CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
145
uncharacterised (
de novo
) mutations and three were not tested.
Bradycardia incidence depended on the definition: 15% of FHR
readings were
≤
110 bpm and 66%
≤
3rd percentile. Confirmation
of foetal LQTS resulted in diagnosing unsuspected LQTS in three
families.
Conclusions:
FHR varies widely in LQTS. A FHR
≤
3rd percen-
tile for gestational age increases the sensitivity to ascertain foetal
LQTS compared to the FHR
≤
110 bpm definition. LQTS should
be suspected if FHR
≤
3rd percentile for gestational age even in the
absence of arrhythmias.
De novo
mutations result in the most severe
foetal bradycardia. These findings may improve detection of foetal
and neonatal LQTS.
160: PREDICTORS OF DISEASE PROGRESSION IN PAEDI-
ATRIC DILATED CARDIOMYOPATHY
Kimberly Molina, for the Paediatric Heart Network Investigators
University of Utah, USA
Background:
Dilated cardiomyopathy (DCM) is the leading indica-
tion for heart transplantation in children. Identifying patients at risk
for disease progression/transplantation remains elusive.
Methods:
The Paediatric Heart Network Ventricular Volume
Variability Study evaluated chronic DCM patients with serial
prospective echocardiographic and clinical data collection over an
18-month follow up. Inclusion criteria were age
<
22 years and DCM
disease duration
>
2 months with exclusion of those needing IV
inotropic or mechanical support, and those listed as status 1A/1B for
transplant. Disease progression was defined as an increase in trans-
plant listing status, hospitalisation for heart failure, IV inotropes,
mechanical support, or death during follow up. Predictors of disease
progression were identified using logistic regression and classifica-
tion and regression tree (CART) analysis.
Results:
Of the 127 patients, 28 (22%) met the criteria for disease
progression during the 18-month follow-up period. Multivariable
analysis (
c
-statistic
=
0.90) identified older age at diagnosis (OR
=
1.16 per year,
p
=
0.003), larger left ventricular (LV) end-diastolic
M-mode dimension
z
-score (LVEDDz) (OR 1.77,
p
<
0.001) and
lower septal peak systolic tissue Doppler velocity
z
-score (OR
=
0.68,
p
=
0.04) as independent predictors of disease progression. CART
analysis risk-stratified patients for significant disease progression
with 89% sensitivity and 94% specificity based on LVEDD z
≥
7.7,
LV ejection fraction
<
38.2%, LV inflow propagation velocity (color
M-mode)
z
-score
<
–0.28, and age at diagnosis
≥
8.5 months.
Conclusion:
In paediatric patients with DCM, diagnosis after late
infancy and echocardiographic parameters of LV size, systolic
and diastolic function were independently associated with disease
progression, and may be used to reliably risk stratify DCM patients.
161: PHYSICAL ACTIVITY RECOMMENDATIONS IN
CONGENITAL AND ELECTROPHYSIOLOGICAL HEART
DISEASE: A SURVEY OF CANADIAN HEALTHCARE
PROVIDERS
Thomas Roston, Astrid De Souza, George Sandor, Shubhayan
Sanatani, James Potts
British Columbia Children’s Hospital and The University of British
Columbia, Canada
Background:
Determining safe levels of physical activity for chil-
dren and adolescents with congenital and arrhythmic heart disease
is a challenging clinical problem. The body of evidence for making
these recommendations is limited and based on the perceived risks
of sudden cardiac death (SCD) with activity. The Bethesda guidelines
were designed to establish consensus guidelines for eligibility and
disqualification from competitive sports in athletes with cardiovas-
cular abnormalities. However, literature on non-competitive physical
activity is not available.
Methods:
A survey was designed to determine practice patterns for
patients with structural and arrhythmic heart disease
.
Between July
2011 and December 2011, approximately 350 healthcare providers
working with this group of patients were recruited by e-mail or while
attending professional meetings. We received 81 responses, primarily
from paediatric cardiologists (70%).
Results:
Our findings indicate that the majority of Canadian cardiac
care providers surveyed are only partially implementing current
recommendations. Areas of disagreement included physical activ-
ity recommendations for hypertrophic cardiomyopathy, long QT
syndrome, catecholaminergic polymorphic ventricular tachycardia
and heart transplantation, among others. The development of consen-
sus guidelines for activity recommendations was supported by 96%
of respondents.
Conclusions:
The heterogeneity of our responses may be attributed
to conflicting information in the literature, an entrenched tendency
towards bed rest in the cardiology community and a lack of aware-
ness by cardiac care providers regarding the role of physical activity
in structural and arrhythmic heart disease. Balancing the risk of SCD
with the long-term morbidity and mortality associated with cardio-
vascular disease needs to be strongly considered.
170: LONG-TERM RESULTS OF PERCUTANEOUS
BALLOON VALVULOPLASTY FOR CRITICAL NEONATAL
AORTIC STENOSIS
Oleg Reich
1
, Petr Tax
1
, Jan Marek
2
, Viktor Tomek
1
, Jan Skovranek
1
,
Jiri Gilik
1
, Roman Gebauer
1
, Vaclav Chaloupecky
1
, Jan Janousek
1
1
Children’s Heart Centre, University Hospital Motol, Prague, Czech
Republic
2
Great Ormond Street Hospital for Children, London, UK
Background:
Since the late eighties, balloon valvuloplasty has been
the first-choice treatment of neonatal aortic stenosis. The aim of the
study was to offer long-term results of the balloon valvuloplasty to
compare with new surgical methods such as valve shaving and plasty
that provide promising short-term results in selected newborns.
Methods:
This was a retrospective follow-up study of all the 126
patients initially treated with the balloon valvuloplasty and regularly
followed up in a single high-volume tertiary referral centre. Only
94 of them (74.6%) fulfilled the revised criteria for biventricular
repair published by Colan
et al
. The age at valvuloplasty was 0 to 28
days (median 2 days) and the follow-up period was up to 22.4 years
(median 4.8, in 111 early survivors 6.9 years).
Results:
Thirty patients (23.8%) died, 28 (22.2%) developed
re-stenosis, 16 (12.7%) severe aortic regurgitation and 20 (15.9%)
both re-stenosis and regurgitation. Surgery was needed in 41 (32.5%)
patients. Mean (SEM) actuarial probabilities 20 years after the proce-
dure were as follows: freedom from re-stenosis 0.43 (0.11), freedom
from severe aortic regurgitation 0.48 (0.07), survival 0.70 (0.07), and
surgery-free survival 0.24 (0.06). Twenty-year survival probability
in patients who fulfilled the Colan criteria was 0.89 (0.03) and in
those who did not 0.32 (0.10),
p
<
0.001. Risk factors for the death
identified by Cox analysis were small body surface area, small aortic
annulus, left ventricular failure/duct-dependent systemic circulation,
severe endocardial fibroelastosis, mitral stenosis, and severe pulmo-
nary hypertension.
Conclusions:
Balloon aortic valvuloplasty is a good life-saving
palliation for critical neonatal aortic stenosis. Less than a quarter
of patients treated will survive childhood without surgery. Small
newborns and those with small aortic annuli and concomitant left
heart diseases are at greater risk of death.
181: EFFECT OF E-HEALTH INDIVIDUALLY TAILORED
ENCOURAGEMENTS TO PHYSICAL EXERCISE ONAERO-
BIC FITNESS AMONG ADOLESCENTS WITH CONGENI-
TAL HEART DISEASE: A RANDOMISED CLINICAL TRIAL.
DESIGN AND RATIONALE FOR THE PREVAIL STUDY
Susanne Hwiid Klausen
1
, Ulla Ramer Mikkelsen
2
, Asle Hirth
3
, Jãrn
Wetterslev
4
, Hanne Kjãrgaard
1
, Lars Søndergaard
5
, Lars Andersen
6
1
Research Unit Women’s and Children’s Health, The Juliane Marie
