CARDIOVASCULAR JOURNAL OF AFRICA • Vol 22, No 6, November/December 2011
AFRICA
307
murmur was the reason for referral in 177 (33%) patients, heart
failure in 62 (12%), chest infection in 36 (7%), and cyanosis in
19 patients (4%).
Of the overall number of patients with CHD, 349 (65%) had
a single cardiac lesion, whereas the remaining 188 (35%) had
multiple defects. The most common single defect was atrial
septal defect (ASD), which was found in 125 (24%) patients,
followed by atrioventricular septal defect (AVSD) in 103 (19%),
and ventricular septal defect (VSD) in 76 (14%) cases. Patent
ductus arteriosus (PDA) occurred in 28 (5%) cases.
Other common defects such as combined ASD plus VSD with
other complex lesions was found in 77 (14%) patients, VSD plus
PDA was found in 42 (8%), and 40 (7%) had ASD plus PDA.
PDA was the most common cardiac defect associated with other
cardiac lesions. It was found in association with VSD and ASD
in 82 patients (15%) (Table 2).
The median age (range) at diagnosis with ASD was three
months (three days – 15 years) and for those with complex and
mixed cardiac lesions it was four months (two days – 18 years)
(Table 2). The rare associations of complex CHD were those of
double-outlet right ventricle and transposition of the great arter-
ies in two patients, pulmonary atresia with VSD in one patient
and a combination of complete atrioventricular septal defect with
tetrology of Fallot in three patients.
Discussion
Approximately 50% of the patients evaluated for DS had an asso-
ciated CHD. This finding is similar to internationally reported
figures.
9
ASD of secondum type was the most common single
congenital cardiac defect and was found in 125 of the 537 DS
patients (24%). This finding was not consistent with what has
previously been reported in Europe, Sudan and the USA, where
ASD was reported to occur in only 5% of the DS patients in
Europe and Sudan and 8% in the USA patients.
14-16
ASD of
secondum type has been reported in Mexico in 38% and Saudi
Arabia in 21% of patients with DS.
17,18
These high distributions
are similar to that in our report.
In most of the European countries, Sudan, Turkey and the
USA, AVSD was the most common cardiac defect.
14-16,19
In Asia
on the other hand, VSD was the most common single cardiac
defect, followed by ASD or AVSD.
18,20,21
In our series, AVSD
with a common atrioventricular valve was the second most
common CHD, found in 103 (19%) patients. In Guatemala, PDA
(29%) followed by VSD (28%) were the most common cardiac
defects
22
The isolated cardiac lesions represented about 65% of all
CHD in our study, compared with 80% in Guatemala,
22
74% in
Mexico,
17
and 78% in Turkey
19
(Table 3). Furthermore, patients’
age at diagnosis with ASD was younger than those with complex
and other associated cardiac lesions. This minimised the possible
bias that patients with more complex lesions died earlier before
diagnosis, compared with those with ASD.
On reviewing the literature (Table 3), it appears that the
frequency and distribution of CHD in DS varies in different
geographical regions. The reason for this difference in the
TABLE 1. CARDIAC EVALUATIONS OF
THE PATIENTS STUDIED
Number
537
M:F ratio
1:1.4
Reason for cardiac evaluation
Screening
237 (44%)
Murmur
177 (33%)
Heart failure
62 (12%)
Cyanosis
19 (4%)
Chest infection
36 (7%)
Others
6 (1%)
Age at diagnosing CHD
0–1 months
149 (28%)
>
1–6 months
230 (43%)
>
6 months – 1 year
66 (12%)
>
1–3 years
44 (8%)
>
3–5 years
18 (3%)
>
5–10 years
19 (4%)
>
10–18 years
11 (2%)
CHD, congenital heart disease.
TABLE 2. THE FREQUENCYAND TYPES OF CONGENITAL HEART DISEASE
Cardiac lesions
Patients,
n
=
537 (%)
Age*
M:F ratio
Single lesion
349 (65%)
4 months (3 days –18 years)
1:1.4
ASD
125 (23%)
3 months (3 days –15 years)
1.1:1
AVSD
103 (19%)
3 months (3 days – 12 years)
1:1.6
VSD
76 (14%)
4 months (4 days – 14 years)
1:1
PDA
28 (5%)
5 months (1 moth – 18 years)
1:2
TOF
13 (2%)
3 months (3 weeks – 9 months)
1:1
COA
4 (1%)
5 months (1 week – 6 months)
1:0.3
Multiple lesions
188 (35%)
4 months (2 days – 18 years)
1:1.4
ASD with VSD plus others
77 (14%)
1 year (2 days – 18 years)
1:1.5
VSD with PDA plus others
42 (8%)
1.5 months (1–3 months)
1:0.8
ASD plus PDA with others
40 (7%)
4 months (1.5 months – 1.5 years)
1:0.8
ASD with pulmonary stenosis
11 (2%)
30 days (4 days – 3 years)
1:3
Other rare associations
19 (4%)
2 months (2 days – 2 years)
1:1
ASD, atrial septal defect; AVSD, atrioventricular septal defect; VSD, ventricular septal defect; PDA, patent ductus arteriosus; TOF, tetralogy of
Fallot; CoA, coarctation of aorta.
*Median (range) age at diagnosing congenital heart disease.
