CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
193
849: COMPUTATIONAL FLUID DYNAMICS IN FONTAN
PATIENTS TO EVALUATE ENERGY LOSS DURING SIMU-
LATED EXERCISE
Sjoerd Bossers
1
, Rena Verhaart
2
, Frank Gijsen
3
, Frans van de Vosse
2
,
Adriaan Moelker
4
, Jolanda Wentzel
3
, Willem Helbing
1
1
Paediatric Cardiology, Erasmus MC, Sophia Children’s Hospital,
Rotterdam, Netherlands
2
Biomedical Engineering, Eindhoven University of Technology,
Eindhoven, Netherlands
3
Biomedical Engineering, Division of Cardiology, Erasmus MC,
Rotterdam, Netherlands
4
Department of Radiology, Erasmus MC, Rotterdam, Netherlands
Background:
In most patients with a functional single ventricle, a
total cavopulmonary connection (TCPC) is created. Exercise intoler-
ance is common in Fontan patients. It has been suggested that energy
loss (Eloss) inside the TCPC may play an important role in reduced
exercise performance. The aim of this study was to establish whether
Eloss inside the TCPC plays a significant role during (simulated)
exercise.
Methods:
In 15 patients a three-dimensional reconstruction of the
TCPC was created using anatomical data from cardiac magnetic
resonance imaging (CMR). Phase-contrast CMR was used to obtain
through-plane flow rates from the inferior vena cava (IVC) and
superior vena case (SVC) during rest and during exercise, simulated
with dobutamine (7.5 μg/kg/min) intravenously. Patients underwent
cardiopulmonary exercise testing to assess maximal oxygen uptake.
CFD simulations were performed using the flow rates obtained by
CMR. Total energy loss (Eloss) inside the TCPC structure was calcu-
lated for both conditions in each patient. A condition where only IVC
flow was increased two-fold compared to the resting condition was
added to the study to reflect a more natural flow distribution, which
would be expected during supine exercise.
Results:
Eloss was higher during simulated exercise in all patients
but one. Mean Eloss was 0.62
±
0.37 mW during rest, 1.07
±
0.64
mW during simulated exercise and 2.97
±
2.49 mW with two-fold
IVC flow. The correlation between cardiac index and Eloss was
exponential (
r
sq
=
0.393,
p
<
0.000). The increase in Eloss depended
on the specific anatomy of each patient. No correlation was found
between the increase in Eloss during dobutamine infusion and exer-
cise capacity as assessed by maximum oxygen uptake.
Conclusions:
Eloss inside the TCPC structure was limited but
increased with (simulated) exercise. This was due to increased blood
flow, but also depended on patient-specific anatomy of the TCPC. We
did not observe a correlation between Eloss and exercise capacity.
850: CROSSED PULMONARY ARTERIES: REPORT OF 20
CASES WITH EMPHASIS ON CLINICAL FEATURES WITH
GENETIC, CARDIAC AND EXTRACARDIAC ABNORMALI-
TIES
Kadir Babao
ğ
lu
1
, Gürkan Altun
1
, Köksal Binneto
ğ
lu
1
, Muhammed
Dönmez
2
, Yonca Anık
2
1
Department of Paediatric Cardiology, Kocaeli University, School of
Medicine, Kocaeli, Turkey
2
Department of Radiology, School of Medicine, Kocaeli University,
Kocaeli, Turkey
Background
: Crossed pulmonary arteries (CPA) is a rare abnor-
mality in which the ostium of the left pulmonary artery originates
superiorly and to the right of the right pulmonary artery. Recognition
of this rare pathology is important because it is usually accompanied
by other congenital heart disease, extracardiac anomalies, and some
genetic problems. To date, there have been few reported cases. Most
are case report with complex cardiac abnormalities.
Methods:
We have detected 20 cases due to increased awarenes of
this anomaly in the last three years. Approximately 9 250 echocar-
diaograms were performed in this period. We describe 20 cases with
this anomaly with an emphasis on the clinical features with genetic,
cardiac and extracardiac abnormalities.
Results:
The ages of the patients ranged from one day to 13 years
at the time of initial diagnosis. There were 10 female and 10 male
patients. Four patients had complex cardiac pathology such as TOF,
truncus, TGA and CAVSD. Eleven of the 20 patients had ventricular
septal defect. ASD and PS were detected in 12 of 20 patients (60%).
Aortic arch abnormalities such as right aortic arch and coarctation
were seen in six patients. One patient had persistent left superior
vena cava; 45% of the cases were associated with genetic syndromes
(Di-george, Noonan and Holt Oram syndrome, VACTER anomalies).
These syndromes were diagnosed on the basis of clinical features.
Karyotypes and FISH for 22Q deletion were studied in 11 patients.
All had normal karyotypes and FISH results. Six patients underwent
succesfull operations. Three of 20 patients died during long-term
follow up. The remaining cases were clinically stable and were being
followed without surgery.
Conclusions
: CPA may be related to different congenital heart
diseases. Detection of CPA may be an important clue to the presence
of structural heart disease and chromosomal abnormalities.
851: RARE CASES OF LEFTVENTRICULARANEURYSM IN
CHILDREN: A SINGLE-CENTRE EXPERIENCE
Pimpak Prachasilchai
1
, Worakan Promphan
1
, Thanarat Layangool
1
,
Chaisit Sangtawesin
1
, Thawatchai Kirawittaya
1
, Suvipaporn
Siripornpitak
2
, Pirapat Mokarapong
3
1
Queen Sirikit National Institute of Child Health, Thailand
2
Ramathibodi Hospital, Mahidol University, Thailand
3
Rajavithi Hospital, Thailand
LV aneurysms in children are very rare and
often result from a
congenital defect in the posterior portion of the mitral valve annulus.
We present two unique cases of children with tuberculosis (TB) and
acquired sub-mitral aneurysms.
Case report:
The first case was an afebrile two-year-old girl with
progressive dyspnoea, CHF and systolic murmur at the apex for eight
days. CXR showed marked cardiomegaly with pulmonary venous
congestion. Echocardiogram revealed a huge LV aneurysm at the sub-
mitral valve area, causing moderate MS, severe MR and TR. EF was
50%. MRI showed a large pseudoaneurysm at thte posterior wall of
the LV. Additional non-contrast CT scan revealed multiple calcified
mediastinal lymph nodes (caseous nodes). The lung parenchyma was
normal. Mitral valve replacement was performed due to complete
destruction of the posterior leaflet and annulus. LV aneurismorhaphy
and tricuspid valve repair were also done. The post-operative course
was uneventful. Pathological examination suggested necrotising gran-
ulomatous inflammation. AFB from the lymph node was negative.
The second case was a 2.5-year-old boy with a history of TB
contact, who presented with prolonged fever and dyspnoea. Physical
examination revealed no signs of CHF or murmur. CXR showed mild
cardiomegaly. He suddenly collapsed after complaining of severe
epigastric pain immediate after administration. CPR was unsuc-
cessful. The autopsy revealed rupture of a huge LV aneurysm at the
sub-mitral valve area, with a massive intrapericardial blood clot,
generalised lymphadenopathy, pleural effusion and ascites. Even
though AFB from myocardial tissue was negative, pathological find-
ings suggested granulomatous inflammation.
TB endocarditis has been reported sporadically. Most cases
presented as LV aneurysm at the sub-mitral valve area. Patients with
LV aneurysm are at potential risk for life-threatening conditions
leading to sudden death. Echocardiography and MRI are crucial
modalities to define the morphology and extent of the aneurysm and
surgical planning. Management should be individualised and mainly
directed toward early diagnosis to prevent moribund outcomes.
854: KAWASAKI’S DISEASE PRESENTINGWITH DILATED
CARDIOMYOPATHY: A CASE REPORT
Adele Dippenaar, Lungile Pepeta
Division of Paediatric Cardiology, Dora Nginza Hospital, Port
Elizabeth Hospital Complex, Walter Sisulu University, Port Elizabeth,
South Africa
