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16
PATTERN OF CONGENITAL HEART DISEASE
AMONG CHILDREN IN MADINA MUNA W ARA
BASHIR AHMAD BHAT, MD; SHEIKH ABDUL MATEEN, MD; ANWAR KHAN, MD;
MOHAMMAD AHMAD IMAM DAD, DCH, Arab Board
A total of 1209 children with 'congenital heart disease (CHD) were studied in the Pediatric
Cardiology Unit of the Madina Maternity' and Children Hospital, in Madina Munawara, between
March 1992 and February 1995, with an aim to provide an overview of CHD in this part of the
Kingdom. Both sexes were equally affected. Ventricular septal defects (VSD) were found to be the
commonest lesion (frequency, 29.7%), followed by atrial septal defect, pulmonary valve stenosis,
and patent ductus arteriosus. The study indicates a high prevalence of atrial septal defects and
pulmonary valve stenosis and relative infrequency of obstructive aortic lesions in Saudi Arabia as
compared to American and European children. A total of 62% of the patients studied were detected
by one year of age, which falls short of cumulative detection rates in Europe, indicating a need for
further improvement in case detection.
CONGENITAL HEART DISEASE (CHD) occurs in
approximately one percent of live born children; it
occurs in a much higher percentage of those aborted
spontaneously or stillborn.l Several reports suggest
that the incidence and pattern of CUD vary in
different geographicallocations.2-7 With these
reports in mind, we investigated the patterns of CHD
in the Madina region in our study at Madina
Maternity and Children Hospital, in Madina
Munawara.
to have CHD were included in the study. Each
patient was diagnosed on the basis of physical
examination,
standard
chest
radiography,
electrocardiography, and echocardiography (2-D and
Doppler). All patients had a final evaluation by the
pediatric cardiologist. In patients where there was
discordance between clinical and echocardiographic
diagnoses, both diagnostic procedures were repeated
to arrive at correct diagnosis.
Results
Materials and Methods
The study was conducted in a period of three
years, beginning in March 1992, in Madina
Maternity and Children Hospital, Madina Munawara,
which is the only referral center for the entire
Madina Region. Every patient referred to the
cardiology clinic was screened and only those found
From the Department of Pediatrics, Madina Maternity and
Children Hospital, Madina Munawara.
Address reprint requests and correspondence to Dr. Abdul
Mateen: Department of Pediatrics, Madina Maternity and Children
Hospital, Madina Munawara.
Journal oCthe Saudi Hcan Association. Vol. 9, No. I, 1997
During the study period of 36 months, a total of
2106 children were referred for screening at the
pediatric cardiology clinic. Of this total, 1209
(57.4%) had CHD. Most of the children were Saudis,
but this was not specifically documented. The various
defects identified in our study are shown in Table 1
with comparative data of the prevalence of various
cardiac defects found in other parts of the world.
Ventricular septal defects (VSD) were found to be
the commonest lesion, followed by atrial septal
defects and pulmonary valve stenosis. Of 38
CONGENITAL HEART DISEASE
17
patients with atrioventricular canal defects, 30
(79%) patients had features of Down's syndrome.
The spectrum of complex defects in this study
showed 13 patients with dextrocardia, including 6
patients with situs inversus, 9 patients with
asymmetrical septal hypertrophy (all infants of
diabetic mothers), 7 patients had hypoplastic left
heart syndrome and double-outlet right ventricle, 5
patients with tricuspid atresia, 4 patients with Ebstein
anomaly, 2 patients with pulmonary atresia and
truncus arteriosus, and I patient with total anomalous
pulmonary venous drainage.
Table 1. Prevalence of various cardiac defects in different
countries.
Defects
California*
(n
-
Blackpoolt
163)10
(n
31.3
Ventricular septal defect
-
Nigeria
338)9
-
(n
28.1
635)4
Denmark
(n
5.0
-
5249)7
India
(n
-
Madinat
2(0)5
24.0
29.0
(n
-
1209)8
29.7
Atrial septal defect
6.1
8.3
7.5
9.4
12.0
26.0
Pulmonary stenosis
13.5
5.5
3.7
2.7
6.5
7.4
9.0
22.0
NM
5.9
12.6
2.6
7.0
11.0
NM
16.1
13.2
3.1
3.7
5.5
3.7
8.6
5.6
5.6
10.0
2.0
4.5
5.8
7.0
4.8
17.0
2.0
4.5
2.2
1.9
1.8
Patent ductus arteriosus
Atrioventricular canal defect
Tetralogy of Fallot
Coarctation of aorta
Transposition of great arteries
Aortic stenosis
3.7
4.1
0.6
4.7
2.5
1.6
Others
23.3
23.1
9.4
23.2
15.0
4.4
'Califomia, United States; tBlackpool, United Kingdom; tMadina, Saudi Arabia; NM - not mentioned.
Table 4. Distribution of various cardiac lesions in newborn
babies.
Table 2. Sex distribution of various cardiac defects.
Defects
Total
Male
Female
MIF
Defects
No. patients Percentage
(M)
(F)
Ratio
Patent ductus arteriosus
73
25
Ventricular septal defect
359
199
160
1.2
Ventricular septal defect
71
24
Atrial septal defect
314
195
159
38
149
99
67
22
165
96
92
16
0.9
1.0
0.7
1.4
Atrial septal defect
Pulmonary stenosis
Transposition of great arteries
Atrioventricular canal defect
65
16
15
10
22
5
5
3
26
23
12
17
14
6
0.8
2.8
Coarctation of aorta
Tetralogy of Fallot
9
5
3
2
16
13
34
6
7
19
2.7
1.7
1.8
Others
Total
31
295
II
100
Others
22
20
53
Total
1209
628
581
1.0
Pulmonary stenosis
Patent ductus arteriosus
Atrioventricular canal defect
Tetralogy of Fallot
Coarctation of aorta
Transposition of great arteries
Aortic stenosis
Table 3. Age at detection of various lesions.
Age
VSD
ASD
PDA
AVCD
PS
Below I wk
33
42
48
4
7
3
I
1-4wk
38
23
25
6
9
6
4
1-3mo
64
59
22
7
21
5
3
3 - 12 mo
96
128
61
129
27
37
13
8
40
118
3
6
8
10
Over 12 mo
-
-
COARC FT
- -
VSD
ventricular septal defect; ASD
atrial septal defect; PDA = patent ductus arteriosus; A VCD
atrioventricular canal defect; PS
pulmonary stenosis; COARC = coarctation of aorta; FT = tetralogy of Fallot; AS
aortic stenosis; TGA
transposition of great arteries.
Journal oflhe Saudi Hean Association, Vol. 9. No. I. 1997
-
-
18
BRAT ETAL
There was no overall difference in the sex
distribution of the various defects (male/female ratio,
1.08: 1.0) but a difference in the sex ratio in some
lesions was prominent (Table 2). Males
predominated in coarctation of aorta, transposition of
great arteries, and aortic stenosis, while patent ductus
arteriosus and tetralogy of Fallot were common in
females.
Age of Patients at Detection
Table 3 shows the ages at which these children
were detected to have CHD.
Fifteen of the 22
patients with transposition of the great arteries and 31
patients with complex defects were detected in the
neonatal period. The majority of patients with
acyanotic congenital heart disease were detected
later. Overall, the cumulative detection frequencies
were 14% at one week, 24% at one month, 40% at
three months, and 62% at one year. In this study,
there were 295 neonates (Table 4), and 58% of the
complex lesions were diagnosed in the neonatal
period. Transposition of the great arteries, tetralogy
of Fallot, coarctation of aorta, atrioventricular canal
defects, and other complex defects, including 7 cases
of hypoplastic left heart syndrome, 5 cases of
tricuspid atresia, 2 cases of pulmonary atTesia, and I
case of total anomalous pulmonary venous drainage,
accounted for 70 (24%) of the 295 cases.
Discussion
To determine the incidence of CHD accurate I y,
it is necessary to have accurate diagnosis of all
children who have the disease. Factors that affect the
underestimation of the incidence of CHD include the
lack of an efficient medical system for diagnosis of
these lesions, presence of minimal asymptomatic
lesions not detected at clinical examination, and
inability to diagnose CHD in infants who die soon
after birth.l
Similarly, large cardiac centers with facilities for
cardiovascular surgery that treat patients referred by
physicians in the periphery only reflect the
population of patients with severe malformations.
Compared to this, the patients minimally selected, as
in our study, referred either because of a known or
suspected cardiac lesion, represent a true pattern of
CHD in our region.
There are many similarities as well as
dissimilarities in our results when compared with
Journal of the Saudi Heart Association, Vol. 9, No. I, 1997
other studies (Table I ). Ventricular septal defect
remains the commonest single defect with a
prevalence of 29.7% in our series. However, atrial
septal defects, pulmonary stenosis, and patent ductus
arteriosus are seen in a higher percentage of our
patients. Obstructive aortic lesions, such as aortic
valve stenosis and coarctation of aorta, were seen less
frequently as compared to a higher percentage of
these lesions among American and European children
(Table I).
Regional differences in the prevalence of various
cardiac lesions have been well documented. I-I 0
Studies of ethnic influences on the pattern of
congenital heart lesions in the United Kingdom have
revealed a higher frequency of coarctation of aorta
among non-Asian infants (9% vs 3.1 %) compared to
Asian infants. I I There have also been similar reports
about paucity of obstructive aortic lesions from the
Kingdom of Saudi Arabia,12 indicating that racial
and genetic factors may influence the frequent
occurrence of obstructive aortic lesions among
different populations), \3
In the continents of Australia, Asia, and Africa,
the indigenous inhabitants may not have access to
good medical care, and cultural factors may prevent
infants from receiving medical care and may not
allow autopsies. Therefore, serious cardiac lesions
that cause both early death and very mild lesions may
be underrepresented in reported series which tend to
have inflated incidences for lesions like tetralogy of
Fallot that do not usually cause death in early infancy
but are conspicuous enough to be diagnosed. I
Regarding etiological factors, trisomy-21 was the
single easily identifiable factor. Thirty (79%) of the
38 patients with atrioventricular canal defects had
trisomy-21; and there is a known association between
the two conditions. 14.15
The age at which CHD is detected in a region
reflects the overall health facilities in that region. In
our study, the detection rates were 24% at I month,
40% at 3 months, and 62% at one year of age.
Although few data are available about the patterns of
CHD in Saudi Arabia, one study conducted by
Jaiyesimi et al,12 in the Qassim province, reported a
detection rate of 60% at one year; this is similar to the
detection rate (62%) in Madina Munawara. However,
this falls short of the cumulative detection rates in
Europe where 82% of the patients were detected by
one year of age in a study conducted by Bound and
Logan.9
19
CONGENITAL HEART DISEASE
It is still necessary to improve our detection
rates. despite the presence of an effective network
of health care in this region. An increasing
awareness of this problem among doctors in the
primary health care institutions. post-natal wards, as
well as major provincial health institutions is
needed. There is also a need for training
pediatricians in major provincial institutions for
echocardiographic evaluation of patients to improve
the detection rate of CHD. Last, but not least, multicenter studies are needed to investigate the true
patterns of CHD in the Kingdom of Saudi Arabia.
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