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National management guidelines recommend that patients with moderate and complex congenital heart disease (CHD) receive life-long cardiac care (LLCC), guided in adulthood by an adult congenital heart disease (ACHD) specialist. However, the percentage of adult CHD patients who receive such care is quite low. Inadequate knowledge regarding LLCC may contribute to care interruption. We, therefore, sought to determine the knowledge of adolescents and young adults regarding LLCC. In this multi-center study, we administered a survey to patients 13–20 years of age with surgically repaired congenital heart disease. We assessed the understanding of both their need for LLCC and awareness of the type of recommended care providers. A total of 290/302 (96%) patients approached in the outpatient clinic setting (10 centers) agreed to study participation; mean age was 16.3 ± 2.3 years; patients were 62% male. While the need for LLCC was recognized by 78% of subjects, only 37% understood that an ACHD specialist in adulthood should guide this care. Only 37% of respondents stated that their current cardiology team had spoken to them about LLCC, but 90% wished to learn more. A substantial number of adolescents and young adults with moderate and complex CHD lack adequate knowledge about LLCC, but most have a desire to learn more about the type of care they will require in adulthood. Transition education/assessment ensuring successful transfer to adult-oriented care for this population should emphasize the importance of LLCC.

The arterial switch operation is the surgical correction of choice for patients born with d-transposition of the great arteries (d-TGA) and an intact ventricular septum. However, prematurity and very low birth weight present both technical and physiologic challenges to this approach. Furthermore, in the setting of d-TGA and an intact ventricular septum, delaying intervention results in deconditioning of the left ventricle, rendering the patient a poor candidate for the arterial switch operation. The report presents an infant born at 27 weeks gestation weighing 1.01 kg who as a newborn underwent a successful urgent balloon atrial septostomy, pulmonary artery banding, and a central shunt on day of life (DOL) 82 and the arterial switch operation on DOL 93.