CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
180
AFRICA
Results:
The age of the Fontan cohort was 11.9 (5.9–16.7) years.
The interval from Fontan surgery to the elastography scan was 8.2
(1.0–13.5) years. None of the Fontan patients were in cardiac failure,
only one had a persisting fenestration, and six had hepatomegaly. All
had an extra-cardiac repair. Liver enzyme values were higher in the
Fontan group (ALT 30 vs 16 U/l;
p
<
0.003 and GGT 49 vs 11 U/l;
p
<
0.0001). Elastography values were significantly higher in Fontan
patients compared with controls (17.1 (11–39) vs 4.7 kPa (3-6),
p
<
0.0001). There was no association between elastography values and
patient age or time since Fontan surgery.
Conclusions:
Elastography is a feasible non-invasive method to
assess liver stiffness in children following Fontan surgery. Paediatric
Fontan patients have markedly elevated liver stiffness, suggesting a
high risk for advanced HF. Elastography has important utility in the
follow up of paediatric Fontan patients.
693: OUTCOMES OF PAEDIATRIC PULMONARY HYPER-
TENSION: DATA FROM THE SPANISH REGISTRY FOR
PAEDIATRIC PULMONARY HYPERTENSION (REHIPED)
Maria Jesus del Cerro, Anna Sabate Rotes, M Quero, I Raposo, A
Moya, S Villagra, A Gil, REHIPED investigators
Hospital La Paz, Madrid, Spain
Complejo Hospitalario Universatrio Juan Canalejo, A Coruna, Spain
Hospital Infantil Universitario La Fe, Valencia, Spain
Hospital de Monteprincipe, Madrid, Spain
Hospital Universitario de Salamanca, Salamanca, Spain
Objectives
: There is sparse data on epidemiology and survival in
paediatric pulmonary hypertension.
Methods
: From January 2009 to June 2012, 21 referral and non-
referral centres collected data of 225 patients, with mean pulmonary
artery pressure (mPAP)
>
25 mmHg and pulmonary vascular resist-
ance index (PVRI)
>
3 WU/m
2
. We included all Dana point (DP)
aethiologies (groups I to V) and analysed differences in survival
according to DP aethiological group, age at diagnosis, functional
class (FC), syncopes, chromosomopathy, right atrial pressure (RAP),
mPAP, cardiac index (CI), PVRI, and PVRI/SVRI.
Results:
Patients were classified as: group I (PAH,
n
=
142, 61%), II
(left heart disease,
n
=
31, 14%), III (respiratory disease,
n
=
41, 18%,
half of them with bronchopulmonary dysplasia), IV (thromboembolic
PH,
n
=
2, 1%), V (
n
=
10, 4.5%, mostly inherited metabolic diseases),
but 31% had multifactorial PH. Median age at diagnosis was 4.3
±
4.9
years (50%
<
2 years); 21% were premature, and chromosomopathies/
syndromes were present in 38%. Functional class at diagnosis was III/
IV in 53%, without significant differences in mPAP, RAP, PVRI/SVRI
or CI between the different DP groups. For the whole cohort, one- and
three-year survival was 80 and 74%. Mortality risk factors (univariate
analysis): DP aethiology group (three-year survival, 81% for PAH,
60% for left heart disease PH, 58% for lung disease PH, and 15%
for Dana point group V,
p
<
0.001), functional class at diagnosis (
p
<
0.001), RAP (
p
=
0. 006), and age at diagnosis (three-year survival,
60% for
<
2 years, 92% between 2 and 8 years, and 85% for
>
8 years,
p
<
0.001). Multivariate analysis: aethiology (
p
<
0.001), age at diag-
nosis (
p
<
0.001), FC at diagnosis (
p
<
0.001) and RAP (
p
=
0.002).
Conclusions:
Age at diagnosis was a significant risk factor: there
was worse survival in younger patients (often frequently missed in
referral centre-based registries). In severe paediatric PH, prognosis
was better in PAH than in the other DP aethiological groups.
698: CHALLENGES FOLLOWING SURGERY IN CONGENI-
TAL HEART DEFECTS: INCREASING INCIDENCE AND
IMPROVED SURVIVAL IN COMPLEX LESIONS
Gunnar Erikssen, Knut Liestoel, Erik Thaulow, Jan-Ludvig
Svennevig, Harald L Lindberg
Rikshospitalet, Oslo University Hospital, Norway
Background
: Few congenital heart defects (CHD) are now beyond
the reach of surgical treatment. There are limited data on the impact
of recent surgical development on the composition of the CHD
population.
Methods
: Between 1971 and 2010, the 7 018 patients aged
0–16 years undergoing corrective CHD surgery at Oslo University
Hospital, Rikshospitalet (80% of all CHD operations in Norway),
were prospectively enrolled. Primary CHD diagnoses were catego-
rised according to a consensus-based severity hierarchy. Date of first
operation was noted, and complete all-cause mortality data were
recorded up to 1 January 2012.
Results
: Number of patients increased from 1 233 in 1971–1980 to
2 286 in 2001–2010. The proportions of patients with tetralogy of
Fallot (TOF; 7.7% in 2001–2010), transposition of the great arter-
ies (TGA; 7.9%), univentricular hearts (UNI; 5.1%) and ventricular
septal defect (VSD; 16.3%) did not change. However, there was
a marked increase in the proportions of patients with hypoplastic
left heart syndrome (HLHS), atrioventricular septal defect (AVSD),
pulmonary atresia (PA) and interrupted aortic arch (IAA) (3.6% in
1971–1980, and 25.4% in 2001–2010), and a decrease in surgical
treatment of anatomically simpler defects. Overall 30-day postopera-
tive survival improved from 89.3% in 1971–1980 to 97.5% in 2001–
2010, with particularly marked changes in groups with complex
lesions. Among the 1 011 patients operated in 1971–1980 who
survived beyond 16 years, 50 (4.5%) had HLHS, AVSD, IAA, PA or
UNI. In contrast, among the 1 682 patients operated in 1991–2000
surviving beyond 16 years or until 1 January 2012, 344 (17.6%) had
such complex defects.
Conclusion
: The number and proportion of patients with complex
CHD surviving childhood are increasing rapidly, since more of
these defects are treated surgically, and with substantially improved
survival.
699: MANAGEMENT AND CLINICAL FEATURES OF
PAEDIATRIC PULMONARY ARTERIAL HYPERTENSION
IN SPAIN: DATA FROM THE SPANISH REGISTRY FOR
PAEDIATRIC PULMONARY HYPERTENSION (REHIPED)
Maria Jesus del Cerro
1
, Antonio Moreno
2
, Anna Sabate Rotes
1
, M.T.
Viadero
3
, MA Izquierdo
4
, O Dominguez
5
, H Falcon
6
, REHIPED
investigators
1
Department of Paediatric Cardiology, Hospital La Paz, Madrid,
Spain
2
Department of Paediatric Cardiology, Hospital Vall d’Hebron,
Barcelona, Spain
3
Department of Paediatrics, Hospital Marques de Valdecilla,
Cantabria, Spain
4
Department of Paediatric Cardiology, Hospital de Donostia, San
Sebastian, Spain
5
Department of Paediatric Cardiology, Hospital Virgen de la Salud,
Toledo, Spain
6
Hospital Materno Infantil de Canarias, Gran Canaria, Spain
Objectives:
There is sparse information on paediatric PAH, but
referral centre-based registries offer data on selected populations.
We collected data on clinical features, management and outcomes of
paediatric PAH in Spain from the REHIPED registry.
Methods:
From January 2009 to June 2012, 21 centres (referral and
non-referral) included cases, aged two months to 18 years at diag-
nosis, with mean pulmonary arterial pressure mPAP
>
25 mmHg,
pulmonary vascular resistance index (PVRI)
>
3 WU/m
2
, and pulmo-
nary wedge pressure
<
15 mmHg.
Results:
We included
142 patients: congenital heart disease (PAH/
CHD,
n
=
105, 74%), idiopathic/familial (IPAH,
n
=
32, 21%),
portopulmonary (
n
=
3, 2.1%), HIV infection (
n
=
1, 0.7%), connec-
tive tissue disease (
n
=
1, 0.7%); 42% were prospective cases. Mean
age at diagnosis: 5.2
±
4.8 years (36% patients
<
2 years.), age at
inclusion: 8.7
±
6.5 years. Female/male ratio: 1.2. Syndromal anoma-
lies were present in 44%; 51% cases were in functional class (FC)
III/IV at diagnosos. Haemodynamic data: mPAP 46
±
18 mmHg,
PVRI 8.7
±
7.8 WU/m
2
, PVRI/SVRI 0.7
±
0.5, cardiac index (CI)
4.6
±
2l /min/m
2
. Only 6% of IPAH children were responders in
