CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
147
Infants
<
37 weeks’ gestation and those who underwent stage II with-
out being discharged after the Norwood procedure were excluded.
The primary outcome was change in WHO weight-for-age
z
-score
(WAZ) between birth and discharge. Growth faltering was defined
as a drop of
≥
0.5 in WAZ and failure to thrive (FTT) as a WAZ
<
–2. The effect of predictor variables on change in WAZ was analysed
using multivariable linear regression with bootstrapping.
Results:
The change in WAZ from birth to discharge (at 1.1
±
1.0
months) for 424 infants in the analytic cohort was –1.40
±
0.80 and
was similar in the two shunt groups (
p
=
0.32). Growth faltering was
present in 91%, with FTT in 37% at discharge. Independent risk
factors associated with a decline in WAZ (
R
2
=
0.33) were male gender
(
P
<
0.001), higher birth weight (
p
<
0.001), pre-Norwood enteral
feeds (
p
=
0.01), longer cardiopulmonary support time (
p
=
0.03),
and more ventilator (
p
=
0.04), and hospitalisation days (
p
<
0.001).
Conclusions:
Nearly all infants discharged after the Norwood proce-
dure in the SVR trial had growth faltering and over a third had FTT,
irrespective of shunt type. Males were at higher risk for a decline in
WAZ and pre-Norwood enteral feeds did not prevent growth fail-
ure. Suboptimal growth was associated with longer intra-operative
support and medical morbidity. Strategies to improve growth during
the Norwood hospitalisation warrant further attention.
203: OUTCOME OF CONGENITAL HEART DEFECTSASSO-
CIATEDWITH 22Q11 DELETION
Orhan Uzun
1
, Kadir Babaoglu
1,2
, David Tucker
3
, Stephen Jolles
1
, Dirk
Wilson
1
1
University Hospital of Wales, Cardiff, Wales, UK
2
Kocaeli University, Izmit, Istanbul, Turkey
3
Congenital Anomaly Register, CARIS, Swansea, Wales, UK
Background
: 22q11.2 deletion is the most common chromosomal
microdeletion syndrome. It has a strong association with conotrun-
cal heart defects and may exert negative influence on the clinical
outcomes. We aimed to define the prevalence of congenital heart
defects in patients with 22q11.2 deletion, timing of detection and
the outcomes.
Methods:
We reviewed the patients with 22q11.2 deletion who either
presented to the University Hospital of Wales with congenital heart
disease or were reported to the Congenital Anomaly Register and
Information Service for Wales (CARIS) between 1990 and 2011.
Results:
One hundred and two cases of 22q11.2 deletion were report-
ed during the study period, suggesting a prevalence of 1.2 per 10 000
total births (one in 8 335 total births); 95 cases had both 22q11.2
deletion and at least one associated congenital heart defect (CHD)
(93%). Interrupted aortic arch, ventricular septal defect, tetralogy of
Fallot and truncus arteriosus, and pulmonary atresia were the most
common defects. Seven cases exhibited a normal heart. In 18 cases,
22q11.2 deletion was detected in foetal life; 13 babies were delivered
alive (72%), pregnancies were terminated in four, and still birth
occurred in one. In 84 patients 22q11.2 deletions were confirmed
postnatally; 88 of 102 patients remained alive during the mean follow
up of 16.2
±
11.4 years (range 1–52 years), but 16 patients died
(19%) of whom eight died after birth, seven in the first year of life,
and one at age 3.8 years.
Conclusions:
Ninety-three per cent of patients with 22q11.2 deletion
exhibited CHD. In spite of the antenatal detection rate being low,
survival rates were similar for all cases irrespective of the presence of
CHD. Conotruncal abnormalities were most prevalent heart defects.
Most deaths occurred within the first year of life but beyond infancy
survival was favourable.
204: MISDIAGNOSIS OF BLAND–WHITE–GARLAND
SYNDROME: REPORT OF TWO CASES WITH DIFFERENT
PRESENTATIONS
Akbar Molaei
1
, Majid Maleki
2
, Bahman Rastkar Hemmati
1
1
Tabriz University of Medical Sciences, Madani Heart Centre,Tabriz,
Iran
2
Tabriz University of Medical Sciences,Tabriz Children’s Hospital,
Tabriz, Iran
Background:
Anomalous origin of the left coronary artery from the
pulmonary artery (ALCAPA) or Bland–White–Garland syndrome is
usually an isolated cardiac anomaly but, in rare incidences, has been
described with patent ductus arteriosus, ventricular septal defect,
tetralogy of Fallot, and aorta. This syndrome may cause sudden death
in infants and young people but in this case report we present two
different types of presentation.
Case report:
The first case was a three-year-old girl diagnosed
with dilated cardiomyopathy since infancy. Her electrocardiogra-
phy showed prominent Q waves in the lateral leads. A dilated right
coronary artery was shown by echocardiography. The second case
was a girl with prolapsed mitral valve and chest pain but similar to
the first case as she had prominent Q waves in the lateral leads on
electrocardiography and a dilated right coronary artery but without
heart failure.
Conclusion:
ALCAPA in children may present with ambiguous pres-
entations from dilated cardiomyopathy and full-blown heart failure to
an atypical chest pain attributed to prolapsed mitral valve.
205: LIVER STIFFNESS: A NEW, RAPID AND NON-INVA-
SIVE METHOD OF CENTRALVENOUS PRESSURE EVALU-
ATION IN PATIENTSWITH CONGENITAL HEART DISEASE
Zakaria Jalal
1
, Xavier Iriart
1
, Julien Vergnol
2
, Juliette Foucher
2
,
Guilhon Emmanuelle
1
, Victor De Ledinghen
2
, Jean-Benoit Thambo
1
1
Paediatric and Congenital Cardiology, CHU, Bordeaux, France
2
Hepatology, CHU, Bordeaux, France
Background:
Transient elastography is a rapid, non-invasive and
reproducible approach to assess liver fibrosis by measuring liver stiff-
ness (LS). However, because the liver is enveloped by a capsule, any
variation in parenchymal fluid content could theoretically affect LS.
LS has been correlated to central venous pressure (CVP) in an animal
model. We aimed to determine the correlation between LS and CVP
in children and adults with congenital heart disease.
Methods:
In this ongoing prospective study, all patients referred for
right heart catheterisation were included. Measurements of mean
right atrial pressure were obtained under general anesthesia (Fi02
=
21%) using an Optitorque 5 French catheter. The patients underwent
10 LS measurements (median value taken as representive) by tran-
sient elastography (Fibroscan, Echosens, France) within the 24 hours
before catheterisation. The results of LS are expressed in kilopascals
(kPa).
Results:
Fourteen children (mean age
=
9
±
6 years, 64% male) and
14 adults (mean age
=
34
±
17 years, 66% male) have been included
so far. Catheterisation indications were pulmonary angioplasty (
n
=
5), Melody valve implantation (
n
=
2), fenestration occlusion
after a Fontan procedure (
n
=
1), aortic coarctation stenting (
n
=
1),
atrial septal defect closure (
n
=
4) and pre-operative assessement of a
complex congenital heart defect (
n
=
16). Mean right atrial pressure
was 8.2
±
3.3 mmHg and mean LS was 8.1
±
4.4 kPa. Correlation
between LS and mean right atrial pressure was excellent for these
first 28 patients (
r
=
0.86,
p
<
0.001).
Conclusion:
Liver stiffness is a new, rapid and reliable method to
evaluate CVP in patients with congenital heart disease. This non-
invasive parameter could potentially be useful for patients in whom
CVP play a key role, especially in patients with a Fontan circulation.
206: IS QRS AXIS PATTERN ASSOCIATEDWITH THE TYPE
OF SURGICAL REPAIR IN ADULTS WITH OPERATED
TETRALOGY OF FALLOT?
Zakaria Jalal
1
, Nicolas Combes
2
, Maxime De Guillebon
1
, Jean-Benoit
Thambo
1
, Frederic Sacher
3
1
Paediatric and Congenital Cardiology, CHU, Bordeaux, France
2
Clinique Pasteur, Toulouse, France
3
Electrophysiology, CHU, Bordeaux, France
