Paeds · cardiology
Adult congenital heart disease transition
Also known as ACHD transition · Transition of care in congenital heart disease · Grown-up congenital heart disease (GUCH) · Paediatric to adult cardiology transfer · Lifelong congenital heart disease follow-up · Transition readiness in congenital heart disease
Fellowship guide to the transition of the adolescent with congenital heart disease from paediatric to lifelong adult ACHD care: why transition is a planned process and not a single transfer clinic; how to build and measure transition readiness from early adolescence using tools such as the TRAQ; the anatomic-physiologic complexity classification that sets the surveillance interval and the care setting; the lesion-specific residua and sequelae that make follow-up lifelong even in the well-feeling patient; the danger of loss to follow-up and the gaps-in-care evidence; contraception matched to thrombotic and haemodynamic risk and pre-pregnancy counselling built on the modified WHO risk classes; the narrowed indications for infective endocarditis prophylaxis in the highest-risk lesions; and the 2018 AHA/ACC and 2020 ESC guidance that anchors ANZ practice.
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Red flags
- A young person with moderate or complex congenital heart disease who has dropped out of specialist care is a red flag — loss to follow-up predicts a late, preventable, advanced complication and mandates active recall, not passive waiting
- New palpitations, exertional breathlessness, syncope, or falling exercise tolerance in a repaired lesion signal atrial arrhythmia, ventricular dysfunction, or a failing conduit or valve and need urgent ACHD review
- Pregnancy in Eisenmenger syndrome, severe pulmonary arterial hypertension, a systemic right ventricle with poor function, or severe left heart obstruction carries very high maternal mortality (modified WHO class IV) — pregnancy is contraindicated and effective contraception is a priority
- Combined oestrogen-containing contraception is dangerous in lesions with a thrombotic or Fontan circulation, cyanosis, pulmonary hypertension, or prior thromboembolism — reach for progestogen-only or a long-acting reversible method
- The highest endocarditis-risk lesions — a prosthetic valve or prosthetic repair material, prior endocarditis, unrepaired cyanotic disease, and the first six months after prosthetic repair — need antibiotic prophylaxis before dental procedures and any breach of infected tissue
Life stages
Care settings
Clinical exam formats
Board mappings
- Cardiology
- Adolescent medicine and transition of care
- Chronic disease and continuity of care
- Paediatric Cardiology — adult congenital heart disease and transition
- General Paediatrics learning goal — plan a structured transition to adult services
- Contraception, pregnancy, and endocarditis prophylaxis in congenital heart disease
- Clinical Applications
- Adolescent health and transition
- Long Cases
- Communication scenarios
- Chronic disease management
- Cardiology: plans lifelong follow-up and transition for the child with congenital heart disease
- Adolescent health: leads a structured transition to adult services
- Health promotion: contraception and pregnancy counselling in chronic disease
- Foundation of Practice (FOP)
- Applied Knowledge in Practice (AKP)
- Transition and adult congenital heart disease
- Clinical
- History
- Communication
- Transition planning and shared decision-making
- General Pediatrics Content Outline — Domain 9: Cardiology
- Subspecialty: Pediatric Cardiology — adult congenital heart disease
- Adolescent medicine — transition of care
- Patient Care 4: Clinical Reasoning and longitudinal care
- Medical Knowledge 1: congenital heart disease across the lifespan
- Systems-Based Practice 1: care coordination and transition to adult services
- Interpersonal and Communication Skills 1: counselling the adolescent and family
- Medical Expert
- Communicator
- Collaborator
- Pediatrics: Core EPA — plan transition to adult care for youth with chronic disease
Overview & Definition
Picture a seventeen-year-old who was born with tetralogy of Fallot, repaired as a toddler, and followed all his childhood at the children's hospital. He feels completely well, plays club soccer, and has just finished school. His paediatric cardiologist retires, the last clinic letter says "review in two years," and no adult appointment is ever booked. Four years later he arrives in an emergency department with palpitations and breathlessness, in atrial flutter, with a dilated right ventricle and free pulmonary regurgitation that a timely valve intervention could have pre-empted. That silent gap between paediatric discharge and adult re-engagement is the problem this topic solves. [3] [4]
ACHD transition is the planned, purposeful, staged preparation of the adolescent with congenital heart disease for a move to adult-oriented, lifelong cardiac care. It is deliberately distinguished from transfer, which is the single administrative event of the first adult appointment. Transition is the years of work on either side of that event: building knowledge, independence, and a therapeutic relationship so that the transfer does not become a cliff edge. [3] [1]
This matters because congenital heart disease is now overwhelmingly a condition of adults. Survival of even complex lesions into adulthood is the norm, and adults with congenital heart disease outnumber children with it. The population that paediatric cardiology once cured and discharged is instead a growing cohort needing decades of specialised surveillance, and the transition is the hinge on which that lifelong care turns. [5] [13]
References14ShowHide
- [1]Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease J Am Coll Cardiol, 2019.PMID 30121239
- [2]Baumgartner H, De Backer J, Babu-Narayan SV, Budts W, Chessa M, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J, 2021.PMID 32860028
- [3]Sable C, Foster E, Uzark K, Bjornsen K, Canobbio MM, et al. Best practices in managing transition to adulthood for adolescents with congenital heart disease: the transition process and medical and psychosocial issues: a scientific statement from the American Heart Association. Circulation, 2011.PMID 21357825
- [4]Mackie AS, Ionescu-Ittu R, Therrien J, Pilote L, Abrahamowicz M, Marelli AJ Children and adults with congenital heart disease lost to follow-up: who and when? Circulation, 2009.PMID 19597053
- [5]Marelli AJ, Ionescu-Ittu R, Mackie AS, Guo L, Dendukuri N, Kaouache M Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation, 2014.PMID 24944314
- [6]Gurvitz M, Valente AM, Broberg C, Cook S, Stout K, et al. Prevalence and predictors of gaps in care among adult congenital heart disease patients: HEART-ACHD J Am Coll Cardiol, 2013.PMID 23542112
- [7]Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J, Blomström-Lundqvist C, Cífková R, et al. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J, 2018.PMID 30165544
- [8]Canobbio MM, Warnes CA, Aboulhosn J, Connolly HM, Khanna A, et al. Management of Pregnancy in Patients With Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association. Circulation, 2017.PMID 28082385
- [9]Silversides CK, Grewal J, Mason J, Sermer M, Kiess M, et al. Pregnancy Outcomes in Women With Heart Disease: The CARPREG II Study. J Am Coll Cardiol, 2018.PMID 29793631
- [10]Roos-Hesselink JW, Ruys TP, Stein JI, Thilén U, Webb GD, et al. Outcome of pregnancy in patients with structural or ischaemic heart disease: results of a registry of the European Society of Cardiology. Eur Heart J, 2013.PMID 22968232
- [11]Wilson W, Taubert KA, Gewitz M, Lockhart PB, Baddour LM, et al. Prevention of infective endocarditis: guidelines from the American Heart Association Circulation, 2007.PMID 17446442
- [12]Habib G, Lancellotti P, Antunes MJ, Bongiorni MG, Casalta JP, et al. 2015 ESC Guidelines for the management of infective endocarditis Eur Heart J, 2015.PMID 26320109
- [13]Bhatt AB, Foster E, Kuehl K, Alpert J, Brabeck S, et al. Congenital heart disease in the older adult: a scientific statement from the American Heart Association. Circulation, 2015.PMID 25896865
- [14]Sawicki GS, Lukens-Bull K, Yin X, Demars N, Huang IC, et al. Measuring the transition readiness of youth with special healthcare needs: validation of the TRAQ J Pediatr Psychol, 2011.PMID 20040605