A total of 888 children attended the cardiac clinic during the study period [see Fig. 2]. Of these, 33.7% [n = 299] had CHD, whereas 36.1% were normal [n = 321] and 30.2% [n = 268 ] had acquired heart disease [RHD, n = 220 and DCMP, n = 22]. The majority of children with rheumatic heart disease [RHD] were diagnosed through a school-based screening program [5]. Females represented 59.2% [n = 177] of cases with CHD. The median age at diagnosis for patients with CHD was 12 months [QR: 4.0–48]. The median age of cases with Acyanotic CHD was 16 months [IQR:5–60] while that of cases with cyanotic CHD was 10 months [IQR:3.5–43]. Neonates [children aged < 28 days] comprised only 7.4% [n = 22] of cases, with infants [children aged 1-12months] representing most cases at 54.6% [n = 125], [see Table 1]. None of the cases had prior prenatal diagnosis.
Table 1
Baseline Characteristics in patients with CHD, N = 299
Variable
|
Number (%)
|
Gender, Female
|
177(59.2%)
|
Median age at diagnosis (IQR), months
|
12 (4–48)
|
Age Category at initial diagnosis
|
|
Neonates (0–28 days)
|
22(7.4%)
|
Infants (1–12 months)
|
125(41.8%)
|
Young child (1–5 years)
|
83 (27.8%)
|
Older Child (6–17 years)
|
69(23.0%)
|
CHD Type
|
|
Acyanotic CHD
|
242(80.9%)
|
Cyanotic CHD
|
57(19.1%)
|
The median age of cases with Acyanotic CHD was 16 months (IQR:5–60) |
The median age of cases with cyanotic CHD was 10 months (IQR:3.5–43) |
Acyanotic CHD comprised of 80.9% of all cases of CHD. The commonest CHD seen was ventricular septal defect [VSD] in 19.4% [n = 58] of cases [see Table 2], followed by atrioventricular septal defect [AVSD] in 17.1% [n = 51] and patent ductus arteriosus (PDA) in 15.7% [n = 47]. Complete AVSD was the commonest subtype of AVSD seen [complete, n = 45; transitional, n = 1 and partial, n = 5]. The clinical phenotype of Down syndrome was identified in 46 of cases with AVSD all of whom had complete AVSD. Isolated atrial septal defects [ASDs] were seen in 10% of cases [n = 30] and a patent foramen ovale [PFO] seen in 9.2% [n = 21]. Obstructive lesions were rarely seen, with pulmonic stenosis seen in 3.7% [n = 11] and coarctation of the aorta in 1% [n = 3].
Table 2
Distribution of Acyanotic CHD, n = 242
Type of Lesion
|
N (Percentage of Total CHD)
|
Ventricular septal defect (VSD)
|
58 (19.4%)
|
Patent Ductus Arteriosus (PDA)
|
47 (15.7%)
|
Atrial septal defects (ASD)
|
30 (10.0%)
|
Atrioventricular septal defects (AVSD)
|
51 (17.1%)
|
Pulmonary stenosis (PS)
|
9 (3.7%)
|
Coarctation of Aorta (COA)
|
3 (1.0%)
|
Others
|
47 (15.7%)
|
Note: Others included defects such as Small Patent Foramen ovale (n = 22), bicuspid aortic valve (4), Isolated persistent LSVC (4 cases), among other defects |
Table 3
Distribution of cyanotic CHD, n = 57
Type of Lesion
|
N (Percentage of Total CHD)
|
Tetralogy of Fallot (TOF)
|
15 (5.0%)
|
Double Outlet RV (DORV)
|
12 (4.0%)
|
Truncus arteriosus (TA)
|
10 (3.4%)
|
d-Transposition of the Great Arteries (d-TGA)
|
4 (1.3%)
|
Tricuspid Atresia
|
5(1.7%)
|
Pulmonary atresia (With one case having intact ventricular septum
|
5(1.7%)
|
Others
|
6 (2.0%)
|
Others included Double inlet left ventricle (n = 2), Hypoplastic Left heart syndrome (n = 1), Ebstein’s anomaly (n = 1), A univentricular heart of RV morphology with pulmonary atresia (n = 1) and a case of left atrial isomerism with common atrium, double out RV and malposed great arteries |
The commonest cyanotic heart diseases seen was tetralogy of Fallot [TOF] in 5% [n = 15] of CHD cases, followed by double outlet right ventricle [DORV] in 4% [n = 12] and truncus arteriosus (TA) in 3.4% [n = 10]. Cases of dextro-transposition of the great Arteries (d-TGA) was seen in 1.3% [n = 4]. During the study only 1% [n = 3] with critical duct dependent lesions were seen [one each with D-TGA with intact ventricular septum, pulmonary atresia with intact ventricular septum and hypoplastic left heart syndrome].
At initial evaluation, 75% [n = 224] of all CHD cases needed definitive intervention, whereas 14% [n = 40] were stable for observation and follow up to determine long-term care plan [see Fig. 3]. Unfortunately, 1% of cases [n = 3] were considered inoperable at initial evaluation [a ten-year-old child with VSD and Eisenmenger syndrome, and two older children having heterotaxy syndrome, complex CHD and severe pulmonary arterial hypertension]. During the study period, 14% of children [n = 32] in need of definitive intervention had accessed surgical or catheter-based therapy. Three quarters of the cases who had access to surgical or transcatheter therapy [n = 24] had definitive care at the UHI including all 12 cases who underwent catheter-based interventions [PDA device occlusion/coil embolization, n = 11 and balloon pulmonary valvuloplasty n = 1]. The catheterization team at the UHI performed all the interventions independently. The cases who underwent surgical care at the UHI included lower risk procedures such as ASD closure [n = 5], PDA ligation [n = 4], coarctation of aorta repair [n = 1], VSD closure [n = 1] and TOF repair [n = 1]. The surgical team at the UHI independently operated on all cases done locally except for 3 ASD cases which were done during surgical camps organized with visiting surgical teams. The local teams combined (both catheterization and surgical) independently performed 87.5% [n = 21] of the cases done locally. The remaining 8 cases obtained definitive surgical care from abroad [VSD closure = 5, ASD closure = 1 and DORV + PS = 1]. All cases who had definitive surgical or transcatheter care where sponsored by grants from charity organizations, except in one case [coarctation of the aorta repair that was subsidized by the UHI]. In two cases, parents declined surgery or transcatheter therapy when they were offered opportunity despite counselling. These included the case of a 15-year-old athletic girl diagnosed with a large secundum ASD through a school based RHD screening program and another case of a 7-year-old girl with a large PDA whose single mother could not find an adult relative to look after 3 younger siblings during a proposed short hospitalization for PDA device closure.