CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
260
AFRICA
was homozygous for the mutation, and the mutation was not detected
in 360 French-Canadian controls. A 6-year-old homozygous muta-
tion carrier developed signs of SSS/POIC during the course of the
study.
Conclusion:
Genetic analysis of the novel SSS/CIPO syndrome
demonstrates a fundamental new link between the two main pace-
makers in humans. A predictive value of
SGOL1
testing was found
in one case and highlights the need to develop preventive strategies
based on molecular findings.
1685: PRESENCE AND PATTERNS OF MYOCARDIAL
GADOLINIUM ENHANCEMENT IN CHILDHOOD DILAT-
ED CARDIOMYOPATHY
Heiner Latus
1
, Kerstin Gummel
1
, Stefan Rupp
1
, Hakan Akintuerk
2
,
Christian Jux
1
, Juergen Bauer
1
, Dietmar Schranz
1
, Christian Apitz
1
1
Pediatric Heart Center, University Children’s Hospital, Giessen,
Germany
2
Division of Cardiovascular Surgery, University Clinic Giessen,
Germany
Background:
Different patterns of myocardial gadolinium enhance-
ment (MGE) including mid-wall fibrosis have been reported in
up to 42% of adult patients with non-ischaemic dilated cardio-
myopathy (NI-DCM). In these studies, MGE was associated with
pronounced LV remodelling and predicted adverse cardiac outcomes.
Accordingly, the purpose of our study was to determine the presence
and patterns of MGE in children with NI-DCM.
Methods:
Patients presenting with severe congestive heart failure
who were admitted for evaluation of heart transplantation at our
centre underwent CMR examination on a 3T system that consisted
of ventricular functional analysis and assessment of MGE for detec-
tion of myocardial scars. Ischaemic DCM was excluded by coronary
angiography and right ventricular endomyocardial biopsies ruled out
ongoing myocarditis.
Results:
Fifteen infants and children (mean age 9.3
±
7.6 months)
with severe LV dilatation (mean indexed LVEDV 160
±
55 ml/m
2
)
and LV dysfunction (mean LV-EF 16
±
7%) were examined. MGE
was detected in 2 of the 15 patients (13%) appearing in patterns
characterised as focal patchy and transmural respectively. None of the
patients exhibited ‘classic’ mid-wall enhancement.
Conclusions:
Despite the small cohort size, the observed differ-
ences in frequency and type of myocardial fibrosis compared to
adult patients might influence the therapeutic strategy in childhood
NI-DCM. However, it remains unclear whether these findings reflect
preserved endogenous repair mechanisms that enable favourable
remodelling. Larger trials are needed to assess the prognostic impli-
cations of MGE in childhood NI-DCM and to determine whether
MGE might be used for risk stratification as demonstrated in adult
NI-DCM.
1687: MID-TERM OUTCOMES OF A HANDMADE TRILE-
AFLET VALVED RIGHT VENTRICLE TO PULMONARY
ARTERY CONDUIT
Zeena Makhija, Rajesh Sharma
Fortis Escorts Heart Institute and Research Center, New Delhi, India
Background:
We have been using a new technique to make bovine
pericardial valved conduits to overcome the shortage of cryopre-
served homografts for right ventricle (RV) to pulmonary artery (PA)
reconstructions. Gore Preclude pericardial membrane (0.1 mm thick-
ness) is used for making the trileaflet valve. We reviewed our experi-
ence to analyse postoperative outcomes and their mid-term results.
Materials and methods
: Between 2007 and 2012, 203 patients (71.9
% male) underwent primary right ventricle outflow tract reconstruc-
tion using bovine pericardial valved conduits in our centre. Their
ages ranged from 6 days to 42 years. Diagnoses included ‘ventricular
septal defect (VSD), pulmonary atresia (PA), MAPCAs’(
n
=
61),
‘VSD, PA’(
n
=
52), ‘truncus arteriosus’ (
n
=
20), ‘double outlet
right ventricle, VSD, pulmonary stenosis (PS)’ (
n
=
18), ‘tetralogy
of Fallot/absent pulmonary valve’ (
n
=
12), ‘corrected transposition,
VSD, PS’ (
n
=
11), ‘transpostion of great arteries, VSD, PS’ (
n
=
7)
and ‘Ross procedure’ (
n
=
11). Fifty-four (26.6%) patients had under-
gone a prior palliative shunt procedure. The sizes of the conduits
implanted ranged from 12 to 24 mm (median 18).
Results:
Conduit-related early complication rate was 0.5% (
n
=
1)
(conduit revision for early pulmonary artery thrombosis). The mean
hospital stay was 13.2
±
31.6 days. Early mortality was 7.4 % (
n
=
15). The mean follow-up period was 61 months. Twenty-three (11.3
%) patients underwent conduit replacement. Of these, acquired distal
conduit stenosis was observed in 10.8 % (
n
=
22) and 0.5 % (
n
=
1)
developed infective endocarditis. Late mortality was 0.5 % (
n
=
1). A
gradient
>
30 mmHg was detected in 13.4 % (
n
=
27) patients, while
15.3 % (
n
31) developed conduit valve regurgitation greater than 2+.
Conclusions:
The handmade valved conduit seems to offer equiva-
lent results as compared to other conduits available today at midterm
follow-up. Long-term results are awaited. Easy availability and low
cost are additional advantages of this conduit.
1690: CONGENITAL HEART DISEASE AND THE EMISSION
OF DEVELOPMENTALTOXICANTS INALBERTA, CANADA
Deliwe Ngwezi
1
, Alvaro Osornio Vargas
1
, Brad Saretsky
2
, Ambika
Mehta
3
, Eric White
4
, Sujata Chandra
5
, Debbie Fruitman
7
, Lisa
Hornberger
1,5
1
Department of Pediatrics, University of Alberta, Stollery Children’s
Hospital, Edmonton, Alberta, Canada
2
Alberta Health Services, Canada
3
Faculty of Science University of Alberta, Edmonton, Canada
4
Faculty of Medicine and Dentistry University of Alberta, Edmonton,
Canada
5
Department of Obstetrics and Gynecology, University of Alberta,
Edmonton, Canada
6
Department of Pediatrics, University of Calgary, Alberta Children’s
Hospital, Calgary, Canada
Background:
Congenital heart disease (CHD) is a significant
global public health issue affecting 1% of all live births and the most
common lethal congenital abnormality in infancy. Although CHD
may occur in the presence of aneuploidy and single gene disorders,
in most affected children the cause is unknown. The role of envi-
ronmental pollutants has recently received attention. We sought
to explore the association of developmental toxicants (DTs) from
industrial sources and CHD in Alberta, Canada through an interdis-
ciplinary multi-step study.
Materials and methods
: In this ecological study we collected the
following data: (1) Chemical emissions between 2003-2010 from
Canada’s National Pollutant Release Inventory; (2) CHD cases (born
between 1/06/04 and 31/08/11) from Stollery Children’s Hospital
Xcelera database; and (3) annual births from Statistics Canada/
Alberta Reproductive Health. We used Scorecard criteria to identify
emitted DTs and corresponding toxic equivalent potential values in
order to normalise emissions (risk score). The location of the emit-
ting facilities and CHD cases were determined using the longitude
and latitude coordinates. Data was aggregated by year and cases were
assigned to the year when pregnancy occurred. Statistical analysis
was done using STATA 12.
Results:
We identified 1 903 cases of CHD and 17 DTs emitted to
air (99% of all emissions) during the study period. The average rate
of CHD was 5.8
±
1.09/1 000 live births with the most commonly
encountered including septal (47.9%), left ventricular outflow tract
obstruction (15.2%) and conotruncal (12.2%) defects. The average
DTs emissions were 7 817 417
±
570 380 tonnes. Annual sulphur
dioxide, ethylene oxide, 1.3-butadiene, hexachlorobenzene and
carbon disulphide average risk scores strongly correlated with CHD
rates (
r
=
0.89;
r
=
0.89;
r
=
0.87;
r
=
0.84 and
r
=
0.84, respectively,
p
≤
0.01).
Conclusions:
These findings suggest that DTs emitted to air in
Alberta could have an impact on the development of CHD. An
