Lifelong care, lasting impact: improving outcomes in adult congenital heart disease

This editorial refers to ‘Preventing cardiovascular complications in adults with congenital heart disease: predictors and outcomes of the levels of follow-up care’, by F. Ombelet et al., https://doi.org/10.1093/eurjpc/zwaf763. Advances in the treatment of congenital heart diseases (CHD) have led to a significant reduction in mortality rates.1 Approximately >97% of patients born with CHD reach adulthood.1 Most adult CHD (ACHD) patients have mild or significant residual sequelae and associated health problems that require regular medical care.1 Longer life expectancy also means a higher risk of acquired diseases.1 Even patients with low-complexity ACHD have a high burden of cardiovascular risk factors and a significantly higher risk of cardiovascular events compared to the general population of the same age.2 In ACHD with two or more cardiovascular risk factors, the age-adjusted incidence rate of serious cardiovascular events is 29%.2 Nationwide cohort study revealed a 50% higher incidence of type 1 diabetes mellitus (T1DM) in patients with CHD compared to matched controls. The combination of CHD and T1DM was associated with a fourfold increase in mortality compared to controls with T1DM alone.3 Endocarditis continues to pose a significant risk to ACHD patients, with a cumulative lifetime risk of 8.5%.1 The risk of developing atrial fibrillation (AF) is high. A registry study showed that at the age of 42, 1 in 12 ACHD patients develop AF and 1 in 10 ACHD patients with AF develop heart failure. The most complex CHDs were associated with the highest risk.4 ACHD patients carry a particularly high risk for various arrhythmias, which occur in nearly 20% of patients.5 To date, arrhythmias are one of the most common reasons for hospital admission for ACHD patients across all levels of lesion complexity, and are risk factors for excess mortality in this population. Early detection and high-quality care, including modern ablation strategies is crucial in the reduction of excess mortality.6 Results from the large-scale Swedish National Patient Register with long-term follow-up and an age- and gender-matched control group provide evidence of an increased risk of developing ischaemic heart disease, heart failure, and stroke in ACHD.1,7,8 The results revealed that ACHD patients aged 40 years and older had a 1.6-fold increased risk of myocardial infarction (MI) compared to the control group, which remained even after adjusting for cardiovascular risk factors. The cumulative incidence of MI up to the age of 65 was 7.4% in ACHD patients compared to 4.4% in control subjects. The results show an increased risk of recurrent MI, new-onset heart failure, or death after the index MI, which was mainly due to a higher incidence of newly diagnosed heart failure.7 It was found that 7.8% of patients with ACHD developed heart failure, compared with 1.1% in the control group. The mean age at diagnosis of HF was 40.3 years in patients with CHD and 66.4 years in control subjects. The overall incidence rate for HF was 31.6 per 10 000 person-years in patients with CHD and 4.0 per 10 000 person-years in control subjects. In the group with complex CHD lesions, the incidence rate was 79 per 10 000 person-years, compared with 26 per 10 000 person-years in the group with non-complex lesions. The overall risk of HF in patients with CHD was 8.7 times higher than in control subjects. In summary, 1 in 13 patients with CHD will develop heart failure during their lifetime. This is an important risk factor that must be taken into account when treating ACHD.8 Results of a recently published systematic review identified patients with CHD to be at a 3.35-fold higher risk to develop stroke than controls. In subgroup analysis, patients with CHD were at a 4.45-fold higher risk of ischaemic stroke and 4.7-fold higher risk of haemorrhagic stroke than the control.9 In summary, these findings underscore the importance of long-term specialized care for ACHD patients. The identification and treatment of modifiable cardiovascular risk factors and early symptoms of acquired cardiovascular and other diseases is important in order to reduce morbidity and mortality and to ensure a longer life expectancy with good health and a high quality of life. All physicians involved in long-time care of ACHD must be well informed about the individual diagnosis and the importance of regular check-ups and advise patients accordingly. The care of ACHD patients is a lifelong process. Against the backdrop of the constantly growing number of patients, this requires local and national strategies for advance planning. International guidelines emphasize the importance of lifelong medical care for ACHD.1 The guideline emphasizes that the complexity of the heart defect should not be the sole criterion for assigning patients to a particular level of care. Patients with complex malformations can easily be assigned to a high level of care, but in certain circumstances, even anatomically simple malformations may require specialist care.1 It is recommended that all ACHD patients be examined once at a specialist centre so that ACHD specialists can determine the most appropriate level of care and follow-up intervals for each individual patient.1 In this context the results of the evaluation of Ombelet et al.10 published in this issue of the EACP give good insight and add valuable knowledge and important evidence to this field. Within the framework of a longitudinal study involving 10 459 patients, covering 79 065 patient years, the authors assessed the care needs of ACHD patients and the predictors and outcomes of level of specialized ACHD follow-up. The results showed that only one in five ACHD patients receive regular specialized ACHD follow-up treatment. Interestingly, this was only the case for half of the patients with complex defects. One in four ACHD received regular non-specialized ACHD aftercare. Most patients receive medical follow-up care in non-cardiac-clinics. The results are in line with findings of earlier studies, which showed follow-up care in specialized ACHD centres or cardiac centres to be underutilized.1 This confirms the significant gaps in the follow-up care of ACHD. Given the known higher risk of acquired cardiovascular disease in ACHD patients, the findings of Ombelet et al.10 are of outstanding importance. They demonstrate the significant preventive effect of regular check-ups at specialized ACDH centres or, at least, at cardiac centres. Follow-up care in a specialized ACHD facility offered significant protection against the onset of heart failure and myocardial ischaemia. Overall, cardiac follow-up care had a preventive effect on the development of myocardial ischaemia and thromboembolic events. Long-term cardiac follow-up care can have a preventive effect on certain clinical events that can significantly impair the quality of life and, in some cases, the life expectancy of these patients. While many risk factors have been addressed in the current study, there is still a shortage of presented data on arrhythmia as a major cause of mortality in this patient group, leaving the opportunity for further research. Overall, this high-quality study provides valuable results that can contribute to improving care of ACHD patients. The steadily growing number of ACHD patients who require lifelong specialized care poses a challenge for the healthcare system. The goal must be to enable ACHD patients to reach old age in the best possible health and with the highest possible quality of life. Particular attention must be paid to the transition of young patients with CHD from paediatric cardiology to adult cardiology.1 Transition programmes with special consultation hours already in adolescence and the early involvement of colleagues from adult cardiology can enable adequate follow-up care for young patients with CHD and prevent them from falling out of follow-up care in young adulthood. Specialists in internal medicine, with their broad focus and knowledge, can provide important support in the care of this patient group. In the long term, however, this can only be achieved through good cooperation with patients, who must have sufficient knowledge of their individual condition and its health consequences in order to recognize and accept the need for regular check-ups and a healthy lifestyle. The results of Ombelet et al.10 are also a wake-up call for physicians who care for this patient group, particularly specialists in ACHD, but also all cardiologists, and specialists in internal medicine who already have to deal with this growing patient group and/or will certainly have to do so in the near future. More cardiologists/internists need to specialize in treating this patient group, and this needs to be given greater consideration in the training curriculum and courses (Figure 1). Providing care for adults with congenital heart defects as a lifelong process. (CV: cardiovascular). Birna Bjarnason-Wehrens (Dr. Prof. Phd (Conceptualization [lead]; Writing—original draft [lead]; Writing—review & editing [lead])), Nicole Müller (Dr. MD PhD (Writing—original draft [supporting]; Writing—review & editing [supporting])), and Christian Paech (Dr. MD Prof Phd (Writing—original draft [supporting]; Writing—review & editing [supporting]))