Young people with congenital cardiac conditions: transition and medical transfer from paediatric to adult care

Initiation and early phase 

• Age 12-14 (identified from the Scottish Paediatric Cardiac Services (SPCS) database) if appropriate for current health status, emotional maturity and cognitive functioning. Provide additional educational and psychological support if needed. 
• The concept of transition is introduced to patient and family/carers. 
• A transition introductory letter and information regarding nurse led transition clinic are provided (Appendix 2).  

Middle stage Transition 14-16 Years  

• Patients are identified via the SPCS database and referred to an appropriate adult care facility by their Paediatric Cardiologist 
• Patients are offered an appointment in the Transition Nurse-led Young Person’s Clinic at RHC.  
• The number of subsequent appointments will be individualised to the individual patient and family needs.  
• A typical appointment is 45-60 mins long (including administration time). Opportunity should be given to see the family/carers as a unit but also to see the patient alone.  
• A typical appointment will focus on:  
  • The patient’s understanding of their condition
  • Understanding of the nature of previous interventions 
  • The importance of specialist life-long follow-up and future interventions 
  • Medication (why they are needed, dosage and frequency) 
  • Understanding the issues around prevention of endocarditis  
  • General lifestyle issues - diet, exercise, alcohol, smoking, use of recreational drugs 
  • Education and career plans  
  • Family planning and pre-pregnancy counselling advice 

The Paediatric Cardiac Nurse Specialist conducting the clinic will complete the transition checklist (Appendix 3) and upload this to the patient’s electronic medical record on Clinical Portal.  

Late stage Transition 16+   

• During this phase formal Transfer of medical care to the adult team occurs. 
• Moderate and complex patients are invited to a nurse led clinic with the Adult Congenital Transition team (virtual or face-to-face). 
• The adult nurse-led clinic builds on the concepts described above in an age appropriate manner. This includes future health care trajectory and discussion about how Adult health care is organised using shared care approach. The importance of lifelong follow-up and advice on how to access services are be included. Other topics include consent and guardianship and DVLA advice if appropriate.  The Adult Congenital Cardiac Nurse Specialists will document these consultations by dictated letter.  

Ready-Steady-Go: The Care plans will have a “Ready, Steady, Go” approach. By the time of transfer this will identify areas that are “ready to Go” and flagging areas that require more support.  

Patients aged 15 and over are invited to the Transition Open Day in the Golden Jubilee Hospital. These will continue to develop to ensure that needs of patients with multiple health or additional learning needs are met. A hybrid platform combining face-to-face and virtual settings, appears to be optimal. These evening educational events for patients, families and carers take place 1-2 times per year.  

A minority of young patient have very particular health challenges or will be transferring their care during a period when there are active ongoing treatment decisions to be made. This includes the less than 5% patients on a clear palliative care track. The transition and transfer process will be tailored to the individual and part of this process will involve a full Clinical Case Conference jointly held between the paediatric and adult congenital teams. These meetings have the additional benefit of ensuring cross-site learning, continuity of approach and dissemination of best practice. It is a bidirectional process between the two teams. 

Patients will be encouraged to engage with specific web-based Transition resources and will be introduced to the charities supporting patients with congenital heart disease. The development of more “teen-friendly” methods of communication and care delivery constitute part of the remit of the transition team.

The transfer of medical care process for congenital heart disease patients starts when the patient is referred by the Paediatric Cardiologist to the ACHD service. This will occur between the ages of 16 to 18 years old depending on the agreement between the family and Paediatric Cardiologist, by means of a referral letter to the SACCS Consultant Team who will ensure the patient is given the appropriate follow-up. The referral letter and clinical documentation pack will be provided to the adult team.  

For mild or simple lesions the referrals are made by the Paediatric cardiologist to the local Adult Cardiologist with Expertise in Adult Congenital Heart Disease copying the letter to the SACCS for database. In addition patients transferring from local services should be referred to the Local Cardiologist with an interest in ACHD (Appendix 5). 

Transition Referral Pack should contain a letter with a summary of past medical history and interventions, current status including medication and most relevant recent clinical investigations results. Enclosed should be Surgical Operation notes, most recent cardiac catheter, ambulatory and exercise ECGs, cardiopulmonary exercise test and cross-sectional imaging reports, where they are not easily accessible within the shared electronic patient record (Regional Portal). 

The Paediatric Cardiac Nurse Specialist leading on transition will ensure continuity of care with their SACCS counterparts. This second handover will minimise the loss to follow-up. 

Medical transfer of care for patient with non-congenital heart disease is beyond the scope of this document (Appendix 8)

There is the ongoing need to build a Scotland-wide congenital heart disease database for all patients from diagnosis through to adult care. The Intra NHS Scotland Sharing Accord for clinical information will facilitate the sharing of such information and minimise barriers to its development.  

The aim of a transition service is to empower the young person and provide them with the necessary skills to take over responsibility for their healthcare needs. Transition services should be designed with a family-centered approach working with all members of the family, as stakeholders, to prepare the young person for this process and for transfer. This process starts in the pediatric services at approximately 12 years of age (this may be adjusted according to the individual patient’s care requirements) and continues within the adult services long after the patient’s medical care has been transferred. In practice, this support continues until the time of physiological brain maturation at or around the age of 25 years and is sometimes described as “Emerging Adulthood”.^{2, 3}    

Several studies have described a high proportion of patients having significant lapses in care during adolescence.^6,7 Patchy provision of specialist transition and tailored adult services contributes adversely impacts on outcomes.⁵ This is true even for patients with complex congenital heart disease. Sub-optimal transition is associated with an increase in lost-to-follow-up rates and to poorer psychological adaptation to chronic health issues. In contrast, the establishment of transition care pathways improves continuity of care and may positively influence other outcome measures such as hospitalisation.⁸  

The leading principle of transition being a process starting when the patient is still well within the paediatric age group, and completing in adult services in early to mid-twenties, distinctly separate from the event of medical care transfer between paediatric and adult services, underpins the guidance document below. 

Benefits of effective transition  

The importance of an effective transition programme goes beyond simply ensuring medical continuity of care. Knowledge deficits, even in long-term clinic attendees are frequent in young people with heart disease, impacting on successful transition to adulthood.¹⁰ Delay in achieving psychosocial milestones is also common in chronic illnesses. There is, therefore, a pressing need for all patients to be empowered to understand their heart condition and make informed decisions about their future health, occupation and lifestyle. Effective transition needs to employ multiple communication modalities to conform to individual preferences, as no two young people are the same.  

The transition process has to address the evolving needs of parents, enabling them to be good advocates for their children with appropriate levels of protectiveness and support. Any discrepancies between professional and parental perspectives¹², in addition to those between parent and adolescent, need to be acknowledged and sensitively negotiated. For some patients, the ability to attend clinic appointments without their parents positively affects attendance⁵. Promoting active involvement of the young patient during the consultation helps foster an environment of trust and enables parents to gradually withdraw from being an equal stakeholder and evolve into a more supportive role.  

Transition and Transfer Model  

A recent multi-centre study¹³ has demonstrated the effectiveness of a nurse-led transition programme, working in parallel with and acting in a complementary way to the medical congenital cardiac care. The primary focus of the nurse-led Transition Service is patient and family education and empowerment of a young person to gradually assume responsibility for their own healthcare decision and function in an adult environment. 

Key principles as outlined in NICE Guidelines¹⁴ 

• The transition process should have the young person’s interests at the centre and should serve to meet the needs of the individual involved 

• Transition support must be appropriate in terms of developmental and emotional maturity, social and personal circumstances 

• Both the young person and caregiver, where appropriate, should be involved in the planning and decisions about the transition process. The aim of this is that the young person takes responsibility for their own health and has the skills to make informed choices and decisions about their health and wellbeing both now and in the future.  National Service Framework for Children, Young people and Maternity Services (2004), Standard 4¹⁵ 

Initiation Phase: Transition introductory letter (Word doc)

1. Data Manager 
   1. Collating information and selecting cohort of patients who have reached the age for commencement of transition process 
   2. Initial categorising of diagnoses in relation to severity of condition 
      
      
2. Secretarial and Outpatient Clinic Administrative Support Staff 
   1. Sending out relevant letters regarding preparation for transition  
   2. Organising and supporting transition clinics  
      
      
3. Paediatric Cardiologist 
   1. Assessment, diagnosis and disease severity categorisation of condition  
   2. Assess suitability for and facilitate referral to Transition nurses  
   3. Supporting the Transition nurse regarding lifestyle advice  
   4. Identify and coordinate multidisciplinary team discussions for patients with complex needs in preparation for transfer 
   5. Ongoing medical care until patient seen in the adult service  
   6. Formal written referral to the adult services for transfer of care including the appropriate documentation.  
      
      
4. Adult Congenital Cardiologist 
   1. Triaging +/- accepting the referrals from the Paediatric Cardiologist.   
   2. Participation in multidisciplinary team discussions regarding patients with specific complex transition and transfer needs  
   3. Supporting the adult Transition Nurse Specialists  
      
      
5. Paediatric and Adult Congenital Cardiac Nurse Specialist 
   1. Assessment for suitability for process of transition 
   2. Coordinate and deliver Transitional care through nurse led clinics 
   3. Disease and lifestyle education 
   4. Assisting the young person navigate services 
   5. Be the link between the young person and the other health professionals involved in their care 
      
      
6. General Practitioner 
   1. Provides day to day care for medical needs 
   2. Refers to specialist services as required 
   3. Continuity of care for the young person through the transition process into adulthood 

Inherited cardiovascular conditions (ICC) 

Regional ICC services should be involved in the transition of all patients with a confirmed cardiac genetic diagnosis (cardiomyopathy, arrhythmia, neuromuscular, mitochondrial, aortopathy) to ensure care is consistent within a family and reproductive options addressed 

Transition of patients who are part of the screening program for ICCs needs to be reviewed on a patient-by-patient basis, whether this is necessary and type of screening 

• Marfan Syndrome and other connective tissue disorders should be referred to their local Adult Cardiology clinic unless they are anticipated to require an intervention in the near future – then transferred to SACCS, GJNH 
• Inherited Cardiomyopathies – local Adult Cardiologist and/or ICC Service 
• Inherited Arrhythmia Syndromes – local Adult Cardiologist and/or ICC Service  

• Neuromuscular disorders: local Adult Cardiologist and/or ICC service 

Non-inherited arrhythmias 

• SVT, VT, bradyarrhythmias: local Adult Cardiologist or local specialised Arrhythmia clinic 
• Patients with implanted cardiac rhythm management devices (pacemakers, ICDs) also require additional separate transfer of device follow up to the regional adult device clinic; patients with ICD require special consideration   

Non-inherited cardiomyopathies 

• Myocarditis, cardiomyopathy – local adult cardiologist or local specialised heart failure clinic 

Acquired Cardiovascular disorders 

• Kawasaki’s disease and other vasculitis resulting in coronary artery lesions - local Adult Cardiologist 
• Rheumatic heart disease - local Adult Cardiologist 

Pulmonary Hypertension 

• Idiopathic Pulmonary Hypertension : Scottish Adult PHTN service (Transition led by GOSH Paediatric PAH team) 
• Pulmonary Hypertension associated with CHD (SACCS, GJNH)