CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
156
AFRICA
Results
: We studied 11 LQTS subjects exhibiting complex foetal
arrhythmias. Mutations in one of the three major LQTS susceptibil-
ity genes were identified in nine: SCN5A (five), KCNH2 (two),
and KCNQ1 (two). Most mutations were
de novo
including four
with SCN5A-R1623Q. TdP occurred in seven foetuses (mean gesta-
tional age
=
30.5 weeks) and eight neonates; 2° AVB occurred in
four foetuses/neonates. TdP exhibited two patterns: incessant and fast
(
>
270 bpm) or intermittent and slow (
<
250 bpm). All cases with
SCN5A mutation had fast-incessant TdP while cases with KCNH2
mutations had slow-intermittent TdP. Cases with KCNQ1 mutations
had 2° AVB. Foetuses with TdP were delivered earlier (33.6 weeks)
than those with 2° AVB (38.5 weeks). Neonatal QTc of subjects with
TdP were longer (652
±
42 ms) than subjects with 2° AV block (507
±
43,
p
=
0.01). Prenatal treatment was administered in six cases with-
out maternal complications; four foetuses improved and TdP ceased
in two. Despite medical and pacemaker therapy, cardiac arrest (
n
=
6) resulting in sudden death (
n
=
1) was common.
Conclusion:
Complex rhythm phenotypes of foetal LQTS have
genotype-suggestive features that, along with QTc duration, may risk
stratify the perinatal management of LQTS.
321: LEFT-AXIS DEVIATION IN THE PAEDIATRIC POPU-
LATION: A MODERN STUDY
Denis Levy
1
, William Keen
2
, Mark Nunes
3
, Ulrika Birgersdotter-
Green
4
, Abraham Rothman
5
1
Division of Paediatric Cardiology, Southern California Permanente
Medical Group, USA
2
Division of Cardiology, Southern California Permanente Medical
Group, USA
3
Division of Genetics, Southern California Permanente Medical
Group, USA
4
Division of Cardiology, University of California, San Diego, USA
5
Department of Paediatrics (Cardiology), University of Nevada
School of Medicine, USA
Background:
Few recent large studies have investigated left-axis
deviation on electrocardiogram with associated medical conditions
in children. Using electronic medical records and the computer data-
base of electrocardiograms within an integrated healthcare system,
we documented these associations.
Methods:
During a five-year period (2007–2012), the computer
database of all electrocardiograms performed (3 267 893 in 1 360
489 individuals) were screened. Patients 0–18 years of age with
left-axis deviation (–30° to –90°) were identified. Each patient’s
electronic medical record was used to identify known associations
with left-axis deviation and these were recorded.
Results:
There were 122 484 electrocardiograms performed on
79 938 individuals (0–18 years of age). Of these individuals, 886
demonstrated left-axis deviation on ECG (1.1%). Of these, 491 were
considered normal as they had no record of heart disease, thoracic
or genetic abnormalities. There were 279 individuals with struc-
tural heart abnormalities: ventricular septal defect (65), complete
atrio-ventricular septal defect (59), partial atrio-ventricular septal
defect (22), tricuspid atresia (10), single-ventricle arrangement (24),
atrial septal defect (19), pulmonary stenosis (18), coarctation of
the aorta (10), left ventrticular hypertrophy/cardiomyopathy, aortic
stenosis (12), tetralogy of Fallot (11) (majority postoperative), patent
ductus arteriosus (five), transposition of the great arteries (five) and
isolated anomalous pulmonary venous return (four). There were 57
with conduction abnormalities: Wolff–Parkinson–White syndrome
(37), paced rhythm (17), long QT syndrome (two) and complete
heart block without a pacemaker (one). There were 10 patients with
Noonan syndrome, and 32 with short stature or other genetic abnor-
malities. Eighteen had scoliosis, pectus excavatum or dextrocardia.
Conclusions:
This study documents the associations of left-axis
deviation in children and demonstrates the utility of using electronic
medical records within an integrated medical system to gather such
data.
326: SURGICALAND INTERVENTIONALMANAGEMENTOF
PATENT DUCTUS ARTERIOSUS IN PREMATURE INFANTS
INWESTERNAUSTRALIA: A NINE-YEAR EXPERIENCE
Jennifer Melvin
3
, Rolland Kohan
2,3
, Stephen Shipton
1
, David
Andrews
1
, Karen Simmer
2,3
, James Ramsay
1
1
University of Notre Dame, Australia
2
King Edward Memorial Hospital for Women, University of Western
Australia, Australia
3
Princess Margaret Hospital for Children, Australia
Background:
There is significant controversy regarding optimum
PDA management in preterm infants. KEMH is the sole tertiary
perinatal hospital caring for all infants born at
<
30 weeks in WA.
KEMH has adopted a conservative approach to neonatal duct ligation
for many years. After discharge it was apparent some preterm infants
required intervention for PDA at the only tertiary children’s hospital
(PMH) in WA.
Methods:
The aim of this study was to review a geographic cohort
of all surviving preterm infants born at
<
30 weeks in WA from
January 2003 to December 2011 requiring surgical or interventional
closure of a PDA. The medical records of these infants were reviewed
to determine complications and outcomes. The neonatal survival
and major duct-related morbidity data for the whole cohort were
compared to data from the Australian and New Zealand Neonatal
Network (ANZNN).
Results:
A total of 1 652 live births (GA
<
30 weeks) had neonatal
management at KEMH, of which 1 475 (89.5%) infants survived to
discharge. Only 2.0% of survivors (median GA 25 weeks and median
BW 735 g) received surgical or interventional closure at PMH
during the study period. Eleven infants had surgical closure during
their initial neonatal hospital admission and 20 after discharge; 17
interventional and three surgical. All surgical and interventional
procedures produced successful closure. Post surgery, three had a
pneumothorax and one vocal cord palsy. One death occurred late and
unrelated to surgical closure. No significant complications occurred
with interventional closures and 14 were day cases. Overall, the
incidence of CLD and NEC in this cohort was similar to the ANZNN
mortality and morbidity data.
Conclusions:
Most PDAs close with conservative medical treatment.
Continued surveillance is needed to manage PDAs that remain open.
There is no evidence of any increase in morbidity or mortality using
this approach.
332: NURSE-LED ECHOCARDIOGRAPHIC SCREENING
FOR RHEUMATIC HEART DISEASE IN FIJI: DESIGN OF A
TRAINING SYLLABUS
Daniel Engelman
1,2
, Joseph Kado
3
, Bo Remenyi
4
, Samantha
Colquhoun
1,4
, Jonathan Carapetis
4
, William May
3
, Nigel Wilson
5
,
Andrew Steer
1,2
1
Centre for International Child Health, University of Melbourne,
Australia
2
Royal Children’s Hospital, Melbourne, Australia
3
Colonial War Memorial Hospital, Suva, Fiji
4
Menzies School of Health Research, Darwin, Australia
5
Starship Children’s Hospital, Auckland, New Zealand
Background:
Rheumatic heart disease causes significant morbid-
ity and mortality in Fiji. Antibiotic prophylaxis is most effective for
mild cases, many of whom are asymptomatic. Screening with echo-
cardiography is the most sensitive screening approach, but is limited
by lack of resources, particularly experienced cardiologists and
technicians. Training nurses in basic echocardiography for screen-
ing is a potential solution. A previous pilot programme showed that
training nurses is feasible, but found that improvements were needed
in screening protocols, referral criteria and the extent of practical
experience. We hypothesised that a structured eight-week syllabus,
including extensive supervised field experience, would provide
appropriate and adequate training for echocardiographic screening
for RHD.
