Final Report > Chapter 3: The Care and Treatment of Children with Congenital Heart Disease > The care and treatment of children with congenital heart disease

The care and treatment of children with congenital heart disease

1 In this chapter we describe briefly the care and treatment which children with congenital heart disease (CHD) might have expected to receive in hospital during the period of our Terms of Reference. We also introduce the other chapters which make up what we call `Setting the scene', and which provide the context for understanding what took place in Bristol between 1984 and 1995. An account of developments in the UK in the diagnosis and treatment of CHD in children between 1984 and 1995, written for the Inquiry by Dr Eric Silove, a member of the Inquiry's Expert Group, appears in Chapter 3 - Developments in the UK, in the Diagnosis and Treatment of Congenital Heart Abnormalities in Children, 1984-1995 of Annex A. It includes an explanation, with diagrams, of specific abnormalities in the heart and the ways in which they have been diagnosed and managed over time. It takes account of all the conditions and procedures referred to in this Report.

2 Between six and eight in every 1,000 children born in the UK are likely to have CHD. The severity of the abnormality which constitutes the CHD can vary enormously. For some children (approximately 50%) it will be relatively mild and may even heal spontaneously as the child develops. For the others, a surgical procedure will almost certainly be necessary to enable them to have a good chance of growing into adulthood. At the extreme, some babies with very severe abnormalities may die within days if they do not have medical and surgical treatment. Some, sadly, are beyond treatment and die.

3 The care and treatment of children with CHD includes, but is not confined to, hospital care. Very often healthcare professionals who work in the community, such as GPs, health visitors and midwives, district nurses, physiotherapists and social workers, may contribute to their care. We acknowledge the importance of such contributions. They are not, however, the subject of this Inquiry. Our focus is on one particular part of the child's journey of care: heart surgery in hospital. When we talk of paediatric cardiac surgical (PCS) services, however, we do not confine them merely to the surgery. We refer to the whole range of services provided by a hospital to children with CHD and to their families. Thus we include not only surgical procedures, but the full range of care and treatment offered by a hospital and its staff associated with surgery: diagnosis, pre- and post-operative care, and support for the children and their families.

4 In many respects there is no `typical' journey of care for a child born with CHD. Every child's condition and every family's circumstances are unique. Yet it is possible to set down, in general terms, the stages of care and treatment which children and their families might encounter. These stages are much the same today as they would have been between 1984 and 1995. An initial diagnosis might be made by the paediatrician at the hospital where the child is born. In other cases, where there are perhaps no immediately obvious signs of a problem, a baby's condition may be diagnosed days or weeks later, when signs are noted by parents, a midwife, a health visitor or GP. Referral to a paediatrician generally follows. The baby will then be referred to a paediatric cardiologist who will make a diagnosis and carry out an assessment of the abnormality of the heart.

5 The cardiologist, in many cases in consultation with a paediatric cardiac surgeon, will advise on necessary care and treatment. The management of the baby's care will depend on the nature and severity of the abnormality: an emergency operation may be required, or initial treatment may involve the use of drugs. For some children, the abnormality in their heart may be such that one operation will be sufficient to correct the defect. For others, a series of operations over a period of years may be required, during which time their care would be managed by a specialist team from the hospital. The child may need open-heart or closed-heart surgery. During open-heart surgery, the child's heart is stopped and the child is supported by a heart-lung bypass machine. During closed-heart surgery, the operation is carried out while the heart is still beating.

6 Post-operatively, the child will be cared for initially in the hospital's intensive care unit (ICU). After discharge from the hospital, long-term care and management of the child by a paediatric cardiologist, often in conjunction with a consultant paediatrician in the child's local hospital, will continue for many years as the child's condition is reviewed and assessed.

7 The child's family will be supported, both practically, for example by the offer of local accommodation where needed, and emotionally, by staff during the child's stay in hospital, and in the longer term.

8 If a child should die following surgery, a pathologist investigates the cause of the death. The pathologist prepares a report on the cause of death for those who were caring for the child, which serves as the basis for discussion with parents about the reason for their child's death. The pathologist's findings can assist the cardiologists and surgeons, not only in their understanding of that particular child's death, but also in shaping their approach to treatment in future cases.

9 These elements together make up the PCS service with which we are concerned. We now turn to developments in the diagnosis and treatment of CHD in children between 1984 and 1995.

10 Very little treatment for children with congenital heart abnormalities was available anywhere in the world until the late 1950s. In 1958 the first open-heart operation on a child was carried out in the UK, using a heart-lung bypass machine. Heart-lung bypass technology was a significant development which made it possible for surgeons to stop the heart, to repair the defective part of the heart, for example, the pumping chambers inside the heart, and then to restart the heart. The number of surgical procedures performed and the number of hospitals carrying them out increased during the 1960s, and by the early 1970s cardiac surgery for children had come to be available in a small number of centres in less than half of the major regions in the UK.

11 The development of PCS services over the last 40 years in the UK, as with the development of any clinical service, has been an evolutionary process. It was not only the volume of operations which increased. Advances in technology, the refinement of surgical procedures and the development of new procedures took place. These, in turn, contributed to better chances of survival. Techniques for diagnosis also improved. Non-invasive methods of diagnosis using ultrasound scanning technology developed rapidly in the 1970s and 1980s, and provided a safer alternative to cardiac catheterisation which, as an invasive procedure, carried a higher risk in small infants. These non-invasive methods included echocardiography (ultrasound scanning) which was developed during the late 1970s and 1980s; the `Doppler' technique, introduced in the mid 1980s; colour mapping of Doppler signals towards the end of the 1980s and into the 1990s; and the introduction of echocardiography in the operating theatre and the ICU.

12 Surgical techniques continued to evolve during the 1980s and 1990s. New procedures, such as the Arterial Switch and the Fontan procedure, were more widely undertaken. Surgery was carried out on children at an earlier age than previously in the case of some heart defects. It also became possible to correct some heart defects, including Ventricular Septal Defects, by one rather than a series of procedures.

13 Advances in equipment and technology also meant that, from about 1982, the technique of cardiac catheterisation could be used, in certain circumstances, for treatment as well as for diagnosis. Thus some children who might previously have required surgery which involved opening the chest in order to expose the heart could now be treated by passing a tube or catheter from a vein in the groin into the heart and inflating a balloon in order to stretch open a narrowed valve.

14 There were also significant changes in the management of intensive care. In 1984, at the beginning of the period covered by our Terms of Reference, it was generally the surgeon who assumed primary responsibility for post-operative care. In the early 1990s anaesthetists began to be more fully involved, and began taking on clinical sessions dedicated to the ICU. In some units, by the mid 1990s, full-time intensivists (clinicians specialising in intensive care, usually anaesthetists) were appointed. This was part of a development in paediatric intensive care generally, and not specific to the care of children who had undergone heart surgery.

15 We have described, broadly, the elements of a paediatric cardiac surgical service which might have been available to a child with CHD who was in need of surgery. We have also referred to some of the key developments which took place in PCS services during the 1980s and 1990s. At this stage in the process of setting the scene it might seem natural now to turn to Bristol and to describe how the PCS service there was organised and delivered. In fact that may not be helpful. It would leave out of the account the wider context within which the PCS service in Bristol was provided. For it is important to remember that the PCS service in Bristol took place within the NHS. The NHS in the 1980s and 1990s was a place of significant change. These changes influenced the hospitals in Bristol. They were as affected as other hospitals by the actions and demands of government. They encountered the same pressures. Their relationship with local health authorities went through the same sort of development. Thus, before we turn to the particular circumstances of the PCS service in Bristol, we should take account of the wider context. We need to get a sense of what was happening in the NHS at the time.

16 The principal reason for setting out this wider context is that it is not possible otherwise to take a view on the quality of the PCS service provided to children in Bristol, how it was assessed at the time, and how it might be assessed today. Bristol did not exist in isolation. It was affected in many things that it did by the conditions which prevailed in the wider NHS. Of central importance in this wider context is how the term `quality', in relation to the notion of the quality of care, was perceived at the time, and how such perceptions changed.

17 A further element contributing to a proper understanding of events in Bristol is that the funding for a major part of the PCS service, the care and treatment of children under 1 year, was unusual: it was funded on a national basis through a system known as the supra regional services. Thus, to make sense of the arrangements in Bristol, and ultimately to increase our understanding of what happened there, we must take account of this particular funding arrangement.

18 When we turn to describe the arrangements for PCS in Bristol, it will be clear that the PCS service was provided within a large, university teaching hospital, with many thousands of employees and a multi-million pound budget. The PCS service was neither free-standing nor self-contained. It was part of a wider system, part of the organisation known as the United Bristol Hospitals (UBH), and, latterly, as the UBHT. The policies and practices of the UBH/T as a whole clearly conditioned and influenced the services within it, including the PCS service. Thus it is important also to be aware of this wider, local context.

19 In setting the scene, therefore, this section of our Report addresses first the national context and then the local context within which PCS services were provided. It is arranged as follows:

• the changing NHS 1984-1995 (Chapter 4);
• management in Bristol (Chapter 5);
• quality, standards and information (Chapter 6);
• the audit and monitoring of the paediatric cardiac surgical service in Bristol (Chapter 7);
• paediatric cardiac surgical services (Chapter 8); and
• the paediatric cardiac surgical service in Bristol (Chapter 9).