A Guide to Child Heart Surgery

Laurence Vick, Legal Director at Enable Law, has written this guide to bringing a claim involving children’s heart surgery. The topics covered include:

What Are Congenital Heart Defects?
Causes of CHDs
Bringing a Claim: Examples of Child Heart Surgery Failings
Bringing a Claim: Brain Damage and Other Non-Fatal Injury Resulting From Heart Surgery: ‘Morbidity’
Research into CHDs
Consent and ‘Data’

What Are Congenital Heart Defects?

Congenital heart defects are the most common type of birth defect, affecting eight out of every 1,000 new-borns. There are an estimated 250,000 adults with CHD in the UK and approximately 1,000,000 in the US. Sadly, CHDs are the leading cause of birth defect-related deaths worldwide. Although nearly twice as many children die from CHDs as from all forms of childhood cancers combined, funding for paediatric cancer research is significantly higher than for CHDs.

There are many types and presentations of CHDs; more than 35 main defects have been identified and the National Institute for Cardiovascular Outcomes Research (NICOR) database in the UK identifies a total of 149 defects, some of which occur in combination. This and the blurring between defect and surgical procedure (e.g. Fontan, Norwood, arterial switch) adds to the difficulty of classification and producing meaningful outcome data.

CHDs range from more straightforward conditions with no outward symptoms to complex issues with severe, life-threatening implications. Some defects can be diagnosed during pregnancy; some will be diagnosed and cause problems soon after birth and in other cases, signs and symptoms may not occur until late childhood. Major treatment, including multiple surgeries and a lifetime of medication and preventative measures to maintain a working heart and healthy body may be the only option. For some, their only hope is a heart transplant.

We have given full support to recent campaigns to raise awareness of CHDs and the crucial importance of early diagnosis and treatment. The good news is that mortality rates for CHDs continue to fall due to advances in research and in the diagnosis and treatment of these conditions.

Causes of CHDs

In most cases, scientists cannot establish what causes hearts to develop abnormally; genetics and environment play a part, but exactly how is uncertain. Most CHDs are believed to occur when something affects the development of the heart in the early weeks of pregnancy.
The CHDs with a genetic link include Down’s syndrome, in which around half of all children will have a CHD – a septal defect (hole in the internal heart wall) in 90% of those cases. Turner and Noonan syndromes and Long QT syndrome, according to US statistics, can be present in 1 in 5,000 of the population. The Rubella virus (German measles) can be a cause of CHDs if a mother contracts the disease during early pregnancy. The use of certain drugs including lithium, anti-seizure and acne medications can also increase the risk of having a baby with a CHD. Untreated type 1 and type 2 diabetes (not gestational) during pregnancy can also increase the CHD risk, most commonly leading to transposition of the great arteries.

Bringing a Claim: Examples of Child Heart Surgery Failings

Any harm suffered by a child during surgical treatment, if that harm was avoidable, may give raise to a medical negligence claim on behalf of the injured child or his or her estate if the surgery has had a fatal outcome. Below are some examples of failures:

• Failures of an inexperienced surgeon, with limited expertise generally or in the particular surgery undertaken
• Failure of the medical and surgical staff to work as a team
• Inadequate post-operative nursing care
• Issues over the timing of surgery and whether this reflected a correct clinical decision, rather than one arising from, or influenced by, the competence or availability of the medical or surgical team.
• Delayed treatment resulting from cancelled appointments. The NHS has a legal duty to provide safe care – lack of resources would not provide a defence if a child suffers identifiable harm as a result
• Diagnosis or, in some cases, misdiagnosis or failure to diagnose the complexity of the defect
• Mismanagement of aortic cross-clamping, cardio-pulmonary bypass (CPB), perfusion and myocardial protection
• Unsuitable hospital equipment and infrastructure
• Poor management systems to ensure follow-up and making/receiving referrals and appointments
In these cases we work with leading medico-legal experts, practising in the relevant fields and in a position to advise on the standards of competence to be expected in a particular procedure, as well as to the known risks and consequences of any failings identified.

In some cases, the damage caused may prove fatal; in others, children can be left severely brain damaged or with cardiological injuries requiring further treatment and specialist care for life. Where it can be shown that a child has suffered avoidable harm resulting in death or injury as a result of sub-standard treatment or surgery, we may be able to assist in securing compensation.

Bringing a Claim: Brain Damage and Other Non-Fatal Injury Resulting From Heart Surgery: ‘Morbidity’
Children who receive sub-standard treatment can be left with a range of physical disabilities, as well as behavioural and cognitive problems and learning difficulties. When children are operated on using cardio-pulmonary bypass (CPB), there is a risk of neurological damage at any heart unit; this risk may be increased, however, by human error on the part of the surgical team.

Certain standards of care in the diagnosis and surgical treatment of heart defects must be adhered to. Where a child is unfortunate enough to have suffered neurological damage, our task is to demonstrate that this was avoidable, and attributable to mismanagement of the child’s treatment, rather than a risk inherent in the surgery. It may also be necessary to establish that the neurological problems suffered by a child are distinct from, or worse than, those that a child may have experienced as a consequence of the underlying congenital heart condition.

Some children have neurological and developmental issues resulting from their underlying congenital heart disease, although these are usually of a minor nature. Our task is to establish ‘causation’ arising from a surgical or diagnostic failure – to show that the failure caused the injury.
A wide variety of complications may arise during surgery. Of the possible long-term complications, brain damage has the most impact on a child and family. Many open-heart operations to correct CHD cannot be accomplished without exposing the brain to some risk. Long-term complications include brain damage, learning difficulties, problems with the mechanics of breathing and psychological consequences of CHD. Short-term complications may increase the length of stay in hospital or in PICU and include infection and respiratory problems. Bearing in mind that, in open-heart surgery, the child’s entire circulation has stopped completely or been grossly slowed down for as much as an hour at a time, it is surprising that the majority of children emerge with no adverse neurological consequences. Sadly, a proportion of children do suffer from temporary or permanent neurological injury, including seizures or movement disorders, or disorders of higher cortical functioning manifesting in learning difficulties.

Some children are already sick following labour and delivery. For some, their CHD is one part of a wider problem that may be of genetic origin, e.g. Down’s syndrome. Some CHDs are unrelated to genetic syndromes but are particularly prone to post-operative neurological complications, e.g. coarctation and Hypoplastic Left Heart Syndrome (‘HLHS’). In HLHS, the foetal brain arteries do not develop in a normal pressure and flow environment, and later may not accommodate stress well. Managing surgery when blood flow to the brain and lower body cannot both be optimal at the same time is extremely challenging.

Research into CHDs

Major research studies in Finland and Norway were published in the journal Circulation in December 2014. The Finnish study examined the results of 13,786 operations, performed on 10,964 children, spanning a 60-year period. Follow-up coverage was an enviable 98%. The 60-year survival for the entire study was 70% against 86% for the general population. This unique study demonstrated that children are now diagnosed and treated at an increasingly younger age, and that advances in diagnosis and research, surgical techniques and post-operative intensive care have led to substantial improvements in survival.

The study carried out in Norway examined outcomes over a 40-year period. The research team in Norway also commented on the improvements in diagnostics, anaesthesia, surgical procedures and post-operative care, which they concluded had reached a level where ‘virtually all CHDs are now within reach of surgical correction with acceptable risk’. They also reported a significant reduction in the need for reoperations, which they felt were a probable consequence of performing more timely surgical corrections as opposed to primary palliations.

Both studies concluded that children with simple cardiac defects have a practically normal life-span after surgery. Long-term post-operative survival has significantly improved for those with complex CHDs when normal physiology and anatomy of the heart can be restored with modern surgical techniques.

These studies underline the crucial importance of a full diagnosis as early as possible, with surgical intervention in optimum time, often very soon after birth. Patients should have regular check-ups for the rest of their lives, with appropriate consideration given to the need for secondary surgical interventions. It goes without saying that fully resourced units with appropriately experienced medical and surgical staff, willing to exchange information and expertise with other units in non-competitive relationships, give children the best opportunity of a favourable outcome.

Consent and ‘Data’

The availability of readily accessible information, enabling families to compare units and understand the risk their children face undergoing surgery at a particular hospital, is still a long way off, despite being a key recommendation of the Kennedy Report of 2001.

These are typical questions any anxious parents will put to their child’s cardiologist or surgeon:
• Is the hospital, and its medical and surgical team, equipped to carry out this particular operation?
• Does the unit have expertise in this procedure, or is it attempting surgery that is beyond the competence of the surgical team?
• Should our child be referred to a unit with expertise in this particular operation, e.g. HLHS repair or the Fontan procedure?

Many families, faced with the prospects of their child undergoing high-risk surgery, simply want to know that the unit is well-equipped and has a medical and surgical team with the appropriate level of expertise and experience in that procedure. They may not wish to delve into the world of statistics and the often impenetrable data on the NICOR website. Some, however, will wish to research the position, and will want to be able to compare the record of other units in that particular procedure.
Parents want to know that they are entrusting their child to a surgical team that will give their child the chance of achieving the best possible outcome, in a unit with the necessary expertise to handle and minimise the risks inherent in that particular operation. Consent may be an issue where parents were given average national outcomes. If a surgeon lacks experience in that specific operation the parents should be informed of this.

This was a particular concern in the operations undertaken by James Wisheart and Janardan Dhasmana at Bristol in the 1990s. In obtaining consent in that era, parents were given average national outcomes that were not based on the risks children actually faced at Bristol. Bristol also saw the concept of the ‘learning curve’. The acceptance or, in the eyes of the families, the infliction of poor outcomes as part of the surgeon’s need to gain experience, was particularly controversial in the 1990s; this was referred to in the Inquiry as ‘one of the tragedies of Bristol’.

Hospitals are now required to comply with a statutory duty of candour. This duty has not yet been tested in the courts, but it is hoped that it would impose a duty to disclose any lack of experience of a surgeon or unit as a whole if it had a bearing on the outcome.

The debate over the reconfiguration of child heart units, a key recommendation of the 2001 Kennedy report, is now well over a decade old. Initially embraced by the children’s cardiac profession, the programme has descended into internecine warfare between units resisting closure, and confusion over presentation of data. Although this is to be addressed in the coming year, 14 years on from Kennedy, there is still no sign of publication of morbidity data, which would enable families to establish the complication rates and non-fatal failures at units. We are left with a situation in which survival for 30 days, in whatever condition, is still deemed a statistical success. Clearly there is still much work to be done.

Laurence Vick and his team, now at Enable Law, have been at the centre of child heart surgery litigation, handling high-profile children’s cardiac claims, for nearly two decades. He continues to contribute actively to the debate over developments in the treatment of children with congenital heart defects (CHD) and the related consent and safety issues. This article was originally published on LinkedIn in 2015 while Laurence was with his previous firm.