CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
143
Ian Ternouth, Kareen McLeod
Taranaki District Health Board, New Plymouth, New Zealand
Background:
Sudden cardiac death can occur in adults (usually as
a result of coronary artery disease) and in younger people under 30
(usually as a result of structural heart disease or channelopathies),
ventricular fibrillation or tachycardia being a common pre-mortem
arrhythmia that can be treated with direct current (DC) cardioversion.
Time to DC shock is proven to be critical to outcome.
Methods
: We report on a project in the Taranaki province of New
Zealand to place ambulatory automatic defibrillators (AEDSs)
throughout the community. Taranaki is on the west coast of North
Island of New Zealand, population 109 000, area 7 257 km
2
. The
population is 84% of European ancestry and 15% Maori. Since
sudden unexpected deaths in the young (SUDY) from presumed long
QT syndrome, we have initiated a project to place AEDs in every
single senior school in the province. The majority of school teachers
also have received basic CPR training. All pupils at the schools are
aware of the sites of the devices. We have also placed the devices in
police vehicles, some volunteer fire brigades, certain shops, several
gyms, golf courses, and some Maraes. The aim is to get as good
a geographical coverage of the region as possible. An iPhone app
shows the site of every device. Devices sourced by us and also those
from other sources are on the site. Emergency services are aware of
all AED placements and will either tell the emergency caller or ask a
nearby business that has one to deliver it.
Results:
Several devices have been used appropriately since being
placed, but the programme has not been successful yet.
Conclusion:
It is feasible to get wide geographical coverage of AEDs
and use modern technology to improve useage.
132: TRIALS, TRIBULATIONS, TERRORS, FEARS, FRUS-
TRATIONS OF SETTING UP A SUDDEN UNEXPECTED
DEATH IN THE YOUNG (SUDY) PROJECT IN A SECOND-
ARY HOSPITAL AND ASSESSMENT OF SYNCOPE IN
YOUNG PERSONS
Ian Ternouth
1
, Jon Skinner
2
, Jackie Crawford
2
1
Taranaki DHB, New Zealand
2
Auckland DHB, New Zealand
Methods:
Taranaki is a province on the west coast of New Zealand’s
North Island, population 109 000, 85% white, 14% Maori. As part of
the National Congenital Inherited Diseases group (CIDG), we have
set out to collect and investigate all SUDY/syncope cases, and screen
relatives where possible. All sudden, unexpected/unexplained deaths
in persons two to 40 years of age are reported to us. Referrals for
syncope are also investigated once/if referred. Investigations include
autopsy, DNA studies in index cases: clinical examination, ECG, ETT,
echocardiography, and where appropriate, cardiac MRI, DNA testing.
Results:
Pathologies found to date include long QT syndrome,
hypertrophic cardiomyopathy, catecholaminergic polymorphic
ventricular tachycardia, arrhythmogenic right ventricular cardio-
myopathy, Brugada syndrome, and aortic stenosis. Challenges have
included reluctance of family members to be investigated, reluctance
to accept therapy where appropriate, inaccurate diagnoses, cultural
issues, missed referrals, and lack of notification. Several asympto-
matic relatives carrying potentially lethal genetic diseases have been
identified; some have accepted therapies as appropriate.
Conclusion
: Investigation of relatives of victims of sudden deaths/
syncope can prevent further deaths but it is not an easy service to
implement, as it is very time and resource consuming.
142: BORN WITH A HUGE MEDIASTINAL MASS, THE
STORY OF AN AMAZING SURVIVAL
Ayman Almasri, Hesham Menshaway, Nasreldeen Almeeri, Ahmed
Dohain, Binu John, Marwa Arwaabdelfattah, Mana; Abdelwahab,
Khaled Abuzaid, Ahmad Sabry, Vadim Ltubomudrov
Paediatric Cardiac Surgery Unit, Chest Disease Hospital, Kuwait
Introduction:
Cardiac tumours are benign or malignant neoplasms
arising primarily in the inner lining, muscle layer, or the surrounding
pericardium of the heart. They can be primary or metastatic. Primary
cardiac tumours are rare in paediatric practice. Intrapericardial tera-
tomas are significantly rare and generally seen in infants and young
children. Interestingly, malignant teratomas are extremely rare with a
prevalence of four to six cases in 10 000. It presents mainly in female
newborns weighing less than 3 kg. Both forms of teratomas may lead
to respiratory distress, pericardial effusion and cardiac compression.
Methods:
We present a 16-day-old female infant with a massive
intrapericardial mass that caused fatal cardiac compression and
respiratory distress. CXR, echocardiography and magnetic resonance
imaging (MRI) of the heart are the main non-invasive diagnostic
tools. Surgery was lifesaving. The removed tissues were examined
pathologically.
Results:
Lifesaving surgery was performed without complete resec-
tion due to the involvement of the great arteries. Pathological samples
revealed a malignant immature teratoma. Chemotherapy was started
on day four post extubation. Thr child has remained in stable condi-
tion to date.
Conclusion:
Immediate post birth detection and surgical manage-
ment were essentially life saving. Yet the prognosis of this child is
considered to be poor in light of the incomplete resection of the
tumour and the course of chemotherapy required.
146: PULMONARY ARTERY BANDING FOR LEFT
VENTRICULAR DILATED CARDIOMYOPATHY: A NOVEL
THERAPEUTIC STRATEGY INSTEAD OF HEART TRANS-
PLANTATION
Dietmar Schranz
Abteilung Kinderkardiologie Zentrum für Kinderheilkunde und
Jugendmedizin Universitätsklinikum Gießen und Marburg GmbH,
Standort Gießen, Germany
Background:
Dilated cardiomyopathy (DCM) in childhood has a
considerable morbidity and mortality rate and high incidence of heart
transplantation (HTX). Pulmonary artery banding (PAB) has been
proposed in patients with ccTGA to retrain the sub-pulmonary left
ventricle (LV) and to improve a failing sub-aortic right ventricle. We
used PAB in young patients with LV-DCM.
Methods:
A retrospective single-centre observational study was
performed to evaluate dilatable surgical PAB in infants and young
children with LV-DCM.
Results:
Since April 2006, 10 infants and two toddlers with LV-DCM
referred for HTX received a PAB. Additional, four patients underwent
repair of the left-sided total TAPVR, re-implantation of an ALCAPA,
mitral valve repair or replacement. There was no hospital mortality,
and clinical improvement in all patients. Median age at operation
was 126 (1–756) days in the 12 patients without additional operation.
The pressure gradient across the PAB increased significantly. The
LV ejection fraction increased from a median 15% pre-PAB to 43%
at discharge home, and 47%, three to six months later. The median
LVEDD and
z
-score decreased from 45 to 30 mm (
p
>
0.001) and
+6.1 to +3.2, respectively. Plasma B-type natriuretic peptide levels
decreased in comparison to the functional class improvement (
p
>
0.001). Six children were subsequently de-banded by trans-catheter
technique and are currently in functional class 1. Two patients, both
with non-compaction DCM, deteriorated five and six months after
PAB de-banding and finally died, one listed for HTX.
Conclusion:
In young children with LV-DCM and preserved right
ventricular function, PAB led to an improvement of LV and mitral
valve function by ventricular interaction and as yet unknown factors.
147: GLOBAL AND REGIONAL CIRCUMFERENTIAL AND
RADIAL MYOCARDIAL DEFORMATION AND TORSION
IN ELITE HIGH SCHOOL ATHLETES: PHYSIOLOGICAL
IMPACT OF HIGH-INTENSITY ATHLETIC TRAINING ON
VENTRICULAR ADAPTATION AND PERFORMANCE
