Yorkshire and Humber ACHD Study Day 18th Jan 2018 The Past, Present and Future care of the ACHD patient Dr Kate English

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

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

What does this success mean for ACHD Services? Survivors of this era now aged 35-39 5500 of ~ 18000 affected babies die before transition Survivors of this era now aged 25 - 29 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 2012-2013 4500 live births with congenital heart disease pa

ACHD in Yorkshire and Humber Population is 5.4 million ~65 000 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

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

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)

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

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

Walton Lillehei 45 patients between 1954-1955 VSD ToF AVSD

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

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

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

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

The wider team

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

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 e-mail 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

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

Feedback

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

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