CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
39
Background:
NeAo valve regurgitation (NAVR) and neoAo root
dilatation (NARD), albeit infrequent, are a matter of concern in late
complications of the Jatene operation as they may progress over time
and require surgical intervention. The switchback Ross operation
(SBRO) was introduced by Hazekamp
et
al.
in 1997 as an innovative
technique to address these conditions when neoAo valve preservation
is deemed unfeasible. Although the technique looked compelling as
it brings the anatomical Ao root (pulmonary autograft) back to the
LVOT, the single case published by the authors developed postop-
erative Ao valve dysfunction that required Ao valve replacement.
This led to generalised skepticism towards the SBRO. We however
decided to apply the SBRO and herein report the first two cases
worldwide, with good medium to long-term results.
Methods:
Case 1: A child with TGA + LVOT stenosis was submitted
to the Jatene operation + LVOT obstruction resection at age eight
years. Moderate NAVR was noticed postoperatively. In 2002, at age
16 years, he became symptomatic on moderate physical exertion.
Significant NARD + severe NAVR + gross LV dilatation were diag-
nosed, and an SBRO was performed.
Case 2, on day 35 of life, had the Jatene operation and transpul-
monary VSD closure for a Taussig-Bing heart. In 2007, at age five
years, he was diagnosed with severe NAVR + sinus of Valvalsa aneu-
rysm + subneoAo valve obstructive membrane + tight pulmonary
anastomotic line stenosis. The SBRO + subneoAo membrane resec-
tion were carried out.
Results:
Bypass and aortic cross-clamp times were, respectively,
300 and 202 min, and 235 and 164 min. Both patients are presently
asymptomatic, with normal-sized left heart structures and only trace
aortic valve insufficiency.
Conclusion:
The SBRO, although challenging, needs renewed
consideration as a possible definitive solution for NAVR and/or
NARD after the Jatene operation.
1023: INTRA-UTERINE EXPOSURETO MATERNAL DIABE-
TES IS ASSOCIATED WITH INCREASED AORTIC STIFF-
NESS IN EARLY INFANCY
Halima AL-Hashemi
1
, Timothy Colen
1
, Akiko Hirose
1
, Venu Jain
2
,
Winnie Savard
1
, Sandra Davidge
2
, Michael Stickland
2
, Lisa.K
Hornberger
1,2
1
Division of Paediatric Cardiology,Department of Paediatrics,
University of Alberta, Canada
2
Department of Obstetric and Gynecology, University of Alberta,
Canada
Background:
Infants of mothers with diabetes (IDM), the most
common complication of pregnancy, have an increased risk of adult
cardiovascular disease (CVD). Although the aetiology and timing of
onset of the cardiovascular changes remain unclear, recent studies
have shown that IDM have increased aortic intimal–medial thickness
in early infancy, which may be an early feature of CVD. Experimental
models also suggest the intra-uterine diabetic environment structur-
ally and functionally alters the aorta of affected offspring. We sought
to determine if there was increased aortic stiffness, a feature of CVD
in adults with diabetes, in IDM.
Methods
: Diabetic pregnancies were recruited prospectively to
examine the role of diabetes in foetal cardiovascular programming.
For this aspect of the study, their infants were evaluated at three to
six weeks by echocardiography for assessment of aortic stiffness, and
the findings were compared to those of healthy infants from uncom-
plicated pregnancies. The pulse-wave velocity (PWV) was calculated
as [D/(T
2
–T
1
)]; where D was the distance of blood flow through the
arch; T
1
, the time measured from QRS to onset of ascending and T
2
,
onset of descending aortic systolic flow.
Results:
Twenty-five maternal–infant pairs were assessed, including
seven IDMs and 18 controls. No statistical difference was observed
in age at examination, BSA and systolic blood pressure between
IDMs and controls. Haemoglobin A
1C
(HbA
1c
) of the diabetic moth-
ers during pregnancy ranged from six to 10.3
μ
g/dl (mean 7.1
±
1.2).
Aortic PWV were significantly higher among IDMs compared to
controls (mean 5.6
±
1.5 vs 3.7
±
1.2 m/s respectively,
p
=
0.008).
IDM PWV in this small cohort tended to correlate positively with
maternal HbA
1c
(
r
=
0.59,
p
=
0.068).
Conclusion
: IDM have evidence of increased aortic stiffness in early
infancy, which may relate to maternal glycaemic control. Whether
the aortic stiffness persists later in life and contributes to adult CVD
is not clear.
1027: CARDIOLOGY
IN SILICO
: USE OF AN ELECTRONIC
MEDICAL RECORD AND ECG DATABASE IN AN INTE-
GRATED HEALTHCARE SYSTEM TO IDENTIFY INDIVID-
UALS WITH UNDIAGNOSED NOONAN SYNDROME
Mark Nunes
1
, Shannon Kwok
2
, Richard Wilson
2
, R Ed Barber
2
, Denis
Levy
3
1
Department of Genetics, Southern California Permanente Medical
Group, USA
2
Data Consulting Services, Kaiser Permanente, San Diego, USA
3
Department of Pediatric Cardiology, Southern California Permanente
Medical Group, USA
Background:
Noonan syndrome (NS) is autosomal dominant, char-
acterised by short stature, dysmorphology, pulmonic stenosis (PS)
and hypertrophic cardiomyopathy (HCM). These and other features
can be coded in an electronic medical record (EMR) using ICD-9.
Left-axis deviation (LAD) on ECG, found in 1% of healthy children,
is found in 50% of those with NS (independent of structural heart
disease).
Aim:
To use an EMR and ECG database to identify previously undi-
agnosed NS.
Methods:
An EMR serving 954 650 individuals
<
19 years old iden-
tified 73 patients with NS (ICD-9 759.89), prevalence 7.6/100 000.
Of these 73, 60 (82%) had an ECG, and 32 (53%) revealed LAD.
Then, to identify previously undiagnosed NS, we screened the EMR
for a combination of commonly coded NS features (short stature, PS,
etc.). Using the ECG database, we identified those with ECG LAD
(–30° to –90°). Lists were cross referenced. Of the 73 individuals
with known NS, seven (9.6%) would have been identified by the
outlined
in
silico
process.
Results:
Two or more NS features and LAD on ECG were identi-
fied in 65 individuals (study group). These EMRs were reviewed by
both a cardiologist and geneticist to identify those with additional
documented features (e.g. hearing loss, ptosis) warranting further
evaluation for NS. Of these, four carried a diagnosis of NS, one
cardiofaciocutaneous syndrome (CFC), and one Kabuki syndrome.
Of the remaining 59, 18 were felt to warrant further clinical evalua-
tion. Of these 18, eight thus far have sufficient documented features
to establish a clinical diagnosis of NS.
Conclusions:
Using an EMR and ECG database, undiagnosed
patients with NS can be identified. This is the first demonstration of
an integrated healthcare system’s EMR being used to identify previ-
ously undiagnosed rare genetic disorders. ECG may be of utility in
evaluating individuals with short stature and other NS-associated
clinical features.
1044: SURGICAL REPAIR OF VENTRICULAR SEPTAL
DEFECTS IN CHILDREN WITH ELEVATED PULMONARY
VASCULAR RESISTANCE: THE DOUBLE-PATCH TECH-
NIQUE
William Novick
1,2
, Frank Molloy
2
, Vasily Lazorryhshynets
3
, Kamal
Saleem
4
, Nestor Sandoval
5
, Christian Gilbert
2
, Thomas Di Sessa
2
1
University of Tennessee Health Sciences Centre, USA
2
International Children’s Heart Foundation, USA
3
Amosov Centre for Cardiac Surgery, Ukrain
4
Armed Forces Institute of Cardiology, Pakistan
5
Fundacion Cardio-Infantile, Columbia
Background:
In developing countries, children with ventricular
septal defects frequently have a delay in their treatment. The result of
