CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
183
for an abnormal screening ultrasound were 100 and 95%, respectively.
VS sensitivity for diagnosing a shunting lesion was 97%. No infants
were found with significant CHD in this evaluation.
Conclusions:
VS screening appears to have sufficient specificity to
exclude significant CHD in experienced hands; sensitivity for surgi-
cally relevant defects beyond the PDA requires additional study.
715: INCIDENCE OF CONGENITAL HEART DEFECTS IN
DUHOK, IRAQ
Alicia Lay
1
, Serdar Pedawi
2
, David Bush
3
, Kirk Milhoan
3
1
University of Texas Health Science Centre, Houston, USA
2
University Teaching Hospital Duhok, Kurdistan, Iraq
3
University of Texas Health Science Centre, San Antonio, USA
Background:
In Iraq, there have been various reports of an increased
incidence of congenital heart defects above the historical world aver-
age of 0.8% of all live births. However, there have been no systematic
studies to capture a true incidence of congenital heart disease (CHD)
in Iraq.
Methods:
During the week of 19 to 25 May 2012, a convenience
sample of 176 newborns was screened at Azadi Teaching Hospital in
Duhok, Iraq, by two paediatric cardiologists, with a complete echocar-
diogram (echo) as part of a demonstration project under the Ministry
of Health. Patent foramen ovale (PFO) and patent ductus arteriosus
(PDA) were considered normal and not recorded as defects.
Results:
A convenience sample of 176 of 276 live-born infants
(64%) was screened during the study period. Of those screened, 24
(13.6%) were found to have at least one abnormality on echo, some
had up to three. There were nine structural defects (5.1%), which
included three cases of tiny muscular ventricular septal defect (VSD)
(1.7%), two cases of atrial septal defect (ASD) (1.1%), three cases
with dysplastic aortic valves (1.7%), one aneurysmal atrial septum
(0.6%), one dysplastic tricuspid valve (0.6%), and one bicuspid
aortic valve (0.6%). None of these defects were considered haemo-
dynamically significant.
Conclusion:
This study is the first of its kind to attempt to screen
newborns with echo in a single hospital to obtain a true incidence
of CHD in Iraq. The overall incidence of structural congenital heart
defects was found to be 5.1%. If only VSDs and ASDs were counted,
an incidence of 2.8% is clearly higher than would be expected.
Systematic efforts at population-based screening in newborns are
warranted, both to define the true incidence of CHD as well as to
delineate any type-specific variations in expected disease rates.
717:EPIDEMIOLOGYOF PULMONARYARTERIALHYPER-
TENSION IN A SPANISH PAEDIATRIC POPULATION:
DATA FROM THE SPANISH REGISTRY FOR PAEDIATRIC
PULMONARY HYPERTENSION (REHIPED) AND SPANISH
REGISTRY FOR PULMONARY ARTERIAL HYPERTEN-
SION IN ADULTS (REHAP)
Maria Jesus del Cerro
1
, Dimpna C Albert
2
, JL Gavilan
3
, E Gomez
4
,
M Lopez
5
, J Espin
6
, P Escribano
7
, REHIPED/REHAP investigators
1
Department of Paediatric Cardiology, Hospital La Paz, Madrid, Spain
2
Department of Paediatric Cardiology, Hospital de
Vall d’Hebron,
Barcelona, Spain
3
Department of Paediatric Cardiology, Hospital Virgen del Rocão,
Sevilla, Spain
4
Department of Paediatric Cardiology, Hospital Reina Sofía de
Córdoba, Spain
5
Department of Cardiology, Hospital Miguel Servet, Zaragoza, Spain
6
Department of Paediatric Cardiology, Hospital Virgen de la Arrixaca,
Murcia, Spain
7
Department of Cardiology, Hospital 12 de Octubre, Madrid, Spain
Nowadays there is a lack of data regarding the epidemiology of
paediatric pulmonary arterial hypertension (PAH). In order to provide
valuable epidemiological data about paediatric PAH in Spain, we
collected clinical data of patients diagnosed with PAH from two
months to 18 years old from the REHIPED and REHAP registries.
Methods:
Voluntary reporting (from referral and non-referral
centres) of their paediatric patients (aged 2 months to 18 years)
diagnosed with PAH was used. Consecutive cases diagnosed from
January 2009 to June 2012 were selected to provide data on average
annual incidence from 2009 to 2012, and point prevalence (June
2012). PAH was defined by a mean pulmonary artery pressure (PAP)
>
25 mmHg, pulmonary vascular resistance (PVR)
>
3 Wood units
(WU) and pulmonary capillary wedge pressure
<
15 mmHg.
Results:
A total of 205 PAH patients (mean age 8
±
5.7 years) was
reported in both registries: iPAH (idiopathic,
n
=
57), PAH/CHD
(congenital heart disease PAH,
n
=
135), veno-occlusive (
n
=
2),
portopulmonary (
n
=
4), HIV (
n
=
1), connective tissue disease (
n
=
2), others (
n
=
4). Yearly incidence rates for PAH diagnoses were 2.56
±
0.25 cases/million/year. For iPAH and CHD-PAH these rates were
respectively 0.49
±
0.29 and 1.87
±
0.18 cases/million/year. We esti-
mated a point prevalence (June 2012) of 14 cases/million for PAH,
2.97 cases/million for iPAH, and 10. cases/million for HAP/CHD.
Conclusions:
The estimated incidence and prevalence of PAH, IPAH
and PAH/CHD in the paediatric Spanish population was comparable
to those provided for this age range by other European countries with
mandatory registration. Congenital heart disease is the most common
cause of PAH in patients from two months to 18 years.
718: DETERMINATION FACTOR OF PULMONARY-TO-
SYSTEMIC FLOW RATIO IN ATRIAL SEPTAL DEFECT
Clara Kurishima
1,2
, Hirofumi Saiki
1
, Hirotaka Ishido
1
, Satoshi
Masutani
1
, Hideaki Senzaki
1
1
Saitama Medical School, Japan
2
Okinawa Chubu Hospital, Japan
Background
: Left ventricular (LV) diastolic stiffness has been postu-
lated as an important determinant of pulmonary-to-systemic flow
ratio (Qp/Qs) in atrial septal defect (ASD). However, to date, there
are no data to directly support this. Therefore, the present study tested
the hypothesis that increased LV diastolic stiffness is an independent
determinant of increased Qp/Qs in ASD.
Methods and Results
: Study subjects were 192 consecutive ASD
patients who underwent catheter closure of ASD with the Amplatzer
septal occluder. Patients’ age ranged from four to 78 years old, and
the average Qp/Qs was 2.38
±
0.90. The size of the defect was deter-
mined as the diameter of the deployed device, and was normalised to
body surface area of the patient. LV diastolic stiffness was calculated
as follows: (LV end-diastolic pressure – LV diastolic minimum pres-
sure)/stroke volume index. Univariate analysis showed that Qp/Qs
was significantly and positively correlated with the size of defect (
r
=
0.49,
p
<
0.0001) and LV diastolic stiffness (
r
=
0.29,
p
<
0.0001).
Multivariate analysis also demonstrated that the size of ASD (
β
=
0.45,
p
<
0.0001), LV stiffness (
β =
0.28,
p
<
0.0001), RV stiffness (
β
=
–0.21,
p
=
0.001) and age (
β
=
0.25,
p
=
0.001) were independent
determinants of Qp/Qs (
r
=
0.65,
p
<
0.0001).
Discussion
: The present study for the first time confirmed that
increased LV stiffness independently increases Qp/Qs in ASD.
Because increased Qp/Qs due to an increased LV stiffness causes
RV dilation and further increase in the LV stiffness via a ventricular
interaction, this could explain the progressive nature of this disease.
721: NEUROCOGNITIVE OUTCOMES OF INFANTS WITH
SINGLE-VENTRICLE PHYSIOLOGY SEEN INA COMPLEX
CONGENITAL HEART DISEASE CLINIC
Sharon Sables-Baus
Children’s Hospital Colorado and University of Colorado, USA
Background
: Earlier intervention and exposure to various peri-
operative techniques and care have contributed to improved survival
rates, but may also adversely impact on future developmental
processes and outcomes. Specific aims were: (1) determination of
which medical characteristics of the baby are predictive of which
