CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
219
I Ayhan
1
, Alison M Hayes
3
, Amos Wong
1
, Dirk G Wilson
1
, Victor D
Ofoe
1
, John L Gibbs
2
1
Department of Paediatric Cardiology, University Hospital of Wales,
Wales, UK
2
Department of Paediatric Cardiology, Leeds General Infirmary,
Leeds, UK
3
Department of Paediatric Cardiology, Bristol Royal Children’s
Hospital, Bristol, UK
Background:
Children presenting with isolated mitral regurgitation
(MR) or dilated cardiomyopathy (DCM) may have anomalous origin
of the left coronary artery from the pulmonary artery (ALCAPA).
Methods:
A 20-year review of ALCAPA from three centres was
undertaken.
Results:
There were 41 patients (27 females and 14 males). Age ranged
from one month to 17 years (median 5 months). It took from one week
to eight years to diagnose ALCAPA in 11 patients. The common-
est reasons for referral were cardiac failure in 23 (56%), respiratory
distress or asthma in 21 (51%), failure to thrive in 15 (37%), and
incidental murmur in 11 (27%) patients. The initial echocardiographic
diagnosis was DCM in 29 (70%), congenital MR in 10 (24%) and
PDA in two (5%). MR was considered as the primary pathology in
eight (18%), hence its association with ALCAPA was overlooked for
as long as five years; 18 patients (43%) exhibited dilated right CA, and
in seven (17%) the origin of the left CA could not be demonstrated. In
15 (37%) patients abnormal retrograde flow in the pulmonary artery
was noted. The commonest ECG findings were non-specific T-wave or
ST-segment changes in 24 (59%) and 18 (43%) patients, respectively;
22 (54%) patients had pathological Q waves in lead aVL. Ninteen
(46%) patients required cardiac catheterisation to confirm ALCAPA.
In two patients, ALCAPA was suspected after PDA ligation or during
mitral valve replacement; 39 (95%) patients had surgery immediately
after diagnosis, but two were not operated on. Post-surgical echocar-
diograms showed resolution of MR and left ventricular dysfunction in
29 (70%). Three (16%) patients died after surgery.
Conclusion:
Isolated MR and left ventricular dilatation are important
and frequent findings in ALCAPA syndrome. Any child with echo-
cardiographic diagnosis of congenital MR or DCM should prompt a
careful search for ALCAPA.
1179: FOETAL AND POSTNATAL OUTCOME OF 22Q11
DELETION AND ASSOCIATED CONGENITAL HEART
DEFECTS
Orhan Uzun
1
, Kadir Babaoglu
1
, Yusuf I Ayhan
1
, David Tucker
2
, Amos
Wong
1
, Dirk G Wilson
1
, Victor D Ofoe
1
1
Department of Paediatric Cardiology, University Hospital of Wales,
Wales, UK
2
Congenital Anomaly Register and Information Service for Wales
(CARIS), 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.
Methods:
A review was done of patients with 22q11.2 deletion who
either presented to the University Hospital of Wales with congenital
heart disease or reported to the Congenital Anomaly Register and
Information Service for Wales (CARIS) between 1990 and 2011.
Results:
During the study period, 102 cases of 22q11.2 deletion were
reported, giving a gross 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 congenital heart defect (CHD) (93%). Seven cases had a normal
heart. In 18 cases, diagnosis was established in foetal life: pregnancy
was terminated in four cases, still birth was found in one and in 13
cases pregnancy resulted in a live birth (72%). Eighty-four patients
were diagnosed at the postnatal period. Mean follow-up duration
was 16.2
±
11.4 years (range 1–52 years, median 14.5 years). Eight
patients died after birth, seven in the first year of life, and one at age
3.8 years.
Conclusions:
Of patients with 22q11.2 deletion, 93% exhibited
CHD. In spite of the antenatal detection rate being low, survival was
similar for all cases irrespective of the presence of CHD. Most deaths
occurred within the first year of life but beyond infancy survival was
favourable
1191:A NEW METHOD FOR MANUFACTURING FLEXIBLE
REPLICAS OF CONGENITAL HEART DISEASE USING
STEREOLITHOGRAPHY AND VACUUM-CASTING TECH-
NIQUE: REHEARSAL OF SURGICAL OPERATION
Isao Shiraishi
1
, Ken-ichi Kurosaki
1
, Suzu Kanzaki
2
, Masatoshi
Takeda
3
, Hajime Ichikawa
4
1
Department of Paediatric Cardiology, National Cerebral and
Cardiovascular Centre,
2
Department of Radiology, National Cerebral and Cardiovascular
Centre, Japan
3
CrossEffect, Inc, Kyoto, Japan
4
Department of Paediatric Cardiac Surgery, National Cerebral and
Cardiovascular Centre, Japan
Background:
Stereolithography is a rapid prototype technology
whereby an ultraviolet laser beam selectively polymerises and
solidifies photosensitive and polymeric liquid plastic. By using this
technique, 3D volumetric image data of multi-slice (MS) CT can be
converted into plastic models that enhance our spatial perception
of real-life anatomy and pathology. However, the materials of the
stereolithography are limited to several photosensitive plastics or
urethane, which are not satisfactory for simulation surgery. Recently,
a vacuum-casting method has been developed, where more delicate
models with different stiff materials can be manufactured.
Methods:
Three-dimensional volumetric data sets of MSCT angiog-
raphy of congenital heart disease (CHD) were converted into stand-
ard triangulated language files to make stereolithographic biomodels
representing both the outer and inner surface of the heart. Then,
urethane with appropriate stiffness was injected using the vacuum-
casing method. After solidification of the urethane, the casts were
carefully removed.
Results:
We have made replicas of CHD including VSD, ASD, TOF,
DORV, SV, criss-cross heart, and ccTGA. The vacuum casting in
association with stereolithography enabled us to manufacture repli-
cas with similar texture and structure to the real heart. This technique
also allowed the surgeon to cut and suture, facilitating the simulation
of the surgical operation.
Conclusion:
The vacuum casting in association with stereolithog-
raphy is a promising technique for the pre-operative practice and
simulation of individual surgery, and planning of novel and innova-
tive surgical procedures of CHD. This technique could be helpful for
rescuing children with CHD.
1192: DAMUS-KAYE-STANSEL ANASTOMOSIS MITIGATES
THE RISK OF SYSTEMIC OUTFLOW TRACT OBSTRUC-
TION IN CHILDRENWITH SINGLEVENTRICLE CARDIAC
ANOMALIES
Bahaaldin Alsoufi, Mubashir Khan, Mamdouh Al-Ahmadi, Avedis
Kalloghlian, Abdullah Al-Wadai, Zohair Al-Halees
Heart Centre, King Faisal Specialist Hospital and Research Centre,
Riyadh, Saudi Arabia
Background:
Children with various single-ventricle (SV) patholo-
gies are at risk of developing systemic ventricular outflow tract
obstruction (SVOTO) following volume unloading with cavo-pulmo-
nary connection (CPC). We aimed to evaluate the efficacy of Damus-
Kaye-Stansel anastomosis (DKS) at time of CPC in decreasing late
SVOTO risk.
Methods:
A retrospective review of SV patients who under-
went DKS concurrent with CPC between 1997 and 2012 was
performed. Clinical, echocardiographic and angiographic outcomes
were analysed.
