CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
169
Purpose:
To evaluate cardiac magnetic resonance imaging (MRI)
findings of familial arrhythmogenic right ventricular dysplasia
(ARVD) in a paediatric population and to correlate MRI data with
other 2010 ARVD task force criteria.
Methods:
This wasw a retrospective study (January 2001 to June
2012) of 70 MRIs in 36 children (one month to 20 years old, mean
10.7 years) from 22 families with proven first-degree relatives with
ARVD. Mean number of MRIs by patient was 1.9; 21 patients had
two or more MRIs for a mean follow up of 2.3 years.
Results:
MRI examinations were normal in 50, non-diagnostic
in two and abnormal in 18 (11 patients). Anomalies consisted of
abnormal cardiac contractility: dyskinesia (
n
=
5), akinesia (
n
=
7)
and hypokinesia (
n
=
6), with only one patient with RV dilatation.
Abnormal contractility was noted in the RV free wall: apex (
n
=
18),
inlet (
n
=
2), outlet (
n
=
0). There was no RV fatty infiltration and
no LV anomaly. Mean age of the positive cases was 13.6 years. Only
three patients were symptomatic (cardiac arrest, syncope, ventricu-
lar tachycardia). Of the other 2010 ARVD task force criteria, two
patients also had epsilon waves in the right precordial leads on ECG.
Six patients underwent implantable defibrillators, with no appropri-
ate shocks, and one with inappropriate shocks.
Conclusion:
According to the 2010 ARVD task force, isolated RV
wall contractility abnormalities, in addition to familial context, lead
to a diagnosis of ARVD. In our small series, contractility abnormali-
ties preceded global dilatation and alteration of function. Since most
positive MRIs were found in adolescents, screening seems optimal at
that age. Larger cohorts are needed to confirm our results.
Clinical application:
Regional RV free wall dyskinesia/akinesia
adjacent to the apex is the first manifestation of familial ARVD and
becomes conspicuous in adolescence.
478: PREVALENCE OF PULMONARY HYPERTENSION
IN CHILDREN WITH ADENOID OR ADENOTONSILLAR
HYPERTROPHY AT THE KENYATTA NATIONAL HOSPI-
TAL
Diana Marangu
1,2
, Christine Jowi
1,2
, Joyce Aswani
1,2
, Sidika
Wambani
1,2
, Ruth Nduati
1,2
, Florence Murila
1,2
1
University of Nairobi, Kenya
2
Kenyatta National Hospital (KNH), Kenya
Background:
Adenotonsillar hypertrophy is a common condition in
childhood, whose serious complications of pulmonary hypertension
and cor-pulmonale are common and devastating but local prevalence
is unknown.
Methods:
This was a cross-sectional descriptive study in children
aged 0–12 years attending ENT clinics and general paediatric wards
at KNH, with clinician-diagnosed adenoid hypertrophy confirmed
on lateral neck radiography. Eligible patients were consecutively
recruited into the study between September and November 2011. The
patients were evaluated for symptoms, physical findings (adenoid,
tonsil and airway sizes on lateral neck radiography) and Doppler
echocardiographic assessment of systolic pulmonary artery pressure
(sPAP). Pulmonary hypertension was defined as mean pulmonary
arterial pressure of
≥
25 mmHg estimated by the Chemla equation
(0.61 sPAP + 2 mmHg).
Results:
The prevalence of pulmonary hypertension in children with
adenoid or adenotonsillar hypertrophy at KNH was 21.1% (95% CI:
14.3–29.4%). Independent factors associated with pulmonary hyper-
tension included daily hyperactivity (OR
=
0.22, 95% CI: 0.06–0.87,
p
=
0.03), oxygen saturation (OR
=
0.72, 95% CI: 0.54–0.97, p
=
0.03) and palpable P2 (OR
=
9.84, 95% CI: 3.2–55.4,
p
=
0.01).
Daily mouth breathing singly or in combination with restless sleep on
history showed the highest sensitivity (88.5%) and negative predic-
tive value (86.4%) for pulmonary hypertension in these children.
Conclusion:
Clinical screening and echocardiography evaluation is
vital in children with adenoid or adenotonsillar hypertrophy for early
identification of pulmonary hypertension.
479: DOCUMENTED CORONARY ARTERY DILATATION
DURING ACUTE VIRAL MYOCARDITIS
Anne Fournier, Ibtissama Boukas, Johanne Thørien, Daniel
Cartwright, Nagib Dahdah
Division of Paediatric Cardiology, Ste-Justine Hospital, Montreal,
Quebec, Canada
Introduction:
Detecting coronary artery (CA) dilatation is essen-
tial in the diagnosis and follow up of Kawasaki disease (KD).
Myocarditis however, is almost always present in acute KD.
Purpose:
To investigate whether myocarditis causes CA dilatation,
we sought to use viral myocarditis as a clinical model.
Methods:
A retrospective series of children with acute viral myocar-
ditis were reviewed to confirm the diagnosis. CA diameters of the
proximal right and the left CA were measured at onset and during
the first two years of follow up. CA
z
-score was calculated based on
our published equations. CA dilatation was defined as a
z
-score
>
2.5. Occult CA dilatation was defined as a
z
-score variation of
>
two
standard deviation points along the follow up in those with
z
-score
always
<
2.5. All other cases were labelled without CA involvement.
Results:
There were 11 girls and three boys between 2000 and
2006 who met the selection criteria. KD was not in the differential
diagnosis of any case. Age was 1.67
±
3.22 years at diagnosis (range
0.02–9.45 years), with a follow-up duration of 16.2
±
16.4 months.
Microbial laboratory tests/cultures confirmed the diagnosis in 11
patients (78.5%), whereas the history of familial/personal acute
infectious illness was present in the remaining. Cardiac enzymes
were elevated in nine patients, normal in two and not available in
three. CA involvement was detectible in 9/14 (64.3%) cases; dilata-
tion in three (21%) and occult dilatation in six (42.9%). Peak CA
z
-score was at the onset of the disease in 7/9. Maximum CA
z
-score
was 1.56
±
0.8 vs 0.42
±
0.9 for cases with or without CA involve-
ment respectively;
p
=
0.036.
Conclusion:
CA dilatation is not uncommon in acute myocarditis.
Our findings represent a potential challenge to the diagnostic signifi-
cance of the clinical criteria of KD especially when ‘supported’ by
the finding of a dilated CA.
480: NATRIURETIC PEPTIDE RELEASE IN ACUTE KAWA-
SAKI DISEASE PREDICTS GAMMAGLOBULINS RESIST-
ANCE AND CORONARY ARTERY INVOLVEMENT
Anne Fournier
1
, Nagib Dahdah
1
, Linda Spigelblat
2
, Jocelyne
Cousineau
3
, Edgar Delvin
3
1
Division of Paediatric Cardiology, Ste-Justine Hospital, Montreal,
Quebec, Canada
2
Paediatric Department, Maisonneuve-Rosemont Hospital, Montreal,
Quebec
3
Biochemistry Department, Ste-Justine Hospital, Montreal, Quebec
Background
: We recently reported on the diagnostic values of
B-type natriuretic peptide (NT-proBNP) in the diagnosing Kawasaki
disease (KD), with a diagnostic odds ratio of 26.7:1 (95% CI:
8.6–82.5) compared to febrile control children. There are also inde-
pendent reports indicating that hyponatraemia is associated with
severe KD and suggesting an inappropriate secretion of the antidiu-
retic hormone (ISADH).
Objectives
: We hypothesised that increased serum NT-proBNP
correlates with the severity of KD in terms of resistance to gamma-
globulins (IVIG) and risk of coronary artery (CA) involvement.
Methods
: Serum NT-proBNP concentrations were measured upon
suspicion of KD (
n
=
74; 3.79
±
2.92 years; diagnosed at 6.58
±
2.82
days of fever), and correlated with serum Na and urine specific grav-
ity. Data related to cardiac involvement were also analysed.
Results
: NT-proBNP level was inversely proportional to serum
sodium concentration (
r
=
0.39,
p
<
0.001), whereas urine specific
gravity correlated proportionally with lower serum Na concentration
(
r
=
0.18,
p
=
0.12). Since ISADH would increase urine specific
gravity and lower serum Na concentrations, our observation refutes
the previously suggested ISADH theory. IVIG resistance was higher
