CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
234
AFRICA
3
The Mater Hospital for Children, Brisbane, Australia
4
Princess Margaret Hospital, Perth
5
The Children’s Hospital at Westmead, Sydney, Australia
6
The Women’s and Children’s Hospital, Adelaide, Australia
Background:
Idiopathic pulmonary arterial hypertension (IPAH)
in children is an uncommon and progressive condition with a poor
natural history. Currently available therapies have not been trialled
in children and little information exists about long-term survival in
treated children.
Methods:
A multicentre, web-based Australian and New Zealand
registry for children with PAH was established to better understand
the profile of children with IPAH, aiming to describe demographics,
presenting features, clinical course and outcomes of children aged
three months to 18 years at the time of the PAH diagnosis, from 1
January 2002. A group of nine historic controls with IPAH diagnosed
between 1970 and 1990 were used for comparison.
Results:
Of the 24 patients enrolled with IPAH, 54% were female
and 75% were Caucasian. The mean (SD) age at diagnosis was
9.3 (4.90) years with 50% in the WHO functional class III at pres-
entation. During follow up, 20 (83%) were treated with an ERA,
(exclusively Bosentan monohydrate), 17 (71%) with PDE5 inhibitor
(sildenafil) and 10 (42%) with either epoprostenol or iloprost; 19
(79%) received warfarin. The mean duration of follow up was 3.45
years, with an annualised mortality of 6%. Study end-points of death/
transplant at nine years post PAH diagnosis was 25% for patients in
the current era compared to 60% for historic controls (
p
<
0.007).
Freedom from death, transplant or epoprostenol initiation for current
era patients was 30% at seven years after diagnosis. The severity of
PAH from cardiac catheterisation was similar between patients and
controls, except for a lower mean CI of 2.57
±
0.93 in current era
group versus 3.85
±
1.85 (
p
<
0.05) for the historic controls.
Conclusions:
Multiple medical therapies, in particular a high utilisa-
tion of epoprostenol, have resulted in improved survival for children
with IPAH. A better understanding of childhood IPAH will facilitate
medical care, permit standardisation of therapeutic guidelines, and
allow better representation in the healthcare arena.
1358: ECHOCARDIOGRAPHIC LONGITUDINAL STUDY
IN CHILDREN WITH ACQUIRED IMMUNODEFICIENCY
SYNDROME
Maria Suely Bezerra Diogenes, Regina Calia Menezes Succi, Valdir
Ambrosio Moisas, Solange Bernardes Tatani, Celia Maria Camelo
Silva and Antonio Carlos Camargo Carvalho
Federal University of São Paulo, Brazil
Background:
Cardiovascular abnormalities in children with acquired
immunodeficiecy syndrome is slow and progressive in occurence.
The most frequent echocardiographic abnormalities are left ventricu-
lar dilation and dysfunction.
Aim:
To study prospectively the cardiovascular profile of children
with acquired immunodeficiecy syndrome (AIDS) by echocardiog-
raphy.
Methods:
We studied 46 HIV 1-positive children born to HIV-infected
mothers who progressed to AIDS, 25 were males and 21 females, and
age ranged from four months to 11 years (average 6 years). The
patients were classified according to CDC’s 1994 revised classifica-
tion system and were longitudinally studied by two-dimensional and
Doppler echocardiography from 1995 to 2012. The mean follow-up
period was six years for each child.
Results:
Cardiac abnormalities were encountered in 12 children
(26%): mild tricuspid regurgitation: 16.6%; mild mitral regurgitation:
8.3%; mild aortic regurgitation: 8.3%; severe dilation of ascending
aorta: 8.3%; mild dilation of left ventricle: 8.3%; pericardial effusion
with cardiac tamponade: 16.6%; mild-to-severe left ventricular dila-
tion and dysfunction: 33.3%; severe pulmonary hypertension with
right ventricular dysfunction: 8.3%. Eight cases were in an advanced
clinical immunological stage (C3) and the others had moderate signs
and symptoms of AIDS. There was one case (C3) of reversibility of
severe left ventricular dilation with dysfunction and the patient is
doing fine at the age of 17 years.
Conclusions:
Cardiac abnormalities initially appeared in children
with moderate symptoms (B1) of AIDS and were more frequent in
the ones with severe symptoms (C3). The most frequent abnormality
was dilated cardiomyopathy and it was reversible in one case.
1361: 10-YEAR EXPERIENCE OF IMPLANTATION OF
CONTEGRA GRAFT IN YOUNG PATIENTS: A SINGLE-
CENTRE STUDY OF OUTCOME
Phan-Kiet Tran, Edin Omerbasic, Jens Johansson-Ramgren, Sune
Johansson, Torsten Malm
Children’s Hospital, University Hospital, Lund, Sweden
Background:
It has been shown that the use of a bovine jugular vein
graft (Contegra) to reconstruct the RVOT is currently, in the mid-term
perspective, the best alternative to homografts. Recent studies also
report on a sustained freedom from re-intervention in the long-term
perspective for graft sizes 16 mm and larger. Here, we investigate the
outcome in a group that consisted of predominantly young patients,
and consequently also a higher number of smaller-sized grafts. We
also investigated the value of post-implantation right ventricular and
pulmonary arterial pressure to predict future stenosis.
Methods:
A total of 94 grafts had been implanted between 2002 and
2012. Mean patient age was 3.3 years (3 days – 18 years, median
1.25 years). Total follow-up time was 226 patient years (25 days – 10
years, median 1.84 years). Diagnoses included: 37 pulmonary atresia,
VSD with or without MAPCA (39%), 15 tetralogy of Fallot (16%),
14 truncus arteriosus (15%), 10 transpositions of the great arteries
(11%), and 18 other diagnoses (19%).
Results:
Implanted graft sizes ranged from 12 to 22 mm, with a
predominance of 12-mm (9%), 14-mm (27%) and 16-mm (38%)
grafts. There were thrree mortalities, all postoperatively in hospital,
and none related to the graft. Freedom of re-intervention was 67%
for graft sizes 12–14 mm and 93% for graft sizes 16 mm or larger.
Post-implantation RV pressure could not predict future risk of steno-
sis development, 39/4 vs 36/2 mmHg,
p
>
0.05, stenosis vs normal,
respectively.
Conclusion:
Compared to previous reports, our study group was
made up of younger patients and a higher proportion of smaller
grafts. Nevertheless, our data support previous studies that have
shown that smaller graft size, 12 and 14 mm, is the main independent
risk factor for re-intervention. The Contegra grafts continue to be a
reliable and readily accessible alternative to homografts.
1364: THE ROLE OF REFLUX OESOPHAGITIS IN PRECIPI-
TATING ISCHAEMIC SYMPTOMS
Datshanna Naidoo, Sunil George
University of KwaZulu Natal, Durban, South Africa
Background:
A link has been postulated between ischaemic heart
disease (IHD) and gastric-oesophageal reflux disease (GORD). To
what extent GORD may precipitate angina is not clear. In 1985, Davies
suggested that reflux may precipitate angina events. We designed a
study to investigate the role of GORD in precipitating angina.
Hypothesis
: It is possible that reflux symptoms may precipitate
angina events. The objectives were to determine whether the pres-
ence of GORD may serve as a trigger for symptoms of ischaemia in
subjects with coronary artery disease (CAD) and to document the
electrocardiographic (ECG) changes that occur during reflux.
Methods:
Patients with endoscopically confirmed GORD, as well
as recent MI admitted to CCU, constituted the study group. Patients
with GORD underwent endoscopy and acid installation to determine
whether GORD could induce ischaemia. Hydrochloric acid (0.1 N)
was instilled during endoscopy and the ECG simultaneously record-
ed. Ischaemia was detected by ST changes during ECG monitoring.
Nuclear imaging with methoxyisobutylisonitrile (MIBI) scanning
was performed to look for objective evidence of ischaemia. Twenty
