Congenital heart defects – how long do we monitor them?
DOI:
https://doi.org/10.13112/pc.1083Keywords:
HEART DEFECTS, CONGENITAL; HEART SEPTAL DEFECTS, ATRIAL; HEART SEPTAL DEFECTS, VENTRICULAR; TETRALOGY OF FALLOT; LONG-TERM CARE; ADULTAbstract
To date, the worldwide prevalence of congenital heart defects (CHD) is around 9 per 1000 live births, with significant geographic variation. While the prevalence of major CHD is declining in many developing countries due to fetal screening and pregnancy termination, the overall prevalence is globally increasing. Due to medical, surgical, and technological advances over the past decades, more than 90 % of individuals born with CHD now survive to adulthood. As a result, the prevalence of adult CHD (GUCH) in the community has increased and now far exceeds the number of children with PSG.
CHD can be classified as mild (eg, bicuspid aortic valve, ASD (small or corrected), pulmonary stenosis), moderate (eg, PAPVR, aortic stenosis, coarctation of the aorta, Ebstein anomaly, tetralogy of Fallot) and severe (eg, univentricular heart with Fontan circulation, pulmonary atresia, heterotaxy, common arterial trunk).
As CHD patients approach adulthood, they require transfer to GUCH care. This transfer should be preceded by a preparatory transitional phase, which continues into adulthood according to the patient's needs. Special healthcare organizations and training programs are needed to meet the needs of these patients. Importantly, caring for GUCH is a lifelong process and also requires advanced care planning strategies. Patients need to be stratified into 3 groups: (i) patients requiring care exclusively in a specialist center, (ii) patients for whom shared care can be established with appropriate general adult cardiology services, and (iii) patients who can be treated in non-specialized clinics (with access to a specialist if necessary).
The complexity of the heart defect should not be the only criterion for assigning patients to a particular level of care. Although patients with complex defects can easily be assigned to a high level of care, even anatomically simple defects may require specialist care under certain circumstances (e.g. atrial septal defect with pulmonary arterial hypertension). It is therefore recommended that all GUCH patients are seen once in a specialized center, which allows GUCH specialists to determine the most appropriate level of care and follow-up intervals for each patient. With the growing population of adults with CHD, more and more patients will first present to general cardiologists for acute conditions such as arrhythmia, heart failure, or endocarditis. Special attention should be attributed to patients who develop arrhythmia after Fontan correction, as even supraventricular arrhythmias are not well tolerated.
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