CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
148
AFRICA
Background
: Tetralogy of Fallot (ToF) is the most common form
of cyanotic congenital heart disease. Until the development of the
transatrial–transpulmonary approach, surgical repair was achieved
through a right ventriculotomy causing right bundle branch block
(RBBB). We aimed to study the QRS axis pattern in adults with
repaired ToF and to correlate it to the type of surgery.
Methods
: Adults with repaired ToF referred at our institution for
ECG, echocardiography and cardiovascular magnetic resonance
were included except paced patients. Surgical history was obtained
from hospital records. Electrocardiographic measures (maximum
PR, QRS and QT duration, QRS axis) were analysed manually from
standard 12-lead electrocardiograms.
Results
: Twenty-nine patients were included (72% male, 33
±
13
years). All patients but one had a transventricular repair (37% had a
transannular patch, 60% had a transverse or longitudinal ventriculot-
omy without transannular patch). Mean CMR right and left ventricle
indexed end-diastolic volumes were, respectively, 150
±
29 and 71
±
17 ml/m
2
. Mean QRS duration was 156
±
16 ms with a RBBB pattern
for all patients. There was no significant difference concerning BMI,
CMR measures or QRS duration between different sub-groups. All
patients from the ventriculotomy group had a normal or right ECG
axis pattern (axis between 34 and 160°). Nine patients (90%) from
the transannular group had a left ECG axis pattern (axis between –10
and –76°)
.
Conclusion:
QRS axis pattern in adults with repaired ToF was corre-
lated with the type of surgery.
208: THE INCIDENCE AND NATURAL HISTORY OF INNO-
CENT HEART MURMUR IN NEWBORN BABIES
Saraiorn Thonginnetra, Chanatip Luevisadpaibul
Srinakharinwirot University,Thailand
Introduction:
This study aimed to determine the incidence, and
define the origin and natural history of the innocent heart murmur
in newborns.
Methods:
In a 21-months prospective study, 2 849 newborn babies
underwent routine examination by paediatricians. Infants with a
murmur were re-examined by paediatric cardiologists. Term babies
with a clinical diagnosis of an innocent heart murmur were studied.
Each baby had a complete echocardiographic study and was followed
up at two and six months until the murmur had disappeared or the
heart was totally normal.
Results:
Clinically suspected innocent heart murmur was found in
30 cases. The incidence of innocent murmur of term babies was
9:1 000 live births. Normal echocardiogram was found in 10 cases
(33%), peripheral pulmonary branch stenosis (PPS) in six (20%),
small patent ductus arteriosus (PDA) in five (17%), small ventricular
septal defect (VSD) in three (10%), mild pulmonary valve stenosis
in one (3%) and isolated mild tricuspid regurgitation in five cases
(17%). Only VSD and pulmonary valve stenosis were considered to
be pathologic. Follow up was performed at the age of two months:
the heart murmur had disappeared in 23 cases (82%), PDA and mild
pulmonary stenosis had resolved in all patients but PPS was still
present in one of six cases. At six months, the murmur had disap-
peared in 26 cases (93%), two of the three with asymptomatic small
VSD still had murmur and the defects were still patent.
Conclusion
: An innocent heart murmur in a term baby is often
related to non-clinical significant conditions. The suspected innocent
heart murmur diagnosed at birth had resolved in most of the babies
at six months.
212: SEVERITY AND PROGNOSTIC INDICES IN CHILD-
HOOD CARDIAC FAILURE
Queennette Daniels
1
, Christy AN Okoromah
2
, SI Omokhodion
3
1
Department of Paediatrics, Lagos University Teaching Hospital,
Lagos, Nigeria
2
Department of Paediatric Cardiology, Lagos University Teaching
Hospital, Lagos, Nigeria
3
Department of Paediatric Cardiology, University College Hospital
Ibadan, Nigeria
Background:
Childhood cardiac failure remains a major cause of
morbidity and mortality in the developing world. The diagnosis and
management outlook of these children in resource-poor countries
remains poor when compared to their developed counterparts. There
is still a paucity of data on the severity and determinants of outcome
in childhood cardiac failure in Nigeria.
Objectives:
The aim of the study was to evaluate the severity of
congestive cardiac failure (CCF) and to highlight the factors that
affect outcome in children presenting at the Lagos University
Teaching Hospital (LUTH), Lagos, Nigeria.
Methods:
Two hundred and twenty consecutive cases of CCF admit-
ted into paediatric wards and the children’s emergency rooms of
LUTH with a diagnosis of heart failure over a one-year period were
studied prospectively. Diagnosis of heart failure was based on the
presence of at least three or four cardiac signs of heart failure. The
severity of CCF was determined using heart failure severity index
scores for children, proposed by Omokhodion
et al
., which was
validated using the Ross heart failure scores for infant and children.
All cases were followed up daily until a definite outcome was deter-
mined.
Results:
Predominant underlying causes of CCF were severe anae-
mia (39.4%), respiratory tract infections (26.1%), intrinsic heart
disease (22.5%), and others (12.0%); 23.8, 30.3 and 45.9% of the
patients presented in mild, moderate and severe heart failure, respec-
tively, and the difference in distribution was statistically significant
(
p
<
0.01). The factors that affected the severity of heart failure were
low packed-cell volume on admission (
p
=
0.04), low socio-economic
class (
p
=
0.03) and the cause of the heart failure (
p
=
0.02). The case
fatality rate was 15.1% among the study population. Poor prognostic
indices identified were renal disease (
p
=
0.03) and acquired heart
disease (
p
=
0.004) as the cause of the heart failure, presence of
severe heart failure on admission (
p
<
0.001), lower socio-economic
status (
p
=
0.003) and re-admission for heart failure within the study
period (
p
<
0.01).
Conclusion:
Heart failure in Nigerian children
is associated with an
unacceptably high mortality rate. Identification of high risk factors
in children with heart failure and routine use of heart failure severity
index to grade heart failure severity may aid in early and effective
interventions.
222: FAMILIAL RECURRENCE OF CONGENITAL HEART
DISEASE CAUSED BY MUTATION IN NKX2-5
Sabrina Eliasson
1
,Vibeke Hjortdal
1
, Jesper Bjerre
1
, Lars Søndergaard
2
,
Henrik Ãrbãk Andersen
2
, Søren Brunak
1
, Anders Boeck Jensen
1
, Lars
Allan Larsen
1
1
University of Copenhagen, Denmark
2
Rigshospitalet, Denmark
Background:
Familial recurrence presents in 3 to 5% of non-syndro-
mic congenital heart disease (CHD). The transcription factor
NKX2-5
is known to cause a variety of CHDs, but most previous studies have
focused on a single specific diagnosis. The aim of this project was to
identify a large number of families with recurrent CHD and to screen
them for mutations in
NKX2-5
regardless of the CHD diagnosis.
Methods:
We included 46 families with 136 affected individuals.
Blood samples were collected from family members after informed
consent. A medical interview was conducted by phone and the
diagnoses confirmed by examination of hospital files. One affected
member of each family was screened for mutations in
NKX2-5
.
Malformations were listed.
Results/Discussion:
One family had a single nucleotide deletion in
exon 1 of
NKX2-5
causing a frameshift. The family had five living
(three with ASD2, one with ASD2 and first-degree AV-block, one
with muscular VSD and ASD) and one deceased individual with
DORV-TOF, CoA, VSD and ASD. The mutation segregated with the
affected individuals, and we also found one healthy carrier of the
