CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
151
infections and 58% had postoperative infections; 42% ofdeaths were
due to delay in diagnosis and pre-operative infections and 14% to
pulmonary hypertensive crisis. There was one late death. In a follow
up of three months to six years (
n
=
53), no patient had residual
pulmonary hypertension.
Conclusion:
Delay in diagnosis and surgery and rampant use of
broad-spectrum antibiotics still contribute to the high mortality rate
of this treatable CHD with good long-term prognosis.
250: CHANGES IN PLASMA HYDROGEN SULPHIDE AND
THE SIGNIFICANCE IN THE DIAGNOSIS OF KAWASAKI
DISEASE
Hui Yan
1
, Yue Yuan
2
, Yan Sun
1
, Xueying Li
3
, Chaoshu Tang
4
, Junbao
Du
1
, Ping Leung
5
, Hongfang Jin
1
1
Department of Paediatrics, Peking University First Hospital, PR
China
2
Department of Paediatric Cardiology, Beijing Children’s Hospital,
PR China
3
Department of Statistics, Peking University First Hospital, PR China
4
Institute of Cardiovascular Research, Peking University First
Hospital, PR China
5
Premier Medical Centre, Hong Kong, China
Objective:
Kawasaki disease (KD) is an acute systemic inflammato-
ry disease in childhood. Our aim was to find out a possible biomarker
which might represent the development of KD and the subsequent
coronary artery lesions.
Method:
A prospective unmatched case–control study was designed.
Forty-eight KD patients, 57 non-KD fever patients and 27 non-fever
children were recruited for the research. KD patients were further
divided into subgroups according to coronary artery abnormalities.
Plasma hydrogen sulphide (H
2
S) was examined by a sulphide-sensi-
tive electrode method.
Results:
Plasma H
2
S levels in KD patients during the acute period
were significantly lower than those during the convalescent period
(34.37
±
8.11 vs 38.17
±
8.63,
p
<
0.05). Plasma H
2
S levels in KD
patients during the acute period were significantly lower than those
in non-KD fever patients (34.37
±
8.11 vs 55.77
±
17.88,
p
<
0.05).
There was a negative correlation between the plasma level of H
2
S and
C-reactive protein, and between the plasma level of H
2
S and eryth-
rocyte sedimentation rate in all participants, respectively (
r
=
–0.511
and –0.481, respectively;
p
<
0.05). Receiver operating characteristic
(ROC) curve analysis revealed a diagnosis of KD (ROC area: 0.905
±
0.028,
p
<
0.001, 95% confidence interval: 0.849–0.960, optimal
cut-off value: 44.705 μmol/l) and a prediction of coronary artery
injury (ROC area: 0.834
±
0.045,
p
<
0.001, 95% confidence interval:
0.745–0.922, optimal cut-off value: 43.78 μmol/l).
Conclusions:
Plasma H
2
S level in the acute period may be a poten-
tially useful biomarker for assisting the diagnosis of KD and predict-
ing coronary lesions.
252: CHILDREN SUFFERING FROM POSTURAL ORTHO-
STATIC TACHYCARDIA SYNDROME WITH A MARKED
INCREASE IN ERYTHROCYTIC HYDROGEN SULPHIDE
HAVE A BETTER THERAPEUTIC RESPONSE TO
MIDODRINE HYDROCHLORIDE
Jinyan Yang
1
, Juan Zhao
1
, Die Liu
1
, Chunhin Fu
2
, Xueying Li
3
, Stella
Chen
4
, Fengwen Zhang
1
, Chaoshu Tang
5
, Junbao Du
1
, Hongfang Jin
1
1
Department of Paediatrics, Peking University First Hospital, Beijing,
PR China
2
Department of Biochemistry, Hong Kong University of Science and
Technology, China
3
Department of Statistics, Peking University First Hospital, Beijing,
PR China
4
Department of Biochemistry and Cellular Biology, University of
California, San Diego, La Jolla, CA, USA
5
Department of Physiology and Pathophysiology, Peking University
Health Sciences Centre, Beijing, PR China
Background:
Midodrine hydrochloride is an important therapeutic
option for children with postural orthostatic tachycardia syndrome
(POTS). However, there are few methods to predict response to the
drug. Endogenous hydrogen sulphide plays an important role in the
pathogenesis of POTS. The present study was to explore the predic-
tive value of erythrocytic hydrogen sulphide in predicting the thera-
peutic efficacy of midodrine hydrochloride for children with POTS.
Methods:
Sixty-eight children were included in the study, of whom
28 suffered from POTS (POTS group) and 40 healthy children served
as control group. Children in the POTS group received midodrine
hydrochloride treatment. The erythrocyte hydrogen sulphide produc-
tion was measured by sensitive sulphur electrode and a receiver
operating characteristic (ROC) curve was used to test if erythro-
cyte hydrogen sulphide could predict the therapeutic response to
midodrine hydrochloride for children with POTS.
Results:
Hydrogen sulphide production from erythrocytes was
significantly higher in children with POTS than in control subjects
(
p
<
0.001). Erythrocyte hydrogen sulphide production in respond-
ers to midodrine hydrochloride was significantly higher than that in
non-responders (
p
<
0.05). ROC curve analysis revealed that the area
under curve was 0.857 with a 95% confidence interval (CI) of 0.715–
0.999. Erythrocytic hydrogen sulphide production yielded both high
sensitivity (81.0%) and specificity (85.7%) in predicting the efficacy
of midodrine hydrochloride therapy for POTS in children.
Conclusion:
Erythrocytichydrogen sulphide could serve as a useful
predictor of therapeutic response to midodrine hydrochloride in
POTS of children.
253: SINGLE-VENTRICLE FUNCTION: PREDICTORS OF
CARDIAC INDEX AND RELATION TO CAVOPULMONARY
HAEMODYNAMICS
Christopher M Haggerty
1
, Lucia Mirabella
1
, James Bethel
2
, Kevin K
Whitehead
3
, Mark A Fogel
3
, Ajit P Yoganathan
1
1
Wallace H Coulter Department of Biomedical Engineering, Georgia
Institute of Technology, USA
2
Westat Inc, Rockville, MD, USA
3
Division of Cardiology, Children’s Hospital of Philadelphia,
Philadelphia, PA, USA
Background:
Single-ventricle (SV) lesions are associated with
gradual attrition following surgical palliation with the total cavopul-
monary connection (TCPC). Abnormalities in ventricular function
(VF) are frequently noted. The role of TCPC haemodynamics, which
are potentially unfavourable, is also still unclear. In this study, we
assessed the ventricular dynamic characteristics of 40 SV patients,
and compared those results to cardiac index (CI), systemic venous
flow (Q
s
), and simulated TCPC power loss (TCPC-EDI) to elucidate
biases and functional relationships.
Methods:
Cardiac magnetic resonance (CMR) data were retrospec-
tively analysed for 40 patients. Cine ventricular short-axis scans were
semi-automatically segmented for all cardiac phases. In addition to
traditional VF volume measures, the maximum time rate of volume
change (dV/dt
max
) was calculated and normalised by end-diastolic
volume (EDV) for systole and diastole. TCPC geometry was acquired
from an axial CMR image stack; relevant flow rates were taken from
phase velocity CMR data. TCPC-EDI was calculated from compu-
tational fluid dynamics simulations. Statistical significance was
assumed for
p
<
0.05.
Results:
The natural logs of EDV and stroke volume (SV), and the
systolic and diastolic dV/dt
max
were significantly related to CI. A multi-
variate regression model was constructed using natural log of EDV
(preload), systolic dV/dt
max
and normalised heart rate, and was strongly
predictive of CI (
R
2
=
0.877). Ventricular volumes were all negatively
correlated with TCPC-EDI; EDV had the strongest relationship. Q
s
did
not significantly correlate with any investigated variables.
Conclusions:
EDV (preload), BSA-normalised HR, and systolic dV/
dt
max
were independent predictors of CI in single ventricles. TCPC
power loss was inversely related to ventricular volumes, particularly
preload.
