CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
114
AFRICA
ADULTS WITH CONGENTIAL HEART DISEASE AND THE PREVENTION OF
AQUIRED HEART DISEASE STARTING IN CHILDHOOD
dure) 94.4, and in group 3 (2 or more cardiac procedures) 86.0.
NYHA classes II and III were more frequent in patient group 2 and
particularly in group 3. Patients in groups 2 and 3 had significantly
more cognitive difficulties than those with no cardiac procedures.
However, more than 50 % of all patients had more than 3 hours’
physical activity/week.
Conclusions
: In this unselected group of patients with congenital
heart disease it is shown that HRQoL is better for children with fewer
cardiac procedures and that HRQoL generally is better than for other
chronically ill children, which confirms previous results on selected
materials. In this single-centre study a web-based registry was used
for evaluation of HRQoL in children and adolescents with heart
disease. In the future it may be used for larger groups, such as the
national population of children with heart disease.
114: COMPLIANCE WITH LIFESTYLE RECOMMENDA-
TIONS IN CHILDRENANDYOUNGADULTSWITH HYPER-
TROPHIC CARDIOMYOPATHY
Ewa-Lena Bratt, Ingegerd Østman-Smith
Department of Paediatric Cardiology, Queen Silvia Children’s
Hospital, and Institute of Clinical Sciences, Sahlgrenska Academy,
University of Gothenburg, Sweden
Background:
Hypertrophic cardiomyopathy (HCM) is the most
common medical cause of sudden death during exercise. Previous
studies suggest that restriction in competitive sports participation
result in lower mortality rates. This study evaluates the effects of
lifestyle recommendations on physical and leisure-time activities in
HCM-patients.
Materials and methods:
Twenty-seven consecutively recruited
asymptomatic patients with HCM diagnosed through family screen-
ing were asked to participate in the study. All received lifestyle
recommendations according to international guidelines. Twenty
(median age 14.5 years, range 5–25) filled out a questionnaire
regarding the frequency of strenuous physical activities (defined as
becoming exhausted), sports participation and leisure time activities
(such as spending time with friends, using a computer, watching TV,
visiting the cinema, theatre or playing or listening to music), before
diagnosis and after 1 year. Patients were classified in 5–6 groups
according to the frequency of activities. Results were analysed using
Wilcoxon signed rank test.
Results:
There was a significant decrease in the frequency of strenu-
ous physical exercise from before diagnosis compared to 1 year later
(
p
=
0.002). Forty per cent performed strenuous exercise
>
7 hours/
week before diagnosis compared to 5% 1 year later. The number of
patients who never participated in sports activities increased from
10% to 20% (
p
=
0.007). No change was detected regarding leisure-
time activities. Time spent watching TV and using a computer did not
increase significantly.
Conclusions:
Lifestyle recommendations significantly affect the
physical activity habits in HCM patients. Our results indicate a high
level of compliance with the recommendations. Leisure-time activi-
ties do not seem to be negatively affected and time spent on sedentary
activities did not increase.
128: VENTRICULAR HYPERTROPHY IN ADULTS OPERAT-
ED FOR VENTRICULAR SEPTAL DEFECT AS TODDLERS
Johan Heiberg, Steffen Ringgaard, Vibeke Hjortdal
Aarhus University Hospital, Denmark
Background:
Cardiac morphology is expected to restore to normal
after surgical closure of ventricular septal defects (VSD) in early
childhood. However, long-term abnormalities in cardiac morphology
may exist and are the subject of this study.
76: ELEVATED LIPOPROTEIN A IN A NEWBORN WITH
THROMBOEMBOLIC DISEASE AND A FAMILY HISTORY
OF ATHEROSCLEROSIS
Nathalie Jeanne M Bravo-Valenzuela
1
, Camila Braga
2
1
Department of Medicine, Taubate University, Brazil
2
Pediatrics, Taubate University, Brazil
Background:
Thromboembolic disease is uncommon in young
children. Catheters, sepsis and inherited diseases are important risk
factors for thrombosis. Familial hyperlipoprotein A is an inherited
condition that leads to higher levels of lipoprotein A (Lp (a)). Lp
(a) is a subclass of low-density lipoproteins, plasminogen-like, with
thrombogenic properties.
Case description:
The male infant was born by caesarean section at
37 weeks’ gestation. The baby was cyanotic with little improvement
in the delivery room. The first echocardiogram (ECHO) demonstrat-
ed pulmonary arterial hypertension (PASP
=
80 mmHg). During the
first days of life, the newborn developed intracranial haemorrhage
and worsening of cyanosis. The follow-up ECHO showed 2 small
clots within right ventricle and a larger one (13.4
×
9.2 mm) near
the left pulmonary artery with flow obstruction. Radionuclide scan-
ning showed signs of pulmonary thromboembolism. An umbilical
venous catheter was removed. Enoxaparin was administered to the
patient (1.5 mg/kg SQ q12h). The results of the collected blood tests
were normal (thyroid hormones, liver enzymes, glucose, white blood
cell and platelet count, haemoglobin electrophoresis, factors IX, V
and VIII). Owing to haemodynamic instability, the newborn did not
undergo cardiac surgery. The conservative treatment showed clini-
cal improvement and gradual reduction of clots. He was discharged
on oral anticoagulation (warfarin). On further investigation, the
prothrombin gene mutation, the anticardiolipin antibodies and the
proteins C and S were collected and were negative. The family
history was positive for premature heart disease. Serum levels of
Lp (a) were obtained from the patient and his parents. The child
(55 mg/dl) and his mother presented with higher levels of Lp (a).
Antiagregant (aspirin) was prescribed. After the neonatal period, the
patient presented no new thrombus.
Conclusions:
Congenital thrombophilia needs to be strongly consid-
ered in neonates with a clinically significant thrombosis. The
increased Lp (a) is a risk factor for thrombosis, coronary artery
disease and cerebrovascular accident.
108: SELF-REPORTED HEALTH-RELATED QUALITY OF
LIFE IN CHILDREN AND ADOLESCENTS WITH HEART
DISEASE: A SWEDISH REGISTRY STUDY
Birgitta Svensson
1
, Gudrun Bjorkhem
1
, Ewa Idvall
2
1
Children’s Heart Center, Lund, Skåne University Hospital, Sweden
2
Malmo University and Skåne University Hospital, Sweden
Background:
During the last decades survival of children with
congenital heart disease has improved significantly. It is important to
assess health-related quality of life (HRQoL) in these patients. Some
of the variables in the Swedish national registry of congenital heart
disease (SWEDCON) include HRQoL measurements.
Aim:
To describe HRQoL in children and adolescents with heart
disease.
Materials and methods:
A descriptive study based on data from
SWEDCON, collected from one paediatric cardiac outpatient clinic.
The sample consisted of 104 girls and 154 boys, 9–18 years old with
registrations of HRQoL variables and the questionnaire DISABKIDS
short version. The patients were divided into three groups depending
on the number of cardiac operations or catheter treatments they had
undergone.
Results
: DISABKIDS mean total score (max 100) in patient group
1 (no cardiac procedures) was 92.9, in group 2 (one cardiac proce-
