CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
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AFRICA
Being rather wide, the PFAA communication was thought initially
to be an aorto-pulmonary window. The patient died from pulmonary
infarction several days after surgical repair.
Conclusions
: Although rare, recognition of PFAA in all its various
forms is important for clinical management. The PFAA in our first
four cases was a vessel arising from an unusually proximal position
from the aorta to connect to the pulmonary artery. This vessel could
be mistaken for an arterial duct but it has unpredictable reactivity to
prostaglandin E
1
. The differential diagnosis for the fifth case was an
aorto-pulmonary window but its length and pulmonary communica-
tion at the bifurcation were against this diagnosis. The co-existence
of arch interruption in this case made diagnosis particularly chal-
lenging.
944: OUTCOMES OF PATIENTS UNDERGOING OPEN-
HEART SURGERY AT THE UGANDA HEART INSTITUTE,
MULAGO HOSPITAL
Twalib Aliku
1,2
, Sulaiman Lubega
2
, Michael Oketcho
2
, Peter Lwabi
2
,
John Omagino
2
, Tom Mwambu
2
1
Gulu University, Uganda
2
Uganda Heart Institute, Uganda
Background
: Heart disease is a disabling condition and the neces-
sary surgical service is often lacking in many developing countries. A
novel approach in which open-heart surgeries are conducted locally
by visiting teams alongside skills transfer to build local capacity has
been adopted at the Uganda Heart Institute (UHI).
Objectives
: We evaluated the progress of open-heart surgery at the
UHI, to describe the postoperative outcomes and challenges faced in
conducting these surgeries.
Methods
: Medical records of patients undergoing open-heart surgery
at the UHI from October 2007 to June 2012 were reviewed.
Results
: A total of 124 patients underwent open-heart surgery during
the study period. The commonest conditions were VSDs in 35.5%
(44/124), ASDs in 34.7% (43/124) and TOF in 10.5% (13/124).
Foreign charities funded 82.3% (102/124) of the operations, and only
four patients (3.2%) paid for their surgeries. A local NGO funded
the rest. There was increasing complexity in cases operated from
predominantly ASDs and VSDs at the beginning, to more complex
cases such as TOFs and TAPVR later on. The local team indepen-
dently operated on 19 patients (15.3%). Postoperative morbidity was
low, with left ventricular dysfunction and re-operations occurring in
six (4.8%) patients each. Sepsis occurred in only two cases (1.6%).
The 30-day mortality was 3.2 %
Conclusion:
Open-heart surgery, although expensive, is feasible in
a developing country. Governments and local charities should direct
funding to support treatment of more children with heart disease
locally as opposed to referral abroad to increase access to the service.
945: PULMONARY ARTERIAL HYPERTENSION ASSOCI-
ATED WITH CONGENITAL HEART DISEASE IN PAEDIAT-
RIC AND ADULT SPANISH POPULATIONS: DATA FROM
THE SPANISH REGISTRY FOR PAEDIATRIC PULMO-
NARY HYPERTENSION (REHIPED) AND SPANISH REGIS-
TRY FOR PULMONARY ARTERIAL HYPERTENSION IN
ADULTS (REHAP)
Maria Jesus del Cerro
1
, J.M. Oliver
1
, A. Mendoza
2
, Laura Dos
3
, A.
Rodriguez
4
, M. Quero
5
, Pilar Escribano
2
, REHAP/REHIPED inves-
tigators
1
Hospital La Paz, Madrid, Spain
2
Hospital 12 de Octubre, Madrid, Spain
3
Congenital Heart Disease Unit for Adolescents and Adults, De la
Santa Creu Hospital, Barcelona, Spain
4
Hospital Gregorio Marañón, Madrid, Spain
5
Hospital Ramon Cajal, Madrid, Spain
Background:
Pulmonary arterial hypertension is a serious complica-
tion of congenital heart disease (PAH/CHD), but there is not enough
data about clinical profiles and survival of these adult and paediatric
patients. Our objective was to analyse clinical and survival data in
adult and paediatric Spanish patients with PAH/CHD.
Methods:
Voluntary reporting was used of 338 adults diagnosed
with PAH/CHD from January 1998 to April 2012 from the REHAP
programme and 105 children diagnosed from January 2009 to June
2012 from REHIPED. Clinical classification for PAH/CHD was
used: (1) Eisenmenger, (2) non-restrictive shunt with high PVR, (3)
small shunt with high PVR , (4) corrected CHD without residual
shunt. Kaplan-Meier curves were analysed, censoring patients at
death/transplantation.
Results:
We included 443 patients. Mean age was 31
±
17 years in
the adult registry and 5.5
±
5 years in the paediatric one. Paediatric
patients had worse functional class (FC) (
p
=
0.003), and more
comorbidities (
p
<
0.05), but they showed higher cardiac index (
p
=
0.015), and lower mean pulmonary arterial pressure (
p
<
0.001). The
most frequent forms in adults were Eisenmenger (68%) and operated
shunts (20%), while in paediatrics, non-restrictive shunts with high
PVR (33%) or operated shunt (33%) were the most common forms
(
p
<
0.001). Nevertheless, survival was similar in paediatric and
adult patients: 91, 85, and 80% at one, three and five five years from
diagnosis. For the whole population, we found significant differences
in five-year survival according to FC at diagnosis (90% in FC I/II,
74% in FC III, 50% in FC IV,
p
<
0.001), and in Eisenmenger versus
operated CHD (94 vs 72%, respectively,
p
=
0.038).
Conclusions:
(1) Clinical profiles in CHD-PAH differ significantly
between paediatric and adult patients, but survival was similar in both
groups. (2) In the whole population, predictors for worse survival
were FC III/IV at diagnosis and operated CHD.
951: THE EPIDEMIOLOGICAL AND CLINICAL PROFILE
OF KAWASAKI DISEASE IN WESTERN AUSTRALIA: A
30-YEAR POPULATION-BASED STUDY
Jelena Saundankar
1
, Deane Yim
1
, Benedicta Itotoh
1
, Ruth Payne
1
,
Gayatri Jape
1
, James Ramsay
1
, Darshan Kothari
1
, Allen Cheng
2
,
David Burgner
3,4
1
Children’s Cardiac Centre, Princess Margaret Hospital for Children,
Perth, Australia
2
Monash University, Melbourne, Australia
3
Murdoch Children’s Research Institute, Royal Children’s Hospital,
Melbourne, Australia
4
School of Paediatrics and Child Health, University of Western
Australia, Perth, Australia
Background:
The current epidemiology of Kawasaki disease (KD)
in Australia is poorly understood. Previous enhanced national
surveillance (1993–1995) gave an estimated incidence of 3.7/100
000 children aged 0–4 years. In non-Asian countries, the incidence
varies between 3.6 in Denmark, 8.1 in the UK, 17.1 in the USA and
26.2 in Canada, although many data are regional rather than national.
Materials and Methods:
We identified all patients hospitalised in
western Australia (population ~2.2 million, of whom 3.4% self-iden-
tified as indigenous) between 1979 and 2009 with an ICD discharge
diagnosis of KD. We retrieved demographic, clinical, laboratory and
echocardiographic data from individual patient files. Age-specific
population estimates were from national census data; 95% confi-
dence intervals were calculated assuming a Poisson distribution.
Results:
We identified 353 KD cases. Male-to-female ratio was 1.7:1
and the median age was three years (IQR 12, 60 months). The mean
annual incidence increased from 2.82 per 100 000 children aged
0–4 years (95% CI: 1.93–3.99) in 1980–1989, to 8.04 (6.55–9.76)
in 1990–1999, to 9.66 (8.01–11.55) in 2000–2009. The highest inci-
dence in the 0–4 age group was 15.7 in 2005. Incomplete KD was
diagnosed in 40/353 (11.4%) patients. Of the 288 children who had
complete echocardiographic studies, 42/288 (14.6%) had coronary
artery (CA) ectasia/dilatation and 26/288 (9%) had coronary aneu-
rysms. The only significant risk factor for CA involvement was Asian
ethnicity. There were no KD cases in indigenous children (5.8% of
WA population).
