1. Yorkshire and Humber ACHD Study Day 18th Jan The Past, Present and Future care of the ACHD patient
Dr Kate English

2. ACHD – a relatively new sub-specialty
0.8% live births born with congenital heart disease
40 yrs ago 80% of those with complex disease were dead by age 16
Now > 80% will survive to reach adulthood
More adults than children living with congenital heart disease
Prevalence ~ 0.3% = 13 million patients worldwide
Prevalence expected to continue to increase year on year til 2050
Estimated that 30-50% of patients are lost to follow up

3. A Massive Success Story – Reflects huge improvements in paediatric cardiac surgery and intervention
Bristol Enquiry

5. What does this success mean for ACHD Services?
Survivors of this era now aged 35-39
5500 of ~ affected babies die before transition
Survivors of this era now aged
2500 of ~18000 affected babies die before transition
Survivors of this era now aged 10-14
<1000 of ~18000 affected babies dies before transition
813,000 live births
4500 live births with congenital heart disease pa

6. ACHD in Yorkshire and Humber
Population is 5.4 million
~ live births per annum
~ 520 babies born with congenital heart disease every year
390 paediatric cardiac operations pa
~ 450 new referrals per year into adult services

7. 2008
2018
3000 patients
6000 patients
3 regular OP clinics weekly
11 regular OP clinics weekly
2500 OP visits pa
8060 OP visits pa
Lots of patients overdue follow up appointments
No overdue follow up appointments
Sheffield outreach 10 days per year
Sheffield outreach 28 days per year
Hull outreach commencing April 2018
Fortnightly obstetric clinic
(1 consultant)
Weekly obstetric clinic (2 consultants)
Fortnightly specialist Pulmonary hypertension clinic
2 x weekly electrical clinics for patients with pacemakers and ICDs
56 ACHD operations
96 ACHD cath interventions
104 ACHD operations
244 ACHD cath interventions
2 wte ACHD consultants
1 wte specialist nurse
5 wte ACHD consultants
4 wte specialist nurses
3 pubmed papers
7 pubmed papers

8. Why have things changed so much?
Development of cardio-pulmonary bypass
Development of new surgical and interventional techniques to treat ever more complex conditions
Improved ICU care
Improved diagnostics – imaging in particular (echo and MRI)

9. Development of cardio-pulmonary bypass
1952
1st open heart procedure
Minnesota
ASD closure in 5 year old girl
Inflow occlusion, moderate total body hypothermia
Survived, had 2 children, still alive

10. 1st successful cross circulation procedure VSD closure in 12 month old - 1954
Patients father
Patient

11. Walton Lillehei
45 patients between
VSD
ToF
AVSD

12. Heterologous biologic oxygenators - 1955
Explanted dog or monkey lungs used as oxygenator
Minnesota – 14 patients, 5 survivors
Monkey lungs – 28 patients, 3 survivors

13. The birth of modern cardio-pulmonary bypass techniques - 1955
The ready availability of the simple and effective disposable Helix Reservoir Bubble Oxygenator had an explosive effect on worldwide growth of open heart surgery.
Children first because pump rates were low and technically this was easier to achieve
Adult capable pumps and oxygenators followed quickly

14. 1938 – PDA ligation
1944 – BT shunt
1944 – coarctation repair

15. The Core Team Kate English James Oliver George Ballard
3 full time pure ACHD Cardiologists
Jo Quirk
Jo Birkett
Emma Pick
3 ACHD Specialist Nurses

16. The wider team

17. The advantages of same site working
Adult Cardiology in-patient facilities
Expertise of paediatric cardiologists on site
Adult cardiac ICU skill set
Full complement of Congenital Cardiac Surgeons on site
Obstetrics on site
Full range of adult specialties
Paediatric Cardiac ICU skill set on site
Take Heart
Expertise of adult Cardiologists on site
Medical Records
Full complement of Congenital Cardiac Anaesthetists on site
Imaging servers
On site neonatal unit
JCC
Full range of adult support staff
Dedicated congenital echo staff
Fetal Cardiology
CHSF
Congenital Psychology and physio support staff
Common Medical Records
Shared catheter labs
Secretarial support
Shared MRI magnets

18. The Service
~ 100 surgical procedures pa (among highest volume centres in country)
~ 130 catheter interventions pa (highest volume centre in country)
~ 5000 patients currently under active follow up
~ 400 new referrals pa
Patients start transition from paediatric services from age 12-13
Transfer to adult services usually by 16th birthday
Transition is facilitated by same site facilities, same medical notes, imaging storage, database, some common personnel
Referrals also from GPs and other adult cardiologists from across the region (5.6 million population)
Adult services are distinctly adult
Daily consultant led ward round of in-patients (medical and surgical)
Daily general ACHD clinics
Speciality Clinics – Pregnancy, Intervention, Pacemakers and Devices, Pulmonary Hypertension
Excellent imaging
dedicated congenital echo team – all modalities
MRI reported by congenital cardiologist
Specialist nurses available in clinics and telephone advice line or by
Patients can be seen within the following day or two in clinic if an acute problem crops up
Weekly JCC runs with paediatric service
Monthly outreach clinic in Sheffield since 2006

19. The Charities TV, phone, internet access
Free of charge relatives accommodation X9
£1M

20. Feedback

24. ACHD Training
Leeds has one of only 5 national sub-specialty training posts in ACHD
Since 2005 have contributed 5 new consultants to national pool
2 currently in training
Provide core curriculum training to all adult and paediatric cardiology trainees across region and beyond
Recent GMC and BJCA survey results demonstrate best access to core training across country
Initiated lots of teaching and training initiatives
Core Curriculum course for trainees
Bi-annual regional consultants training day through network
Annual training day for Cardiology SpRs
Website since 2006
International Society for ACHD website and core curriculum lecture course
Staff frequent invited speakers at regional, national and international meetings

26. ACHD at LTHT – Current Challenges
Financial pressures in NHS
Bed pressures at LTHT
Antiquated IT resources – difficulties in clinic bookings, letters, communications
Small fish in very large pond
MRI waiting list
24/7 on call service
Very small national pool of appropriately trained medical and allied staff to recruit from
Expanding outreach services