CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
203
Conclusions:
KD epidemiology in WA mirrors that in other indus-
trialised, predominantly Caucasian populations. The rising incidence
reflects both improved ascertainment and a real increase in disease
burden. The current Australian incidence of KD is two- to three-fold
higher than previously reported and comparable to that in the UK and
US. The CA outcomes, which include the pre-IVIG era, are compara-
ble to those reported elsewhere.
958: MIDTERM RESULTS OF MITRAL VALVE REPAIR FOR
RHEUMATIC HEART DISEASE IN CHILDREN
Sivasubramanian Muthukumar
1
, Ranjith Karthekeyan
2
, Ramya Shri
3
,
Jebaraj
3
, Dheeraj Reddy
1
, Thangavelu Periyasamy
1
1
Department of Cardiothoracic Surgery, Sri Ramachandra University,
Chennai, India
2
Department of Cardiac Anesthesia, Sri Ramachandra University,
Chennai, India
3
Department of Cardiology, Sri Ramachandra University, Chennai,
India
Objective:
To analyse the techniques and midterm results of mitral
valve repair in children with rheumatic mitral valve disease.
Methods:
The study population includes 67 patients who underwent
mitral valve repair for rheumatic mitral valve disease in the paediatric
age group of under 18 years in our institution. The group included 36
female and 31 male patients. The age varied from six to 18 years with
a mean age of 14 years; 43 patients had only mitral regurgitation, 18
had mixed lesions, and five had pure mitral stenosis; 71 % of patients
were in NYHA class III or IV.
Discussion:
All patients underwent mitral valve repair under CPB
with aortic and bicaval cannulation, with the core cooled to 28°C.
The approach to the mitral valve included both left atrial and supe-
rior septal. We performed annuloplasty using rings, Teflon strip and
suture only in 63 patients. Neo-chordae was created in 22 patients
using PTFE suture. Chordal transfer was done in 11 patients. Open
mitral valvotomy was done in 13 patients. Intra-operatively, the
valve was checked by saline injection and TEE. Two years follow-up
study was done for those patients. Six patients were lost to follow
up. Postoperative echo revealed severe MR in three patients, moder-
ate MR in nine, mild MR in 31 and trivial MR in 18 patients. Four
patients were re-operated.
Conclusion:
Mitral valve repair in rheumatic patients can be
performed with acceptable mortality and good midterm results.
We believe valve repair should be preferred over valve replacement
for rheumatic patient in the paediatric age group to avoid problems
related to anticoagulation and prosthetic valve function.
975: LONG-TERM SURVIVAL AND FONTAN COMPLETION
RATES AFTER SURGERY FOR UNIVENTRICULAR HEART
DEFECTS
Jan Sunnegårdh, Mats Synnergren, Håkan Berggren
The Queen Silvia Children’s Hospital, Sahlgrenska University
Hospital, Sweden
Background:
To analyse long-term outcome after surgery for
univentricular heart defects.
Methods
: All 320 patients (133 girls, 187 boys) operated due to
univentricular heart defects in our institution before the age of 18
years from 1 January 1994 to 1 January 2010 were included. Patient
files were studied and cross-checked as of 1 January 2012 against
the National Population Registry in Sweden, allowing for reliable and
complete data on survival. Two patients (0.6%) emigrated and were
lost to follow up three and six years after Fontan completion.
Results:
Median age and weight at first surgery was 11 days (0–15.1
years) and 3.6 kg (1.2–30). Median age of survivors at follow up was
10.8 years (2.1–30.7); 87 deaths (27.1%) occurred with a median
age at death of 69 days (3 days – 22.7 years). Median survival time
in deceased patients was 29 days (0–11.3 years) after the last major
surgery. In all 276 patients with their first surgery in 1994–2009,
mortality was 53/124 (42.7%) in patients with classic hypoplastic
left heart syndrome, unbalanced atrial septal defect or critical aortic
stenosis with left ventricular hypoplasia (A), 12/94 (12.7%) in
patients with tricuspid atresia, double-inlet left ventricle or pulmo-
nary atresia with intact septum (B) (
p
<
0.001). Mortality rate in
children with other complex heart defects (C) was 19/68 (27.9%).
In these subgroups the number of patients alive with completed
Fontan circulation was 59/124 (47.6%) (A), 73/94 (77.7%) (B), 38/68
(58.9%) (C). Nineteen patients had a heart transplantation with four
late deaths.
Conclusions:
Overall survival was 72.9%. Patients with a morpho-
logical left ventricle as systemic ventricle had a high survival (87.3%)
and Fontan completion rate (77.8%). The corresponding figures were
less favourable in patients with a systemic right ventricle (57.3 and
47.6%) and in those with other complex univentricular heart defects
(72.1 and 58.9%).
982: ANATOMY OF THE CORONARY ARTERIES AND
AORTIC ARCH AND ITS RELATION TO TRUNCAL VALVE
DYSPLASIA
Gabriel Liguori
1
, Yen Ho
2
, Marcelo Jatene
1
, Vera Aiello
1
1
Heart Institute (InCor), University of Sao Paulo Medical School,
Brazil
2
Royal Brompton Hospital, Imperial College London, UK
Background
: Surgical correction of the common arterial trunk
(CAT) requires knowledge of the particularities of the malformation,
such as the anatomy of the truncal valve, coronary arteries and aortic
arch. The truncal valve may show dysplastic leaflets. Anomalies
of the coronary arteries and of the aortic arch are also described
as frequent findings in CAT. In this study, we sought to check for
possible associations of truncal valve dysplasia with coronary and
aortic arch anatomy.
Methods:
Forty-three heart specimens were analysed with CAT.
Coronary orifices were expressed regarding their number, location
and shape. Anatomy of the aortic arch was described as normal,
interrupted or coarcted, double, right-sided and presenting an aber-
rant subclavian artery. The truncal valve was described regarding the
number of leaflets and, according to its thickness and symmetry, clas-
sified as mildly dysplastic, moderately dysplastic, severely dysplastic
or without dysplasia.
Results:
Thirty-one (72%) specimens presented at least a mild
degree of dysplasia, three (6%) presented an abnormal number of
coronary orifices (two single and one triple ostium) and 17 (40%)
presented at least one type of aortic arch anomaly. Truncal valve
dysplasia was associated with the number of leaflets other than
three (
p
=
0,022). Anomalous shape of the coronary orifices was
predominantly described as slit-like for the right coronary orifices
and as funnelled for the left coronary orifices. Anomalous shape of
the right coronary orifice was associated with both dysplasia and
abnormal number of leaflets (
p
=
0.038 and
p
=
0.025, respectively).
Specimens with abnormal anatomy of the aortic arch also showed a
trend to present abnormal numbers of leaflets (
p
=
0.072).
Conclusion:
Anomalies of the coronary ostia and aortic arch seemed
to be related to the dysplasia of the truncal valve. This correspond-
ence should alert both the echocardiographist and the cardiac surgeon
to the diagnosis and management of associated lesions.
988: AN INFANT WITH MITRAL VALVE ENDOCARDITIS
CAUSED BY SACCHAROMYCES SP: AN UNUSUAL CASE
AND SUCCESSFUL MEDICAL TREATMENT
Adriana Castillo
2
, Ana Erazo
2
, Martha Alvarez-Olmos
1
, Claudia
Stapper
1
, Nestor Sandoval
1
, Alberto Guerra
1
, Karina Tovar
1
1
Fundacion Cardioinfantil, Bogota, Colombia
2
Universidad Del Rosario, Bogota, Colombia
Background:
Invasive fungal infection (IFI) is an emerging compli-
cation in paediatric population. Its incidence has recently increased,
