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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.

Shape memory biodegradable elastomers are an emergent class of biomaterials well-suited for percutaneous cardiovascular repair requiring nonlinear elastic materials with facile handling. We have previously developed a chemically crosslinked shape memory elastomer, poly (glycerol dodecanedioate) (PGD), exhibiting tunable transition temperatures around body temperature (34-38 °C), exhibiting nonlinear elastic properties approximating cardiac tissues, and favorable degradation rates in vitro. Degree of tissue coverage, degradation and consequent changes in polymer thermomechanical properties, and inflammatory response in preclinical animal models are unknown material attributes required for translating this material into cardiovascular devices. This study investigates changes in the polymer structure, tissue coverage, endothelialization, and inflammation of percutaneously implanted PGD patches (20 mm × 9 mm x 0.5 mm) into the branch pulmonary arteries of Yorkshire pigs for three months. After three months in vivo, 5/8 samples exhibited (100%) tissue coverage, 2/8 samples exhibited 85-95% tissue coverage, and 1/8 samples exhibited limited (20%) tissue coverage with mild-moderate inflammation. PGD explants showed a (60-70%) volume loss and (25-30%) mass loss, and a reduction in polymer crosslinks. Lumenal and mural surfaces and the cross-section of the explant demonstrated evidence of degradation. This study validates PGD as an appropriate cardiovascular engineering material due to its propensity for rapid tissue coverage and uneventful inflammatory response in a preclinical animal model, establishing a precedent for consideration in cardiovascular repair applications.

I had the opportunity to see a 17-year-old male who had been referred to the pediatric cardiology clinic after his foster care intake exam resulted with an abnormal EKG. He came to the appointment with a group home staff member who knew none of his past medical history, allergies, or current prescribed medications. He was new to our health system and his electronic medical records were outdated and sparse. The answer to every question I asked this young man to ascertain his cardiovascular risk was “I don’t know.” He had no family history to share and none of his birth history or even surgical history was known to him.