Key points

  • Highlights the dental standards within the Paediatric Congenital Heart Disease Standards and Specifications (PCHDSS).

  • Discusses the importance of treatment planning and the provision of care for children at increased risk of infective endocarditis, including those who are planned for elective cardiac surgery or cardiac intervention.

  • Outlines which children would benefit from a specialist paediatric dental assessment or management.

Introduction

The Paediatric Congenital Heart Disease Standards and Specifications (PCHDSS) were published in May 2016 by NHS England.1 The standards should ensure consistent and high quality cardiac services for all children. They are also the first cardiac standards to outline the oral health care paediatric patients with congenital heart disease (CHD) should receive from cardiac units throughout England with immediate effect.

The PCHDSS complement the National Institute for Health and Care Excellence (NICE) prophylaxis against infective endocarditis (IE) guideline.2 The standards highlight that it is not only the dental community's responsibility but also the cardiology team to inform patients of the importance of good oral health. IE is a rare complication that can be associated with both invasive dental procedures and poor oral health, particularly in children with CHD.2 Numerous publications have sought to address and clarify when and if antibiotic prophylaxis for dental treatment is required for those at risk of IE. Although this topic has a lack of consensus, there is currently insufficient evidence for antibiotic prophylaxis in the United Kingdom.2,3

Congenital heart disease

Congenital heart disease (CHD) is one of the most common congenital defects; present at birth, it can affect the walls, valves or the great vessels of the heart.4 In England and Wales five to nine per 1,000 live births are associated with a congenital heart defect each year.5 Improvements in diagnosis, neonatal care, intervention and surgical management has led to significantly higher survival rates into adulthood.6

Classification

CHD can be classified in a variety of ways: the site of anomaly, pathophysiology, obstructive or cyanotic and acyanotic defects.7,8 For the purpose of this paper, the terminology cyanotic and acyanotic heart defects will be used.

Acyanotic defects, such as a ventricular septal defect (VSD), allow for the mixing of blood between both systemic and pulmonary circulations. The pressure gradient, however, still causes blood movement from the left to the right side of the heart (oxygenated to deoxygenated). Cyanotic defects such as tricuspid atresia are commonly associated with blood being shunted from the right side of the heart to the left, causing deoxygenated blood to flow into the oxygenated systemic system causing cyanosis.

Congenital arrhythmias such as long QT and Wolff-Parkinson-White are not classified as CHD and these patients are not at increased risk of IE. Dental management, however, can be more challenging and therefore optimal preventive care and regular attendance is advised. The most common acyanotic and cyanotic defects are listed in Table 1.

Table 1 CHD defects9,10
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Diagnosis

Cardiac defects are commonly diagnosed at the 18-20 week antenatal anomalies ultrasound scan. In the UK, up to 50% of patients have an antenatal cardiac anomaly diagnosis.11

Aetiology

CHD has a rangve of aetiologies including; chromosomal abnormalities, maternal illness, and maternal drug use, in addition to multifactorial and isolated causes. CHD is a common feature in a number of chromosomal abnormalities such as 22q11 chromosome deletion,12 Noonan syndrome, Williams syndrome, trisomy 21, and Marfan syndrome.13 Maternal systemic illnesses, phenylketonuria and pre-existing maternal diabetes are also strongly associated with significant cardiac defects.14,15 In addition, a number of medications taken during pregnancy have been implicated with cardiac abnormalities including:

  1. 1.

    Anti-convulsant drugs, carbamazepine and sodium valproate

  2. 2.

    Acne medication, isotretonin

  3. 3.

    Non-steroidal anti-inflammatory drugs

  4. 4.

    Angiotensin-converting enzyme drugs

  5. 5.

    Alcohol.

Infective endocarditis in the paediatric population

Infective endocarditis is an infection of the endocardium or valves of the heart. A multistep process is involved in triggering IE. This begins initially with damage to the endocardium via abnormal cardiac structures producing turbulent blood flow within the heart. Platelets and fibrin attach to the damaged surface, producing a sterile vegetation. The thrombus then acts as a site for bacteria within the blood stream to adhere to and multiply.21

Children with CHD are at increased risk of IE and 11% to 12% of children suffer from IE throughout their life.22,23 The mortality rate for children who develop IE remains high (5-40%), which is a similar statistic to the adult population.22,24 The transient bacteraemia that is allied with IE can be hospital acquired, oral health-related or spontaneously acquired.25 The most common pathogens associated with 80% of IE cases are Staphylococcus aureus, Staphylococcus epidermis, Streptococcus viridans and Enterococcus species.26 Oral bacteraemia Streptococcus mitis, Streptococcus mutans and Streptococcus sanguis are implicated in 20-40% of IE cases.27,28 It has been suggested that children with CHD and poor oral hygiene have a higher oral bacteraemia and, therefore, are at increased risk of IE compared to children with CHD and good oral hygiene.29

Children at increased risk of IE

Children with CHD are at increased risk of IE.2,30,31 NICE prophylaxis against IE guideline2 outlines which cardiac conditions should be considered as being at increased risk of developing IE:

  1. 1.

    Acquired valvular heart disease with stenosis or regurgitation

  2. 2.

    Hypertrophic cardiomyopathy

  3. 3.

    Previous infective endocarditis

  4. 4.

    Structural congenital heart disease, including surgically corrected or palliated structural conditions, but excluding isolated atrial septal defect, fully repaired ventricular septal defect or fully repaired patent ductus arteriosus, and closure devices that are judged to be endothelialised

  5. 5.

    Valve replacement.

If doubt exists over a child's cardiac diagnosis or their IE risk status, further information should be sought. The American Heart Association and the European Society of Cardiology have defined similar conditions for which patients should be categorised as high risk for IE. Disparities exist between the guidelines, however, all agree that children with CHD are at increased risk of IE.

Common cardiac surgical procedures

Cardiac surgery and interventional catheterisation are undertaken for a wide range of congenital abnormalities and can be palliative or curative. Many operations occur in the first six months of life before the eruption of teeth. Although active dental management is minimal at this stage, it is important that enhanced oral health prevention is given from birth due to the long-term risk of IE. However, the majority of surgeries take place later on in childhood. Most operations are planned and ideally should allow for robust dental assessment and treatment before surgery. Although, in practise, this is not always the case.

Examples of common procedures:

  1. 1.

    Catheterisation:

    • PDA or ASD closure

    • Balloon valvuloplasty for PS/aortic stenosis

    • Pulmonary artery stenting.

  2. 2.

    Surgery:

    • ASD, VSD or Tetralogy of Fallot repair

    • Valve repair or replacement

    • Glenn shunt and Fontan completion.

Oral health of children with CHD

Children with CHD have been found to have similar or poorer oral health compared to children without a cardiac defect when assessing caries experience and oral hygiene measures.32,33,34,35 Children in the primary dentition have been found to have significantly more enamel defects, specifically enamel hypoplasia, compared to children without CHD. Positional anomalies and crowding in the permanent dentition have also shown to be more prevalent in the cardiac group.32 Parental knowledge of the relationship between oral health and IE is variable, with both poor and excellent carer knowledge reported. The high level of knowledge was found in a group of parents whose children were deemed at very high risk of IE.30,36,37

Worryingly, a number of studies have shown that children with CHD have higher levels of untreated oral disease compared to healthy children.34,35,37 There are concerns that reduced access to dental care is due to a combination of limited parental knowledge, medical treatment taking priority and general dental practitioners (GDPs) not feeling confident in managing medically compromised patients.38 In addition, children with CHD are significantly more dentally anxious than their healthy counterparts. This has been suggested to be due to previous hospital treatment and overnight admissions related to their cardiac condition.39

Prophylaxis against infective endocarditis for dental treatment

NICE guidance on prophylaxis against IE for people undergoing dental procedures was updated in July 2016.2 The update changed the wording of the statement from 'antibiotic prophylaxis against infective endocarditis is not recommended' to 'antibiotic prophylaxis against infective endocarditis is not recommended routinely'.

A summary of the use of antibiotic prophylaxis in the United Kingdom is shown in Table 2. In line with the Montgomery ruling and obtaining informed consent, clinicians 'should take reasonable care to ensure that the patient/carer is aware of any material risks involved in any treatment, and of any reasonable alternative or variant treatments'.40 Clinicians should inform carers of the change in NICE guidance, take the cardiologist's view into consideration and discuss the risks and benefits. Informed consent on the use of antibiotic prophylaxis for dental treatment can then be obtained. The need for this has been expressed in the recent Scottish Dental Clinical Effectiveness Programme (SDCEP) for implementation advice on antibiotic prophylaxis against IE.41 The guidance was produced to help implement the NICE 2016 guidelines. It discusses the need for robust record keeping regarding discussions with carers/parents about the risks and benefits of antibiotic prophylaxis. Useful information leaflets for dentists and carers have been produced.

Table 2 A timeline highlighting the key changes in antibiotic prophylaxis use
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There may be differences of opinion within cardiology units throughout the country on the use of antibiotic prophylaxis for dental procedures for at risk patients. Following the recent NICE guidance change and SDCEP advice, it is important that clinicians are aware of their local cardiac unit's policy on the recent amendment. A number of cardiac units have not altered their standpoint and continue to not recommend antibiotic prophylaxis for dental treatment.42

High risk dental procedures such as those that manipulate the gingivae or periapical tissues cause an increase in oral bacteraemia levels post-operatively.27,43 In addition, every day oral habits such as toothbrushing and chewing also produce a regular low level of oral bacteraemia.28,29 It is important, therefore, that a strong emphasis is placed on prevention and maintaining good oral health to prevent the need for invasive dental treatment.

Paediatric Congenital Heart Disease Standards and Service Specification (PCHDSS)

The PCHDSS published in May 2016 by NHS England describes in detail the cardiac care patients should receive within England and include a dedicated dental section.1 This dictates what oral health care patients should receive with immediate effect and concerns both cardiologists and dentists. The dental standards are documented in Table 3. Of particular interest to primary dental care providers are the following two points:

Table 3 Dental standards in the Paediatric Congenital Heart Disease Standards and Service Specification1
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  1. 1.

    Point two: all children and young people with planned elective cardiac surgery or intervention must have a dental assessment as part of pre-procedure planning to ensure that they are dentally fit for their planned intervention

  2. 2.

    Point three: all children at increased risk of endocarditis must be referred for specialist dental assessment at two years of age, and have a tailored programme for specialist follow-up.

In relation to point two, primary care providers will likely be asked to complete a dental examination before the child's cardiac surgery/intervention by the cardiology team to ensure the child is free from oral disease. This should include a full assessment, including radiographs where possible, to ensure interproximal caries is diagnosed.45 In some cardiology units, children will not be listed for cardiac surgery until there is written documentation from a dentist that the child is free from oral disease. If this assessment and documentation of the child's oral health is not completed in a timely manner, it may delay cardiac surgery. It is, therefore, important that these children are assessed quickly and, if required, referred for specialist care promptly.

If any outstanding dental care is required under local anaesthetic, this will usually be safe to complete in primary care. If the child requires more extensive treatment or would benefit from specialist care, then a referral to a consultant-led community dental service or paediatric dentistry department in a teaching or children's hospital is indicated. Children below the age of two years, who are at risk of IE, should be referred to specialist paediatric dental services for a complete assessment initially in line with the PCHDSS. Ongoing prevention and care, however, should be completed by the child's primary dental care provider or carried out on a shared care basis with specialist input if required.

Treatment planning

Paediatric patients with cardiac disease should all be treated as high risk of developing caries in the first instance. This is due to the increased risk of an oral infection leading to a serious medical complication. In addition to their high caries risk status, children with cardiac disease are often more challenging to manage due to their medical history, possible comorbidities and increased dental anxiety.39 For some children with CHD a general anaesthetic may pose a significant risk to life. Early intensive prevention and diagnosis of oral disease is vital to reduce the need for dental treatment under general anaesthetic. In accordance with the Department of Health's 'Delivering better oral health toolkit', children with CHD will be in the 'giving concern' category and should receive enhanced preventive advice.46

The SDCEP 'Prevention and management of dental caries in children' guideline advocates for a minimally invasive approach to be used in children with caries who are pre-cooperative.47 While a preventive only approach or biological management of caries, such as the Hall technique preformed metal crown (HTPMC), can be a valid treatment option for a specific group of children, this method is not recommended in children with cardiac disease.48 A more conventional caries management approach should be adopted. There is, however, a lack of consensus regarding the use of the HTPMC within the speciality for this cohort and some paediatric dental units do undertake them on a case-by-case basis. If it is felt that a patient may benefit from this technique, it would be sensible to discuss the treatment plan with a paediatric dentist.

Children with cardiac disease often require radical treatment plans to ensure the mouth is free from any oral sources of infection. It should be noted that this may be different from being 'dentally fit'. For example, a child with early dentinal caries could be deemed dentally fit for surgery given that the risk of the tooth becoming pulpally involved or causing pain and/or infection is likely to be minimal before cardiac intervention. A carious tooth, if left untreated, does leave the patient at risk of pain and/or infection following the cardiac intervention and is an oral source of infection. Carious teeth need to be definitively restored or extracted to ensure the patient is free from any oral sources of infection before intervention. This will reduce the risk of further, more complicated dental care being required post-operatively. This is of upmost importance when children are awaiting cardiac surgery. Key stages in information gathering and treatment planning for children with cardiac disease are outlined in the flowchart (Fig. 1). Tables 4 and 5 highlight when a specialist referral may be advisable and what dental treatments are contraindicated in children with CHD.

Fig. 1
figure 1

Treatment planning for children with cardiac disease

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Table 4 Circumstances when a referral to a specialist/consultant in paediatric dentistry should be considered
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Table 5 Contraindicated dental treatments
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Summary

Patients with cardiac disease can pose a challenge to dental care professionals due to their high dental needs, complex medical histories and dental-related anxiety. It is vital that children with CHD access high quality dental care. This will enable them to achieve and maintain good oral health throughout their life, limiting the need for complicated dental treatment and reducing their risk of IE. Primary dental care providers should feel confident and have a critical role to play to regularly review patients with CHD, provide enhanced prevention, diagnose and manage caries early and be aware of when to refer.

The introduction of national cardiac guidance on the oral health of paediatric patients has highlighted the importance of good oral hygiene and cross-disciplinary working to dental and cardiology teams. Dentists in both primary and secondary care should expect to have more engagement from cardiac units and an increased number of referrals for patients who are waiting for elective cardiac intervention. The guidelines also highlight the need for an early referral to specialist paediatric dental care for children at increased risk of IE before the age of two years. Careful medical history taking and liaison with the patient's medical team will allow for the successful management of most patients in primary care.