CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
140
AFRICA
Background:
Long-term survival after the Fontan procedure shows
excellent results but is associated with a persistent risk of arrhyth-
mias, exercise intolerance and other severe complications. We aimed
to analyse the current burden of clinically relevant arrhythmia and
severe exercise intolerance in Danish Fontan patients and, further-
more, to estimate the future burden from analysis of mortality and
from the current burden related to age.
Methods:
All Danish citizens with Fontan completion from 1981 to
2009 were identified (
n
=
235). Surviving patients performed exer-
cise test, Holter monitoring, echocardiography, pulmonary function
test, and blood sampling and medical history were retrieved from
medical records.
Results:
Twenty-six (11%) patients died or had heart transplanta-
tion (HTx) after a mean (
±
SD) post-Fontan follow up of 8.3
±
5.7
years. Excluding peri-operative deaths (
n
=
8), a linear probability
of HTx-free survival was observed and estimated to 99.1% per year.
Prevalence of clinically relevant arrhythmia and severe exercise
intolerance increased significantly with age and was found in 32 and
85% of patients
≥
20 years, respectively. Furthermore, resting and
maximum cardiac index, resting stroke volume index and pulmonary
diffusing capacity decreased significantly with age while diastolic
and systolic ventricular function were unchanged. From survival
data and logistic regression models the future prevalence of patients,
clinically relevant arrhythmia and severe exercise intolerance were
estimated, revealing a considerable augmentation. For example,
assuming Fontan completion at four years of age and survival of the
peri-operative period, the probability of being alive at 40 years old is
72%, the probability of clinically relevant arrhythmia is 45% and the
probability of severe exercise intolerance is 88%.
Conclusions:
The prevalence of clinically relevant arrhythmia and
severe exercise intolerance increased significantly with age in Danish
Fontan patients. The future Fontan burden was estimated showing a
considerable increase in the prevalence of older patients, clinically
relevant arrhythmia, and severe exercise intolerance.
104: PREDICTIVE VALUE OF RESPONSE TO ACUTE VASO-
REACTIVITY TESTING IN CHILDREN WITH IDIOPATHIC
PULMONARY ARTERIAL HYPERTENSION
Christian Apitz, Christian Jux, Heiner Latus, Jochen Kreuder,
Dietmar Schranz
Paediatric Heart Centre, University Children’s Hospital, Giessen,
Germany
Background:
Acute testing of vasoreactivity during catheterisation
is an important factor in the evaluation of idiopathic pulmonary arte-
rial hypertension (IPAH). However, although the presence of an acute
response has important clinical consequences, its definition remains
controversial, especially in childhood, where a response is generally
defined as decrease of mean pulmonary artery pressure (mPAP) of
≥
20%. The purpose of our study was to assess the predictive value
of vasoreactivity testing and to compare different response criteria.
Methods:
Forty-two children with IPAH (mean age 10.1
±
5.4 years)
were included in the study. The assessment of pulmonary vasore-
activity was performed according to the guidelines of the German
Association for Paediatric Cardiology. Receiver-operating charac-
teristic curve (ROC) and Kaplan-Meier analysis were used to define
the predictive value of three different response criteria: reduction of
mPAP
≥
20%, mPAP
≥
30%, mPAP
≤
40 mmHg.
Results:
Baseline mPAP was 65.2
±
18.3 mmHg, mean change in
mPAP during vasoreactivity testing was 25.8
±
19.1%; 24 of the 42
patients showed a more than 20% reduction of the mPAP, 18 of them
to an mPAP below 40 mmHg. Mean follow up after catheterisation
was 55.3
±
40.5 months. Freedom from serious cardiovascular events
(lung transplantation or death) was 86% after two years, 76% after
three years, and 57% after five years. ROC curve revealed a reduc-
tion of mPAP
≥
30% as best cut-off value [area under the curve 0.753
(95% confidence interval 0.603–0.904);
p
=
0.006]. A predictive
value of mPAP
≥
30% and
≤
40 mmHg was superior to a reduction
of mPAP
≥
20% [log rank (Mantel Cox) Chi-square 9.98;
p
=
0.002].
Conclusions:
Acute vasoreactivity testing has an impact on outcome
in paediatric IPAH patients, however, a stricter definition of the
response criteria seems to be more reliable to predict serious cardio-
vascular events.
109: RHEUMATIC FEVER IN ESTONIAN CHILDREN
DURING THE LAST TWO DECADES (1992–2011) BASED
ON DATA FROM THE CHILDREN’S CLINIC OF TARTU
UNIVERSITY HOSPITAL
Lilja Ginter
1
, Hille Liivamägi
1
, Silvia Virro
2
1
Children’s Clinic, Tartu University Hospital, Estonia
2
Heart Clinic, Tartu University Hospital, Estonia
Backround:
There are almost no data published on RF among
Estonian children. We report our data from the south Estonian region
with a population of approximately 100
000.
Methods:
We carried out a retrospecive analysis of hospital and
outpatient cases.
Results:
There were 37 cases of RF (24 girls, 13 boys). The diagnosis
of RF was based on modified Jones criteria. Echocardiography was
performed on al patients.The first attack of RF occurred at the age
of thee to 16 years. Most cases occurred from November to April.
Cardiac involvement was present in 26 cases (70%): endocarditis
in 24 cases with mitral valve involvement, in eight cases with aortic
valve involvement, in five cases with tricuspid and in 11 cases with
multiple valve involvement, pericarditis in three cases, and myocardi-
tis in one case. Huntington’s chorea was diagnosed in 15 cases (41%);
and only chorea in seven (19%). Joint involvement was present in
22 cases (59%). All children were treated with conventional anti-
rheumatic and symptomatic therapy. They received prophylaxis
with benzathine benzylpenicillin. After the first attack in children
with cardiac involvement, six recovered without seqalae. Others had
only mild mitral or aortic valve regurgitation and in two cases, mild
dilatation of LV persisted, requiring administration of enalapriI. In
five cases there were no futher data after the first attack. A second
attack occurred in seven cases: five with cardiac involvement and
two with chorea. Even after the second attack only two mild mitral
insufficiencies persisted. None of our children needed to be operated
during childhood.
Conclusions:
RF is a rare disease in Estonia nowadays. In 1995 a
small outbreak of 8.5:100 000 was evident. Since 2000 there have
been up to two cases diagnosed per year in our hospital. However
there may still be some new cases in years to come and it is impor-
tant to take the possibility of RF into consideration, and implement
timeous, appropriate treatment to prevent subsequent complications.
110: NO DIFFERENCE IN CARDIAC PERFORMANCE
BETWEEN CRITICAL NEONATAL AND NON-NEONATAL
PATIENTS ONE YEAR POST COARCTECTOMY
Liselotte M Klitsie
1
, Arno AW Roest
1
, Irene M Kuipers
2
, Mark G
Hazekamp
1
, Nico A Blom
1
, Arend DJ Ten Harkel
1
1
Leiden University Medical Centre, Leiden, The Netherlands
2
Academic Medical Centre, Amsterdam, The Netherlands
Background
: Nowadays, repair of a critical neonatal coarctation
has low surgical mortality. It is unknown if subsequent postopera-
tive cardiac performance in this subgroup of coarctation patients is
different from patients who have undergone correction at an older
age. Accordingly, we aimed to characterise in both neonatal and
non-neonatal coarctation patients the changes in right (RV) and left
ventricular (LV) systolic and diastolic performance within the first
year of coarctectomy.
Methods
: Children (0–17 years) undergoing an aortic coarctec-
tomy were included and subsequently classified as critical neonatal
(prostin dependent
<
1 month old) or non-neonatal coarctation
patients. To evaluate RV and LV systolic and diastolic performance
peak systolic (S’) and early diastolic (E’) tissue Doppler imaging
velocities and E/E’ were assessed in the basal LV lateral wall and
