CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
245
degree relatives, the prevalence of TAAD was increased to 0.7%
(9/1339) compared to 0.016% in the general population (
p
<
0.001).
The recurrence rate of CHD was higher (2.7%) in first-degree rela-
tives than in second-degree relatives (0.79%).
Conclusion:
These data provide evidence for a genetic associa-
tion between PDA and aortic disease. The prevalence of PDA and
other CHD is increased in first-degree relatives of the index patient,
suggestive of common genetic factors in the determination of CHD
and aortic disease.
1488: THREE-DIMENSIONAL EN FACE ECHOCARDIO-
GRAPHICRECONSTRUCTIONOF SIMPLEANDCOMPLEX
VENTRICULAR SEPTAL DEFECTS ON RIGHT VENTRICU-
LAR SEPTALSURFACE USINGA SIMPLE BEDSIDE PROTO-
COL
Sivakumar Kothandam, Anil kumar Singhi, Sreeja Pavitran
Paediatric Cardiology, MIOT Hospitals, Chennai, India
Background:
Increasing clinical use of three-dimensional echocar-
diogram (3DE) has enabled volume rendering of ventricular septal
defects (VSD) on the right ventricular septal surface to guide under-
standing of the cardiac morphology. This will help decision making
on surgical or interventional closure. A simple 3DE protocol to show
all VSDs from right ventricular surface in a uniform format will aid
understanding among all team members.
Methods
: We studied 40 patients with simple (single or multiple
VSD) and complex VSD (tetralogy of Fallot, atrio-ventricular canal,
double-outlet right ventricle, transposition of the great arteries and
truncus arteriosus) using 3DE enface reconstruction. We adopted a
uniform subxiphoid live 3DE acquisition of a sector width that covers
the entire interventricular septum. This acquisition gives good repro-
ducible images in most patients under 10–12 years using x7-2 matrix
probe (Philipsmedical, Andover, MA). The probe is positioned in the
coronal plane in subxiphoid window to display the entire ventricu-
lar septum from right ventricular inflow to outflow. Using volume
rendering, an enface reconstruction of the right ventricular septal
surface is presented to give anatomical location of the ventricular
septal defect.
Results:
Out of 40 patients, good 3DE acquisition was achieved in
all except one with optimal images. Twenty-two patients had differ-
ent types of simple VSD (single in 17 and multiple in five), four
patients had atrio-ventricular canal, two had tetralogy of Fallot, six
had double-outlet right ventricle, three had truncus arteriosus and two
had transposition of the great arteries. The 3DE reconstruction was
accurate in showing the VSD location to the interventionist (two peri-
membranous VSD and two muscular VSD) and surgeons.
Conclusion
: A simple right ventricular septal surface enface recon-
struction of the VSD by a bedside live 3DE greatly enhances under-
standing of the location of the VSD and aids in planning closure.
1490: MYOCARDIAL FUNCTION FOLLOWING REPAIR
OF ANOMALOUS ORIGIN OF LEFT CORONARY ARTERY
FROM THE PULMONARY ARTERY (ALCAPA) IN CHIL-
DREN
Nitha Naqvi
1,2
, Nagarajan Muthialu
1
, Sonya Babu-Narayan
2
, Shreesha
Maiya
2
, Prathiba Chandershekar
1
, Victor Tsang
1
, Jan Marek
1
1
Great Ormond Street Hospital, London, UK
2
Royal Brompton Hospital, London, UK
Background
: We hypothesised that speckle tracking
may improve
detection of myocardial dysfunction and provide new pathophysi-
ological insights in anomalous coronary artery from the pulmonary
artery (ALCAPA).
Methods:
Echocardiography including speckle tracking was
performed in 22 children with ALCAPA (eight males, median age
at surgery 0.4 years; IQR: 0.21–1.05) pre- and postoperatively and
in 22 healthy controls. Measurements included global and segmental
longitudinal, radial and circumferential peak deformation (strain) and
synchronicity index (SI) defined as agreement of time to peak strain
measurements between segments per subject, summarised using
intraclass correlation coefficient.
Results:
Global strains were lower in unoperated patients than
controls (longitudinal: –46 vs –123;
p
<
0.001; circumferential: –48
vs –118;
p
<
0.001, radial: 110 vs 357;
p
<
0.001) and improved
postoperatively (longitudinal: –46 pre vs –82 post;
p
=
0.002, circum-
ferential: –48 vs –96;
p
=
0.012, radial: 110 vs 317;
p
=
0.001).
Unoperated patients with normal 2D function (
n
=
8) had signifi-
cantly impaired strain. Global dyssynchrony significantly improved
postoperatively (longitudinal SI 0.93 pre vs 0.94 post, circumferen-
tial 0.85 pre vs 0.9 post, radial 0.71 pre vs 0.88 post). Global time
to peak shortened (longitudinal 2 236 pre vs 1 589 post;
p
<
0.001,
circumferential 2 037 vs 1 447;
p
=
0.005, radial 2 169 vs 1 602;
p
=
0.01 ms). Despite overall global improvement some abnormalities
remained. Strain improved in the majority of segments but apical
septal and anterolateral segments remained abnormal. Post-systolic
index improved in some segments but pre-systolic stretch persisted.
Conclusions:
Both global contractility (strain) and global synchro-
ny (coordinated contraction) improved after repair of ALCAPA,
suggesting recovery of hibernating myocardium. Contractility in
some segments supplied by the anomalous left coronary artery failed
to improve following ALCAPA repair, suggesting a degree of irre-
versible myocardial damage. 2D speckle tracking identified impair-
ment of
function not revealed by standard echocardiography.
1494: AN UNUSUAL CASE OF COARCTATION THE AORTA
AND PATENT DUCTUS ARTERIOSUS
Daria Kulikova
1
, Igor Polivenok
1
, Olga Buchneva
1
, Yulia Psiklova
1
,
Yaroslav Shafer
1
, Frank Molloy
2
, Robert Arnold
3
, Christian Gilbert
2
,
Valery Bojko
1
, William Novick
2,4
1
AMS of Ukraine, Kharkov, Ukraine
2
International Children’s Heart Foundation, Memphis, TN, USA
3
Royal Liverpool Children’s Hospital, Liverpool, UK
4
University of Tennessee, Health Sciences Centre
Background:
We describe a highly unusual variant of juxta-ductal
coarctation of the aorta (CoAo) and the lessons learned by our team.
Case report
: The patient, M, was a five-month-old female, who
was hospitalised with bilateral pneumonia and referred for cardiac
evaluation. Echo showed marked biventicular hypertrophy, CoAo
gradient 45 mmHg, patent ductus arteriosus (PDA) 3 mm, pulmonary
hypertension (PH) 75 mmHg. ECG suggested biventricular hypertro-
phy. Cardiac catheterisation: Asc Ao
=
142/60 mmHg, RV
=
110/10
mmHg, PA
=
110/50 mmHg. Thoracotomy revealed CoAo and PDA.
During PDA ligation with simultaneous aortic clamping there was
severe bradycardia and collapse, without signs of ischaemia on ECG.
Inotropes were ineffective. The patient recovered with direct cardiac
massage, declamping of the aorta and removal of PDA ligature. Our
interpretation was vagal stimulation. After stabilisation, PDA ligation
was repeated with resection of coarctation with ‘end-to-end’ anasto-
mosis. Further temporary haemodynamic instability occurred during
chest closure but she was stabilized. However, three hours after
surgery, the child suddenly developed refractory cardiac arrest. Post
mortem study: PDA ligated, anastomosis of the aorta ‘end-to-end’. A
single coronary artery was found on the front of the pulmonary trunk
in 0.7 cm from the valve, which divided into anterior and posterior
descending branches and circumflex branch. No coronaries were
present from the aorta. Right ventricular wall: 8 mm, left: 11 mm.
Discussion:
We suppose that ischaemic symptoms of anomalous
origin of the coronary supply were not seen in this case due to CoAo
with PDA resulting in high PA pressure maintaining high coronary
perfusion pressure. After correction of CoAo and PDA, PA pressure
fell and led to an acute fall in coronary perfusion. Following post
mortem we reviewed the pre-operative cardiac catheterisation and
aortogram, which clearly showed the absence of coronary arteries
from the aorta, which were not seen by our team focusing on the
aortic arch and ductus.
