CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
224
AFRICA
Results:
BNP was significantly higher (
p
<
0.01) at pre-SCPC (
n
=
173) compared to 14 months (
n
=
134); median (IQR) 80.8 pg/ml
(35–187) vs 34.5 pg/ml (17–67). BNP
>
100 pg/ml was present in
72 (42%) of subjects pre-SCPC and 21 (16%) at 14 months. Patients
with BNP at both visits (
n
=
117) had a median (IQR) decrease of 32
(1–79,
p
<
0.01). In longitudinal analysis, higher levels of BNP were
associated with increased echocardiographic end-systolic volume
z
-score (
p
=
0.01), greater degree of AV valve regurgitation (
p
<
0.01), lower weight
z
-score (
p
<
0.01), and lower length
z
-score (
p
=
0.02). In multivariable analyses at 14 months, higher BNP level
was associated with presence of arrhythmia post-SCPC surgery (
p
<
0.01), prior Norwood procedure (
p
<
0.01), and longer length of
hospital stay post-SCPC surgery (
p
=
0.04), as well as lower Bayley
Psychomotor Developmental Index (
p
=
0.015).
Conclusion:
BNP decreased in infants with SV after SCPC surgery.
Elevations in BNP were associated with increased ventricular dila-
tion, increased atrio-ventricular valve regurgitation, and poorer
growth and neurodevelopmental outcomes. Therefore, BNP may be
a useful seromarker in identifying infants with SV at risk for worse
outcomes.
1240: MAGNETIC RESONANCE ASSESSMENT OF
MYOCARDIAL SCARRING AND VENTRICULAR FUNC-
TION BEFORE AND AFTER REPAIR OF ANOMALOUS
LEFT CORONARY ARTERY FROM THE PULMONARY
ARTERY
Heiner Latus
1
, Kerstin Gummel
1
, Stefan Rupp
1
, Klaus Valeske
2
,
Hakan Akintuerk
2
, Christian Jux
1
, Juergen Bauer
1
, Dietmar Schranz
1
,
Christian Apitz
1
1
Paediatric Heart Centre, Children’s Hospital Giessen, Germany
2
Division of Cardiovascular Surgery, University Clinic Giessen,
Germany
Background:
Anomalous left coronary artery from the pulmonary
artery (ALCAPA) is a rare cardiovascular anomaly. Left ventricu-
lar (LV) dilatation and dysfunction (DCM) evolves due to dimin-
ished myocardial perfusion caused by coronary steal phenomenon.
Myocardial scarring has been shown in ALCAPA patients late after
repair, however, the incidence of myocardial scarring before surgery
and its impact on the postoperative course is unknown. Accordingly,
the aim of the study was to assess myocardial function and viability
using CMR imaging before and in the short-term follow up after
ALCAPA repair.
Methods:
Eight patients (mean age 10.0
±
5.8 months) with diag-
nosed ALCAPA underwent CMR before and after (mean time inter-
val 4.9
±
2.5 months) coronary re-implantation procedures (six direct
re-implantation, two Takeuchi method). CMR included functional
analysis and late gadolinium enhancement (LGE) for detection of
myocardial scars.
Results:
Severe LV dilatation (mean indexed LVEDV 171
±
94 ml/
m
2
) and dysfunction (mean LV-EF 22
±
10%) were present in all
patients and improved significantly after surgery in seven of eight
patients (mean LVEDV 68
±
42 ml/m
2
, p
=
0.02; mean LV-EF 58
±
19%,
p
<
0.01). Myocardial scarring (one apical subendocardial, one
midventricular transmural) was present in two of the eight patients
(25%) and did not predict postoperative course or functional recov-
ery. Early follow-up CMR showed an unchanged degree of infarcted
myocardium in these patients. Although functional recovery was
sufficient, transmural scarring not evident before re-implantation was
found at follow-up CMR in two patients.
Conclusions:
Despite often severely compromised LV function pre-
operatively, myocardial scarring was only present in the minority
of our patients. According to our experience, myocardial dimen-
sion and function recovered quickly after coronary re-implantation,
independently of myocardial scarring. Further studies are needed to
elucidate the endogenous repair mechanisms that are responsible for
the re-remodelling of the LV in patients with ALCAPA.
1244: INTER-PARAMETRIC CORRELATION BETWEEN
ECHOCARDIOGRAPHIC MARKERS IN PRETERM
INFANTS WITH PATENT DUCTUS ARTERIOSUS
Arvind Sehgal
1,2
, Samuel Menahem
3
1
Monash Newborn, Monash Medical Centre, VIC, Australia
2
Department of Paediatrics, Monash University, Melbourne, Australia
3
MonashHeart, Melbourne, Australia
Background:
Various echocardiographic parameters are studied in
the assessment and evaluation of patent ductus arteriosus. In isola-
tion, many of these parameters have low sensitivity and specificity
for ductal significance when compared to ductal size.
Aim:
To correlate various echocardiographic parameters with ductal
size (transductal diameter) in infants with symptomatic patent ductus
arteriosus, and ascertain the sensitivity, specificity, positive and nega-
tive predictive values and likelihood ratios of various parameters to
predict a duct
≥
3mm in size.
Methods:
Preterm infants less than 32 weeks’ gestation who were
evaluated for the presence of a patent ductus arteriosus were assessed.
The following echocardiographic parameters were measured: trans-
ductal diameter (TDD), ductal velocity, end-diastolic left pulmonary
artery (LPA) flow, ductal diameter-to-left pulmonary artery ratio
(TDD:LPA), left atrial-to-aortic root ratio (LA:Ao), left ventricular
output-to-superior vena cava flow ratio (LVO:SVC), transmitral E/A
ratio and isovolumic relaxation time (IVRT).
Results:
Fifty-two infants were enrolled with a mean gestational
age of 26
±
2 weeks and mean birth weight of 837
±
240 g. The
mean transductal diameter was 2.8
±
0.5 mm. Transductal diameter
correlated significantly with ductal velocity, end-diastolic LPA flow,
TDD:LPA, LA:Ao and LVO:SVC ratio. No significant correlation
with transmitral indices was noted. Among the parameters, LVO:SVC
ratio had the highest specificity (0.83) and sensitivity (0.95) to detect
a duct of
≥
3 mm. The area under the curve was 0.95 (95% CI: 0.85–
0.99), indicating a 95% probability that a randomly selected patient
with LVO:SVC ratio
≥
4 will have a ductal size
≥
3 mm.
Conclusions:
Significant correlations between ductal size and surro-
gate markers of pulmonary over circulation were noted. A combina-
tion of echocardiographic markers may help assess the magnitude of
the haemodynamic impact of a patent ductus arteriosus.
1245: MARFAN SYNDROME IN AN EIGHT-YEAR-OLD BOY
Renny Suwarniaty, Haryudi Aji Cahyono, Nur Ramadhan
Department of Child Health Medical School, University of Brawijaya,
Saiful Anwar, Malang, Indonesia
Background:
Marfan syndrome is a systemic disorder of the connec-
tive tissue associated with mutations. Early diagnosis and advances
in medical technology have improved the quality of life for people
with this syndrome. It can be determined by multisystem disorder
with manifestations typically involving the cardiovascular, skeletal
and ocular systems. Rupture of the aorta was a serious complication
of Marfan syndrome.
Objective:
An eight-year-old boy weighing 27 kg, height 138 cm
came to the hospital on 20 March 2009 for endocrinology counselling.
The boy looked healthy. He was taller than expected for his genetic
background. A craniofacial physician found a small chin and malar
flattening. There was no chest deformity and in the extremities, a wrist
and thumb sign. An ophtalmologist diagnosed simple ectopia lentis.
Echocardiography revealed elongation of the aorta. He is the second
child, and his father went blind about three years ago. This patient
fulfills the Ghent criteria (three out of five criteria).He was prescribed
a beta-blocker to prevent progression of the elongation of the aorta,
followed by serial echocardiography regularly every six months.
Conclusions:
This diagnosis of Marfan syndrome was done using
clinical manifestations (Ghent criteria) without genetic testing.
Medication with a beta-blocker and serial echocardiography was
necessary to detect elongation of the aorta, which may cause sponta-
neous rupture of the aorta.
