CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
199
XIII rs5982 (19 vs 6%,
p
=
0.04), GG polymorphism in fibrinogen
alpha chain rs2070006 (62 vs 38%,
p
=
0.02), CC polymorphism in
coagulation factor V rs3753305 (31 vs 17%,
p
=
0.05), and AG/GG
polymorphism in plasminogen rs13231 (44 vs 65%,
p
=
0.04).
Conclusions:
Patients with congenital heart disease have an imbal-
ance of prothrombotic gene polymorphisms that is magnified in the
subset of patients with trisomy 21. Clinical significance of these
genetic changes regarding thrombosis risk and thromboprophylaxis
effectiveness should be investigated further.
902: EISENMENGER IN INFANCY: IS IT TRIGGERED BY
COMBINED PRESSURE-VOLUME PULMONARY BLOOD
FLOW RATHER THAN BY INCREASED PULMONARY
VENOUS PRESSUREALONE? IMMEDIATEANDMIDTERM
NORMALISED PULMONARY ARTERY PRESSURES IN A
SIX-YEAR-OLD CHILD WITH COR TRIATRIATUM AFTER
REPAIR: A CASE REPORT
Damian Hutter
1
, Mladen Pavlovic
1
, Jean-Pierre Pfammatter
1
,
Alexander Kadner
1
, Bendicht Wagner
2
1
University Hospital, Bern, Switzerland
2
University Children’s Hospital, Bern, Switzerland
Introduction
: Eisenmenger disease describes a condition with fixed
pulmonary hypertension. Mostly congenital heart malformations
with increased pulmonary arterial pressure and blood flow (VSD,
ASD, PDA) or elevated pulmonary venous pressure (mitral stenosis,
cor triatriatum, obstructed pulmonary veins) are thought to be respon-
sible for the irreversible remodelling of the pulmonary vasculature.
If such a condition is left untreated for approximately two years,
failure of normal regression of the intimal smooth muscles occurs.
We report on a six-year-old child from Togo with severe pulmonary
hypertension due to an untreated cor triatriatum. At rest he had slight
tachypnoea of 40
–
45/min, thoracic deformation (cardiac voussure),
and saturation in room air
>
96%.
Methods
: Echocardiography gave a diagnosis of a cor triatriatum
with severe pulmonary arterial hypertension (TI gradient 150 mmHg,
BP 100/45 mmHg, gradient over membrane of cor triatriatum 55/15
mmHg). There was no atrial or ventricular septal defect. At mild
exertion (walking to out-patient clinic) there was immediate desatu-
ration to 80% in room air, with fatigue.
Results
: After surgical repair the patient showed immediate recovery
from pulmonary hypertension: 1/3 pulmonary arterial pressure while
coming off bypass circulation with Milrinon but without antihy-
pertensive treatment (NO, prostacyline). After six weeks, there was
improved physical performance with no desaturation while walking.
Echocardiography showed no evidence of pulmonary hypertension.
Conclusion
: Excessive high pressure-volume pulmonary blood flow
is most harmful for the pulmonary vascular bed and leads to early
fixed pulmonary hypertension. This case illustrates that increased
pulmonary venous pressure alone related to obstructive lesions such
as a cor triatriatum behave haemodynamically similar to severe mitral
stenosis in adults. In contrast to the high pressure-volume state in
large shunt lesions that develop usually over a period of 24 to 48
months, in fixed pulmonary hypertension, these patients obviously
have a greater potential to recover from pulmonary hypertension
regardless their age.
903: INCREASE IN USE OF POOLED HUMANANTITHROM-
BIN REPLACEMENT THERAPY IN PAEDIATRIC PATIENTS
Colleen E Gruenwald, Cedric Manlhiot, Leonardo R Brandao, Helen
M Holtby, Christopher A Caldarone, Steven M Schwartz, V Ben
Sivarajan, Lynn Lean, Glen S van Arsdell, Brian W McCrindle
Hospital for Sick Children, University of Toronto, Toronto, Canada
Background:
Antithrombin is an essential part of the coagula-
tion system. A number of paediatric patients, including those with
congenital heart disease, may have low antithrombin activity. For
patients with critically low antithrombin activity, endogenous human
antithrombin from pooled donors is available for replacement thera-
py. We sought to determine current trends in the use of antithrombin
supplementation in paediatric patients.
Methods:
Hospital records of all patients who received antithrombin
supplements at the Hospital for Sick Children between 2002 and
2011 were reviewed. Indication for antithrombin use was classified
as replacement therapy for cardiac patients [non-extracorporeal
membrane oxygenation (ECMO)], patients supported on ECMO and
for non-cardiac/non-ECMO patients.
Results:
A total of 551 paediatric patients received 1 912 courses of
antithrombin replacement therapy, of which 315 (57%) were cardiac
patients not on ECMO, 116 (21%) were patients supported on ECMO
and 121 (22%) were non-cardiac patients. Nearly half (48%) of all
patients receiving antithrombin were neonates (
<
31 days), 32%
infants (31 days – 1 year), 10% young children (1
–9
years) and 10%
adolescents (10
–
18 years). A higher proportion of neonates were
in the cardiac, non-ECMO patient group (52 vs 43%,
p
=
0.03).
Median baseline blood antithrombin level was 46% (25th
–
75th
percentiles: 32
–
61%). Number of patients receiving antithrombin
increased from 33 in 2002 to 81 in 2011 (+5 patients/year,
p
=
0.002).
During this period, use of antithrombin for cardiac patients not on
ECMO remained stable (+0.6 patient/year,
p
=
0.38) as did use for
non-cardiac patients (+0.4 patient/year,
p
=
0.16). However, use of
antithrombin supplementation in the setting of ECMO significantly
increased (+3 patients/year,
p
=
0.02) during the study period.
Conclusions:
Antithrombin use has been increasing in recent years,
primarily in patients on ECMO, despite the lack of high-quality
studies evaluating safety and efficacy. Future studies are needed to
determine proper indications and outcomes in these populations.
905: INTRACARDIAC THROMBUS IN PAEDIATRIC
PATIENTS WITH DILATED CARDIOMYOPATHY
Andiswa Nzimela
1
, Antoinette M Cilliers
2
, Ebrahim GM Hoosen
1
,
Paul Adams
2
, Gcina Dumani
2
, Himal Dama
1
1
Paediatric Cardiology, Inkosi Albert Luthuli Central Hospital and
Department of Paediatrics, University of KwaZulu-Natal, Durban,
South Africa
2
Division of Paediatric Cardiology, Chris Hani Baragwanath Hospital
and University of the Witwatersrand, Johannesburg, South Africa
Introduction:
Intracardiac thrombosis (ICT) in patients with dilated
cardiomyopathy (DCMO) is a serious complication with associated
significant morbidity and potential mortality. This study investigates
the incidence, risk factors and outcome of ICT in children with
DCMO. A retrospective review of clinical records was performed at
two tertiary centres for all children with DCMO.
Method:
This was a
r
etrospective review of paediatric cardiology
databases. All DCMO patients between 0 and 14 years seen between
January 1983 and December 2010 at Chris Hani Baragwanath
Academic Hospital (CBAH), Johannesburg, and between January
2003 and December 2011 at Inkosi Albert Luthuli Central Hospital
(IALCH), Durban, both tertiary institutions in South Africa, were
assessed.
Results:
An ICT was found in 39 (10.8%) of the 361 patients seen
at CBAH and in 13 (10.5%) of the 123 patients seen at IALCH. The
ICT was located within the left ventricular cavity in the majority of
patients. Only one patient had ICT in the right ventricular outflow
tract. All patients had poor left ventricular systolic function with
fractional shortening (FS) below 18%; 32% presented with an acute
thromboembolic event. All patients with ICT were anticoagulated
with heparin initially before changing to warfarin.
Discussion:
The development of ICT in patients with DCMO occurs
with stasis of blood from impaired left ventricular function, an abnor-
mal pro-coagulant endocardial surface, arrhythmias and heritable
hypercoagulable states. Clinical studies report a frequency of 4
–
16%
of ICT in patients with DCMO, with a much higher incidence of
43
–
57% in post mortem reports. Our retrospective review shows a
similar high incidence of ICT in patients presenting with DCMO who
are at risk of thromboembolic complications.
