CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
152
AFRICA
256: CARDIOVASCULAR PROFILE IN CHILDREN WITH
CHRONIC KIDNEY DISEASE
Shakuntala Prabhu
1
, Vaishnavi Iyengar
1
, Sumitra Venkatesh
1
, Snehal
Kulkarni
1
, Uma Ali
2
1
Department of Paediatrics, Division of Paediatric Cardiology,
Mumbai, India
2
Division of Paediatric Nephrology, Mumbai, India
Objective:
To study the cardiovascular profile in children with
chronic kidney diseases (CKD).
Methods:
Clinical data, biochemical profile and staging of 43
children with CKD were correlated with echocardiographic/colour
Doppler profile and large-vessel disease [carotid intimal–medial
thickness (CIMT)].
Results:
Male:female ratio was 1:4 with mean age eight years (4.5
months to 16 years) (
n
=
43). The mean GFR was 23.22 ml/1.73m
2
/
min. Malnutrition was universal in all stages, with reflux nephropa-
thy as the commonest aetiology. Nine/41 patients with LVH (LV
mass index
>
38 g/m
2.7)
and eight/39 patients with high CIMT were
symptomatic; 68% had LV dilatation with hypertrophy. The mean
EF in early CKD (stage II and III) was 54.3
±
4.46% vs 63.7
±
1.9%
seen in advanced CKD (stage IV and V) (
p
=
0.03). Patients with
ESRD had higher mean CIMT values (0.14 cm,
p
=
0.03) than other
stages; 57% of patients with uncontrolled systolic hypertension and
43% with uncontrolled diastolic hypertension had LV dysfunction (
p
=
0.04). Significantly higher CIMT was noted in those with uncon-
trolled systolic and diastolic hypertension (0.15 cm), which persisted
even after control. Children with LVH had significantly higher mean
CIMT (0.124
±
0.006 cm) than those without (
p
<
0.0001). Vitamin
D intake was associated with better LV function (
p
=
0.03).
Conclusion
: Cardiovascular disease was mainly subclinical and
noted in all stages of CKD, with large-vessel disease worsening
exponentially with ESRD. Eccentric LV hypertrophy with hyperten-
sion was an important risk factor contributing to LV dysfunction
and persistent large-vessel disease. Vitamin D therapy had a positive
impact on global LV function.
261: SENNING OPERATION FOR CORRECTION OFTRANS-
POSITION OF THE GREAT ARTERIES: IS THE SURGI-
CAL TECHNIQUE ADOPTED RESPONSIBLE FOR A LONG
PATIENT SURVIVAL?
Miguel Angel Maluf
Cardiovascular Division, São Paulo Federal University, Brazil
Objective:
Long-term results after the Senning operation for trans-
position of the great arteries are not known. Sinus node dysfunction
and systemic ventricular dysfunction are crucial in patient survival.
We evaluated the results, long-term outcome and quality of life in a
group of 39 patients.
Methods:
The study was a retrospective analysis of 39 (39/40
=
97.5%) surviving surgical patients submitted to Senning operation,
with a mean follow-up time of 14.7
±
3.1 years. Electrocardiogram,
echocardiogram and chest radiograph series were performed every
six months. Thirty-six patients of this series underwent Holter study
and ergometric tests to evaluate the physical capacity. Three patients
living overseas were excluded.
Results:
There was only one late death (1/39) (late mortality
=
2.5%): a 16-year-old patient had an accidental death. The actuarial
survival was 95.0% (38/40) (simple or with little VSD, TGA). The
probability of staying in sinus rhythm in the 39 surviving patients
was 77.1% (30) or normal right ventricular function was 76.5% (29),
10 to 20 years after operation. The incidence of sinus node and right
ventricular dysfunction increased gradually over time. No re-opera-
tion and pacemaker implantations were performed. Functional class
I
=
30 (85.7%) cases and functional class II
=
five (14.3%) cases.
Conclusions
: Patients with simple TGA submitted for the Senning
procedure, in our experience, presented during late follow up with:
(1) low incidence of right ventricular dysfunction and active arrhyth-
mias, (2) low mortality and no sudden death recorded, (3) good qual-
ity of life, and (4) satisfactory surgical results (free of re-operation or
definitive pacemaker implantation).
263: REGRESSION OF AORTIC DILATION IN PATIENTS
WITH HEART BLOCK AFTER PACEMAKER IMPLANTA-
TION
Anne Fournier, Gabriel Altit, Johanne Thørien, Nagib Dahdah
Paediatric Cardiology Division, Ste-Justine Hospital, Montreal,
Quebec, Canada
Introduction:
The appearance of ascending aortic dilation in patients
with structurally normal hearts and congenital heart block (CHB)
has been recently described, but the aetiology and management have
yet to be determined. We hypothesised that aortic dilation occurs in
children with CHB and that it will regress following the implantation
of a permanent pacemaker. We also attempted to identify an associa-
tion between the presence of maternal antibodies and the degree of
aortic dilation.
Methods:
We carried out a retrospective review of charts and
echocardiograms of patients with CHB, followed at our institution
over the past 27 years. Comparison of the diameter of the target
structures was based on
z
-scores derived from normal populations
and dilation was defined as
z
-score
≥
2.
Results:
Seventeen patients met the inclusion criteria in our prelimi-
nary review. Mean age at diagnosis was 5.2 months and 53% were
males. Positive maternal antibodies were found in five cases, two
were negative and 10 were unknown. All the patients underwent
pacemaker implantation (mean age of 17 months). Aortic dilatation
was found in seven (41%) patients. In those patients, there was a
trend towards reduction of the
z
-score when comparing ascend-
ing aortic diameters before (
z-
score
=
5.10) and after implantation
(
z-
score
=
2.89,
p
=
0.01). Also, four patients among the seven had
positive maternal antibodies (the other three had unknown serologi-
cal status).
Conclusion:
Although these are preliminary data, ascending aortic
dilatation seems to be present in a large proportion of patients with
congenital heart block. This associated aortic dilatation seemed to
regress after implantation of a pacemaker. Mechanisms involved
in this phenomenon might be related to the regularisation of stroke
volume. Positive serological status in a large proportion of affected
patients might indicate susceptibility secondary to possible
in utero
inflammation of aortic wall tissues. Further studies are needed.
265: DECREASED MORTALITY AND PRESERVATION OF
LEFT VENTRICULAR FUNCTION IN DUCHENNE MUSCU-
LAR DYSTROPHY TREATEDWITH STEROIDS
Anne Fournier
1
, Gernot Schram
2
, Hugues Leduc
2
, Nagib Dahdah
1
,
Johanne Thørien
1
, Paul Khairy
2
1
Division of Paediatric Cardiology, Ste-Justine Hospital, Montreal,
Quebec, Canada
2
Department of Cardiology, Montreal Heart Institute, Montreal,
Quebec, Canada
Background:
Duchenne muscular dystrophy is a debilitating X-linked
recessive disease that afflicts one in 3 500 boys. Cardiomyopathy is a
major source of morbidity and mortality. While steroid therapy slows
musculoskeletal impairment, the effects on cardiac function and
mortality remain to be determined.
Methods and Results:
We conducted a retrospective cohort study on
86 patients with Duchenne muscular dystrophy, age 9.1
±
3.5 years,
all of whom received antagonists of the renin–angiotensin–aldoster-
one system. Over a follow up of 11.3
±
4.1 years, seven of 63 (11%)
patients on steroids died, in comparison to 10 of 23 (43%) without
steroids (
p
=
0.0010). Survival rates at five, 10, and 15 years of
follow up were 100, 98 and 79% for patients with steroids versus 100,
72, and 28% for patients without steroids (log rank
p
=
0.0005). In
multivariate analyses, steroid use was associated with an 85% lower
mortality rate (hazard ratio 0.15, 95% confidence interval 0.04–0.56,
