CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
15
In bilateral venous stenosis patients, survival was 0% (0/2) in group
C and 83.3% (6/7) in group S (
p
=
0.035). Among three patients
(one in group C and two in group S) who developed PVS in all four
pulmonary veins, only one in group S survived.
Conclusion
: PVS relief with a sutureless technique was superior to
conventional procedures, particularly in more severe cases such as
bilateral PVS or PVS in all four pulmonary veins. The sutureless
technique for postoperative PVS could be applied aggressively, even
at the initial repair for TAPVC, to decrease the risk of postoperative
PVS.
248: CLINICAL ANALYSIS OF ORTHOSTATIC HYPERTEN-
SION IN CHILDREN
Juan Zhao, Jinyan Yang, Hongfang Jin, Junbao Du
Department of Paediatrics, Peking University First Hospital, China
Objective:
To study the clinical characteristics of orthostatic hyper-
tension (OHT) in children.
Methods:
A total of 96 OHT children who met the diagnostic crite-
ria and clinical manifestations were recruited in the Department of
Paediatrics, Peking University First Hospital. Age and gender distri-
butions were noted. The duration of disease, frequencies of symp-
toms and the predisposing factors were recorded. The haemodynamic
changes from supine to upright positions were also analysed.
Result:
There were 50 boys and 46 girls in our study group. The
mean age was 11.8
±
2.7 years; 32 children were between six and 10
years old, accounting for 33.3% of all subjects, while 64 patients were
from 11 to 17 years old, accounting for 66.7%. Duration of symptoms
of OHT were less than one month in 22.9% children, from one month
to one year in 51.1% of children and longer than one year in 26.0%
children. The most common clinical manifestations were syncope
and dizziness. The incidence of this were 70.8 and 46.9%, respec-
tively. Other clinical manifestations included transitional amaurosis,
nausea and/or vomiting, and pallor. These often occurred with posi-
tional changes (24.0%) and standing for long periods (57.3%). Other
predisposing factors included exercise, emotional changes and envi-
ronment. The baseline systolic and diastolic blood pressure was 103
±
8 mmHg (1 mm Hg
=
0.133 kPa) and 59
±
6 mmHg, respectively.
The standing systolic and diastolic blood pressure at 3 min was 113
±
8 mmHg and 73
±
6 mmHg and the differences were significant (
t
=
27.674,
p
<
0.01;
t
=
17.936,
p
<
0.01). The baseline heart rate in the
supine position was 81
±
11 beats/min and the maximum heart rate
in the upright position was 113
±
12 beats/min (
t
=
33.092,
p
<
0.01).
Conclusion:
OHT is commonly seen in puberty in children. The
main symptoms are syncope and dizziness. They were mostly
induced by positional changes and long periods of standing.
260: LATE SURGICAL CORRECTION OF ANOMALOUS
LEFT CORONARY ARTERY FROM PULMONARY TRUNK
IN CHILDREN, USING AUTOGENOUS AORTIC AND
PULMONARY ENDOTHELIALiSED TUBE
World
Journal
of Cardiovascular Disease
2012;
2
: 136–140
Miguel A. Maluf
Sao Paulo Federal University, Brazil
Background:
Direct re-implantation of an anomalous left coronary
artery into the aorta is the preferred surgical option for creating a
dual coronary arterial system in patients in whom the anomalous
artery originated from the pulmonary trunk.
Methods:
Three patients presented with the origin of the left coro-
nary artery from the left posterior pulmonary sinus, associated with
moderated mitral valve insufficiency caused by left ventricular
dysfunction. For surgical correction we used a trapdoor-like coronary
artery elongation with autogenous aortic and pulmonary endothelial-
ised tube to connect the left coronary ostium to the ascending aorta,
avoiding the mitral valve intervention.
Results:
There were no early or late deaths. All patients were in
functional class I, with good biventricular function and a competent
mitral valve at a median follow up of 94 months, ranging from 108
to 132 months. Postoperative computerised tomography of the aorta
in our first patient showed good arterial flow without any distortion.
A local and distal stenosis of the left pulmonary artery was observed
and submitted for stent treatment.
Conclusions:
The potential benefits of the trapdoor-like technique
and its modifications allow for excellent operative exposure. The
use of an autogenous endothelialised tube is a viable tissue capable
of further growth, and this avoids injury to the aortic and pulmonary
valvular apparatus or production of an obstruction within the right
ventricular outflow tract.
264: CARDIAC EFFECT OF LOW- AND HIGH-DOSE
IDEBENONE THERAPY IN FRIEDREICH’S ATAXIA
PATIENTS
Anne Fournier, Daniel Velasco-Sanchez, Johanne Therrien, Nagib
Dahdah
Division of Paediatric Cardiology, Ste-Justine Hospital, Montreal,
Quebec, Canada
Introduction:
Cardiac involvement in Friedreich’s ataxia (FRDA) is
present in 63% of patients, mostly represented by hypertrophic cardi-
omyopathy. The use of antioxidants, such as idebenone, has shown
promising results in improving cardiac hypertrophy parameters at
low to intermediate doses. Higher doses of idebenone are suggested
but the related long-term cardiac effects have not been studied, which
was the objective of this study.
Methods:
This was a prospective, non-controlled, comparative,
open-label trial of a 12-month regimen of low versus high doses of
idebenone between two cohorts with FRDA. Cardiac evaluation was
performed before initiation of therapy, and at six, nine and 12 months
of therapy.
Results:
Significant left ventricular mass reduction from baseline
was observed in both groups after six, nine and 12 months (
p
≤
0.05). Systolic function parameters were within the normal range
in all patients, with no significant differences between baseline and
last follow up in either group. Diastolic function was marked by an
improved mitral deceleration time in the high-idebenone group at
the last follow up (
p
=
0.029), but not in the low-idebenone group
(
p
=
0.13).
Conclusion:
There were comparable effects of high- and low-
idebenone therapy in terms of reduction of left ventricular hyper-
trophy parameters. Both therapeutic regimens seemed to preserve
systolic cardiac function, with an advantage of the high-idebenone
dose improving diastolic filling.
269: OUTCOMES OF EXTRACORPOREAL LIFE SUPPORT
FOLLOWING CARDIAC SURGERY IN CHILDREN WHO
FAIL TOWEAN FROM CARDIOPULMONARY BYPASS
Peta MA Alexander
1
, Derek Best
1
, Clarke Thuys
2
, Johnny Millar
1
,
Yves d’Udekem
2,3
, Warwick Butt
1
1
Paediatric Intensive Care Unit, The Royal Children’s Hospital,
Melbourne, Australia
2
Department of Cardiac Surgery, The Royal Children’s Hospital,
Melbourne, Australia
3
Murdoch Children’s Research Institute, Melbourne, Australia
Background:
Extracorporeal life support (ECLS) following paedi-
atric cardiac surgery varies according to centre availability and
philosophy. We assessed local outcome of patients receiving ECLS
for failure to wean from cardiopulmonary bypass (CPB), with the
aim of identifying factors associated with outcome.
Methods:
Institutional databases from our tertiary referral centre
identified children who received postoperative ECLS. Retrospective
medical record review preceded standard statistical analyses, includ-
ing factors associated with survival.
Results:
Between 1 January 2002 and 1 January 2011, 65 children
(median age 1.5 months) received ECLS for failure to wean from
