CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
201
2
Division of Paediatric Cardiology, University Hospital, Lund,
Sweden
3
Division of Paediatric Cardiology,Uppsala Academic Hospital,
Uppssala, Sweden
4
Division of Paediatric Cardiology, Astrid Lindgren Children
Hospital, Stockholm
5
Division of Paediatric Cardiology, Umeå University Hospital, Umeå,
Sweden
Background:
Hypertrophic cardiomyopathy (HCM) is the most
common cause of sudden unexpected death in childhood and among
athletes. Success at attempting to prevent sudden arrhythmia death
rests on identifying individuals at increased risk, but most clinically
used strategies for risk stratifications are based on research in adult
HCM patients
Methods:
From the complete Swedish national material on patients
who have died suddenly due to HCM, patients diagnosed before 20
years of age, and with sudden death (SuD) occurring before 30 years
of age were retrieved (SuD group;
n
=
26) and compared with a well
characterised complete regional cohort of paediatric HCM survi-
vors from west Götaland region (Surv group;
n
=
46). Previously
published risk features were compared.
Results:
The groups were comparable in terms of age at diagnosis
(SuD group 9.7
±
5.9 years; Surv group 9.1
±
6.5, mean
±
SD), and
duration of follow up was not significantly different (7.4
±
5.9 vs
10.2
±
8.8 years). The adult criterion of wall thickness of
≥
3 cm has
a low sensitivity of 33%, and a relative risk of 2.9 (95% CI: 1.5–5.2).
Two paediatric wall thickness criteria are much better: a wall thick-
ness
≥
2 cm, relative risk 8.2 (3.1–32.3), sensitivity 91%; and a septal
thickness of
≥
190% of 95th centile value for age: relative risk 7.1
(2.3–21.9) and sensitivity of 84%; the latter has a better specificity
of 74% compared to 63% for
≥
2 cm. Among electrocardiographic
risk markers, an electrocardiographic risk score
≥
6 points (European
Heart J 2010; 31:439) gives a relative risk of 21.2 (3.0–148), a sensi-
tivity of 96% and a specificity of 78%. A 12-lead QRS amplitude
×
duration product
≥
2.2 mV/s has a relative risk of 74.7 (4.3–1303),
a sensitivity of 100% but a somewhat less good specificity of 59%.
Conclusions:
The best electrocardiographic risk markers discrimi-
nate better than any wall thickness criteria for the risk of sudden
arrhythmia death in paediatric HCM.
916: CONGENITAL HEART DISEASE IN NIGERIAN CHIL-
DREN: A MULTICENTRE EXPERIENCE WITH 605 CHIL-
DREN
Wilson Sadoh
1
, Chinyere Uzodimma
2
, Queennette Daniels
3
1
University of Benin Teaching Hospital, Benin City, Nigeria
2
Lagoon Hospital, Lagos, Nigeria
3
Zankli Hospital, Abuja, Nigeria
Background:
Congenital heart disease (CHDx) is among the leading
causes of morbidity and mortality in childhood with a global inci-
dence of 3.5 to 11.3 per 1 000 live births. We report the findings on
the spectrum of echocardiographically diagnosed CHDx from three
different centres across Nigeria.
Methods
: Over a period of 42 months, children who were referred
for echocardigraphic evaluation in the centres located in three
large metropolitan cities were consecutively recruited if they were
confirmed to have identifiable CHDx. The data was collected on
age, gender, types of CHDx and analysed using SPSS 16 (Chicago
IL, USA).
Results:
A total of 605 children were recruited. Of these 296 (48.9%)
were males and 494 (81.7%) had acyanotic congenital heart disease
(ACHDx). The mean age of the study population was 2.1
±
3.5 (range
0–17) years. Almost half of the children (42.5%) were diagnosed
within the first year, only 17% within the neonatal period. The gender
was evenly distributed. The commonest CHD was VSD (46.6%)
followed by ASD (11.7%), PDA (10.6%) and TOF (7.8%). Over half
(55.0%) of the VSD were peri-membranous, and the outlet variety
accounted for 24.5%. Complex CHDx accounted for 7.7%.
Conclusion:
VSD is the commonest CHDx, as has been previously
reported. There is a high proportion of the outlet variety of VSD in
our study population. There is increasing awareness, availability and
use of diagnostic facilities as mirrored in the age distribution of the
children. However access to definitive surgery is poor and draws
attention to the urgent need for affordable surgical facilities in the
country.
924: THE SUCCESSFUL MANAGEMENT OF FAUCIAL
DIPHTHERIA TOGETHER WITH MYOCARDITIS AND
SEPTIC DIPHTHERIA AS COMPLICATIONS
Renny Suwarniaty, Natalia E Jahja, Irene Ratridewi, Soemakto, Setya
Budhy
Department of Child Health Medical School, Brawijaya University,
Saiful Anwar Hospital
Malang, Indonesia
Background:
Severe diphtheria epidemics with high mortality rates
have been recorded since the 16th century and the implementation of
diphtheria immunisation has led to marked decreases in some coun-
tries. But in 2011, Indonesia had the second highest number of cases.
Objective:
To describe a comprehensive management of faucial
diphtheria with myocarditis and septic diphtheria as complications in
an unimmunised child.
Case report:
A five-year-old girl was referred from Bangil General
Hospital because of seizures, parotid gland enlargement due to a
suspected diphtheria infection and encephalitis. The physical exami-
nation found her delirius with nasal flares, a bull neck and pseudo
membranes on both enlarged tonsils. Initial laboratory findings
showed slight anaemia and severe neutropenia with lymphocyte
dominance. For early treatment she was put on a ventilator, given
ADS 100 000 IU, dexamethasone intravenously and an intramuscular
of penicillin procaine injection 1.5 million IU. On the fourth day, she
had myocarditis as a complication. Methylprednisolone 4 mg/kg/day
was administered and then continued at 3 mg/kg/day for a week. The
first routine throat swab culture was positive for
C diphtheriae
and
her father was a carrier. After 11 days of treatment, the methylpred-
nisolone was tapered off. The results of the second to fourth routine
throat swab culture showed no more
C diphtheriae
. The FNAB from
the persistant bull neck found a neck abscess. Incision and drainage
were performed. After one month of treatment, she was given DPT
immunisation and discharged.
Conclusion:
A complicated case of diphtheria required comprehen-
sive management to achieve an optimum outcome.
927: PERSISTENT FIFTH AORTIC ARCH: A CLINICAL
CONUNDRUM
David Lloyd, Rodney Franklin, Yen Ho, Hideki Uemura, Alan Magee
Royal Brompton Hospital, London, UK
Background
: Persistence of the embryonic fifth aortic arch is a
rare and enigmatic condition with variable anatomical forms and
physiological consequences. As such, it may be significantly under-
diagnosed as a primary pathology.
Methods and Results
: A search of the surgical database revealed
five cases of persistent fifth aortic arch (PFAA). Four cases were
diagnosed with congenital heart disease antenatally; one case
presented at six weeks of age. In two cases there was isolated PFAA
with significant left-to-right shunting causing heart failure. In
another two cases the PFAA provided the only source of pulmonary
blood flow in obstructive right heart lesions. In these four cases the
PFAA mimicked the arterial duct, which was absent in all. The PFAA
was sensitive to prostaglandin E
1
in one case with pulmonary atresia.
The two cases with isolated PFAA were treated by surgical ligation
avoiding the need for cardiopulmonary bypass and the two cases with
pulmonary atresia have been successfully repaired. The PFAA in the
fifth case was wide and co-existed with a patent arterial duct, inter-
ruption of the aortic arch (‘type A’), and severe pulmonary stenosis.
