CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
223
ac morbidity in Fontan patients. Moreover, the association between
the OUES and cardiac morbidity was independent of relevant base-
line clinical information.
1230: LONG-TERM MANAGEMENT OF CO-MORBIDITIES
ASSOCIATED OBESITIY IN CHILDREN WITH PREADER-
WILLI SYNDROME
Renny Suwarniaty, Anik Puryatni, Haryudi Aji Cahyono, Januar
Wijaya
Department of Child health, University of Brawijaya, Saiful Anwar
Hospital, Malang, Indonesia
Background
: Prader–Willi syndrome (PWS) is the most common
syndromic form of obesity, caused by deletion in the 15q11-13.
Management of obesity and its complications is a critical part of care.
Objective:
To present long-term management of co-morbidities
associated with obesity in children with PWS.
Case:
A seven-year-old girl was admitted to Saiful Anwar General
Hospital due to dyspnoea on effort. She was born to non-cosanguin-
eous and healthy biological parents. Her developmental progress was
delayed. She had mild mental retardation, speech articulation defect
and global developmental delay. She developed feeding difficulties
in infancy but became preoccupied with food, accompanied by a
compulsion to eat at three years old. At five years she had morbid
obesity. Physical examination showed dysmorphic features. Genetic
testing indicated deletion in the 15q11-13. We found dilated cardio-
myopathy and type 2 diabetes mellitus as a complication of obesity.
Echocardiography showed decreased ejection fraction and mild MI/
MR. Long-term management included monitoring and management
of the co-morbid conditions, such as medication for the cardiomyopa-
thy, a well-balanced, low-calorie diet, regular exercise, environmental
modification and antidiabetic agents. Early intervention and special
education, followed by supportive employment, were appropriate to
address the developmental disabilities. Physical, occupational and
speech therapy were started after the establishment of a diagnosis.
This resulted in a decrease in BMI, improvement in the cardio-
myopathy, improvement in developmental and behavioural problems,
including food-seeking behaviour, thus improving the quality of life.
Conclusion.
Comprehensive management of Prader–Willi syndrome
resulted in improvement in the quality of life of the patient.
1234: THE USE OF COMPUTED TOMOGRAPHY ANGIOG-
RAPHY (CTA) IN THE EVALUATION OF CONGENITAL
HEART DISEASE
Darshan Reddy
Department of Cardiothoracic Surgery, Inkosi Albert Luthuli Central
Hospital, Durban, South Africa
Background:
While echocardiography is the commonest non-
invasive imaging modality used in the diagnosis of congenital heart
disease, CT angiography may be useful to further elucidate anatomi-
cal detail that is crucial to accurate surgical planning.
Methods:
From 2009 to the present, at our institute CT angiography
has been used selectively to evaluate pulmonary artery size, pulmo-
nary venous drainage, aortic arch abnormalities and coronary artery
abnormalies in patients with congenital heart disease. Furthermore,
select patients who underwent corrective or palliative surgery were
evaluated by CTA.
Results:
The use of CTA as an adjunct to echocardiography has
enabled accurate pre-operative assessment and facilitated surgical
decision-making.
Conclusions:
The frequent use of CTA has enabled cardiologists,
congenital cardiac surgeons and radiologists alike to familiarise
themselves with the anatomical details evident on CT angiography,
and correlation with the intra-operative findings enhances the inter-
pretation of the study.
1236: INTERVENTION FOR RE-COARCTATION IN THE
SINGLE-VENTRICLE RECONSTRUCTION TRIAL: INCI-
DENCE, RISK AND OUTCOMES
Kevin Hill
Duke University Medical Centre, USA
Objectives:
To determine the incidence of re-coarctation (re-CoA),
risk factors and outcomes in patients with single right ventricle
lesions after the Norwood procedure.
Methods:
The cohort included subjects randomised to right ventri-
cle–pulmonary artery shunt (RVPAS) or modified Blalock–Taussig
shunt (MBTS) in the Single Ventricle Reconstruction (SVR) Trial.
Re-CoA was defined by intervention, either catheter-based or surgi-
cal. Univariate analysis and multivariable Cox proportional hazard
models were performed adjusting for centre.
Results:
Of the 549 SVR subjects, 97 (18%) underwent 131 interven-
tions (92 balloon aortoplasty; 39 surgical) for re-CoA. Intervention
typically occurred at pre-stage II catheterisation (
n
=
71, 54%) or at
stage II surgery (
n
=
38, 29%). Median (range) age and catheterisa-
tion gradient at first intervention were 4.9 months (1.1–10.5) and
17 mmHg (0–60). Centre intervention rates varied from 0–50%.
In multivariable analysis, re-CoA was not associated with assigned
shunt type, but was associated with actual shunt type received (HR
2.0 for RVPAS vs MBTS,
p
=
0.02), and Norwood discharge peak
echo-Doppler arch gradient (HR 1.07 per 1 mmHg,
p
<
0.01).
No other demographic, anatomical or surgical variables predicted
intervention. Subjects with re-CoA demonstrated co-morbidities at
pre-stage II evaluation, including higher pulmonary arterial pressures
(15.4
±
3.0 vs 14.5
±
3.5 mmHg;
p
=
0.05), higher PVR (2.6
±
1.6
vs 2.0
±
1.0 WU
×
m²;
p
=
0.04) and increased echocardiographic
volumes (end-diastolic volume: 126
±
39 vs 112
±
33 ml/BSA
1.3
;
p
=
0.02). There was no difference in 12-month post-randomisation
transplant-free survival for those with and without re-CoA (91 vs
93%;
p
=
0.7).
Conclusions:
Intervention for re-CoA was common and varied by
centre. Intra-operative crossover due to complex arch anatomy may
explain the association with receipt of an RVPAS. Although those
undergoing intervention demonstrated co-morbidities prior to stage II,
there was no difference in one-year transplant/mortality. Further eval-
uation is warranted to evaluate effects of the morbidity rate of re-CoA.
1239: FACTORS ASSOCIATED WITH SERUM B-TYPE
NATRIURETIC PEPTIDE LEVELS IN INFANTS WITH
SINGLE VENTRICLE
Ryan Butts
1
, Daphne Hsu
2
, Victor Zak
3
, David Hehir
4
, Paul Kantor
5
,
Jami Levine
6
, Renee Margossian
6
, Derek Williams
7
, Andrew Atz
1
,
Paediatric Heart Network investigators
1
Medical University of South Carolina Children’s Hospital, USA
2
The Children’s Hospital at Montefiore, USA
3
New England Research Institutes, USA
4
Children’s Hospital of Wisconsin, USA
5
The Hospital for Sick Children, USA
6
Children’s Hospital of Boston, USA
7
Brenner Children’s Hospital, USA
Background:
Data regarding the value of B-type natriuretic peptide
(BNP) in infants with single-ventricle (SV) physiology are lacking.
The objective of this analysis was to describe the changes in BNP in
infants with SV physiology before and after superior cavopulmonary
connection (SCPC) surgery.
Methods
: BNP levels were measured by a core laboratory pre-SCPC
(5.0
±
1.6 months) and at age 14 months during a multicentre trial
of ACE inhibition therapy in infants with SV. Associations between
BNP, clinical and laboratory variables were assessed using log-trans-
formed BNP. To identify the predictors of BNP, longitudinal analysis
was performed for each set of grouped variables (echo, catheterisa-
tion, growth), and multivariable analysis was performed using patient
characteristics at both visits and neurodevelopmental predictors at
the 14-month visit.
