CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
251
1565: INVESTIGATION OF IDIOPATHIC DILATED PULMO-
NARY ARTERY: THE ROLE OF DIFFERENT NON-INVA-
SIVE IMAGING MODALITIES: A CASE REPORT
Shuk Han Lee, Louisa Poon
Department of Paediatrics, Queen Elizabeth Hospital, Hong Kong
Case description
:A 14-year-old Chinese girl with Noonan syndrome,
mild mental retardation and left renal agenesis was followed up for
idiopathic pulmonary artery dilatation. At 7 years old, her echocar-
diogram and right heart catheterisation showed idiopathic pulmonary
artery dilatation.
Trans-thoracic echocardiogram was repeated at 14 years of age. It
showed dilated main pulmonary artery, measuring 3.38 cm in diam-
eter and dilated left coronary artery. The left main coronary artery
measured 6.2 mm and the left anterior descending artery measured
5.2 mm in diameter. There were multiple low velocity turbulent
jets in the mid right ventricular free wall. There was no evidence of
pulmonary hypertension from Doppler pressure gradient assessment
of pulmonary regurgitation.
Left and right cardiac catheterisation was performed with a suspi-
cion of coronary arteriovenous fistula to the right ventricle. Left
coronary angiogram did not show any fistula but retrograde contrast
flow to the right coronary artery. Attempt to cannulate the right
coronary artery was unsuccessful. Detailed review of the angiogram
showed suspicious anomalous origin of right coronary artery from
the main pulmonary artery but we could not confirm the diagnosis
by manipulating a catheter in it.
A computed tomography (CT) coronary angiogram with 3D
reconstruction was later performed. It showed beautifully the anoma-
lous origin of the right coronary artery from the main pulmonary
artery. Magnetic resonance imaging (MRI) perfusion scan with
adenosine stress test showed no evidence of myocardial ischaemia.
The patient was treated conservatively.
Conclusion:
One should be careful with the diagnosis of idiopathic
pulmonary artery dilatation. In this case, the combined role of non-
invasive imaging including echocardiogram, CT coronary angiogram
and MRI perfusion scan can replace traditional invasive cardiac cath-
eterisation to reveal the true underlying cause.
1571: COURSE OF THE DFT IN THE MIDTERM FOLLOW-
UP AFTER ICD-IMPLANTATION USING THE EXTRACAR-
DIAC TECHNIQUE
Thomas Kriebel
1
, Wolfgang Ruschewski
2
, Heike Schneider
1
, Matthias
Müller
1
, Matthias Pietzka
1
, Ulrich Krause
1
, Thomas Paul
1
1
Department of Pediatric Cardiology and Intensive Care Medicine,
Georg-August-University of Göttingen, Göttingen, Germany
2
Department of Thoracic and Cardiovascular Surgery, Georg-August-
University of Göttingen, Göttingen, Germany
Introduction:
Up to now, there is limited experience in
implantable
cardioverter defibrillator
(ICD) implantation in infants and small
children. The previously described extracardiac technique offers
an effective and safe ICD implantation technique in these patients.
However, data about midterm follow-up, especially the course of the
defibrillation thresholds (DFT), during the growth of the patients are
lacking.
Patients and methods:
An extracardiac ICD-system was implanted
in 19 patients (mean age: 5.6 [range 0.2–9.8] years) as previously
described. In brief: Under fluoroscopic guidance a defibrillator
lead was tunnelled subpleurally along the course of the 2-6
th
rib
until almost reaching the vertebral column. After a partial inferior
sternotomy, bipolar steroid-eluting sensing and pacing leads were
sutured to the atrial wall in selected patients and to the anterior wall
of the right ventricle in all patients. The ICD device was implanted
as ‘active can’ in the upper abdomen. Sensing, pacing, and DFT as
well as impedances were verified intraoperatively, 3 months later and
then every 12 months.
Results:
In all patients, intraoperative DFT between the extracardiac
lead and device was
<
15 J (mean 11.2 J). No serious complications
were noted. After a mean follow-up of 1.8 years DFT remained stable
or below
<
20 J in 15/19 patients. Revision was required in 3 patients
because of unacceptable DFT
>
20 J. Using multiple regression
analysis a significant correlation between the size of the patient and
mean DFT was noted. In 3 patients inadaequate ventricular sensing
was noted. Revision of the system could be avoided in 2/3 patients
by an alternative sensing option between the ventricular leads and
subpleural shock electrode.
Conclusions:
The extracardiac technique offers a safe and effec-
tive approach for ICD implantation in infants and small children.
However, regular DFT testing is mandatory to recognise failure of
the system.
1573: RISK FACTORSAND NATURAL HISTORY OF ISOLAT-
ED NON-COMPACTION MYOCARDIOPATHY IN CHILD-
HOOD
Alejandra Villa, Carlos Rosental, Marisa Di Santo, Natalia Napoli,
Alejandra Mori
Garrahan Children’s Hospital, Buenos Aires, Argentina
Background:
Left ventricular non-compaction (LVNC) is an uncom-
monly reported genetic disorder of endocardial morphogenesis.
Clinical and prognostic heterogeneity is described. The purpose of
this study was to identify the clinical characteristics and risk factors
of children with LVNC.
Methods and result:
We retrospectively reviewed 24 children with
LVNC evaluated at the Garrahan Children’s Hospital from January
1996 to December 2011. The median age at presentation was 2 years
(range 15 days to 13 years). Median follow-up was 4 years (2–15
years). Seventeen patients (70%) had electrocardiograph (ECG)
abnormalities, 6 patients left bundle branch block (LBBB), two
patients Wolff-ParkinsonWhite syndrome (WPW). Both ventricles
were involved in 8 patients (33.3%) and only the left ventricle in 16
patients (69.1%). Left ventricular systolic function was depressed in
12 patients (50%), with a median ejection fraction of 30% (20–66%)
at diagnosis. Of 9 patients who presented with depressed left
ventricular contractility, 66% had functional recovery. During follow-
up, 8 patients (33.3%) suffered an adverse event. Five patients with
biventricular and three with left ventricular compromise were put on
the heart transplantation programme and two required a ventricular
assistance device.
Conclusion:
In this cohort of patients with LVNC 69.9% remain in
functional class I–II. Risk factors for adverse events, heart transplan-
tation or death were biventricular involvement (
p
<
0.05) and conduc-
tion disturbances (
p
=
0.001).
1574: PERIPHERAL MICROVASCULAR FUNCTION IS
AFFECTED IN YOUNG INDIVIDUALS AT RISK FOR HCM
AND CORRELATES WITH FINE CHANGES IN MYOCAR-
DIAL FUNCTION AND ELECTROPHYSIOLOGY
Eva I Fernlund
1
, Todd T Schlegel
2
, Pyotr Platonov
3
, Jonas Carlson
3
,
Annika Maxedius
1
, Petru Liuba
1
1
Pediatric Cardiology Department, Lund University, Lund, Sweden
2
NASA Johnson Space Center, Houston, Texas, USA
3
Cardiology Department, Lund University, Lund, Sweden
Background
: Recent data from our centre suggested that advanced
electrocardiograph (ECG) analysis combined with tissue Doppler
(TD) has increased sensitivity in identifying individuals at risk for
developing hypertrophic cardiomyopathy (HCM), and in distinguish-
ing HCM from athlete’s heart. Adult patients with HCM appear
to have functional abnormalities not only in coronaries but also in
peripheral vessels. Whether the latter are present in young individu-
als, and whether they can be used as an additional index for stratify-
ing the risk for HCM have not been yet studied.
Methods:
The study population, part of a cohort from an ongoing
prospective study, consisted of children and adolescents (age 4–27,
median 13.9 years) with familial HCM heredity, either without left
