CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
159
Echocardiography images were reviewed on-site by a paediatric
cardiologist and were reported on using the World Heart Federation
echocardiographic criteria. The local team was assisted by an RHD
coordinator from Fiji to build capacity, support and expand the local
RHD programme.
Results:
Of the total of 2 374 school-aged children of Nauru, 462
pupils aged six to 15 years underwent echocardiographic screen-
ing for RHD; 53% were female. Seven cases of definite RHD were
identified including two previously known cases; a prevalence of
15.1 per 1 000. Five had mild disease, one had moderate and one
had severe. Nine additional borderline RHD cases were detected. All
pupils with RHD had a clinical review, received counselling and were
registered with the RHD programme. Those with definite RHD were
commenced on secondary prophylaxis. Pupils with borderline RHD
and a suspected history of rheumatic fever based on clinical review
were also commenced on benzathine penicillin. All cases were
offered follow up by the visiting cardiologist in 12 months.
Conclusion:
A prevalence of definite RHD of 15.1 per 1 000 is
consistent with similar screening studies conducted in neighbouring
Pacific Island countries. A contributing factor to the prevalence of
RHD may include the overcrowded living conditions in Nauru.
367: CURRENT OUTCOME VARIABLES IN NEONATAL
EBSTEIN’S ANOMALY
Jeong Jin Yu
1
, Tae-Jin Yun
2
, Hye-Sung Won
3
, Yu Mi Im
2
, So Yeon
Kang
1
, Hong Ki Ko
1
, Chun Soo Park
2
, Jeong-Jun Park
2
, Young-Hwue
Kim
1
, Jae-Kon Ko
1
1
Division of Paediatric Cardiology, College of Medicine, University
of Ulsan, Korea
2
Division of Paediatric Cardiac Surgery, College of Medicine,
University of Ulsan, Korea
3
Department of Obstetrics and Gynecology, College of Medicine,
University of Ulsan, Korea
Background
: Neonates with Ebstein’s anomaly have a high mortal-
ity rate. Currently, cases of Ebstein’s anomaly have frequently been
detected before birth, and they make up the main group of the popu-
lation of neonatal Ebstein’s anomaly. We performed this study to find
the outcome variables in current populations of neonatal Ebstein’s
anomaly.
Methods
: The records of 59 patients with neonatal Ebstein’s anomaly
who were managed in Asan Medical Centre between January 2001
and June 2012, were investigated retrospectively. In 46 of them, a
prenatal diagnosis was done.
Results
: Operative procedures: pulmonary valvotomy, palliative
shunt operation, tricuspid valvuloplasty or right ventricular exclu-
sion, were performed in 27 patients. Median follow-up period was
1.96 (range 0.0–10.4) years. The overall mortality rate was 23.7%
(14/59). Ten of 14 died during the neonatal period. One-year and
five-year survival rates were 78.6 and 76.3%, respectively. Variables
found to be related to the time to death on univariate analysis were
foetal distress (
p
=
0.002), prematurity (
p
=
0.036), low birth weight
(
p
=
0.003), diameter of atrial septal defect (
p
=
0.022), pulmonary
stenosis/atresia (
p
=
0.001). Carpentier-Edwards classification (
p
=
0.175) and Celermajer index (
p
=
0.958) were not significant vari-
ables. Multivariate analysis showed that foetal distress (
p
=
0.004)
and pulmonary stenosis/atresia (
p
<
0.001) remained significant.
Conclusion
: Foetuses in whom Ebstein’s anomaly are diagnosed
should be closely monitored throughout pregnancy, especially those
with pulmonary obstruction. Strict cooperation between obstetrician,
neonatologist, paediatric cardiologist and paediatric cardiac surgeon
is essential for their survival.
368: SECONDARY QT PROLONGATION DUE TO EXOGEN-
IC STRENGTH TO PRECORDIAL REGION MAY INDUCE
COMMOTIOCORDIS
Seiichi Sato
1
, Tsukasa Torigoe
2
, Fujito Numano
1
, Satoshi Hoshina
3
,
Hiroshi Kanazawa
4
1
Department of Paediatrics and Neonatal Care Centre, Niigata City
General Hospital, Japan
2
Department of of Paediatrics, Nagaoka Red Cross Hospital, Japan
3
Department of of Paediatrics, Niigata University, Japan
4
Department of of Cardiac Surgery, Niigata City General Hospital,
Japan
Background:
Commotiocordis (CC) is defined as the combination
of ventricular fibrillation (Vf) and sudden cardiac death secondary to
blunt chest wall impact, and it occurs especially in healthy children.
We have recently experienced 10 patients who exhibited typical QT
prolongation (LQT) in electrocardiogram (ECG) after receiving
external force against thea thoraco-abdominal region. We studied
the clinical, electrocardiographic and laboratory features to clarify
whether there is a relationship between CC and secondary LQT.
Methods:
We reviewed the charts and ECGs of 10 patients (two girls
and eight boys, six to 15 years) who showed LQT in the first ECG
following blunt trauma against the chest and abdomen, and analysed
their clinical backgrounds, laboratory data and ECGs.
Results:
Clinical features: the cause of the hard knocks was as
follows: traffic accident (four patients), collision during sport (three
patients), and a fall (three patients). Five patients suffered from liver,
spleen and intestinal tract damages, three patients showed a skull
fracture, one exhibited hemi-pneumothorax, and another showed
severe dyspnoea.
ECG findings: the first ECG on arrival disclosed long QTc (442–
503) and two peaks of T wave in V2–3 leads. The second ECG
demonstrated normal QTc (396–429) and normal T wave forms. No
ECGs of the patients showed Vf or cardiac arrest.
Laboratory data: laboratory studies on arrival showed increased
blood glucose concentrations (132–222) and decreased potassium
levels (2.8–3.9), which both subsequently normalised.
Conclusions:
CC usually results from Vf following a mechanical
stimulus on the precordial region. The over-excited sympathetic nerv-
ous system after a severe shock causes over-secretion of adrenaline.
This condition could increase the intra- and extracellular gradient of
potassium ions, which results in LQT. Children may incur sudden
death following blunt trauma to the chest from TdP due to second-
ary LQT.
374: PROGNOSTIC IMPLICATIONS OF TWO-DIMENSION-
AL, M-MODE AND DOPPLER ECHO INDICES OF RIGHT
VENTRICULAR FUNCTION IN CHILDREN WITH PULMO-
NARY ARTERIAL HYPERTENSION
Eias Kassem, Mark Friedberg
Paediatric Cardiology, Hospital for Sick Children and University of
Toronto, Canada
Background:
Right ventricular (RV) function may be a key determi-
nant of mortality in paediatric idiopathic pulmonary artery hyperten-
sion (iPAH) and that associated with congenital heart disease (cPAH),
but echo indices of RV function have not been adequately studied.
Methods:
Children (0–18 years) with iPAH and cPAH were retro-
spectively studied. RV function indices (indexed RV end-diastolic
area, fractional area change, tricuspid annular excursion, right atrial
volume) were analysed at diagnosis and at last follow up. Indices
were compared between iPAH and cPAH patients at baseline and
follow up. Living iPAH patients (group 1) were compared with
deceased/transplanted patients (group 2) at the time of the study.
Cut-off points predictive of survival were generated (ROC) and
Kaplan–Meier survival as analysed.
Results:
Fifty-four patients [36 cPAH (7.5
±
5.9 years; M:F 12:24);
18 iPAH (8.9
±
5.7 years; M:F 7:11); group 1 ‘alive’,
n
=
12, group
2 ‘dead\lung transplant’,
n
=
6) were studied. Median follow up
was 4.3 (0.2–7.4) years. In iPAH patients, RV indices were similar
between groups 1 and 2 at presentation. However, at follow up,
despite similar pulmonary artery pressures and PVRi, RV function
was significantly worse in group 2. A small pericardial effusion was
seen in three patients in group 2 vs 0 in group 1. RV function was
