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International Archives of Medicine
Congenital heart block associated with Sjögren syndrome: case
Karwan A Moutasim*1, Penelope J Shirlaw2, Michael P Escudier3 and
Timothy WJ Poate4
Address: 1Cancer Sciences Division, School of Medicine, University of Southampton, Southampton SO16 6YD, UK, 2Department of Oral
Medicine, Guy's and St. Thomas' NHS Foundation Trust, London SE1 9RT, UK, 3King's College London Dental Institute at Guy's, King's and St.
Thomas' Hospitals, London SE1 9RT, UK and 4King's College Hospital NHS Foundation Trust, Denmark Hill, London SE5 9RS, UK
Email: Karwan A Moutasim* - firstname.lastname@example.org; Penelope J Shirlaw - email@example.com;
Michael P Escudier - firstname.lastname@example.org; Timothy WJ Poate - email@example.com
* Corresponding author
Background: Congenital heart block is a rare complication of pregnancy associated with Sjögren
Syndrome that may result in the death of the foetus or infant, or the need for pacing in the newborn
or at a later stage.
Case report: The case is presented of a 64-year-old patient with primary Sjögren Syndrome and
a history of having given birth to two sons with congenital heart block, both of whom required
pacing several years later.
Conclusion: The literature relating to this association is discussed including the suggested
mechanism, long-term outcome of mothers of children with congenital heart block and preventive
Sjögren Syndrome is an autoimmune disease of exocrine
glands that occurs with a prevalence of 0.5 – 1% . It has
a female preponderance and is commonly diagnosed in
the 4th–5th decades of life. As it primarily affects the lach-
rymal and salivary glands, the chief clinical features are
dry eyes and a dry mouth .
Congenital heart block occurs in a frequency of 1 in
20,000 live births. It has been reported to occur in 2% of
Ro-positive mothers ; 5% of mothers with a diagnosis
of mixed connective tissue and/or Sjögren Syndrome 
and in 8% of Ro-positive mothers . 53% of cases are
diagnosed at 16–24 weeks of gestation; whilst 24% are
diagnosed later at 25–30 weeks of gestation . Here, we
present the case of a female patient diagnosed with
Sjogren Syndrome that had given birth to two sons with
congenital heart block with different presentation and
A 64-year-old Caucasian lady with Sjögren Syndrome was
referred to the Oral Medicine department from Rheuma-
tology. She complained of dry mouth, eyes and vagina;
intermittent swelling of the salivary glands and aching
joints. She was diagnosed with primary Sjögren Syndrome
35 years earlier. At presentation, her management regime
consisted of 5 mg prednisolone, Oral Balance Gel, sugar-
Published: 28 July 2009
International Archives of Medicine 2009, 2:21 doi:10.1186/1755-7682-2-21
Received: 2 July 2009
Accepted: 28 July 2009
This article is available from: http://www.intarchmed.com/content/2/1/21
© 2009 Moutasim et al; licensee BioMed Central Ltd.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0),
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
International Archives of Medicine 2009, 2:21 http://www.intarchmed.com/content/2/1/21
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free chewing gum and Viscotears. Her other medications
were lansoprazole 15 mg, hydroxychloroquine 200 mg
and thyroxine 75 mcg daily. She was a retired journalist.
She did not smoke. She drank 14 units of alcohol a week,
mainly red wine. She was married with 5 sons; 2 of whom
had congenital heart block. The first son was 41 years old
and had required no treatment. The second son was 25
years old and had required a pacemarker at age 4 years
and later required a heart transplant at 13 due to cardio-
myopathy. Extraoral examination revealed mild skin
bruising. Intraorally, the mucosa was dry with no salivary
pooling and no milkable saliva from the major gland ori-
fices. Blood investigations revealed lymphopenia at 0.9 ×
109 (reference range: 1.3–3.5 × 109), a raised ESR of 38
(reference range: 1–15) and a raised CRP of 8 mg/L (refer-
ence range:0–4 mg/L). She was anti-Ro and anti-La posi-
tive and IgG levels were raised at 26.2 (5.3–16.5 g/L). Her
whole flow rate was 0 ml/min and the parotid flow rate
was markedly low at <0.1 ml/min. C. albicans counts were
raised. Her management was based on maintaining good
hydration. The superimposed candidal infection was
treated by 1-week courses of Nystatin 100,000 IU/ml oral
suspension, repeated every 4 weeks.
Discussion and Conclusion
An association between congenital heart block (CHB) and
connective tissue diseases such as lupus and Sjögren Syn-
drome has been reported in the literature . There is a
strong correlation between congenital heart block and
maternal autoantibodies to 48 kD La, 52 kD Ro and 60 kD
Ro ribonucleoproteins. The resultant fibrosis of foetal AV
node is thought to be due to immune mediated tissue
damage to foetal heart following transplacental passage of
maternal IgG autoantibodies .
A study by Buyon et al examined 105 mothers with Ro
and/or La positive sera, and reported that 113 infants were
diagnosed with CHB. Of the 87 pregnancies with ade-
quate records, bradyarrhythmias detected before 30 weeks
gestation in 71 (82%) – median 23 weeks. Of the 113
infants, 22 (19%) died, mostly within 3 months of birth.
Of the 107 live-born children, 67 (63%) required pacing;
35 within 9 days, 15 within 1 year and 17 after 1 year. Of
the 49 mothers who had subsequent pregnancies, 8
(16%) had another child with CHB .
The underlying mechanism behind congenital heart block
in Sjögren Syndrome is suggested to be due to Ro and La
antigens present on apoptotic blebs on surface of actively
remodelling foetal cardiocytes. Opsonized Ro & La anti-
bodies can then lead to macrophage activation, TNF-α &
TGF-β secretion which favours differentiation of fibrob-
lasts into collagen-secreting myofibroblasts which pro-
mote scarring . The site of action of Ro & La antibodies
may be the α-1 subunit of the L-type calcium channel,
which is involved in action potential propagation in AV
node & excitation coupling in the heart. These channels
are present in lower density in foetal heart cells; which
may explain why the foetal heart is affected and the mater-
nal heart unaffected [7,8].
It is thought that foetal CHB arises due to increased sus-
ceptibility of the foetal heart due to increased apoptotic
remodelling of foetal heart cells or lower density of L-type
calcium channels. Other antibodies such as muscarinic
type-1 receptor (M1R) antibodies described in maternal
blood may also have a role. Foetal heart expresses M1R
while maternal heart expresses mainly M2R. The majority
of cases of CHB are subclinical at birth; with a case
reported of normal ECG at birth and subsequent develop-
ment of asymptomatic heart block at 2 years .
Discordance for CHB has been reported in monozygotic
twins despite identical genetics and environmental expo-
sure to maternal anti-Ro antibody . Late-onset dilated
cardiomyopathy has been reported despite early pacing in
16 children with congenital heart block . Dilated car-
diomyopathy and heart failure developed between 2
weeks and 9 years requiring cardiac transplant in those
It is recommended that infants with CHB require close
follow up, not only of cardiac rate & rhythm, but also of
ventricular function. Press et al studied the long-term out-
come of 64 children with complete heart block, and
found that 60% (32 out of 53) of the mothers tested were
anti-Ro and/or anti-La positive. The mean follow-up
period was 121 months, and the mean maternal age was
38. At the time of delivery, the majority of mothers (66%)
were healthy .
Potentially serious outcomes are rare but possible, with
63% of affected offspring requiring pacing. The general
recommendation is to monitor foetal cardiac function
from 16–24 weeks in utero, continuing after birth to mon-
itor rate, rhythm and ventricular function .
The optimal treatment is still debatable but corticoster-
oids may be of benefit in established cases and may fur-
ther have a role in the prevention of congenital heart
block in mothers with previously affected pregnancy.
Thus, treatment of congenital heart block is based on
fluorinated steroids that cross the placenta. The use of glu-
cocorticoid intervention for 3–19 weeks in cases of foetal
second degree heart block may increase the likelihood of
reverting to first degree heart block rather than progres-
sion to third degree heart block . Dexamethasone
commenced at time of diagnosis of CHB and maintained
International Archives of Medicine 2009, 2:21 http://www.intarchmed.com/content/2/1/21 Download full-text
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for duration of pregnancy may also improve foetal sur-
vival in cases of prenatally diagnosed complete heart
The evidence to support prophylactic treatment to prevent
congenital heart block during subsequent pregnancies is
weak, and concerns regarding the safety of fluorinated
steroids have been raised, particularly neurological toxic-
ity, spontaneous abortion, stillbirths & intrauterine
growth restriction. Other interventions, such as intrave-
nous immunoglobulins, plasmapeheresis and azathio-
prine have been suggested .
In summary, congenital heart block is a common occur-
rence in offspring of mothers affected with Sjögren Syn-
drome. Whilst there is no consensus on management,
appropriate referral and follow-up is recommended.
Finally, a diagnosis of Sjögren Syndrome should be con-
sidered in a mother who has a child diagnosed with con-
genital heart block.
Written informed consent was obtained from the patient
as per the standard protocol at Guy's Hospital NHS Foun-
The authors declare that they have no competing interests.
All authors were involved in patient diagnosis and man-
agement, with PJS and MPE being the senior clinicians
guiding overall management at the time. KAM wrote the
manuscript under the general guidance and supervision of
K.A.M., BDS, MFDRCSI, MSc is currently a Clinical
Research Training Fellow at the University of Southamp-
ton. Prior to that, he had been a postgraduate student and
research fellow at Guy's Hospital (the study site). He is a
member of the British Society for Oral Medicine, British
Society for Oral and Maxillofacial Pathology, Interna-
tional Association for Oral Pathology and the Interna-
tional Association for Dental Research. He has won
numerous clinical and research prizes including the Royal
Society of Medicine's President's Prize of the Odontology
Section (2009; UK) and the British Society for Oral Medi-
cine's clinical and research papers prize (2009).
P.J.S., BDS, LDSRCS, FDSRCS, is a Consultant in Oral
Medicine and the Clinical Director for Dental Services at
Guy's Hospital, London (UK).
M.E.P. BDS, MBBS, FDSRCS, MRCP, FDSRCS(OM), MD is
a Senior Lecturer and Honorary Consultant in Oral Medi-
cine at Guy's Hospital (UK).
T.W.J.P. BDS, MBBS, FDSRCS, MRCP, FDSRCS(OM) is
currently a Consultant in Oral Medicine at King's College
Hospital, London. He has trained in both dentistry and
medicine and at the time of the study was a Specialist Reg-
istrar and Honorary Lecturer in Oral Medicine.
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