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Dental Health and Management for Children with Congenital Heart Disease

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 Congenital Heart Disease and Primary Dental Care

Dental Health and Management for Children with

Congenital Heart Disease
Kirsten FitzGerald, Pádraig Fleming and Orla Franklin

Key Words: Paediatric Dentistry, Congenital Heart Defects, Endocarditis, Oral Health, Children © Primary Dental Care 2010;17(1):21-25

Congenital heart disease (CHD) is one of the most common
developmental anomalies. Children with CHD are at increased
risk of developing oral disease, and are at increased risk from the
systemic effects of oral disease. Recent changes in guidelines related
to prophylaxis against infective endocarditis have highlighted the
importance of establishing and maintaining oral health for this group
of patients. The management of children with CHD can be complex
and, unfortunately, many of these children do not receive the care
they require. The challenges that these children pose are discussed,
and suggestions are made for the appropriate management of these
patients and the key role that all those working in primary dental
care have to play.

INTRODUCTION
Congenital heart disease (CHD) is a
defect in the structure of the heart and/
or great vessels that is present at birth.
The incidence of CHD detectable by
routine clinical examination has been
estimated to be 7.5 per 1000 live births.1
Severe cardiac anomalies, which are lethal
in the absence of cardiac catheterisation
or surgical intervention, occur in 2.5
to 3 infants per 1000 births.2,3 Most
CHDs either obstruct the flow of blood
or cause it to flow in an abnormal
pattern. Examples of complex forms of
CHD, with a normal heart for com-
p a r ison, are shown in Figures 1-3. A
significant minority of children with
cardiac malformations have an associated
syndrome (Table 1).
Advances in diagnostics, neonatal
care, and surgical management have
increased the survival rates of children
born with CHD. With this increase in
survival comes an increased burden of
complexity when managing these chil-
dren’s oral health and disease. The im- management of children with CHD,
portance of achieving and maintaining many of which impact on oral health
oral health for individuals with CHD by altering saliva, plaque, mucosa, and
has been highlighted recently by much gingivae. There is potential for drug
debate around changes in guidelines interaction when prescribing for dental
relating to prophylaxis against infective purposes. It is essential that prior to
endocarditis.4,5 Regardless of which prescribing, any potential for interaction
guidelines are in use, one thing has not be evaluated and appropriate consul -
changed: the dental team is charged with tation made with the family medical
guiding the child with CHD towards
enjoying a lifetime of optimum oral Table 1: Common syndromes
health. associated with CHD
(listed alphabetically)
MEDICAL AND SURGICAL
DiGeorge (22q11.2 deletion)
MANAGEMENT OF
Down (trisomy 21)
CHILDREN WITH CHD
Edwards
Once recognised and diagnosed, the Goldenhar (hemifacial microsomia)
child with CHD is managed with the
Hurler
appropr iate combination of medical
Kabuki
treatment, therapeutic catheterisation,
and open-heart surgery. For complex Noonan
anomalies, a staged approach that palli- Treacher Collins
ates but may not repair a major structural Turner
anomaly is often needed.
Williams
Various medications are used in the

K FitzGerald BDentSc, MFD, MS. Clinical Fellow in Paediatric Dentistry.* O Franklin MB BCh BAO, DCH, MRCPCH, MPCPI. Consultant Paediatric Cardiologist.*
P Fleming BDentSc, FDS, MSc, FFD. Consultant Paediatric Dental Surgeon.* * Our Lady’s Children’s Hospital, Crumlin, Dublin 12, Republic of Ireland.

Primary Dental Care • January 2010

21
 Children with Congenital Heart Disease

at times life-threatening.The need to gain

Key: weight to be fit for surgery may override
SVC = superior vena cava
oral health concerns, and frequent con-
RA = right atrium
TV = tricuspid valve sumption of high-calorie sugary foods
RV = right ventricle and fluids is common. Night-time feed-
IVC = inferior vena cava
ing is often prolonged, leading rapidly to
PV = pulmonary valve
PA = pulmonary artery severe early childhood caries. These spe-
PVn = pulmonary vein cific nutritional issues are in addition to
A = aorta
LA = left atrium
the prevailing background of an influen-
MV = mitral valve tial media environment that has been
LV = left ventricle found to promote products that can be
AV = aortic valve
detrimental to dental and general health.7
Medications may produce changes in
saliva, and an association between the
use of digoxin and dental caries has been
made.8 Digoxin is only available in a
sucrose-based suspension, but fortunately
is now only rarely used in the medical
Adapted from a diagram by Dake and Eric Pearce
management of CHD. Psychosocial issues
Figure 1 Normal human heart. and a child’s fear of medical treatment
may prevent parents from bringing their
practitioner or the cardiology team. its effects, can lead to difficulties in child for dental care, and access to care
Interventional catheterisation tech- achieving optimum oral health for these may be limited by a lack of dental staff
niques are now the accepted standard of children. In many cases, the entire family able and confident to provide appropriate
care for the treatment of many types of may have a low level of awareness of the care.9 Neurological deficits have been
CHD. A wide variety of procedures and importance of oral health in general, and documented in some children with com-
devices has been developed to occlude may be completely unaware of the link plex CHD, further increasing the diffi-
septal defects and vascular structures, to between the mouth and the heart.6 In culties encountered in providing care for
create, enlarge and maintain intra-atrial addition, the priority placed on oral care this vulnerable group.10,11 The develop-
communications, and to widen and may be low, especially when the child’s ing dentition may be affected by the sys-
support narrowed vessels. Open-heart clinical situation has been complex and temic effects of CHD and its treatment.
surgery is carried out while the blood-
stream is diverted through a heart–lung
machine, and complex rerouting of Key:
A = patent ductus arteriosus (PDA)
vessels and device placements can be
B = hypoplastic aorta
achieved. Prosthetic valves, patches, and C = atrial septal defect (ASD)
donor valves are usually placed via an D= hypoplastic left ventricle

open-heart technique.

CHALLENGES FOR THE

DENTAL TEAM
Clinicians in pr imary dental care,
including dental hygienists, dental ther-
apists, general dentists, and paediatric
dentists, are faced with a number of
challenges when caring for a child with
CHD. These challenges may be directly
or indirectly related to the child’s med-
ical condition. Oral health and disease
Adapted from a diagram by Dake and Eric Pearce
have a multifactorial basis, which, com-
bined with the complexity of CHD and Figure 2 Hypoplastic left heart.

Primary Dental Care • January 2010

22

Key:
A = pulmonary stenosis
B = overriding aorta
C = ventricular septal defect
D = right ventricular hypertrophy
Adapted from a diagram by Dake and Eric Pearce
Figure 3 Tetralogy of Fallot.
A number of studies have shown enamel
defects to be of increased prevalence
in children with CHD.12,13 These teeth
with deficient enamel are more sus -
ceptible to dental caries and are more
difficult to restore.
Taking these challenges into account,
it is no surprise that children with CHD
are found to have increased levels of
dental caries when compared with the
general population.12,13 What is perhaps
most alarming is that this group has a
greater level of untreated oral disease
and decreased provision of preventive
care.6,12 Whether their families do not
seek dental care, or dental teams fail to
provide it, the result is the same: many
of the children most at risk from the
consequences of oral disease do not
receive the treatment that they require.
INFECTIVE
ENDOCARDITIS
Some children and adults with CHD are
known to be at risk of developing infec-
tive endocarditis (IE). Recent revisions
of guidelines in relation to antibiotic
prophylaxis against IE have brought the
subject to the fore.4,5 It is tempting to
focus on the changes that have been
made in these guidelines, but it might
be more appropriate to focus on what
remains the same: children and adults
with CHD still remain at risk of IE.
Viridans group streptococci (Streptococcus
mutans, S. sanguis, S. mitis) and Staphylo-
coccus aureus, all common oral bacteria,
are significant causative agents for IE
in paediatric patients.14 The fact that
antibiotic prophylaxis prior to dental
treatment is no longer recommended in
the United Kingdom does not imply
that these patients are no longer at risk
of developing IE. The emphasis is now
firmly on the maintenance of oral health
to lower the incidence of spontaneous,
everyday bacteraemias. With this in
mind, a shift towards preventive den-
tistry is emphasised as the mainstay of
management for children at risk of IE.
DENTAL CARE FOR
INDIVIDUAL PATIENTS
WITH CHD
As for all patients presenting to the clin-
ician in primary dental care, the first step
in providing appropriate care is to obtain
and document a comprehensive medical
and social history. Specific enquiry
should be made into the nature of the
K FitzGerald et al
original defect, previous hospitalisations,
current medications, the presence of
prosthetic valves/patches, exercise toler-
ance, and the presence of hypertension.
Discussion of home oral care and dietary
practices forms a part of this history and
provides valuable information for risk
analysis and planning. Where clinically
indicated, radiographs should be exposed
to augment the clinical examination.
Once a thorough history and examina-
tion have been carried out, an accurate
diagnosis can be made and a tentative
treatment plan formulated. At this point,
liaison with the family medical practi-
tioner or the cardiology team responsible
may be required, prior to providing
active treatment. This should generally
take the form of a letter to the medical
provider that is specific in its enquiries.
Simply asking for an ‘update’ on a
patient’s history, or asking about a
patient’s ‘suitability for dental treat-
ment’ without specifying the nature of
that treatment will yield little useful
information.
Once a thorough knowledge of the
child’s cardiac status is gained and any
special care required is identified, a
definitive treatment plan can be estab-
lished. Preventive dentistry in the form
of dietary advice, home and office
fluoride therapy, and oral hygiene advice
can and should be provided for all
patients. There are straightforward, evi-
dence-based guidelines available for the
prevention and management of dental
car ies in both young children and
children at high caries risk,15-17 which
should be followed. This group should
receive every appropriate measure in the
preventive armamentarium in order to
minimise their risk of developing dental
caries. Fissure sealant placement may or
may not be appropriate, depending on
age and cooperation, but should be con-
sidered as soon as it is feasible. Placement
of resin-modified or conventional glass-
ionomer sealants may be considered as
an interim measure for teeth especially
at risk of caries that are not yet fully
erupted, or for children who are unable
Primary Dental Care • January 2010
23
 Children with Congenital Heart Disease
to tolerate the placement of a conven-
tional resin sealant.18
Should disease occur, identification of
potential foci of infection in the mouth
is the cornerstone of treatment planning
for this group. Treatment of dental
caries, whether surgical or restorative,
must be provided in the context of the
risk of IE. Definitive treatment is prefer-
able to temporary or short-to-medium-
term solutions. Extraction is generally
favoured over pulp therapy, especially
for the primary dentition. Restorative
treatment should be definitive, and the
placement of stainless steel crowns
(SSCs) is often preferable to direct intra-
coronal restorations, especially for cari-
ous primary teeth. The SSC is extremely
durable, relatively inexpensive, subject
to minimal technique sensitivity during
placement, and offers the advantage of
full coronal coverage.19,20 Primary den-
tal care providers should be adept at the
placement of SSCs, and should consider
their use for children with CHD who
present with caries. Their durability as a
restoration is highly favourable, but per-
haps their most useful feature is their
ability to protect the remainder of the
tooth from caries attack for its lifetime.
For children with CHD, who will
remain at high risk of developing dental
caries, this is a significant advantage.
Some dental care providers might be
anxious about placing SSCs, being a
restoration they may have used only
infrequently. Observation of a practi-
tioner who is experienced and skilled
in their placement is very valuable, and
with a little practice, SSCs become easy
and quick to place.
The management of first permanent
molars of poor prognosis is always a
challenge. These teeth may have been
affected by caries and/or molar/incisor
hypomineralisation (MIH). Regardless
of the cause, appropriate care of these
teeth can involve sealants, temporary
restorations (with materials such as glass
ionomer), direct restorations (such as
composite and amalgam), indirect restor-
ations (such as SSCs, onlays, and inlays),
Primary Dental Care • January 2010
24
and extraction. A clear-cut decision tree
has been suggested as an outline for the
management of molars exhibiting MIH,
with decisions based on the varying
degrees of severity and treatment needs
over time.21 The decision to extract is
sometimes difficult but, when timed
correctly, can lead to a much improved
long-term situation. Primary dental care
providers must consider the certainty of
a lifetime burden of restorative care for
such teeth if they are to be maintained
in the mouth, and the likelihood of
eventual failure and possible extraction.
Treatment planning should take into
account the risk of IE. A timely decision
to extract is sometimes in the patient’s
best interests. Further guidance on the
management of extraction therapy for
first permanent molars in children is
available in guideline form.22
Extractions and other surgical treat-
ment should be carefully planned and
consideration given to potential coagu-
lation problems. An increase in post-
extraction bleeding may occur in
patients on anticoagulant therapy (such
as warfarin and aspirin), or may be sec-
ondary to prolonged cyanosis. For war-
farinised patients, dental extractions can
be carried out with an international
normalised ratio (INR) up to 4. Regard-
less of the indication for oral anticoag-
ulant, the therapeutic aim for most of
these patients is an INR of 2-4, so there
is no need to alter their anticoagulation
regimen. An INR should be obtained
within 24 hours of the proposed surgery.
Because excessive bleeding may be seen
in a small number of children with poly-
cythaemia due to cyanotic CHD, a full
blood count and coagulation screen may
be prudent for this group prior to sur-
gical therapy. Post-operative bleeding
can be controlled using additional local
haemostatic measures such as placement
of haemostatic gauze, sponges and
sutures, and avoidance of rinsing follow-
ing extractions.
Orthodontic treatment, including the
placement of space maintainers, can be
provided for children with CHD, but
only with the strictest observation and
maintenance of excellent oral hygiene to
reduce the risks of developing IE.23
Behaviour-guidance approaches for
patients with CHD can be challenging.
Decisions relating to this aspect of care
should not be made solely by the dentist.
The child (if appropriate), the parent
(who can often provide useful insight
into the child’s likely behaviour), and
the significant members of the health-
care team should all be involved. As for
all children, communicative approaches
are used to introduce the child to the
dental setting. For children who have
little or no anxiety related to dental and
medical treatment, this may be suffi-
cient, and treatment can proceed nor-
mally. Unfortunately, communicative
techniques alone are insufficient for
many children with CHD. Their long
history of medical and surgical inter -
ventions, coupled with a possible neuro-
logical impairment, which may be a
complication of cardiac surgery,10,11 can
leave the dental practitioner with a
patient who is difficult to manage in the
traditional dental setting. Sedation and
general anaesthesia are more compli-
cated and can carry greater risks for
children with CHD than for healthy
children, but the goal of achieving qual-
ity dental care in a non-stressful envi-
ronment may necessitate the use of these
pharmacological behaviour-modifying
techniques. Appropriate consultation with
the cardiac and anaesthetic teams at the
planning stage forms the basis for mini-
mising the risks during sedation and
general anaesthesia.
SUMMARY
Children with CHD should be able to
enjoy a lifetime of optimal oral health.
Prevention of oral disease is the mainstay
of care for this group. Although children
with CHD are cared for by hospital spe-
cialists for their medical and oral prob-
lems, primary dental care clinicians have
a major role to play in the routine oral
healthcare of such children. This role
involves the prevention of oral disease
 K FitzGerald et al

and prompt management if disease
occurs. As explained earlier, there are
considerable challenges associated with
providing dental care for these children,
requiring primary dental care clinicians
to work in partnership with all others
involved in the care of the children
concerned. Excellent communication
between all healthcare professionals
caring for these children is essential.
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Correspondence: K FitzGerald,
Dental Department, Our Lady’s Children’s Hospital,
Crumlin, Dublin 12, Ireland.
E-mail: kirsten.fitzgerald@olchc.ie

management. Recommended patient information sheets are a valuable

Oral and Maxillofacial Medicine:
The Basis of Diagnosis and
Treatment
Crispian Scully
London: Churchill Livingstone (Elsevier); 2008 (2nd ed)
£44.99; Softcover; 408 pp
ISBN: 9 78044 306 8188
When Professor Scully is the author of a textbook, then expectations
are immediately very high. This book fulfils those expectations completely.
The clarity of the writing runs throughout. Early in the text, the
13 intended outcomes of the book are stated. These aims for the reader
include systemic history-taking, diagnostic methods, identification of
lesions, the need to refer or not to refer, and the importance of working
with colleagues in other disciplines. Initially, this may seem a daunting list
for the student but due to the accessibility of the subjects, all of the aims
can be achieved with less effort than would be the case with many
textbooks. Starting with diagnosis, examination, and appropriate records,
the sequence that the author gives for each topic is supportive. By
following these methods, the practitioner is guided to take the course
of action to the best benefit of the patient.
Sections on common complaints, common and important oral
conditions, relevant systemic disorders, other conditions and adverse
inclusion. Tables of medications are clear and concise. The diagnostic
algorithms are particularly helpful either for learning or for reference in
the clinical situation. The book is liberally illustrated with clinical pictures,
histopathology sections, and pack shots of medications and reagents.
The first edition of this book was awarded first prize of the Royal
Society of Medicine and Society of Authors for a new authored book.
This edition has additions of histopathology, diagnostic algorithms,
sections on sialorrhoea and drooling, other conditions, and adverse drug
reactions. These extra items make this book extremely comprehensive
and justify obtaining this edition, even if you have the first version.
The publishers recommend the book to senior dental students, dental
practitioners, and for trainees and practitioners in oral and maxillofacial
medicine, surgery and pathology. In the light of a greater number of
patients seeking oral and dental advice from their GPs rather than
National Health Service dentists, perhaps this list should be revised to
include general medical practitioners who would find it a valuable
reference. In a registrar-training general practice, it could be regarded as
essential. In the primary dental care environment, this book would be a
valuable as a reference to identify rarely encountered conditions. The
advice on treatment or onward referral is reassuring when we might be
out of our comfort zone. This is an exceptionally accomplished textbook,
and is highly recommended.
JOHN COVENTRY MSC, BDS, MRD, LDS.
re ook

drug reactions are presented in a completely logical form. Conditions SPECIALIST IN PERIODONTICS, SHREWSBURY.
w
B
vie

are discussed by causes, clinical features, complications, diagnosis, and

Primary Dental Care • January 2010

25
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