Adult congenital heart disease transition — formative SAQs

SAQ 1 (10 marks)

A 17-year-old man with tetralogy of Fallot, repaired at age two, attends a paediatric cardiology clinic for what will be one of his last paediatric visits. He feels well, plays club soccer, and has no symptoms. His most recent echocardiogram shows moderate pulmonary regurgitation with mild right-ventricular dilation. He can name his condition only as "a hole in the heart fixed as a baby" and has never booked his own appointment. ^{[1] [3]}

1. Define transition and explain why it differs from transfer, and outline how you would structure his transition over the remaining paediatric visits. (4) ^{[3] [14]}
2. State the surveillance he needs as an adult with repaired tetralogy of Fallot, the complexity grade and follow-up interval, and why lifelong follow-up is essential even though he feels well. (3) ^{[1] [2]}
3. He is at risk of loss to follow-up. List the risk factors for loss to follow-up in this population and the practical measures that reduce it. (3) ^{[4] [6]}

Model answer — SAQ 1

(1) Transition versus transfer, and the structure (4). Transition is the planned, staged, purposeful preparation of the adolescent and family for a move to lifelong adult care; transfer is the single administrative event of the first adult appointment. Transition is the years of work around that event. I would start early, teach him to describe his own anatomy and operation, hand over responsibility for knowing his medications and booking appointments, see him alone for part of each visit, cover adult-life topics (contraception, exercise, alcohol, careers), measure his readiness with a validated tool such as the TRAQ, prepare a written health summary, identify and book a named adult ACHD clinic, and confirm attendance rather than assuming a referral equals engagement. ^{[3] [14]}

(2) Surveillance and why lifelong (3). Repaired tetralogy of Fallot is a moderate-complexity lesion needing review at an ACHD centre every one to two years. Surveillance includes a twelve-lead ECG and oxygen saturation, transthoracic echocardiography, and cardiac MRI to quantify right-ventricular volumes and pulmonary regurgitant fraction, which time pulmonary valve replacement; Holter monitoring for arrhythmia and cardiopulmonary exercise testing track decline. Lifelong follow-up is essential because no repair is a cure: chronic pulmonary regurgitation slowly dilates and impairs the right ventricle and generates atrial and ventricular arrhythmia, and this progresses silently — the well-feeling patient is exactly the one in whom timely intervention is missed. ^{[1] [2]}

(3) Loss to follow-up (3). Risk factors include a transfer that is never completed, feeling well, geographic distance and cost, poor understanding of the diagnosis, mental-health and neurodevelopmental difficulty, socioeconomic disadvantage, and no named adult clinic. Gaps peak in adolescence and young adulthood. Practical measures: a structured transition programme, a portable written health summary, a booked and confirmed first adult appointment, joint or overlapping clinics, telehealth-linked shared care for rural and remote patients, and active recall of anyone who lapses. ^{[4] [6]}