CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
254
AFRICA
done to 28°C. Total CPB time was 325 min and aortic cross clamp
time 235 min. Total ventilatory support was given for 86 hours and
inotropes for 6 days. Intensive care unit (ICU) stay was until the 8
th
post-operative day (POD).
Conclusion:
Baby was discharged on 16
th
POD with stable haemody-
namics and on normal oral feeds.
1602: DELAYED RECOGNITION OF UNILATERAL PULMO-
NARYVEIN OBSTRUCTIONAFTER REPAIR OF CORTRIA-
TRIATUM
Jae Hyeon YU, Nan Yeol Kim, Yeun Ju Lee, Yu Young Jeong, Hyun
Kong Oh, Min Woong Kang, Myung Hun Na, Seung Pyung Lim
Department of Cardiac Surgery, Chungnam National University
Hospital, CNU, Korea
Background:
Cor triatriatum is a rare condition where the left atrium
(LA) is subdivided by a membrane. Although cor triatriatum can be
an isolated lesion, in many cases, it is associated with other congeni-
tal cardiovascular anomalies: atrial septal defect, a left superior vena
cava (SVC), and pulmonary venous anomalies, etc. We report a case
of unilateral pulmonary vein stenosis diagnosed 7 years after the
repair of cor triatriatum.
Methods:
A 2-month-old boy was diagnosed with cor triatriatum
and severe pulmonary hypertension (PAH). The LA was divided by
an obstructing membrane with 2 mm opening. The membrane was
excised and the postoperative course was uneventful. Seven years
after the operation, we recognised that the vascular markings and
the volume of the left lung were markedly decreased. Therefore, an
examination was done to obtain detailed information.
Results:
On the echocardiogram, the left pulmonary artery (LPA)
was relatively small (7 mm, z
=
-3), but there was no PAH. The lung
perfusion scan showed the markedly decreased perfusion on the left
(95% vs 5%). In the cardiac catheterisation, LPAwas small and left
pulmonary vein is not recognised. Right ventricle (RV) pressure was
25/0/7, MPA 25/15/13, wedge pressure of RPA 10, LPA 15 and mean
LA pressure was 10 mmHg. The 3D CT showed small LPA and no
left pulmonary veins.
Conclusion:
In this case, unilateral pulmonary vein obstruction was
hard to diagnose because pulmonary hypertension and symptoms
did not appear. We assume that the pulmonary vein stenosis may
have been associated with cor triatriatum at the time of surgery, and
progressed slowly during the follow-up period. Thus, particular care
should be taken to identify such anomalies in patients with cor tria-
triatum before and after repair.
1615: END POINT SEPARATION BETWEEN MITRALVALVE
AND SEPTUM OVER THE DIASTOLIC LEFT VENTRI-
CLE DIAMETER (EPSS/LVDD) OF THE LONG-AXIS VIEW
OF ECHOCARDIOGRAPHY IS AN EASY AND RELIA-
BLE PARAMETER FOR ASSESSMENT OF GLOBAL LV
DYSFUNCTION IN PATIENTS OF THALASSAEMIA MAJOR
Jeng-Sheng Chang
1
, Yu-Chin Huang
1
, Kang-Hsi Wu
2
, Ching-Tien
Peng
2
1
Pediatric Cardiology, Children’s Hospital, China Medical University
Hospital, Taiwan
2
Pediatric Oncology, Children’s Hospital, China Medical University
Hospital, Taiwan
Background:
Myocardial dysfunction is a common complication
in patients of thalassaemia major (TM). Although echocardiography
is a convenient instrument, none of its parameters so far has been
considered as an ideal surrogate for prediction of impending heart
failure in TM. However, our previous study of using the long-axis,
M-mode echocardiographic measurements in TM patients with acute
heart failure proved that the distance of end point separation between
mitral valve and septum (EPSS) and the ratio of EPSS divided by
diastolic diameter of the LV (EPSS/LVDd) was significantly corre-
lated with their clinical and laboratory improvements. This study
intended to extend the clinical application of EPSS and EPSS/LVDd
to TM patients of long-term follow up.
Materials and method:
From January 2003 through July 2012, the
thalassaemia registry of China Medical University Hospital, Taiwan,
enrolled 34 TM patients for daily oral iron chelator therapy and
echocardiographic studies, which were performed every 6 months
to 1 year by two double-blinded technicians. The normal ranges of
EPSS (2.5
±
1.7 mm) and EPSS/ LVDd (0.08
±
0.06) were used as
references. Compared to the baseline data at enrolment, the trends
of changes in EPSS, LVDd and EPSS/LVDd were assessed by the
generalized estimation equation (GEE).
Result:
Their average ages were 13.4
±
3.7 years at enrolment and
the average follow-up duration was 7.2
±
2.0 years. In accord with
improvements of EPSS/LVDd, favourable response to iron chelation
occurred in 24 of the 34 patients (13 with direct improvement, 5
stayed as before (normal) and 6 showed delay in improvement after
initial worsening), and unfavourable response occurred in 10 patients
(5 remained as before (abnormal) and 5 even deteriorated). GEE
analysis showed a general trend of rising EPSS/LVDd after 5 years
of follow up (
p
<
0.01).
Conclusions:
EPSS/LVDd is a convenient parameter to monitor the
global cardiac function of TM patients.
1616: OUTCOMES OF IMPLANTABLE CARDIOVERTER-
DEFIBRILLATOR USE IN PAEDIATRIC AND CONGENITAL
HEART DISEASE PATIENTS - THE NORTH WEST EXPERI-
ENCE
Atul Kalantre,Abbas Khushnood,Arjamand Shauq,AdamFitzpatrick,
Derrick Todd, Mark Hall, Edmund Ladusans
Alder Hey Children’s Hospital, Liverpool, UK
Objective:
We reviewed our experience over 20 years of the use,
complications and outcomes of implantable cardioverter defibrilla-
tors (ICD) in a large paediatric population.
Method:
Retrospective analysis was done of 35 patients requir-
ing ICD insertion from 1993 to 2012 at a single tertiary paediatric
cardiac centre. Indications for implant were long QT syndrome (
n
=
16), catecholaminergic polymorphic ventricular tachycardia (
n
=
5), hypertrophic obstructive cardiomyopathy (
n
=
5), idiopathic
ventricular tachycardia (VT) (
n
=
3), ventricular tachycardia (VT)
with underlying congenital heart disease (
n
=
4), Brugada (
n
=
1),
arrhythmogenic right ventricular dysplasia (
n
=
1).
Results:
There were 38 ICD implantations (19 males). Median age at
presentation was 10 years (range 5–20) and median weight of 37 kg
(range 21–70). The presentation was 15 with cardiac arrest, 17 with
syncope, 1 with pre-syncope, 1 with palpitations and 1 with a seizure.
Concomitant antiarrhythmic drug therapy was with beta-blocker
alone in 10, beta-blocker and amiodarone in 2 and amiodarone alone
in 1. A single chamber ICD was used in 18 and dual chamber in
20. Median age at implant was 11 years (range 6–23). At follow-up
(median 4 years, range 0.5–12 years), monitoring revealed 7 episodes
(18%) of appropriate shocks and 9 (24%) of inappropriate shocks as
a result of lead failure (
n
=
4), oversensitivity (
n
=
2) and sinus/atrial
tachycardia (
n
=
3). Twenty-two (58%) patients did not receive any
shocks. There were 9 lead replacements, 3 of which were elective as
a result of box change. There were 2 episodes of infection, one minor
and one needing box change. Two (5%) patients had died, 1 with a
head injury after collapse secondary to VT successfully cardioverted
and 1 with catecholamine storm and repeated cardioversions.
Conclusion:
ICD implantation in the paediatric population can be
life-saving but is associated with a significant incidence of inappropri-
ate shock and requirements for generator and electrode replacement.
1619: AETIOLOGY OF PULMONARY HYPERTENSION IN
AFRICA - PRELIMINARY DATA ANALYSIS AFTER ONE
YEAR OF RECRUITMENT: THE PAN AFRICAN PULMO-
NARY HYPERTENSION COHORT STUDY (PAPUCO)
Friedrich Thienemann
1,2,3
, Lori Blauwet
4
,Anastase Dzudie
5
,Kamilu M
