CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
196
AFRICA
876: ECHOCARDIOGRAPHIC PATTERN AND SEVERITY
OF VALVE ABNORMALITIES IN CHILDREN WITH RHEU-
MATIC HEART DISEASE SEENAT UGANDA HEART INSTI-
TUTE, MULAGO HOSPITAL
Sulaiman Lubega
1
, Twalib Aliku
1,2
, Peter Lwabi
1
1
Uganda Heart Institute, Mulago Hospital, Uganda
2
Department of Paediatrics, Gulu University, Uganda
Background:
Rheumatic heart disease (RHD) is the commonest
acquired heart disease in children worldwide but in Uganda, data
are scarce regarding its morbidity and mortality. The disease has a
progressive course and patients will usually require valve surgery.
Objectives:
To describe the frequency of the different heart valves
affected, and the relationship of the disease severity with age and
gender.
Methods:
This was a retrospective descriptive study done at the
Uganda Heart Institute. Echo reports of children 15 years and below
with a diagnosis of RHD, done from January 2007 to December
2011, were retrieved from the data base and analysed.
Results:
A total of 376 children had RHD. The mean age (
±
SD) of
the children was 11.0
±
2.7 years (range 4–15 years) and 216 (57.5%)
were females. Severe mitral regurgitation (MR) was the commonest
lesion seen in 277 (73.7%) of the children, 28 (7.4%) had severe
aortic regurgitation (AR), 22 (5.9%) had severe mitral stenosis (MS),
32 (8.5%) had severe tricuspid regurgitation (TR), while only one
(0.3%) had severe aortic stenosis. Twenty-seven of the 28 children
with severe AR had concomitant severe MR. Severe MR was found
to be significantly more prevalent in females (
p
=
0.04), while severe
AR was significantly more common in males (
p
=
0.007). There was
no difference in disease severity between children below 10 years and
those 10 years and above.
Conclusions:
The commonest severe valvular abnormality was MR
(74%). Girls were more likely to have severe MR whereas boys were
more likely to have severe AR.
877: THE EFFECT OF BETA-BLOCKER THERAPY ON
LEFT VENTRICULAR VOLUME IN PAEDIATRIC FAMIL-
IAL HYPERTROPHIC CARDIOMYOPATHY
Pari Allahyari, Ingegerd Østman-Smith
Division of Paediatric Cardiology, Queen Silvia Children’s Hospital,
Gothenburg, Sweden
Background
: We have previously shown that paediatric patients
with hypertrophic cardiomyopathy (HCM) had significantly reduced
left ventricular volumes compared with age- and gender-matched
normals. We aimed to study how cardiac end-diastolic volumes and
cardiac output at rest were affected by high-dose beta-blocker therapy
in children and adolescents with HCM.
Methods:
The study included 14 patients (two females), mean age 9.2
(range 1.8–17.7) years, with familial HCM and moderate-to-severe
hypertrophy. They were paired in seven untreated and treated pairs
(treatment: metoprolol or propranolol 5–20 mg/kg) according to age,
gender and severity of hypertrophy, using septum-to-cavity (sepcavr),
and left ventricular wall-to-cavity ratios (lvcavr) as age-independent
measures of hypertrophy at diagnosis. The beta-blocker group had
received treatment for at least one year. Ultrasound examinations were
performed with Philips IE33 and analysed with 3-D QLab advanced
software (version 7). We took 3-D four-chamber images and meas-
ured end-diastolic and end-systolic volumes and recorded heart rate.
Measurements were compared with Wilcoxon signed-rank.
Results
: There were no significant differences between the untreated
and treated patients in degree of cardiac hypertrophy before treatment
was commenced, with sepcavr 0.58 (0.30–0.86 and 0.63 (0.31–1.28;
p
=
0.51) and lvacvr 0.22 (0.17–0.68) and 0.27 (0.17–0.31;
p
=
1.0),
respectively. The group receiving beta-blockers had a 30% higher
left ventricular end-diastolic volume/m
2
BSA, untreated: 31.9 (22.3–
43.6) ml/m
2
, beta-blocker group: 41.4 (30.2–55.2;
p
=
0.022), and
stroke volume showed a somewhat smaller increase, 21.9 (14.3–31.4)
versus 27.9 (18.4–39.4), which did not reach significance (
p
=
0.08).
Resting heart rates were not different, 76 (53–95) versus 76 (56–94).
Calculated resting cardiac output was non-significantly 32% higher
in the beta-blocker group, 2.13 (1.41–3.31) versus 1.61 (1.13–2.01)
l/m
2
BSA for the untreated group (
p
=
0.07).
Conclusions:
Beta-blocker therapy did not reduce cardiac output at
rest, and the trend for improvement suggests that the increase in rest-
ing left ventricular volumes was beneficial.
878: OUTCOMES OF PATIENTS WITH TETRALOGY OF
FALLOTWITHABSENT PULMONARYVALVE SYNDROME:
37 YEARS OF EXPERIENCE
Deane Yim
1
, Matthew S. Yong
1,2,
Christian P. Brizard
1,2
, Yves
D’Udekem
1,2
, Andrew Bullock
3
, Terry Robertson
4
, Igor E
Konstantinov
1,2
1
Department of Cardiology, Royal Children’s Hospital, Melbourne,
Australia
2
University of Melbourne, Australia
3
Children’s Cardiac Centre, Princess Margaret Hospital for Children,
Perth, Australia
4
Department of Cardiology, Royal Women’ and Children’s Hospital,
Adelaide, Australia
Background:
Absent pulmonary valve syndrome (APVS) is associ-
ated with varying degrees of aneurysmal dilatation of the pulmonary
arteries and compression of the tracheobronchial tree, and may lead
to significant respiratory compromise. We describe the outcomes of
patients with APVS who underwent surgery in our unit.
Methods:
A retrospective review of 51 patients with APVS who
underwent surgical correction between August 1975 and August
2012 was conducted. The median age and weight at repair were
0.9 years (4 days –24.2 years) and 6.9 kg (1.8–56 kg), respectively.
Pre-operative intubation was required in 15 (30%) patients and 21
(41%) required urgent surgery. The pulmonary valve was replaced
with valved conduit (15, 30%) or monocusp valve (16, 31%). No
valve repair was performed in 20 patients (39%). Pulmonary artery
reduction/plication was performed in 38 (75%); two (4%) underwent
a Lecompte manoeuvre.
Results:
Operative mortality was 14% (7/51); 1975–1989, 19%
(3/16); 1990–2000, 19% (4/21), and 2001–2012, 0% (0/14). Late
mortality was 6.8% (3/44); 1975–1989, 15% (2/13); 1990–2000,
0% (0/17); 2001–2012, 7% (1/14). There were more Contegra
valved conduits (5/14, 36%) and less valveless repairs (4/14, 29%)
performed during 2001–2012 compared with earlier eras (1975–
1989, 0%, 50%; 1990–2000, 0%, 38%). Less pulmonary artery
reduction/plication surgery was performed in the latest era (50 vs 88
and 81% in the early and middle eras), with better antenatal diagnosis
(64 vs 6 and 19%, respectively). Overall survival at five, 10 and 20
years was 81.4
±
5.6%. On multivariate analysis, pre-operative venti-
lation (
p
=
0.009), prematurity (
p
=
0.04) and repair using a homo-
graft (
p
=
0.009) were risk factors for overall mortality. Freedom
from late re-operation at five, 10 and 20 years was 79.7
±
6.9%,
69.4
±
8.2% and 52.1
±
9.8%, respectively. No difference was found
between conduit, monocusp or valveless techniques. Risk factors
for late re-operation include prematurity (
p
=
0.001) and neonatal
primary repair (
p
=
0.007). Longer postoperative ventilation periods
were predicted by pre-operative ventilation (
p
<
0.0001) and surgery
during infancy (
p
=
0.008).
Conclusion:
Long-term survival for APVS has improved signifi-
cantly over the last decade. Pre-operative ventilation predicted longer
postoperative ventilation and mortality.
879: COMPREHENSIVE IMAGING TOOLS: AN UNUSUAL
CASE OF RIGHT ISOMERISMWITH COARCTATION
Cornelia Woerner
1
, Bahiyah Alnafisi
2
, Mike Seed
1
, Katherine Taylor
2
,
Shi-Joon Yoo
2
1
Labatt Heart Centre, Hospital for Sick Children, University of
Toronto, Canada
2
Hospital for Sick Children, Canada
