CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
211
phy provided a median of 96 projections (78–105) and the following
3D reconstruction was successful in all studies. Both allowed for
precise visualisation of the external tunnel, superior vena cava and
pulmonary arteries. Overall quality of 3DRA images was graded as
good in 32 (78%) studies and satisfactory in nine (22%). None of the
3DRAs was graded as poor, however eight additional angiographies
(three 3DRA, five fixed-plane) were performed to better visualise the
left pulmonary artery.
Conclusions:
In patients after TCPC, 3DRA provided a large number
of projections with relatively small amounts of contrast and allowed
for perfect visualisation of the Fontan circuit.
1083: ASSESSMENT OF TRICUSPID ANNULAR-PLANE
SYSTOLIC EXCURSION IN PATIENTS WITH HYPOPLAS-
TIC LEFT HEART SYNDROME IN FONTAN CIRCULATION
Eileen Pardun
1
, Dominik Gabbert
1
, Michael Jerosch-Herold
2
,
Christopher Hart
1
, Inga Voges
1
, Miriam Michel
1
, Ana Andrade
1
, Minh
Pham
1
, Hans-Heiner Kramer
1
, Carsten Rickers
1
1
Department of Congential Heart Disease and Paediatric Cardiology,
Kiel, Germany
2
Brigham and Women’s Hospital, Boston, USA
Background:
The tricuspid annular plane systolic excursion (TAPSE)
is an established echocardiographic parameter to assess right ventricu-
lar (RV) systolic long-axis function in biventricular hearts. The aim of
this study was to investigate long- and short-axis function in patients
with
hypoplastic left heart syndrome
(
HLHS) utilising MRI.
Methods:
40 children (5.8
±
2.5 years) with HLHS in Fontan circula-
tion and 10 healthy controls (10.6
±
5.2 years) underwent a compre-
hensive cardiac MRI study to evaluate global and regional RV func-
tion. We used CMR cine-imaging (TR/TE/
α
=
1.1/1.6/60, FOV: 240
×
260) for analysis of global (EF, and cardiac index: CI) and regional
ventricular function. TAPSE was analysed with an in-house developed
software that allowed 3D reconstruction of ventricular geometry.
Results:
HLHS patients had a lower TAPSE and cardiac index
compared with healthy subjects. In contrast with healthy subjects,
TAPSE and RV-EF did not show significant correlation in HLHS
patients with a rudimentary LV. In HLHS patients without a visible
LV cavum we found a significant correlation between RV-EF and
TAPSE (
r
=
0.62;
p
=
0,01). Furthermore, HLHS patients with a
rudimentary LV had a significantly reduced septal wall motion and a
cardiac index compared with HLHS patients without a rudimentary
LV (4.6
±
1.5 vs 6.9
±
2.1 mm;
p
=
0.001; 3.4
±
0.8 vs 2.8
±
0.9 ml/
m
2
/min;
p
=
0,01).
Conclusion:
Patients with HLHS had impaired long-axis RV func-
tion compared to healthy controls. A rudimentary LV impaired in
particular, contraction in the septal segment, resulting in a reduced
long-axis and global RV function. This may be of prognostic signifi-
cance for the long-term outcome in HLHS patients.
1090: CAN MINOR CONGENITAL MITRAL VALVE VARI-
ANTS BE DIFFERENTIATED FROM RHEUMATIC MITRAL
VALVE DISEASE?
Nigel Wilson, Tom Gentles, Paul Tan, Anthony Concannon, John
Stirling
Starship Children’s Hospital, Auckland, New Zealand
Background:
The echocardiographic features of congenital mitral
valve prolapse and severe mitral rheumatic heart disease (RHD) are
well established. Mild forms of these entities are harder to differenti-
ate. A frequent clinical scenario in regions with high prevalence RHD
is whether mild mitral regurgitation is of congenital or rheumatic
aetiology.
Methods:
Fifteen echocardiograms with mild mitral regurgitation
(MR) were reported by two experienced observers and two trainee
cardiologists without knowledge of the patient demographics. The
aetiology of the MR was judged congenital if there was classical
mitral valve bi-leaflet prolapse, accessory mitral valve leaflet or
scallop, diastolic excessive movement, or mitral valve cleft. Five
echocardiograms had minor congenital mitral valve features previ-
ously defined by a panel of three cardiologists.
Results:
The range of congenital mitral anomalies diagnosed was
one to four of the five positive studies (kappa 0.25–0.46).
Conclusions:
Minor congenital mitral valve variations may cause
mitral valve regurgitation but they are not easily differentiated from
MR due to RHD. Minor congenital mitral valve anomalies can cause
a false-positive diagnosis of RHD.
1102: LONG-QT MOLECULARAUTOPSY: RESULTS OF SIX
YEARS OF A POPULATION-BASED CLINICAL SERVICE
FOR AUTOPSY-NEGATIVE SUDDEN DEATH IN 1–24 YEAR
OLDS
Jonathan Skinner
1,2
, Jackie Crawford
1,2
, Warren Smith
1
, Paul
Morrow
1,3
, Ian Hayes
1,4
, Donald Love
1,3
1
Cardiac Inherited Disease Group, New Zealand
2
Starship Children’s Hospital, Auckland, New Zealand
3
Lab Plus, Auckland District Health Board, New Zealand
4
National Clinical Genetic Service, New Zealand
Background:
Families and forensic pathologists seek a diagnosis
with urgency following the tragedy of a sudden, unexpected and
unexplained death in children and youth. We report the results from
six years of a national molecular autopsy service in New Zealand
(population 4.3 million) focusing on this age group.
Methods:
Following a national protocol, pathologists save DNA at
all sudden unexpected natural deaths in young people, and refer for
cardiac genetic opinion and molecular autopsy when the standard
autopsy and ancillary tests are uninformative.
Results:
BetweenApril 2006 and February 2012, DNA was preserved
on 132 1–24 year olds who had died suddenly and unexpectedly
without an overt cause at initial autopsy. In 67 cases, ancillary tests
(histology, toxicology, microbiology, etc) revealed a cause of death
(mostly infection and poisoning). The remaining 65 DNA samples
underwent genetic sequencing of six genes linked to long-QT
syndrome or Brugada syndrome; 39/65 (68%) were of European
ancestry and 17/65 (26%) Maori. Rare variants were identified in 13
of 65 cases (20%), seven in SCN5A, two in KCNQ1 and one each
in KCNH2, KCNE1, KCNE2 and KCNJ2. Nine of these 13 deaths
(69%) were nocturnal. Uncertainty remains over the pathogenicity
of five of these variants. Familial evidence of cardiac ion channel
disease on cardiological testing is present in five families thus far.
Conclusions:
Sudden unexpected natural death in 1–24 year olds
occurs with a minimal incidence of five per million general popu-
lation per year, half of which remain unexplained after standard
autopsy. Abnormalities in genes linked to long-QT and Brugada
syndromes occur in 20% of these, with SCN5A being the commonest
involved, and with most deaths occurring during sleep. Interpretation
of the molecular genetic results remains a significant challenge and
involves a multidisciplinary approach, including engaging with the
family early in the process.
1103: DETECTION OF CORONARY ARTERY LESIONS
LATE AFTER THE ARTERIAL SWITCH OPERATION FOR
TRANSPOSITION OF THE GREAT ARTERIES IN CHIL-
DREN AND ADOLESCENTS: USE OF LOW-DOSE MULTI-
DETECTOR COMPUTED TOMOGRAPHY (MDCT)
Hopewell Ntsinjana
1,2
, Oliver Tann
1,2
, Graham Derrick
2
, Catherine
Owens
2
, Silvia Schievano
1,2
, Andrew Taylor
1,2
1
UCL Institute of Cardiovascular Science, Centre for Cardiovascular
Imaging, London, UK
2
Great Ormond Street Hospital for Children, London, (UK
Background:
One of the most catastrophic late sequelae of the arte-
rial switch operation (ASO) for transposition of the great arteries
(TGA) is the development of coronary artery stenosis in approxi-
mately 7.2% of cases. The ‘gold-standard’ imaging modality to detect
