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............................................................................................................................................................................. • Comprehensive evaluation and systematic review of potential complications. • Blood tests (see section 3.4.8.8) • Education about risk reduction strategies (Table 9).

3.4.8.10 Additional considerations

• Air flight: commercial air travel is well tolerated.104,105Riskreduction strategies include avoidance of travel- and non-travelrelated stress, dehydration, alcoholic drinks, and measures to prevent deep vein thrombosis. • Exposure to high altitude: acute exposure to high altitude (>2500 m) should be avoided. Gradual ascent up to 2500 m may be tolerated. • Pregnancy: pregnancy in cyanotic patients, without PH, results in significant maternal and foetal complications and requires followup by a Pregnancy Heart Team. Oxygen saturation (>85%) and haemoglobin (<200 g/L) before pregnancy were the strongest predictors for live birth in one series.106 Pregnancy should be strongly discouraged in Eisenmenger syndrome and in cyanotic patients without PAH, but an arterial oxygen saturation <90%43 (see section 3.5.7). • IE prophylaxis: recommended in all patients (see section 3.4.6).

3.5 Additional considerations

3.5.1 Sex differences Women have been appropriately included in trials, and sex-stratified analyses are available to some extent. Data regarding sex difference on prevalence of CHD, morbidity, and mortality are conflicting.76,107110 Although no differences in mortality were noted in the CONgenital CORvitia (CONCOR) study, there were significant sex differences in morbidity (increased risk for PH for women, but lower risk for IE, aortic complications, and ICD implantation).111 Whether these differences relate to genetic and inherent biological difference, smaller body size, or other not yet defined differences warrants further exploration.112 Sex differences are important for diagnostic evaluation and clinical decision making. Although recommendations are usually not sex specific, body surface area (BSA)-normalized cut-offs of aortic and cardiac chamber dimensions may correct for smaller BSA in women.113 Personalized counselling is essential in women contemplating pregnancy, as indications for intervention in females may set lower absolute values for aortic surgery (hereditary/CHD-associated aortopathy) or for pulmonary valve replacement (PVRep) in patients with TOF.112 There are data showing different impacts for men than women for employment,114 gaps in medical care,115 and physical activity.116 Clinical evaluation, decision making, and counselling may require tailoring to the individual to help ensure equity of outcomes.

3.5.2 Adult congenital heart disease at more advanced age To date, 90% of patients with mild, 75% with moderate, and 40% with complex heart defects reach the age of 60 years.117 These proportions are expected to rise further in the upcoming decades. Hence, there is a growing population of older persons with CHD and unique healthcare needs. These older patients are characterized by more comorbidities, inherent age-related risk for arrhythmia (especially AF), accelerated ageing, acquired disease, changed responses to medication, earlier onset of geriatric syndromes (e.g. cognitive decline, immobility/falls, failure to thrive, sensory alterations), and an altered psychosocial profile.118,119

Dedicated guidelines for older patients with CHD ought to be consulted to provide adequate care to this more vulnerable patient population.120 The onset of acquired diseases starts early in life, therefore, prevention strategies ought to be implemented in the first decades of life (at paediatric cardiology).

3.5.3 Advance care planning and end-of-life care Most patients, independent of defect complexity, wish to discuss life expectancy prior to being confronted with life-threatening complications.121 Such complications may occur during high-risk interventions or reflect an uncertain illness trajectory. Timely discussion of advance care planning is a critical component of patient-centred, comprehensive care.121,122 Initiation of this demanding conversation is difficult. Unplanned hospital admissions, ICD insertion, or functional decline can serve as a trigger for such conversations. Most patients do not start advance care planning discussions on their own and wait for the provider’s initiative. The content of advance care planning discussions depends on the patient’s physical and psychological health and preferences. Initially, discussion about life expectancy and treatment preferences may be all the patient wants. With deteriorating health, a holistic assessment of the patient’s wishes and values, advance directives, nomination of a surrogate decision maker, and decisions regarding device therapies in ICD patients are required. During the process of care, active disease-specific treatment may progressively become supported and eventually substituted by palliative care. Involvement of palliative care specialists can be supportive. At any time, it is important to emphasize that active disease-specific care might continue during the advance care planning process, even near the end of life, keeping in line with patients’ preferences and goals. The role of palliative care and family support continues after the patient’s death in managing bereavement care.123 Whenever possible, family members should be involved in all steps. Patient preferences can change over time and periodic reevaluation of the patient’s wishes is necessary.

3.5.4 Insurance and employment For ACHD patients, it is often difficult to obtain life-, health-, or travel insurance, and mortgages.124126 If insurance is granted, patients frequently need to pay an extra charge or the heart defect as a preexisting condition excludes them from insurance. In general, obtaining insurance or not, and the higher fee, are mostly unrelated to the complexity of the defect, the functional status of the patient, or the prognosis,124,126 but are rather a function of the policies of insurance companies and reflect a large inter- and intra-country variation. Patients currently need to shop around, and both clinicians and patient associations may be of help. Insurability is a definite issue that should be discussed during patient counselling. Also, employment, in particular the requirements for specific professions, needs attention.127 Starting in adolescence, choices about education should be Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026