional contraceptive measures like a condom [9, 16, 25].

COC may induce some fluid retention, but there is no evidence that contraceptive steroid hormones affect cardiac function directly. Still, combined oral contraceptives are contraindicated in women with a reduced ejection fraction after a myocardial infarction, especially in the presence of other risk factors, like smoking and hypertension.

There is no formal contraindication for COC use in women with isolated arrhythmias (isolated supraventricular or ventricular extra beats, AVNT or VT in long QT syndrome).

Contraceptive pills, containing progesterone only, prevent sperm penetration by cervical mucus thickening and prevent implantation by reducing endometrial receptivity. If used in a higher dose, ovulation may be inhibited [17, 26]. No increased risk of thrombosis in women using progesterone-only pills is reported [27]. These pills can contain different types of progestogens with varying efficacy and safety window and are commonly used as additional contraception in lactating women. Desogestrel (Cerazette)-containing tablets are effective in inhibiting ovulation with a safety window of 12 h and have similar efficacy as the combined oral contraceptive pill [9, 16, 25, 26, 28]. They are therefore the only pills of this type recommended in women with (severe) cardiac disease.

3 Long-Acting Reversible Contraceptives (LARC)

Long-acting reversible contraceptives consist of intrauterine devices (IUDs), subdermal implants and intramuscular injections of DMPA (depot-medroxyprogesterone acetate). By eliminating patient adherence, the efficacy of LARC is excellent, even exceeding sterilisation [10, 29]. Two models of IUDs exist: levonorgestrel-releasing intrauterine system (LNG-IUS) and copper-bearing IUDs. LNG-IUS induces endometrial atrophy and causes formation of a cervical mucus plug, impeding sperm penetration and implantation over a 5-year period. Copper is toxic to the ova and sperm and induces endometrial inflammation which prevents implantation, offering contraception for 10 years [30]. IUDs can be used in both nulliparous and multiparous women. IUDs can be inserted at any point of the cycle or directly postpartum [31, 32]. Insertion during menstruation offers immediate contraception and is facilitated by the physiological opening of the cervical ostium.

Fertility rapidly returns upon removal [29]. Uterine perforation and spontaneous expulsion are rare but are recognised complications. Importantly, IUDs are devoid of increased thrombotic risk. LNG-IUSs increase the levels of high-density lipoprotein, making it a good option for women with ischaemic heart disease and hyperlipidaemia [16, 20]. They often reduce menstrual blood loss, sometimes resulting in complete amenorrhea, but the bleeding pattern may become irregular in some women. It can help to control menstrual bleeding problems which occur more often in women using anticoagulants [33–35]. While the natural menstrual cycle is maintained with copper IUDs, an increase in blood loss and discomfort are often observed with menstruation.

The risk of pelvic infection is increased during the first 3 months after insertion, and transient bacteraemia has been documented at replacement but is rare in uncomplicated insertion or removal [36, 37]. Recent studies and guidelines do not recommend the standard use of prophylactic antibiotics to prevent either PID (pelvic inflammatory disease) or endocarditis at insertion [38–43]. Nevertheless, since the introduction of this guideline which limited the indications for antibiotic prophylaxis, an increase in prevalence of endocarditis has been observed in women with cardiac disease in general [44].

As such, the administration of prophylactic antibiotics (ampicillin 2 g and gentamicin 80 mg given intravenously 1 h before IUD insertion) may be considered in women at high risk of endocarditis [45]. Insertion of an IUD requires extra caution in women with pulmonary hypertension or Fontan repair. Vasovagal reactions may occur upon insertion due to pain and cervical manipulation, which is potentially dangerous in these women [9, 16, 21, 25, 46–48]. Insertion is therefore best performed with pain relief (e.g. IV opioids), cardiovascular monitoring and anaesthetic support on standby, to prevent or adequately anticipate the consequences of a vagal reaction [48].

Subdermal implants, containing etonogestrel (Implanon) or levonorgestrel (Norplant), gradually release their progesterone over the course of 3 years, inhibiting ovulation and altering cervical mucus and endometrial receptivity. Subcutaneous insertion just below the medial groove between the biceps and triceps after local anaesthetic infiltration makes it a simple procedure, leading to contraception with an efficacy exceeding that of sterilisation [10]. Failure rates are low due to the elimination of the “patient adherence” factor. The newer easy-to-use insertion devices and incorporation of radioactive filaments prevent failure due to unnoticed loss and facilitate retrieval in the rare occasion of spontaneous migration of the device [29, 49]. The simplicity of insertion (no vasovagal reaction accompanying cervical manipulation), high efficacy and absence of increased thrombogenic risk make that subdermal implants are an excellent option for women with mechanical valves, pulmonary hypertension or Fontan repair [9, 11, 16, 21, 25, 29, 50, 51]. Women often experience a reduction in vaginal blood loss in terms of amount, frequency and duration of bleeding. Endometrial atrophy, which may occur after prolonged exposure to progesterone, may cause vascular fragility, occasionally leading to irregular and unpredictable bleeding or spotting [29, 52–55]. As previously mentioned, additional contraceptive measures should be taken in women using Bosentan.

Depot-medroxyprogesterone acetate offers reliable contraception if used every 13 weeks, with a grace period of 4 weeks, but effects usually last much longer. DMPA injections can induce intramuscular haematoma, but even in women on anticoagulants, this rarely seems to be of clinical significance [9, 16, 21, 25]. Uncertainty regarding a potential thrombogenic effect remains, as evidence is conflicting [27, 51, 56, 57].

4 Sterilisation

Sterilisation is a good option for patients with a contraindication for pregnancy or for couples with a completed family [9, 21, 25]. The role of sterilisation has decreased as other highly reliable and reversible contraceptive methods have emerged. Sterilisation does not offer non-contraceptive benefits like reduction of blood loss or cycle control.

Although sterilisation is considered a definitive method of contraception, reversal surgery may restore fertility in some cases. Laparoscopic tubal ligation and hysteroscopic insertion of intratubal stents are all considered good options, but these procedures carry risks. Laparoscopic tubal ligation is performed under general anaesthesia and requires a pneumoperitoneum with elevated intra-abdominal pressures and is therefore contraindicated in the presence of pulmonary hypertension or Fontan repair. Alternatively, an open or laparoscopic procedure with minimal inflation can be considered. Perioperative cessation of anticoagulation increases risk of thrombosis, and anaesthesia and the procedure itself create an inherent risk of haemorrhage and infection [9, 21, 25, 58]. Sterilisation at the time of Caesarean section is associated with a slightly higher regret and failure rate as compared to a laparoscopic procedure [59, 60]. The psychological impact of definitive contraception, even in the case of severe heart disease with an absolute contraindication for pregnancy, should be taken into consideration. Vasectomy imposes no risk for the woman, but in case of a cardiac condition with a high chance of early demise, vasectomy may lead to male fertility problems in a future relationship.

Hysteroscopic tubal occlusion can also be achieved using various techniques. It does not require a skin incision or abdominal entry. Technical developments mean that hysteroscopes are now very thin and insertion devices allow these procedures to be performed with limited discomfort in an outpatient setting. However, pain relief (e.g. IV opioids), cardiovascular monitoring and anaesthetic support on standby remain necessary in women with pulmonary hypertension or Fontan repair, to prevent or adequately anticipate the consequences of a vagal reaction [48]. Currently, the most commonly used method consists of coils (Essure), which are inserted in the proximal part of the fallopian tube and induce fibrosis and occlusion. This method was approved in Europe and by the FDA and seems to have a low complication and low failure rate after tubal patency is assessed by ultrasound or hysterosalpingography after 3 months [61]. Nevertheless, recently some controversy on its safety emerged after the reports of numerous adverse events by women. This resulted in serious concerns about the risk of chronic pain, device migration and contraceptive efficacy [21, 25, 62–65]. Until these concerns are resolved, it is prudent to avoid this method.

5 Emergency Contraception

Emergency or postcoital contraception is essential in women not using any method or in whom a contraceptive method was not used appropriately (e.g. irregular pill intake, condom failure). The method with the highest contraceptive efficacy (failure rate of 0.09 %) is the insertion of a copper IUD within 120 h after intercourse.

A single dose of 1.5 mg levonorgestrel inhibits follicular growth or rupture and is therefore only effective before ovulation. It has a failure rate of 1.1 % if taken within 72 h after unprotected coitus. Obesity (BMI >30 kg/m2) increases the failure rate, and a copper IUD should therefore be preferred in obese women requiring emergency contraception. Drug interactions with warfarin are reported, requiring close control of the INR [24].

Mifepristone 25 mg or ulipristal acetate 30 mg can be taken up to 120 h after unprotected intercourse. The superior efficacy of mifepristone and ulipristal over levonorgestrel may arise from ovulation inhibition and its additional postovulatory mechanism of action in inhibiting endometrial receptivity and tubal contractility. Proper counselling on reliable ongoing contraception should be offered at the time of provision of emergency contraception.

Conclusion

Contraceptive counselling in women with cardiovascular disease should begin early, preferably soon after menarche. Choices should be made by a multidisciplinary team, based on impact of pregnancy, safety of use, associated benefits of the contraceptive and the individual’s preferences. Efficacy and ease of use are important factors for adequate contraception. Continuation rates are highest when the patient feels well using a particular method. Progestogen-only long-acting reversible contraceptive methods are a good option for patients with cardiovascular disease.