Cardiovascular Journal of Africa: Vol 24 No 1 (February 2013) - page 183

CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013 AFRICA 181
the vasoreactivity test. Of the whole group, 92% received PH drugs
(46% in combination, 23% prostanoids), 7% oral anticoagulation
and 14% anti-aggregation. For the whole group, one-, three- and
five-year survival was 89, 85 and 79%, respectively, without differ-
ences between IPAH and PAH/CHD. Mortality risk factors (univari-
ate analysis): younger age at diagnosis (p = 0.013), FC III/IV (p <
0,001), CI (p = 0.005) and RAP ( p = 0.014). Multivariate analysis:
age at diagnosis [HR 0.84 (95% CI: 0.73–0.96), p = 0.009], FC (p
< 0.001), IC [HR 0.46 (95% CI: 0.3–0.7), p < 0.001], RAP (HR 1.2
(95% CI: 1.06–1.38), p = 0.012].
Conclusions: REHIPED registry patients had a younger age at diag-
nosis, higher percentage of prospective cases, and lower percentage
of IPAH responders than other referral-centre registries, but similar
survival, with high prevalence of combination therapy. In paediatric
PAH, besides the already known risk factors (FC, CI, RAP), younger
age at diagnosis was also a risk factor for mortality.
704: CLINICAL EXPERIENCE OF SUBCUTANEOUS AND
TRANSVENOUS IMPLANTABLE CARDIOVERTER-DEFI-
BRILLATORS INYOUNG PATIENTS
Stephen J Pettit1, AndrewMcLean2, Ian Colquhoun1, Derek Connelly2,
Karen McLeod1
1Golden Jubilee National Hospital, Agamemnon Street, Clydebank,
Glasgow, UK
2Royal Hospital for Sick Children, Dalnair Street, Yorkhill, Glasgow,
UK
Background: Subcutaneous ICDs do not require a lead to be in
contact with the heart. Potential advantages over transvenous ICDs
are the elimination of vascular complications and reduction of lead
fractures. We compared the short-term performance of subcutaneous
and transvenous ICDs in patients < 20 years old.
Methods: The study was a retrospective, observational study of all
patients < 20 years of age who received an ICD over a four-year
period (July 2007 to July 2011) in the west of Scotland. Baseline
characteristics, complications and ICD therapy were recorded. The
primary outcome measure was survival. The secondary outcome
measure was survival free from inappropriate ICD therapy or system
revision.
Results : Nine patients received a subcutaneous ICD and six patients
received a transvenous ICD. Underlying diagnoses and age at implant
were similar for the two groups. There were no implant complications
with subcutaneous ICDs, but one patient sustained a pneumothorax
during transvenous ICD implantation. Median follow up was 20
months (range 12–32 months) for subcutaneous ICDs and 36 months
(range 24–55 months) for transvenous ICDs. Appropriate shocks for
VF occurred in two patients with a subcutaneous ICD and one with
a transvenous ICD. No subcutaneous ICD required revision, but two
transvenous ICDs were extracted due to infection (n = 1) and lead
fracture (n = 1). Survival was 100% in both groups. Survival free
from inappropriate ICD therapy or system revision was 89% for
subcutaneous ICDs and 25% for transvenous ICDs (log rank test, p
= 0.0237).
Conclusions: In our series of young patients, subcutaneous ICDs
performed well on short-term follow up, with a lower incidence
of inappropriate shocks and complications requiring system revi-
sion, compared to transvenous ICDs. In the absence of randomised
trials, subcutaneous ICDs should be compared prospectively with
transvenous systems in large multi-centre registries with comparable
periods of follow up.
705: PREVALENCE OF MYOCARDIAL FIBROSIS BY
CARDIAC MAGNETIC RESONANCE IMAGING PREDICTS
CLINICAL PRESENTATION AND OUTCOME IN CHIL-
DRENWITH HYPERTROPHIC CARDIOMYOPATHY
Lidia Ziolkowska1, Wanda Kawalec1, Jolanta Misko2, Joanna Petryka2,
Mateusz Spiewak2, Lukasz Malek2, Lukasz Mazurkiewicz2, Andrzej
Kosciesza 3, Agnieszka Boruc1, Katarzyna Bojarska 1
1Department of Paediatric Cardiology, Children’s Memorial Health
Institute, Warsaw, Poland
2Cardiac Magnetic Resonance Unit, Institute of Cardiology, Warsaw,
Poland
3Cardiac Magnetic Resonance Unit, The Children’s Memorial Health
Institute, Warsaw, Poland
Background: Patients with hypertrophic cardiomyopathy (HCM)
frequently demonstrate non-ischaemic pattern myocardial fibrosis
(MF). Cardiac magnetic resonance (CMR) imaging with late gado-
linium enhancement (LGE) can detect a small and focal MF. The
aim of the study was to assess the occurrence of MF in children with
HCM and to evaluate its clinical significance.
Methods: Fifty-six children with HCM underwent CMR from
January 2010 to June 2012. All 56 patients were divided into two
groups: group I: 30 (54%) children, mean age 12.7 years with
myocardial fibrosis by LGE and group II: 26 (46%) patients, mean
age 11.3 years without MF. Patients’ demographics, clinical symp-
toms as well as the results of LGE CMR were analysed and compared
between the groups.
Results: The patients in the fibrosis group more often had symptoms
such as syncopes (13 vs 4%), presyncopes (37 vs 23%), chest pain
(43 vs 23%), and fatigue (80 vs 65%). Among children with MF,
LV wall thickness (mean 22 vs 14 mm; p = 0.0001) and LV mass
index (mean 105 vs 86 g/m²; p = 0.003) were significantly greater
compared with those without MF. In 79% patients in group I and
in 36% in g roup II, LV mass was increased (p = 0.003). In group
I, nsVT episodes were more frequent than in group II (10 vs 4% of
patients). Patients in both groups differed significantly regarding the
occurrence of major risk factors for SCD (67 vs 31%; p = 0.016).
LV wall thickness ≥ 30 mm and cardiac arrest were observed only in
group I (20 and 7%, respectively; p = 0.025), and family history of
SCD was more frequently positive (30 vs 15%). In group I, 13% of
patients underwent surgical myectomy compared with 4% in group
II. ICD was implanted only in children with MF (33%; p = 0.001),
in two patients as secondary prevention and in eight as primary
prevention.
Conclusion: The presence of myocardial fibrosis was related to an
unfavourable clinical course of HCM in children. Visualising LGE
in CMR may contribute to better risk stratification of SCD and may
help in taking therapeutic decisions in HCM patients.
707: PULMONARY HYPERTENSION IN BRONCHOPULMO-
NARY DYSPLASIA: CLINICAL FINDINGS, ASSOCIATED
CARDIOVASCULAR ABNORMALITIES AND OUTCOMES
Maria Jesus del Cerro, Anna Sabate Rotes, Antonio Carton, Lucia
Deiros, Carlos Labrandero, Montserrat Bret, Maria Isabel Barrio,
Juan Jose Mendez, Malaika Cordeiro, Federico Gutierrez-Larraya
Paediatric Cardiology, Hospital La Paz, Madrid, Spain
Background: Severe pulmonary hypertension (PH) worsens the
prognosis of bronchopulmonary dysplasia (BPD). Clinical char-
acterisation, incidence of cardiovascular abnormalities (CVA) and
outcome in this setting have not yet been fully established.
Methods: This was a retrospective study of the clinical records,
CT imaging and catheterisation data of 29 patients with BPD and
PH referred to our pulmonary hypertension unit from March 2006
to December 2011. Median gestational age was 27 weeks (IQR
24–27.3) and median birth weight 740 g (IQR 620–830). Sixteen
were male and 13 female (ratio 1.2).
Results: PH diagnosis was made at a median age of 4.5 months
(IQR 2.4–7.8), in 48% after initial hospital discharge, with echocar-
diography estimating the median right ventricular pressure/systemic
pressure ratio of 70% (IQR 60–80%). CT scan was performed in
21 patients and catheterisation in 13, finding CVA in 19 patients
(65.5%): nine systemic-to-pulmonary collaterals, seven pulmo-
nary vein stenosis, four ASD, one restrictive VSD and nine PDA.
Haemodynamic data were (median and IQR): pulmonary arterial
systolic pressure 78.5% of systemic (57.8–94.7), PVR 4.6 UW/m2