Introduction
Congenital heart disease (CHD) is the most common type of birth defect, affecting 13 children born every day in the UK,1 but only around half are detected on routine antenatal screening.2 Many children require open heart surgery or other intervention, often within the first year of life and survival continues to improve2 3; yet early morbidities remain common after surgery4 and infants have an ongoing risk of death following discharge.5 Nowadays around 97% of children born with CHD are expected to survive into adulthood6 and there are estimated to be over 250 000 adults living with CHD in the UK,7 including an emerging population with conditions that previously were rarely compatible with survival, presenting new diagnostic and therapeutic challenges. Almost all patients require lifelong follow-up in specialist clinics to manage their unresolved burden of disease8; many develop late complications related to their condition or previous procedures, and have an ongoing need for increasingly hazardous reinterventions, to treat recurrent or progressive lesions, or replace prosthetic implants such as valves, conduits or pacemakers. Uncertainty over further interventions and long-term outlook may contribute to increased levels of anxiety, depression and other mental health issues in CHD.9 While a growing number are living with CHD into their 60s and beyond, developing acquired morbidities and senescence,10 long-term survival remains reduced compared with the general population, especially in those with uncorrected, palliated, complex or cyanotic heart conditions, with heart failure the most common cause of premature death.11
There is a lack of evidence to inform clinical decision-making in children and adults with CHD. The Cochrane Library contains fewer than 20 reviews on CHD topics,12 and there is a need for high-quality, multicentre clinical trials that answer important questions to improve their daily lives and outcomes.13 The British Heart Foundation (BHF) identified that ‘we urgently need research breakthroughs to ensure survivors (of CHD) lead longer and healthier lives’.14
The James Lind Alliance (JLA) is a non-profit-making initiative, established in 2004 and coordinated by the National Institute for Health and Care Research (NIHR), to bring together patients, carers and clinicians as equals in a Priority Setting Partnership (PSP).15 Through a defined method with shared decision making,16 PSPs identify and prioritise unanswered questions for research, or evidence uncertainties, providing a platform for researchers and funders to prioritise the research that matters most to research beneficiaries. We, therefore, brought together patients with lived experience of CHD, parents, national charities and healthcare professionals in the UK to form the national Congenital Heart Disease PSP.