p.27 抽出テキスト

............................................................................................................................................................................. made based on the possibilities to perform heavy exercise and work night shifts, and the use of specific medication such as oral anticoagulation.

3.5.5 Exercise and sports Recommendations for exercise and sports need to be based on the underlying congenital heart defect and its potential complications, the haemodynamic and EP status of the patient, and their pre-existing fitness.24 Counselling must consider the type of sport and the anticipated effort levels. In general, physicians have been over-conservative in their advice, especially as physical activity has well-documented benefits for fitness, psychological well-being, and social interaction, as well as a positive effect on the future risk of acquired heart disease; symptoms do not exclude physical activity. Dynamic exercise is more suitable than static exercise. In addition, in patients with known cardiac conditions, complications during exercise, including SCD, are rare.128 Recommendations for participation in low- to high-intensity sports are discussed in the position paper of Budts et al.24 Guidelines on elite sports in athletes with CHD are described in the 2020 ESC Guidelines on sports cardiology.129 Assessment of physical exercise capacity should be done before recommending recreational exercise or sport in order to avoid intense exercise in untrained patients. Most patients with CHD can safely engage in regular, moderate physical activity. A few conditions, such as systemic ventricular systolic dysfunction, systemic ventricular outflow tract obstruction, PH, haemodynamically significant arrhythmias, or aortic dilation, warrant more caution.

3.5.6 Non-cardiac surgery The evaluation and management of ACHD patients should follow the principles of the 2014 ESC Guidelines on non-cardiac surgery130 and consider the specificities of CHD. Factors associated with increased risk of perioperative morbidity and mortality are cyanosis, congestive heart failure, poor general health, younger age, PH, operations on the respiratory and nervous systems, complex CHD, and urgent/emergency procedures. In patients with complex CHD (Fontan, Eisenmenger syndrome, cyanotic patients), non-cardiac surgical and interventional procedures should be performed in an expert centre.130,131 Issues to consider are endocarditis prophylaxis, complications related to underlying haemodynamics, abnormal venous and/ or arterial anatomy affecting venous and arterial access, persistent shunts, valvular disease, arrhythmias including bradyarrhythmias, erythrocytosis, pulmonary vascular disease (PVD), prevention of venous thrombosis, monitoring of renal and liver function, periprocedural anticoagulation, possible need for non-conventional drug dosing, increased prevalence of hepatitis C infection because of prior procedures and remote blood transfusions and, finally, developmental disability.

3.5.7 Pregnancy, contraception, and genetic counselling 3.5.7.1 Pregnancy and contraception The majority of ACHD patients tolerate pregnancy well, but women with complex CHD have higher risks. Detailed ESC Guidelines on pregnancy and heart disease were published in 2018.43 Pre-pregnancy counselling should be provided to all women with CHD. Specialist care is best provided in a multidisciplinary team setting by the Pregnancy Heart Team. This team should have input from ACHD cardiology, obstetrics, and anaesthesia, and where necessary from other specialists, including clinical geneticists. The team should be involved in all patients with at least moderate to complex heart disease before pregnancy for timely counselling and advice during pregnancy in order to plan antenatal care, including delivery and post-partum follow-up and need for cardiac monitoring. Risk estimation should be individualized and based on the modified WHO (mWHO) classification (Table 10).43

Functional status before pregnancy, ventricular function, severity of the lesions, and history of previous cardiac events are also of prognostic value.132 CPET performed before conception can predict maternal and neonatal outcomes. A blunted heart rate response to exercise is associated with a higher risk of maternal cardiac and neonatal adverse events. Maternal mortality is 01% and heart failure complicates pregnancy in 11% of women with heart disease, with PAH being associated with the highest risks.133,134 Cyanosis poses a significant risk to the foetus, with live birth unlikely (<12%) if oxygen saturation is <85%.106 Women with cardiac disease also have an increased risk of obstetric complications, including premature labour, pre-eclampsia, and postpartum haemorrhage.135 The potential for drugs affecting the foetus should always be considered; angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor blockers (ARBs), in particular, should not be used. Women requiring oral anticoagulation deserve special attention. VKAs are teratogenic, especially at higher doses. A dose-adjusted treatment algorithm is proposed in the 2018 ESC Guidelines on pregnancy.43 The pregnancy duration and mode of delivery should be decided by the Pregnancy Heart Team, taking the CHD severity into account. Contraception should be discussed in a timely fashion with specific attention to effectiveness and safety.136 Barrier methods are safe and protect against sexually transmitted diseases, however, they only have a high contraceptive efficacy with compliant couples. Hormonal contraceptives are highly efficacious, but there are few data on their safety in the ACHD population. The combined oral contraceptive is highly effective (99.9%), but is best avoided in patients with a preexisting thrombotic risk (cyanosis, Fontan physiology, mechanical valves, prior thrombotic events, PAH), especially as there are few data to suggest that concomitant oral anticoagulation therapy will negate this risk. Progesterone-only contraceptives, on the other hand, do not pose such a high thrombosis risk, and newer preparations available for oral administration or with intrauterine implants have a high efficacy (>95%). The risk of endocarditis after insertion of progesterone-coated intrauterine devices is probably low. However, there is a risk of vasovagal reactions (5%) at the time of insertion or removal. Patients with a fragile physiology (e.g. patients with a Fontan circulation, PH, cyanotic CHD, Eisenmenger syndrome) should have their intrauterine device inserted/removed in a safe environment (i.e. in an environment with expertise in ACHD). Female sterilization or male partner sterilization should only be considered after careful discussion, with particular reference to long-term prognosis. Assisted reproduction has added risks above those of pregnancy alone, and consultation with an ACHD specialist must be performed before treatment. Superovulation is prothrombotic and can be complicated by ovarian hyperstimulation syndrome, with marked fluid shifts and an even greater risk of thrombosis.137 The risk of ovarian Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026