CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
149
mutation. It is known that mutations in
NKX2-5
can cause ASD and
AV-block. In mice the AV-block is progressive and sudden death has
been reported.
Conclusion:
Screening for known mutations in 46 CHD families
revealed a mutation in
NKX2-5
in one family. Further investigations
are needed to determine if the combination of ASD and AV-block
should lead to genetic investigations.
223: GLOBAL STRAIN RATE IS AN INDEX OF RIGHT
VENTRICULAR CONTRACTILITY IN HYPOPLASTIC
LEFT HEART SYNDROME (HLHS)
Jana Schlangen, Colin Petko, Gunther Fischer, Miriam Michel, Jan
Hinnerk Hansen, Hans-Heiner Kramer
University Hospital of Schleswig-Holstein, Kiel, Germany
Background:
Systemic right ventricular (RV) function in patients
with HLHS is an important aspect during long term-follow up
after Fontan repair. Echocardiographic evaluation of RV function is
affected by loading conditions. The only load-independent parameter
of ventricular function, the end-systolic elastance (Ees), can only
be generated with invasive catheterisation. Therefore we sought to
determine if parameters obtained by two-dimensional speckle track-
ing (2DST) are affected by acute changes in preload and correlate
with intrinsic RV contractility measured by Ees in HLHS patients
after Fontan palliation.
Methods:
In 34 patients [median age 5.0 (range 2.9–12.7) years]
2DST and conductance catheter studies were perfomed simultane-
ously. A balloon catheter in the intra-atrial lateral tunnel was used
to modify preload. Measurements were repeated with dobutamine
infusion.
Results:
RV Ees correlated with global strain rate (SR) (
r
s
=
–0.4,
p
<
0.01), but not with global strain (S) (
r
s
=
0.01). S and SR did not
change with preload reduction (S: –17.6
±
3.6 vs –17.3
±
4.1%,
p
=
0.6; SR: –0.92
±
0.20 vs –0.95
±
0.26 1/s,
p
=
0.4). S did not change
with dobutamine infusion (–17.6
±
3.6 vs –18.6
±
4.0%,
p
=
0.09)
whereas SR increased significantly (–0.92
±
0.20 vs –1.675
±
0.49
1/s,
p
<
0.001).
Conclusion:
SR was not affected by preload and correlated with
Ees of the systemic right ventricle. It may therefore be a useful non-
invasive parameter of RV contractility suitable for routine follow up
in patients with HLHS after Fontan palliation.
231: CURRENT OUTCOMES OF POSTOPERATIVE EXTRA-
CORPOREAL MEMBRANE OXYGENATION SUPPORT IN
CHILDREN WITH FUNCTIONAL SINGLE VENTRICLE
PATHOLOGIES
Bahaaldin Alsoufi
1
, Abid Awan
1
, Cedric Manlhiot
2
, Zohair Al-halees
1
,
Mamdouh Al-Ahmadi
1
, Brian McCrindle
2
, Avedis Kalloghlian
1
1
Heart Centre, King Faisal Specialist Hospital and Research Centre,
Riyadh, Saudi Arabia
2
Labatt Family Heart Centre, Hospital for Sick Children, Toronto,
ON, Canada
Background:
Improved survival with postoperative extracorporeal
membrane oxygenation (ECMO) support has expanded its applica-
tion to children with complex single-ventricle (SV) pathologies. We
sought to examine current era outcomes of postoperative ECMO and
compare results between children with SV versus biventricle (BV)
pathologies.
Methods:
Demographics, anatomical, surgical and support details
of children who received postoperative ECMO (2007–2012) were
entered into multi-variable regression analysis to determine factors
affecting survival.
Results:
There were 95 patients (3 days to 16 years) who were
divided into SV (
n
=
28) and BV (
n
=
67) groups. ECMO was initi-
ated in the OR for failure to wean off bypass (
n
=
30) or ICU for
haemodynamic compromise (
n
=
65). Thirty-four patients (36%)
received rescue ECMO (ECPR) during chest compression and 13
(14%) required re-operation while on ECMO. Forty patients (42%)
survived
>
24 hours after ECMO discontinuation and 37 (39%) were
discharged alive. Mean ECMO duration was 4.9
±
3.1 days (3.8
and 5.5 days in survivors vs non-survivors,
p
=
0.003). Survival for
ECMO initiated in the OR vs ICU was 43 and 40% (
p
=
0.66), and
38 and 39% for ECPR and non-ECPR patients (
p
=
1.0). Survival of
SV and BV patients was 32 and 42% (
p
=
0.18). In the SV group,
outcomes diverged with best survival after BT shunt (60%) or
Norwood procedure (50%). On the other hand, survival was dismal
following PA band, Glenn, Fontan, TAPVC in heterotaxy patients
(0%). On multi-variable analysis, cardiac re-operation and leaving
cannulation snares were predictors of survival while longer CPR
duration, higher pre-arrest and post-ECMO lactate and longer time
to lactate normalisation were predictors of mortality. In addition,
markers of end-organ injury such as higher creatinine and bilirubin
levels, in addition to pulmonary haemorrhage, dialysis requirement
and ischaemic brain injury were associated with death.
Conclusions:
ECMO plays a valuable role in children requiring
postoperative support, including SV patients. Results in SV vary with
Norwood procedure and BT shunt having a better prognosis. Timely
ECMO prior to emergence of complications and surgical correction
of residual lesions may improve survival.
240: INCIDENCE OF ISOLATED AORTIC DILATION IN
PATIENTS WITH TURNER SYNDROME
Nassiba Alami Laroussi, Anne Fournier, Johanne Thørien, Nagib
Dahdah
Paediatric Cardiology Division, Ste-Justine Hospital, Montreal,
Quebec, Canada
Introduction:
Dilatation of the ascending aorta (AoD) is described
in Turner’s syndrome (TS) with variable prevalence (6.8–32%).
Reported series include patients with associated cardiac anomalies,
e.g. aortic coarctation, left outflow tract obstruction and bicuspid
aortic valve.
Methods:
A retrospective study was carried out with data collected
from medical records and echocardiography studies. Patients with
TS seen at our centre from 1992 to 2010, free of structural cardiac
malformations were eligible when they had
≥
two echocardiogra-
phies. Patients with previous cardiac surgery were excluded. Age
ranged from infancy to adulthood. From 120 patients, 33 (27.5%)
had one or more cardiac anomaly; 18 (15%) with bicuspid aortic
valve, 14 (11.6%) with coarctation of the aorta, seven (5.9%) with
aortic valve stenosis and three (2.7%) with left superior vena cava.
Ascending aorta diameter measurements were collected for all
patients and adjusted for body surface area based on our institutional
regression equation derived from 1 300 healthy children. AoD was
defined as a
z
-score
>
2.
Results:
Of the 87 subjects, 28 (31%) were further excluded due to
missing data or no follow-up echo. Age was (13.6
±
8.6 years) at first
echo and (20.0
±
8.1 years) at last follow up. Follow-up duration was
(6.4
±
3.8 years). At initial echo, 10 (16.9%) patients had AoD. A
total of 18/59 (30.5%) patients had AoD throughout follow up, with
actuarial survival analysis showing freedom from AoD in 90, 77 and
50% at 10, 20 and 30 years old, respectively.
Conclusion:
The prevalence of AoD increases with age in TS even
in the absence of bicuspid or obstructive left-sided lesions. Specific
attention should be brought to healthcare providers attending to TS
patients. The impact of confounding factors such as genetic variants
(mosaicism), growth hormone therapy and vasoactive medication is
yet to be determined.
242: USEFULNESS OF pro-BNP AS A MARKER FOR
MYOPERICARDITIS IN THE EARLY DIAGNOSIS OF
KAWASAKI DISEASE
Jae-Young Park, Min-Seob Song
Department of Paediatrics, College of Medicine, Inje University,
Korea
