CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
215
Introduction:
The double-chambered right ventricle (DCRV) is an
uncommon congenital heart disease; it represents 0.5% of these
congenital cardiopathies.
Objective:
A series of 11 cases with diagnosis of double-chambered
right ventricle is presented from the paediatric age to adulthood
(2–64 years).
Methods
: The median of age of the studied patients was 10 years
(range: 2–64); 36.45% of these cases were asymptomatic and the diag-
nosis was made because of an incidental finding of cardiac murmur.
In other cases patients did not thrive in the breatfeeding stage and in
older children a worsening of NYHA functional class was observed.
The echocardiogram showed double-chambered right ventricle as
seen by a muscular band in all patients. The most frequent associ-
ated anomalies were: ventricular septal defect, patent foramen ovale,
subvalvular aortic stenosis, and tetralogy of Fallot. Seven patients had
surgical treatment and six are now in NYHA functional class I.
Conclusions:
This study has special importance because is the
largest series of patients with double-chambered right ventricle in
Mexico and the results are comparable to studies reported in the
literature. Worsening of NYHA functional class was the predomi-
nant symptom. Clinical manifestations can present from infancy
to adulthood and the degree of obstruction may be progressive.
Echocardiography is the method of choice in the diagnosis of these
patients. The 91% of patients with DCRV who had surgical treatment
had excellent haemodynamic and functional results at mid-follow up.
1144: MORTALITY AND MORBITY IN YOUNG PACEMAK-
ER AND INTRACARDIAC DEFIBRILLATOR CARRIERS: A
FOCUS ON EPICARDIAL VS ENDOCARDIAL STRATEGY
IN LONG-TERM FOLLOW UP
Luca Tomasi
1
, Alessandra Cristofaletti
1
, Luca Barozzi
2
, Paolo
Moggio
1
, Maria Antonia Prioli
1
, Lucia Rossetti
1
, Fabrizio De Rita
2
,
Giovanni Battista Luciani
2
, Giovanni Morani
1
, Corrado Vassanelli
1
1
Department of Cardiology, University Hospital Civile Maggiore,
Verona, Italy
2
Department of Cardiosurgery, University Hospital Civile Maggiore,
Verona, Italy
Background:
The choice of the most appropriate procedural tech-
nique for paediatric cardiac pacing is still debated.
Aim:
To evaluate the outcome, in terms of mortality and morbidity,
of young PM or ICD carriers.
Methods:
All PM or ICD carriers in our centre less than 18
years were included. Indications for device therapy, peri-procedural
complications and long-term follow up were revised. An echocar-
diogram was performed in patients with isolated congenital atrio-
ventricular block (iAVB) focused on the presence of aortic dilation.
Results:
We followed
34 patients, 28 with PM and six with ICD, for
a median time of 7.8 and 2.4 years, respectively. The overall survival
in patients carrying an ICD was 100% (93
±
5) in the PM group. All
four deaths were complex congenital heart disease patients (CHD).
The major complications were lead failures (19%) and infections
(12%). The complication-free survival was 57
±
12% at 10 years in
all patients. Infections were responsible for a worse outcome in CHD.
Time to first complication was significantly shorter for epicardial
pacing systems (3.35 years vs
5.63 years,
p
=
0.016). The difference
between epicardial and endocardial systems was not significant in
terms of complications and electrical parameters. iAVB was associ-
ated with significant dilation of the ascending aorta (median
z
-score
2.32, Q1–Q3:
0.55–3.73,
p
=
0.047).
Conclusions:
PM/ICD carriers experienced a significant number of
complications. Systemic infections were responsible for the majority
of deaths, especially in patients with CHD and endocardial leads.
For this reason, epicardial leads used as long as possible in CHD
patient might be a reasonable choice. The challenge for the future
is to increase longevity of pacing systems and to reduce number
of re-intervention. The positive correlation between dilation of the
ascending aorta and iAVB in stimulated hearts needs further inves-
tigations.
1145: THE UTILITY OF REMOTE MONITORING OF
IMPLANTABLE CARDIAC DEFIBRILLATORS AND PACE-
MAKERS IN A PAEDIATRIC TERTIARY CARE CENTRE
Maully Shah
1
, Karen Smoots
1
, Jason Imundo
2
1
The Children’s Hopsital of Philadelphia, University of Pennsylvania,
USA
2
Hershey Children’s Hospital, Pennsylvania State University, USA
Background:
The number of paediatric and adult congenital heart
disease patients with implantable cardiac defibrillators and pacemak-
ers continues to grow on a yearly basis. Cardiovascular implantable
electronic devices (CIED) require regular follow up to ascertain tech-
nical integrity. There is a paucity of published literature in the paedi-
atric population regarding remote monitoring in patients with CIED.
Methods:
A single-centre, retrospective, observational chart review
was performed. Data were obtained and reviewed for patients
who have CIED and were followed at the Children’s Hospital of
Philadelphia from 1 January 1991 to 1 November 2011. The primary
objective was to describe the use of remote monitoring in paediatric
patients with CIED at a large tertiary care children’s hospital. The
secondary objective was to determine the potential clinical benefit to
patient safety and care, and determine if remote monitoring decreases
the number of hospital visits due to CIED related issues.
Results:
Fifty patients were included: 15 with pacemakers, 35 with
implantable cardiac defibrillators. The mean age was 19 years old.
The data sent by the remote monitoring system was monitored for
12 months for determination of clinically actionable events (CAE).
Of 633 total transmissions, 41 resulted in a CAE in 19 patients. The
CAE comprised the following: 29 arrhythmias, five lead malfunc-
tions, and seven other issues. In the pacemaker group, the alerts that
were most frequently activated were those concerning atrial tachyar-
rhythmia, battery exhaustion, lead impedance, as well as sensing and
threshold measurements. In the ICD therapy group, the alerts nearly
always activated were those concerning a detection set-off, battery
exhaustion, or critical values of impedance.
Conclusions:
Remote monitoring is a reliable method for following
up patients with CIEDs. Accurate alert settings personalised to the
patient’s clinical features are essential for effective management and
avoidance of excessive flow of data.
1146: UNUSUAL ATRIO-FASCICULAR ACCESORY PATH-
WAY IN AN ADOLESCENT WITH PRE-EXCITATION AND
LONG-TERM TACHYCARDIA
M Cecilia Gonzalez
1
, Thierry Sluysmans
1
, Jean-Benoït le Polain de
Waroux
2
, Christophe Scavée
2
1
Paediatric Cardiology, Cliniques Universitaires St Luc, Brussels,
Belgium
2
Cardiology Department, Cliniques Universitaires St Luc, Brussels,
Belgium
Background:
Mahaim fibres are rare decremental and antegrade-
only fibres connecting the right atrium to the right ventricle. We
present an extremely rare case of an atrio-fascicular fibre with proxi-
mal insertion in a para-Hissian position.
Case presentation:
A 17-year-old patient with pre-excitation and
history of tachycardia leading to heart failure at age 2 was referred
with recurrence of the arrhythmias despite therapy. The electro-
physiology study showed at baseline a negative HV interval in
sinus rhythm. Decremental atrial pacing lead to progressive AH
prolongation and loss of pre-excitation with normalisation of the HV
interval. Dual AV nodal physiology was excluded. Atrial stimulation
induced a usual wide-complex tachycardia with persistence of a
retrograde His. Late atrial extrastimuli during tachycardia advanced
the QRS complex proving pre-excitation of the ventricle via the AP.
Atrial mapping on sinus rhythm indentified the earliest signals in
the right antero-septal position. On the ventricular site, the earliest
potentials were identified on the moderator band. Ventricular pacing
excluded retrograde accessory pathway conduction. Cryo and RF
ablation of the atrial insertion was unsuccessful. We finally cryo-
