Cardiovascular Journal of Africa: Vol 24 No 1 (February 2013) - page 32

CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013 30 AFRICA
671: LOW-VOLUME BLOOD SAMPLING TUBES CAN BE
USED FOR THROMBELASTOMETRY IN PAEDIATRIC
PATIENTS
Jo Bonding Andreasen1 , Ingrid Hell Knudsen2 , Thea Unger Pistor-
Riebold2, Hanne Berg Ravn1, Anne-Mette Hvas2
1Department of Anaesthesiology and Intensive Care, Aarhus
University Hospital, Skejby, Denmark
2Department of Clinical Biochemistry, Aarhus University Hospital,
Skejby, Denmark
Background: Minimising the volume of blood used for diagnostic
procedures in children undergoing congenital heart surgery is desir-
able. We investigated to what extent the size and the type of sample
tube affected the results of thrombelastometry (RoTEM® ).
Methods: In 20 healthy individuals, we compared four sampling
tubes (evacuated 0.109 M sodium-citrate plastic tubes): Venosafe®
1.8, 2.7 and 3.6 ml and BD Vacutainer® 1.8 ml. Using four paral-
lel RoTEM® devices we studied clotting time, maximum velocity
and maximum clot firmness in three assays: ExTEM ®, InTEM® and
FibTEM® .
Results: No difference was found in any of the RoTEM® param-
eters using four different tubes for blood sampling. Intra- and inter-
individual variation was acceptable. When comparing the two low-
volume tubes, we found that the technical design of the tube did not
influence RoTEM® results.
Conclusion: RoTEM® results were not affected by the size and the
type of the sampling tube. Therefore, to minimise the volume of blood
drawn, smaller tubes of 1.8 ml are preferable in paediatric patients.
672: IDEBENONE IN FRIEDREICH ATAXIA: IMPROVED
FUNCTIONAND REDUCED MYOCARDIAL HYPERTHROPHY
Sanja Dorner, Krasanka Hafner, Monja Gverieri, Iva Cucic
Paediatric Cardiology Clinical Hospital Centre, Osijek, Croatia
We present a patient at the age of nine years who suffers from
Friedreich ataxia with hypertrophic cardiomyopathy. She was born
after a normal pregnancy and delivery. She began walking at 13
months but with difficulty. At the age of three years she often
complained of pain in the legs while walking and she kept falling.
She sweated a lot and in every respiratory infection symptoms got
worse. Friedreich ataxia was diagnosed and a cardiological examina-
tion was done. Ultrasound showed hypertrophic cardiomyopathy with
depressed contractility and there were repolarisation disturbances in
the electrocardiogram. We treated her with idebenon after which the
cardiac muscle contractility was normalised.
Friedreich ataxia is an autosomal recessive multisystem progres-
sive disease characterised by disturbances in walking, and extremity
ataxia caused by deterioration of the rear column and spinocerebellar
tracts in the spinal cord. Mutation of the gene for frataxin (FXN)
causes the disease. In most patients, the disease affects the heart and
in combination with neurological problems, significantly reduces
the patient’s abilities and often causes premature death, particu-
larly in patients who have the disease before the age of 20 years.
Hypertrophic cardiomyopathy with normal ejection fraction is the
most frequent ultrasound finding in the heart in 75% of the cases.
Unfortunately, there is no cure for this disease and the function of
fraxatin protein is unknown. The present studies show that frataxin
protein is important in regulating the transfer of iron in mitochondria.
Idebenone is a short-chain bensoquinon similar to coenzyme Q10. It
is a powerful antioxidant and electron carrier in the respiratory chain
and helps the creation of ATP. The recent studies have shown that
idebenone is effective in doses of 5–10 mg/kg/day in patients with
FA and cardiomyopathy.
674: A NEWBORN WITH HIGH-OUTPUT CARDIAC FAIL-
URE DUE TOA LARGEVEIN OF GALEN MALFORMATION
Sanja Dorner1, Darjan Kardum1, Ivan Malcic2, Vesna Benjak 2, Monja
Gverieri1 , Zeljka Kardum1
1Clinical Hospital Centre, Osijek, Croatia
2Clinical Hospital Centre, Zagreb, Croatia
Neonatal heart failure in newborns is often caused by asphyxial
cardiomyopathy, left-sided obstructive lesions, large mixing cardiac
defects and myocarditis. The most frequent haemodynamically
significant extracardiac arterio–venous shunt found in newborns is
a vein of Galen malformation. However, it is not a frequent cause
of severe cardiac failure in infancy. Effective treatment has not yet
been found.
We report on a patient with a large vein of Galen malformation
who presented with high-output cardiac failure, pulmonary artery
hypertension and respiratory distress soon after birth. During an
emergency cardiac catheterisation, we diagnosed a large vein of
Galen malformation. The cardiac status was improved after partial
embolisation.
Nine months later the echocardiographic finding was normal and
further therapy was discontinued. Unfortunately, as in similar cases,
hypertensive hydrocephalus developed. A ventriculoperitoneal shunt
was placed but there were major neurodevelopmental delays.
676: THE CASE OF NEONATAL LUPUS SYNDROME:
TRANSIENT COMPLETE CONGENITAL AV BLOCK IN A
NEWBORN
Monja Gverieri, Iva Cucic, Sanja Dorner
Clinical Hospital Centre, Osijek, Croatia
The congenital heart block in neonatal lupus is a form of passively
acquired autoimune disease in which maternal auto-antibodies to the
intracellular ribonucleoproteins Ro (SS-A) and LA (SS-B), cross the
placenta and injure the foetal heart. Studies show that AV block in
neonatal lupus is irreversible. The aim was to present a patient who
had spontaneous recovery from complete heart block.
The patient was born after a normal, controlled pregnancy. In
the 36th week of gestational age she was delivered by emergency
caesarean section because of foetal bradycardia in a cardiotoco-
graphic recording, BW 2 750 g, Apgar score 9/9. After the birth,
electrocardiography results showed complete AV dissociation with
atrial frequency around 150 bpm and ventricular around 55–60 bpm.
Echocardiography showed normal heart structure and function.
Serology for autoimmune diseases was performed and showed
that the newborn had maternal anti-Ro-SSA and anti-La-SSB anti-
bodies in the circulation. Anamnesis of the mother showed that she
had years of dermatological treatment due to skin lesions on her legs,
and occasional pain in the joints. We advised her to have rheumatolo-
gy tests done. During her stay, the newborn had no cyanotic crisis and
no signs of loosing heart function. The pulse rate ranged from 65–90
bpm and ECG showed a persisting complete atrioventricular block.
At the age of three months the patient was re-admitted to the
clinic to run control tests. During the 24-hour ECG, she was in sinus
rhythm, with an average frequency of 133 bpm. The lowest frequency
was recorded while sleeping, at 67 bpm. There were no episodes of
complete AV block or other arrhythmias. There was spontaneous
recovery of the cardiac conduction system.
690: A RETROSPECTIVE REVIEW OF PALLIATIVE
SYSTEMIC-TO-ARTERIAL SHUNTS IN A RESOURCE-
SCARCE AFRICAN ENVIRONMENT
Darshan Reddy, Julian Buckels
Inkosi Albert Luthuli Central Hospital, Durban, South Africa
Background: Due to the lack of cardiac surgical programmes
undertaking corrective surgery in the neonatal period in resource-
scarce African countries, the vast majority of patients presenting
with cyanosis in the newborn period are palliated by a systemic-to-
pulmonary arterial shunt.
Methods: We reviewed the case records of all patients who under-
went systemic-to-pulmonary arterial shunting over a seven-year