CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
141
RV free wall. Echocardiographic studies were performed pre-opera-
tively, one day postoperatively and one year postoperatively (11.4
±
8.3 months postoperatively). Additionally, controls age-matched to
patients were included for echocardiographic evaluation.
Results
: In both neonatal (
n
=
20) and non-neonatal (
n
=
19) coarc-
tation patients LV systolic and diastolic performance significantly
improved within the first year following repair. One year postopera-
tively LV systolic performance had normalised, while LV diastolic
performance was still impaired compared to controls in both neonatal
(LV E’ 8.7
±
3.1 vs 13.3
±
3.8 cm/s,
p
=
0.005; LV E/E’ 20.0
±
13.8
vs 9.1
±
3.4,
p
<
0.001) and non-neonatal patients (LV E’ 12.1
±
3.5
vs 15.1
±
2.4 cm/s,
p
=
0.008; LV E/E’ 11.4
±
4.2 vs 7.4
±
1.6,
p
=
0.001). In neonatal coarctation patients, RV systolic and diastolic
performance significantly increased within the first year following
repair. Subsequently, one year postoperatively no differences were
observed in RV systolic or diastolic performance between neonatal
or non-neonatal coarctation patients and controls.
Conclusions
: One year postoperatively LV diastolic performance was
still impaired in both neonatal and non-neonatal coarctation patients,
while RV systolic and diastolic performance were normal. Hence,
current results reveal that a similar pattern of cardiac dysfunction is
present in neonatal compared to non-neonatal coarctation patients
one year after coarctectomy.
112: IMPLANTABLE CARDIOVERTER DEFIBRILLATOR
THERAPY IN PAEDIATRIC PATIENTS IN THE NETHER-
LANDS
Arend Derk Jan Ten Harkel
1
, Annemieke Van der Kooi
1
, Sally-Ann
Clur
2
, Frederik DuPlessis
5
, Freek Van den Heuvel
3
, Christian Blank
4
,
Nico Blom
1
1
Leiden University Medical Centre, Paediatric Cardiology, Leiden,
The Netherlands
2
Academic Medical Centre, Emma Children’s Hospital, Paediatric
Cardiology, The Netherlands
3
University Medical Centre, Beatrice Children’s Hospital, Paediatric
Cardiology, The Netherlands
4
University Medical Centre, Wilhelmina Children’s Hospital,
Paediatric Cardiology, The Netherlands
5
Erasmus Medical Centre, Paediatric Cardiology, Rotterdam,The
Netherlands
Introduction:
In paediatric patients, implantable cardioverter defi-
brillators (ICDs) are increasingly used but unfortunately, the high
incidence of shocks limits its use in this particular population. This
multicentre retrospective study was undertaken to evaluate the clini-
cal outcome of ICD therapy, the incidence of complications, and the
frequency of (in)appropriate shocks.
Methods and Results
: We retrospectively reviewed the medical
records of all children [
n
=
95; female: 38; male: 57; median age 13.0
years (0.23–19)] who underwent ICD implantation between 1990 and
2012 in The Netherlands. Median follow-up period was 3.8 years
(0.1–16). Six patients died during follow up. ICD was implanted as
a primary prevention in 56, and after aborted cardiac arrest in 39
patients. Underlying cardiac disorders were primary electrical heart
disease (
n
=
41), cardiomyopathy (
n
=
37), congenital heart disease
(
n
=
7) and several other diagnoses in 10 patients. Re-interventions
in 56 patients included ICD change because of end-of-life (N
=
28)
and lead-related problems (
n
=
17); 31 (33%) patients received an
appropriate shock after a median time of 6.6 months and 14 (15%)
children experienced an inappropriate shock after a median period
of 5.8 months. Patients with primary electrical heart disease were
significantly more likely to receive an appropriate shock (
p
=
0.036).
With regard to the occurrence of appropriate shocks, no differences
could be found between the primary and secondary prevention group.
Patients younger than six years were more at risk of an appropriate
shock than older children. Methods to prevent further shocks need to
be investigated, as increasing beta-blocker dosage or changing ICD
settings were rather unsuccessful, since 80% of the patients received
further shocks.
Conclusion
: ICD therapy is effective and safe in paediatric patients.
Regardless of the type of treatment change, patients who received a
shock once remain at risk for further shocks. Lead-related problems
are an important reason for re-interventions.
119: VARIANTS OF SCIMITAR SYNDROME
Neville Solomon, Ganapathy Subramaniam, Shapna Varma, CS
Muthukumaran, Prasad Manne, Kothai
Apollo Children’s Hospital, Chennai, India
Background
: Scimitar syndrome is a rare condition with anoma-
lous drainage of the right inferior pulmonary vein into the inferior
vena cava. This can present either in infancy as a sick child or as a
relatively asymptomatic older child. There can be other associated
conditions that determine the timing of presentation.
Case report 1
: A 45-day-old neonate presented with respiratory
distress and echo and X-ray evidence of scimitar syndrome with
large patent ductus arteriosus and hypoplastic right lung. The PDA
was ligated through a left thoracotomy with partial improvement in
the clinical condition. Catheterisation showed multiple collaterals
from the descending aorta to the right lung which was embolised.
The condition again transiently improved but the child continues to
have severe PAH.The child is four months old and is now scheduled
for pneumonectomy.
Case report 2
: A seven-year-old relatively asymptomatic child
presented with symptoms of mild failure to thrive. X-ray and echo
raised a suspicion of scimitar syndrome which was confirmed by CT
angiography. A dilated inferior pulmonary vein was draining into the
inferior vena cava and there were no systemic collaterals. This child
underwent successful re-implantation of the inferior pulmonary vein
into the left atrium without cardiopulmonary bypass through a right
thoracotomy, with an uneventful postoperative course.
Conclusion:
Scimitar syndrome can present as extremes, with one
child having persistent severe pulmonary hypertension and respira-
tory issues, presenting as a neonate, and ending up requiring a pneu-
monectomy, and the other as a relatively asymptomatic child where
off-pump re-implantation of the pulmonary vein to the left atrium
was possible. The degree of associated pulmonary artery and lung
hypoplasia and the presence of systemic collaterals to the lung deter-
mined the timing of presentation.
121: ARRHYTHMIA IN PATIENTS WITH CONGENITAL
CORRECTED TRANSPOSITION OF THE GREAT ARTER-
IES AFTER DOUBLE SWITCH OPERATIONAND CONVEN-
TIONAL RASTELLI
Keiko Toyohara
1
, Morio Shoda
2
Takeshi Hiramatsu
3
, Mitsugi
Nagashima
3
, Daiji Takeuchi
1
, Kei Inai
1
, Tokuko Shinohara
1
, Hirofumi
Tomimatsu
1
, Toshio Nakanishi
1
1
Department of Paediatric Cardiology, Tokyo Women’s Medical
University, Japan
2
Department of Cardiology, Tokyo Women’s Medical University,
Japan
3
Department of Cardiovascular Surgery, Tokyo Women’s Medical
University, Japan
Background:
Patients with congenitally corrected transposition of
the great arteries (ccTGA) often develop arrhythmia.
Methods:
We analysed the incidence of arrhythmia in 64 and 29
patients after they underwent double-switch operation (DSO) and
conventional Rastelli (CR), respectively.
Results:
Fourteen of 64 patients (21%) after DSO and seven of the
29 (24%) after CR showed significant tachycardia. Of these, 12 after
DSO and five after CR had atrial tachycardia (AT). The mean dura-
tion from the repair to the first AT was 7
±
5 and 21
±
7 years after
DSO and CR, respectively (
p
<
0.02). Two cases (3%) after DSO and
two cases (7%) after CR developed ventricular tachycardia, one with
DSO was treated medically and the other was given an implantable
cardioverter defibrillator. Thirteen of the 64 patients (20%) after
