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Adults with congenital heart disease represent a highly diverse, ever-growing population. Optimal approaches to management of problems such as arrhythmia, sudden cardiac death, heart failure, transplant, application of advanced therapies and unrepaired shunt lesions are incompletely established. Efforts to strengthen our understanding of these complex clinical challenges and inform evidence-based practices are ongoing. This narrative review summarizes evidence underpinning current approaches to congenital heart disease management while highlighting areas requiring further investigation. A search of literature published in ‘Medline,’ ‘EMBASE,’ and ‘PubMed’ using search terms ‘congenital heart disease,’ ‘arrhythmia,’ ‘sudden cardiac death,’ ‘heart failure,’ ‘heart transplant,’ ‘advanced heart failure therapy,’ ‘ventricular assist device (VAD),’ ‘mechanical circulatory support (MSC),’ ‘intracardiac shunt’ and combinations thereof was undertaken. Application of novel technologies in the diagnosis and management of arrhythmia has and will continue to improve outcomes in this population. Sudden death remains a prevalent problem with many persistent unknowns. Heart failure is a leading cause of morbidity and mortality. Improved access to specialist care, advanced therapies and cardiac transplant is needed. The emerging field of cardio-obstetrics will continue to define state-of-the-art care for the reproductive health of women with heart disease.

Updates in the management of congenital heart disease in adult patients