CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
221
Christopher Knott-Craig, Steven Goldberg, TK Susheel Kumar, Jean
Ballweg, Thomas Chin
University of Tennessee, Le Bonheur Children’s Hospital, USA
Background
: Ebstein’s anomaly (EA) presenting in the severely
symptomatic neonate remains a complex entity with high mortality
rates. There is no accepted standard approach to surgical repair, and
opinions vary between complete biventricular repair and single-
ventricle (Fontan) pathways. More than half of neonates with EA
have associated anatomical pulmonary atresia (PA). The outcome in
this subset is not well defined. We report on our experience with the
early and intermediate outcome of EA/PA.
Methods
: From 1994 to 2011, 35 consecutive patients (26 neonates,
nine young infants) with EA were operated on by a single surgeon
(CKC). Of the 26 neonates, 16 had associated anatomical pulmonary
atresia (61%, 16/26) and seven had functional PA. For the entire
cohort (
n
=
35), follow up was complete in 93% of patients, extend-
ing to 18 years (mean 6.4
±
4.6).
Results
: Thirty-two of the 35 patients had a complete two-ventricle
repair, and three patients had a single-ventricle palliation (BT shunt,
n
=
2, Starnes palliation,
n
=
1). Hospital mortality for the group
was 20% (7/35). For patients with associated PA, mortality was
37.5% (6/16) compared to 5.2% (1/19) for patients without PA (
p
<
0.05). The hospital mortality for patients with EA/PA undergoing
biventricular repair was 46% (6/13), and the late mortality was 22%
(2/9). In our more recent experience (2005–2011) we altered our
management of patients with EA/PA to include either a RV-PA valved
conduit or a single-ventricle palliation (
n
=
7) with no early mortality.
Kaplan-Meier 15-year survival estimates were 79
±
15% for neonates
without pulmonary atresia, and 40
±
15% for those with pulmonary
atresia (
p
=
0.03).
Conclusions
: The surgical management of EA with pulmonary atre-
sia in neonates continues to evolve. The early survival of neonates
with EA/PA is improved when a RV-PA conduit is included in the
repair or the patient is stratified to a single-ventricle palliation.
1206: RARE COMPLICATIONS OF CONGENITAL BICUS-
PID AORTIC VALVE DISEASE, POSSIBLE CORONARY
ARTERIOPATHY
Ian Ternouth
1
, Sanjeevan Pasupati
2
, Rajesh Nair
2
, Adam El Gamel
2
1
Taranaki DHB, New Zealand
2
Waikato DHB, New Zealand
We present a case of a patient with unusual complications post
Bentall repair and aortic valve replacement, possibly due to arte-
riopathy involving the proximal coronary trees. A 46-year-old well
woman was referred for assessment of palpitations. She was found
to have a bicuspid aortic valve with mild aortic stenosis, mild-to-
moderate aortic regurgitation, but significantly dilated aortic root and
left ventricular dilatation, all of which increased in size under surveil-
lance. CTCA showed normal coronary arteries. She was referred for
cardiac surgery, which was complicated by emergency vein graft to
the LAD as the patient did not come off-pump easily.
She was initially well, then four months later presented with acute
chest pain. A small 4-mm defect in the anterior wall of the prosthesis
with a large pseudo-aneurysm was found on echo, confirmed on
angiography and CTA, and subsequently closed with an Amplatz
device. Angiography then showed mild RCA (FFR0.91) and left main
coronary artery stenosis. The vein graft was occluded. The patient
subsequently presented two months later with crescendo angina, and
was found to have critical 95% + left main coronary artery stenosis,
and was stented successfully. The RCA stenosis was confirmed but
did not merit PCI.
This patient seems to have developed rapid severe intimal hyper-
plasia, possibly triggered by instrumentation on the substrate of
bicuspid aortopathy/arteriopathy. Bicuspid aortic valves are known
to be associated with cystic medial necrosis and premature coronary
artery disease, abnormal vascular reactivity, coarctation, and cranial
aneurysms but this patient developed severe coronary disease within
a few months of having normal coronary anatomy, suggesting possi-
ble rapid intimal hyperplasia. We have not found any similar reports
in the literature. Echo and three-dimensional CT images will be
displayed.
1208: PULMONARY VENO-OCCLUSIVE DISEASE IN CHIL-
DREN
Cornelia Woerner
1
, Ernest Cutz
3
, Hartmut Grasemann
4
, Shi-Joon
Yoo
2
, Tilman Humpl
1
1
Labatt Heart Centre, Hospital for Sick Children, University of
Toronto, Canada
2
Division of Cardiac Imaging, Hospital for Sick Children, Toronto,
Canada
3
Department of Pathology, Hospital for Sick Children, Toronto,
Canada
4
Department of Respirology, Hospital for Sick Children, Toronto,
Canada
Background:
Pulmonary veno-occlusive disease (PVOD) is a rare
lung disease and accounts for five to 10% of all cases with pulmo-
nary hypertension (PH) of unknown aetiology. Incidence, prevalence
and aetiology of PVOD in children are not well defined. The mortal-
ity remains high, which is partly related to limited treatment options.
Methods and Results:
This retrospective analysis (1985–2011)
summarises symptoms, associated factors, treatment and outcomes
of nine paediatric patients (five female, four male) with histological
confirmation of PVOD. PH was diagnosed at a mean age of 13.5 years
(range 8–16 years), followed by the definitive diagnosis of PVOD at
a mean age of 14.3 years (range 10–16 years). Symptoms, such as
decreased exercise tolerance (
n
=
6) and/or shortness of breath (
n
=
9) preceded the diagnosis by two years on average; the mean survival
time after diagnosis was 14 months (range 0–47 months). Computed
tomography (CT) of the lungs showed typical radiological features.
Treatment included home-oxygen (
n
=
5), diuretics (
n
=
9), warfarin
(
n
=
4) and pulmonary vasodilators (
n
=
4). Four children were listed
and three successfully lung transplanted. Eight patients died, includ-
ing two after lung transplant. One transplant patient survived with
good quality of life.
Conclusions:
PVOD is an important differential diagnosis for
patients with PH. CT is a valuable tool to image lung abnormalities;
the definitive diagnosis can be made only by lung biopsy, which
subjects the patient to additional risk. Early listing for lung trans-
plantation is essential, as the mean survival time is only 14 months.
1212: ANOMALOUS ORIGIN OF A CORONARY ARTERY
FROM THE AORTA: OUTCOMES OF TREATMENT STRAT-
EGIES
Francesca Romana Pluchinotta
1,2
, Massimiliano Carrozzini
2
, Gabriele
Egidy Assenza
1
, Elisabeth Beran
1
, Stephen P. Sanders
1
, Francis Fynn-
Thompson
1
1
Boston Children’s Hospital, Boston, MA, USA
2
University of Padova, Italy, USA
Background
: Anomalous origin of a coronary artery from the
aorta (AOCA) is often an incidental finding but can be associated
with myocardial ischaemia and sudden death, especially in athletes.
Because AOCA is rare and the natural history unclear, there are
no established management guidelines. We sought to investigate
outcomes of various treatment strategies.
Methods:
We reviewed records of patients with the diagnosis of
AOCA seen in our hospital between 1995 and 2012. We included all
patients with coronary origin from an inappropriate sinus or single
coronary ostium, and excluded patients with other congenital or
acquired coronary abnormalities or a CHD. Diagnosis, presentation,
cardiac testing results, treatment, and outcome were recorded.
Results
: We identified 114 patients, 72 (63%) male, ages 0 to 49
years (mean 9.7
±
9.7 years). The LCA arose from the right sinus
in 20 (18%), the RCA from the left sinus in 73 (64%), and the
