CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
177
2
Department of Medicine and Therapeutics, Chinese University of
Hong Kong, China
We report on a 15-year-old adolescent girl with good previous
health, who presented with sudden onset of palpitation and dizziness.
Examination revealed fast, irregular apical beats (170 beats/min) with
normal blood pressure. Electrocardiogram showed typical fast atrial
fibrillation (AF). Transthoracic echocardiogram confirmed there was
no structural abnormality, but the cardiac function was suboptimal
with biventricular hypokinesia. No intracardiac thrombus was identi-
fied. Successful synchronised cardioversion was performed without
anticoagulation, and the rhythm was converted to sinus without any
thromboembolic events.
The girl was put on new-generation anticoagulant and anti-
arrhythmic medication, dabigatran and dronedarone, respectively, for
a short period of time, and there was no recurrence of AF. Lone AF
is rare in the paediatric population. The thromboembolic risks are
exceedingly low. Therefore, aggressive rhythm control would be the
approach in its management, and anticoagulation before cardiover-
sion may not be indicated. Genetic predisposition has become a new
trend in the study of young-onset lone AF. The updated evidence of
managing lone AF in children and adolescents is discussed, including
the use of dabigatran and dronedarone.
625: VENTRICULAR HYPERTROPHY WITH OUTFLOW
TRACT OBSTRUCTION VERSUS DILATED CARDIOMYO-
PATHY IN NEUROBLASTOMA
Sit Yee Kwok, Frankie Wai Tsoi Cheng, Amy Fung Cheung Lo, Man
Ching Yam, Chi Kong Li
Department of Paediatrics, Prince of Wales Hospital, Chinese
University of Hong Kong, China
Catecholamine-associated hypertension secondary to neuroblastoma
can occasionally be demonstrated, but cardiomyopathies caused by
neuroblastoma have been rarely reported. We report two cases of
neuroblastomas with
different extremes of severe
cardiomyopathic
changes and complications.
The first case was a two-month-old girl with a retroperitoneal mass,
which was incidentally found in an ultrasonic examination
during a
postnatal evaluation of antenatal hydronephrosis. Hypertension was
detected and echocardiogram showed severe concentric left ventricu-
lar hypertrophy with mid-ventricular obstruction, with a peak pressure
gradient of 51 mmHg, simulating hypertrophic obstructive cardio-
myopathy (HCOM).
The second case was a 30-month-old girl who
presented with abdominal discomfort, subsequently developed refrac-
tory hypertension and heart failure. Echocardiogram
revealed a mark-
edly dilated left ventricle with poor cardiac contractility. Computed
tomographic scan indicated a huge abdominal mass.
Both girls were diagnosed with neuroblastoma, and high levels
of urinary catecholamine and its metabolites were identified. Choice
of antihypertensives was different. Beta-blocker was used in the first
case to promote left ventricular filling, while angiotensin converting
enzyme inhibitor was used in the second case. The anthracycline
group of chemotherapy was avoided in the initial phase of treat-
ment. Chemotherapy and subsequent removal of the tumour led to
successful normalisation of blood pressure and regression of abnor-
mal cardiomyopathic changes.
HOCM-like features secondary to
catecholamine-secreting neuroblastoma is exceedingly rare. The very
early onset of HOCM suggested that the remodelling of the heart
had already started during her foetal period. Updated management of
catecholamine-induced
cardiomyopathy associated with neuroblas-
toma is also discussed.
627: ROUTINE HEAD ULTRASOUND SCANS ARE NOT
INDICATED IN THE PRE-OPERATIVE EVALUATION OF
INFANTS WITH CONGENITAL HEART DISEASE
Danielle Rios
1
, Stephen Welty
1
, Julia Gunn
2
, John Beca
3
, Charles
Minard
4
, Michelle Goldsworthy
5
, Lee Coleman
6
, Jill Hunter
7
, Dean
Andropoulos
8
, Lara Shekerdemian
5
1
Section of Neonatology, Baylor College of Medicine and Texas
Children’s Hospital, Texas, USA
2
Murdoch Children’s Research Institute, Australia
3
Department of Paediatric Intensive Care, Starship Children’s
Hospital, Aukland, New Zealand
4
Dan L Duncan Institute for Clinical and Translational Research,
Baylor College of Medicine and Texas Children’s Hospital, Texas,
USA
5
Critical Care Medicine, Baylor College of Medicine and Texas
Children’s Hospital, Texas, USA
6
Department of Radiology, Royal Children’s Hospital Melbourne,
Australia
7
Diagnostic Imaging, Baylor College of Medicine and Texas
Children’s Hospital, Texas, USA
8
Anesthesiology, Baylor College of Medicine and Texas Children’s
Hospital, Texas, USA
Background:
Head ultrasound scans (HUS) are a routine part of
the pre-operative evaluation of young infants with CHD in many
institutions. HUS have the advantage of being inexpensive, easily
performed and readily available. However, the utility of HUS in the
cardiac population is unknown. More recently, MRI has become a
valuable tool in the early detection of brain injury in infants with
CHD. The purpose of this study was to assess the utility of pre-
operative HUS in a cohort of young infants who also underwent pre-
operative MRI as part of a prospective research study of brain injury
in infants undergoing surgery for CHD.
Methods:
One hundred and sixty-seven infants born at 35 weeks’
gestation or greater and due to undergo surgery for CHD were
included in this three-centre study. None of the patients had any clini-
cal signs or symptoms of pre-operative brain injury and all received
HUS and brain MRI. All imaging was reported by experienced
neuroradiologists who were blinded to any specific clinical details of
the study participants. The findings were compared to evaluate for
the presence of brain injury.
Results:
Pre-operative brain injury was found on HUS in five infants
(3%) and MRI in 44 infants (26%) (
p
<
0.001). The abnormalities
on HUS were: intraventricular haemorrhage in four that was not
confirmed on MRI, performed within a few days after HUS, and
periventricular leukomalacia in one patient. The predominant MRI
abnormality was white matter injury (32 infants). Other findings
included infarct (14), haemorrhage (five), and lesion or infarct of
deep nuclear grey matter (two).
Conclusions:
Pre-operative brain injury on MRI was present in 26%
of infants with CHD, but only 3% had any evidence of brain injury on
HUS. Among positive HUS, 80% were false positives. Our findings
suggest that routine HUS are not indicated in asymptomatic term or
near-term neonates undergoing surgery for CHD.
629: CONGENITAL HEART DISEASE IN MILLER–DIEKER
SYNDROME
Faraz Quraishi
1
, Joshua Dyme
2
, Crystal McLeod
1
, Mohy Kotb
3
, Neily
Oundjian
2
1
Department of Paediatrics, University of Medicine and Dentistry of
New Jersey, USA
2
Department of Paediatrics, Hackensack University Medical Centre,
USA
3
Division of Paediatric Cardiology, University of Rochester Medical
Centre, USA
Background:
Miller–Dieker syndrome (MDS) is a contiguous gene
deletion syndrome caused by deletion of 17p13.3. It is characterised
by severe lissencephaly, characteristic facial features, severe neuro-
logical abnormalities, and occasionally other congenital anomalies
such as renal, gastrointestinal and cardiac defects. The lissencephaly
in MDS is due to deletion of the
LIS1
gene. The dysmorphisms and
other features are believed to be due the deletion of genes distal to
LIS1
. The incidence of CHD among individuals with MDS has been
