International Journal of Contemporary Pediatrics

Jan M et al. Int J Contemp Pediatr. 2023 Jun;10(6):806-812

http://www.ijpediatrics.com pISSN 2349-3283 | eISSN 2349-3291

DOI: https://dx.doi.org/10.18203/2349-3291.ijcp20231482
Original Research Article

Extra cardiac birth defects in children with congenital heart anomalies

Muzafar Jan, Parvaiz Rafieq Sohil, Younus Ramzan Khan*, Amber Bashir

Department of Pediatrics, Government Medical College Srinagar, Jammu and Kashmir, India

Received: 28 March 2023

Accepted: 03 May 2023

*Correspondence:
Dr. Younus Ramzan Khan,
E-mail: dr.younusramzan97@gmail.com

Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under
the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial
use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

Background: Congenital heart disease (CHD) may be present with a syndrome containing known anomalies with
specific physical findings or can be present with manifestations of a systemic defect that affects the heart and other
systems. Aim of the study was to determine the prevalence and spectrum of extra-cardiac defects associated with
CHD.
Methods: This prospective and observational study was conducted in the age group of 1 day to 12 years with CHD
chosen by purposive sampling technique. Informed consent was obtained from parents or guardians. The CHD was
diagnosed on the basis of detailed clinical examination, chest X-ray and Electrocardiography and confirmed by 2D
echocardiography. The extra-cardiac defects were diagnosed based on standard definitions and various investigations
like CBC, LFT, KFT, USG abdomen and skull, CT, MRI, G-Band karyotype, fluorescence in situ hybridization
(FISH) studies for micro-deletions, audiometry, ophthalmologic evaluation and thyroid hormone profile.
Results: A total of 61,254 patients were examined during the study and 640 children were identified with CHD. So,
the prevalence was 10.14 per 1000 live births. Out 640 children enrolled in the study 352 (55%) were males and 288
(45%) were females with a male female ratio of 1.2:1 and 520 (81.3%) had acyanotic CHD (ACHD) and 120 (18.3%)
had cyanotic CHD (CCHD). The majority of the children with CHD belonged to the second birth order 360 (56.2%).
The extra-cardiac anomalies were present in 128 (20%) cases. Out of them 77 (12%) cases had extra-cardiac
anomalies associated with acyanotic CHD and 51 (8%) cases had extracardiac anomalies associated with cyanotic
CHD. Out of 128 cases with extra-cardiac defects 64 (50%) cases were syndromic, 51 (39.8) cases had multiple
congenital defects and 13 (10.2%) had laterality defects.
Conclusions: The CHD is often associated with various extra-cardiac abnormalities.

Keywords: CHD, Extra-cardiac defects, Syndrome, Echocardiography

INTRODUCTION and other systems.5-9 To assess the possible systemic

disease or congenital malformations syndromes, all
Congenital heart defect (CHD) is the most common systems of the body are examined. Extra-cardiac
congenital malformation among all birth defects leading abnormalities are found in 20-45% of children with
to morbidity and mortality among children. The burden CHDs.10 About 5-10% of patients have known
of CHD is high in developing countries like India, due to chromosomal abnormalities.11 Extra-cardiac defects are
the high birth rate and critical nature of CHD requiring termed as MAJOR if they had medical or surgical
expensive surgical and non-surgical interventions. The significance or serious cosmetic significance and termed
incidence of moderate to severe structural CHD in live- as MINOR if there are unusual morphologic features not
born infants is 6 to 8 per 1000 live births. 1-4 CHD may be having any serious medical, surgical, and cosmetic
present with a syndrome containing known anomalies significance. Extra-cardiac defects are divided into three
with specific physical findings or can be present with distinct groups: (A). Multiple congenital defects:
manifestations of a systemic defect that affects the heart Multiple congenital defects are defined as those having at
least one major or three minor congenital birth defects or

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 Jan M et al. Int J Contemp Pediatr. 2023 Jun;10(6):806-812

malformations that cannot be explained by an underlying variables were summarized as frequency and percentage.
syndrome or sequence.12 (B) Syndromes: It is group of Continuous variables were summarized as mean and SD.
signs and symptoms that occur together and characterize
a particular abnormality or condition. Those with RESULTS
chromosomal anomalies, single gene defects, or
teratogenic syndromes are counted among syndromes for A total of 61,254 patients were examined during the
example CATCH 22 syndrome, Williams Beurn, Down, study. Out of that, 640 children were identified with
Turer, Noonan, Holtoram, Alagille etc. 10,13 (C). Laterality CHD. So, the prevalence was 10.14 per 1000 live births.
defects; These are also known as embryonic left-right A total of 640 children with CHD who satisfied the
axis patterning or malposition complexes. They include a inclusion criteria were enrolled in this study. Out of them
spectrum of disorders that range in increasing severity 352 (55%) were males and 288 (45%) were females with
from isolated dextrocardia or situs inversus to situs a male female ratio of 1.2:1. In this study, 472 (73.8%)
inversus total is or heterotaxy.9,13-17 Thus, recognition of belonged to age group <6 years, out of them 260 (55.1%)
CHD in the newborn is important as this group of were male and 212 (44.9%) were female and 168 (26.2%)
abnormalities constitutes a significant proportion of belonged to age group > 6 years, out of them 92 (54.8%)
congenital malformations that present in neonatal life, were males and 76 (45.2%) were females as depicted in
and their early detection is important for appropriate table no.1. In this study, the majority of the children with
management, and short-term follow up for decision- CHD belonged to the second birth order 360 (56.2%)
making regarding a referral or waiting. 18-21 The main aim whereas 200 (31.2%) and 80 (12.5%) cases belonged to
of our study was to determine the various extra-cardiac first and third birth order respectively. In this study only
defects associated with CHD’s. 40 (6.2%) were preterm (<37 weeks) and 600 (93.8%)
were term (>37weeks) and 112 (17.5%) children were
METHODS low birth weight and 528 (82.5%) had normal birth
weight. Out of the 640 children enrolled with CHD, 520
This prospective and observational study was conducted (81.3%) had ACHD and 120 (18.3%) had CCHD. In
in the age group of 1 day to 12 years with CHD chosen ACHD group, 226 (35%) had VSD, 192 (30%) had ASD,
by purposive sampling technique in the post graduate 64 (10%) had PDA and 40 (6.3%) had other heart
department of pediatrics, GB pant children hospital disease. In CCHD group, 42 (6.5 %) had d-TGA, 32
government medical college Srinagar (GMC) over a (5.0%) had TOF and 14 (2.3 %) had TAPVC and 48 (5%)
period of two years between October-2020 to October- had other heart disease. In this study out of 640 only 8
2022. Our study was approved by the ethical committee (1.2%) children had a history of CHD (ASD and VSD) in
of GMC Srinagar via communication no. F (Min- their family member maternal or paternal side and
BOPGS) Acad/KU/22 02-02-2022 held on 29 and 30th consanguinity had seen in 132 (20.6%) cases. In this
September, 2021 under serial number 15. The children study, 6.2% of the mothers were below 19 years of age,
with CHDs included as study population after Informed 92.5% belonged to 19-35 years of age and 1.2% were
consent obtained from parents or guardians. CHDs was above 35 years of age at the time of conception. In this
diagnosed on basis of detailed clinical examination, chest study, the majority of the children 560 (87.4%) with
X-ray and Electrocardiography and confirmed by 2D CHD belonged to the lower class with Upper lower class
Echocardiography. Extra-cardiac defects were diagnosed 424 (66.2%) and lower class 136 (21.2%) respectively.
based on standard definitions and various investigations
like CBC, LFT, KFT, USG abdomen and skull, CT, MRI, Table 1: Age and gender distribution of study
G-Band karyotype, fluorescence in situ hybridization population.
(FISH) studies for micro-deletions, audiometry,
ophthalmologic evaluation and thyroid hormone profile. Percentage
Age (Years) Gender N
(%)
Inclusion criteria Male 260 55.10
<6 Female 212 44.90
Children (age 1 day to 12 years) with CHD with and Total 472 74
without extra-cardiac defects were included in study. Male 92 54.80
>6 Female 76 45.20
Exclusion criteria Total 168 26

Patients with patent ductus arteriosus in preterm In our study extra-cardiac anomalies were present in 128
neonates, bicuspid aortic valve and patent foramen ovale (20%) cases where as 512 (80%) cases had no extra-
were excluded. cardiac anomalies. Out of them 77 (12%) cases had extra-
cardiac anomalies associated with acyanotic CHD and 51
Statistical analysis (8%) cases had extra-cardiac anomalies associated with
cyanotic CHD as depicted in Table 2. Out of 128 cases
Data was entered in a Microsoft excel spreadsheet and with extra-cardiac defects 64 (50%) cases were
analyzed using SPSS version 22 software. Categorical syndromic, 51 (39.8) cases had multiple congenital

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 Jan M et al. Int J Contemp Pediatr. 2023 Jun;10(6):806-812

defects and 13 (10.2%) had laterality defects. The various

syndromes associated with CHD in our study (Table 3).

Table 2: Type of CHD and associated extra-cardiac

anomalies.

With
Without
extra- Total
CHD extra-CHD
CHD
N % N % N %
Acyanotic 77 12 443 69.2 520 81.2
Cyanotic 51 8 69 10.8 120 18.8
Total 128 20 512 80 640 100

Table 3: Distribution of syndromes in CHD'S, (n=64).

Percentage
Syndromes N
(%)
Downs 35 54.70
Edward 3 4.70
Digeorge 3 4.70
Holt Oram 2 3.10
Williams 3 4.70
Turner 1 1.60
Noonans 2 3.10 Figure 2: Patient with Edward syndrome having
Achondroplasia 3 4.70 clenched hand, overlapping fingers, rocker bottom
Congenital rubella syndrome 3 4.70 feet and echo documented Peri-membranous VSD and
Pierre robin syndrome 3 4.70 TOF.
Treacher Collins synddome 2 3.10
Cri-du-chat syndrome 1 1.60 In laterality defects 7 (53.8%) had right sided isomerism
Marfan syndrome 2 3.10 and 6 (46.2%) had left sided isomerism. Asplenia was
Patau syndrome 1 1.60 present in 7 cases while interrupted inferior vena cava
with polysplenia was seen in 6 cases. In multiple
congenital defects musculoskeletal defects were
commonest associated defect seen in 22 patients [43.1%].
The other systems affected were gastrointestinal system
in 9 (17.6%) cases, genitourinary system in 7 (13.7%)
cases, respiratory system in 4 (7.8%) cases, central
nervous system in 3 (5.9%) cases and other systems in 6
(11.7%) cases as depicted in Table 4.

Table 4: Various extracardiac anomalies in CHD.

Percent
Extracardiac anomalies N
(%)
Asplenia 7 54
Laterality
Polysplenia 6 46
defects
Total 13 100
Musculoskeletal 22 43
Gastrointestinal 9 17
Multiple genitourinary 7 14
congenital Respiratory 4 8
defects CNS 3 6
Others 6 12
Total 51 100

Among musculoskeletal defects 8 had club foot, 7 had

Figure 1: Patient with downs syndrome and echo
cleft lip with cleft palate, 5 had polydactyly and
documented nonrestrictive inlet VSD and karyotyping
syndactyly, 4 had eye and ear deformities, 3 had
showing Trisomy 21, 47XY.

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 Jan M et al. Int J Contemp Pediatr. 2023 Jun;10(6):806-812

congenital dysplasia of hip, 3 had choanal atresia, 3 had

ankyloglossia, 2 had scoliosis and 2 had arthrogryposis.
Among gastrointestinal anomalies 3 had hernia (inguinal
and umbilical), 2 had congenital hypertrophic pyloric
stenosis, 1 had duodenal atresia, 1 had biliary atresia and
1 had malrotation of gut. Among genitourinary anomalies
3 had cryptorchidism, 2 had hypospadias, 2 had posterior
urethral value and 1 had polycystic kidney disease.
Among respiratory anomalies 2 had laryngomalacia, 1
had trachea-esophageal fistula and 1 had diaphragmatic
hernia. Among CNS anomalies 2 had neural tube defects,
1 had hydrocephalus and corpus callosum agenesis.

Figure 5: Patient with arthrogryposis with echo

documented large inlet VSD and ASD.

Figure 3: Patient with cleft lip and palate with echo

documented 7 mm ASD and 2.5 mm PDA.

Figure 6: Patient with congenital diaphragmatic

hernia and echo documented bicuspid aortic valve,
mild aortic stenosis with tortuous arch.

DISCUSSION

CHD is the most common congenital malformation

among all birth defects leading to morbidity and mortality
among children. The burden of CHD is high in
Figure 4: Patient with polydactyly and CTEV with developing countries like India, due to the high birth rate
echo documented inlet VSD with perimembranous and critical nature of CHD requiring expensive surgical
extrusion. and non-surgical interventions. The estimated prevalence

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 Jan M et al. Int J Contemp Pediatr. 2023 Jun;10(6):806-812

of 8 per 1000 live births is accepted as the best anomalies.35 Similarly Bosi et al retrospectively evaluated
approximation.22 Available Indian studies had reported a CHD in Italy and found 24% of these patients had extra-
wide variation in the prevalence of CHD from 2.25 to 26 cardiac malformations.36 Similarly study done by
per 1000 live births.23-25 A recent systemic review also Abdullah et al in Saudi Arabia reported 28.5% incidence
reported a higher prevalence in Asia due to a high birth of extra-cardiac anomalies.37
rate and consanguinity, especially in Iran and India. In
this study, the prevalence is 10.14 per 1000 live births. In our study out of 128 cases with extra-cardiac defects
This may be high by being the largest referral center in 64 (50%) were syndromic, 51 (39.8) cases had multiple
Kashmir, availability of better diagnostic modalities, congenital defects and 13 (10.2%) had laterality defects.
improved survival of premature infants, and high birth Laterality defects were the least common found only in
rate. 13 cases of which 7 cases were right isomerism and 6
cases were left isomerism. Miller et al conducted a
In this study, out of 640 cases 352 (55%) were male and population-based surveillance study of 7984 live-born
288 (45%) were female children with a male female ratio and stillborn infants and fetuses with CHDs:
of 1.2:1. In a study by Begum et al, Singh et al, male to 1080(13.5%) had multiple, 1048 (13.1%) had syndromic
female ratio was found to be 0.9:1 and 1.1:1 respectively, and 161 (2.0%) had laterality defects.48 Similarly
and a study by Vaidyanathan et al and Tandan et al Tennstedt et al conducted a necropsy study in 815 fetuses
observed male to female ratio of 1:1 and 0.8:1. 26-29 Out of and found 85 (66%) had extracardiac birth defects with
the 640 children enrolled with CHD, the majority 520 central nervous system birth defects being the most
(81.3%) had ACHD and 120 (18.7%) had CCHD. The frequent.38 Gucer et al conducted autopsies in 305
majority had VSD (35%) followed by ASD in (30%) and children with CHDs and reported 140 (46%) had
PDA in (10%) which was in accordance with similar extracardiac birth defects with craniofacial birth defects
studies by Smitha et al who reported (40.7%) had VSD, being the most frequent.39 In all these studies the multiple
(19.06%) had ASD and (9.53%) had PDA. 30 In this congenital defects were more than syndromes. Downs
present study, the majority of the children with congenital syndrome was the commonest syndrome associated with
heart disease belonged to the lower (87.4%) class with CHD in our study occurring in 35 cases comprising
Upper lower class (66.2%) and lower class (21.2%) 54.7%. The prevalence of congenital heart disease in
according to modified Kuppusamy Socio-Economic downs syndrome was similar to the findings from other
Scale. A study by Agha et al and Tandon et al also studies, which have been between 44% and 62%.40 The
reported a high prevalence of CHD in children belonging study done by Greenwood et al found that 137 of 1566
to low socio-economic status. In this study out of 640 infants (8.8%) had congenital heart disease associated
only 8 (1.2%) children had a history of congenital heart skeletal abnormalities.41 Similarly Wallgren et al found
disease (ASD and VSD) in their family member maternal such an association in 161 out of 1000 children with
or paternal side.29,31 According to Nelson textbook of congenital heart disease (16.1%), with an excess of
pediatrics, the risk of occurrence increases if a 1st degree tetralogy of Fallot (27% of affected infants). 42 In our
relative (parent/sibling) is affected in (2-6%) whereas Study Among musculoskeletal anomalies, the most
when two first degree relatives are affected with CHD, common was club foot (8 cases), followed by cleft lip
the risk of subsequent child may reach to 20-30%.32 with or without cleft palate (7 cases) followed by
polydactyly and syndactyly (5 cases). In our study out of
In this study, 6.2% of the mothers were below 19 years, 640 patients with CHD 7 (13.7%) had genitourinary
92.5% belonged to 19-35 years and 1.2% were above 35 anomalies which was similar to study done by Wallgren
years at the time of conception. Similarly Best et al et al who reported out of 1000 patients with CHD 131
reported that advanced maternal age is not a risk factor (13.1%) had genitourinary anomalies, out of these 131
for CHD however there was a marginal risk of infants patients, 30 (23%) had hydronephrosis, 24 (18%) had
developing certain types of CHD among mothers aged renal agenesis, 15 (11%) had horseshoe kidney, 28 (21%)
more than 35 years.33 Our results were also supported by had genital malformations, and 56 (43%) had other
Miller et al who also observed that infants born to anomalies.48 In our study 9 (17.6%) cases had
mothers older than 35 years of age seemed to be at 20% gastrointestinal anomalies, among them 3 had hernia
increased risk of CHDs, while infants born to younger (inguinal and umbilical), 2 had congenital hypertrophic
mothers tended to be at decreased risk of CHDs. 13 In the pyloric stenosis, 1 had duodenal atresia, 1 had biliary
present study, consanguinity was noted in 20% of cases. atresia and 1 had malrotation of gut. Similarly study by
Similarly Gnanalingam et al also observed that parental Touloukiarr reported that four of five infants with both
consanguinity was noted in 12.5% of the control group trisomy 21 and duodenal atresia had malformations of the
compared to 31.1% of the CHD group.34 In our study atrioventricular septum.43 Fonkalsrud et al noted that 17%
extra-cardiac anomalies were present in 128 cases of infants with duodenal obstruction had coexisting
[20.0%] which was in accordance with retrospective congenital heart disease. On the other hand, congenital
study done by Pradat et al who observed the data of heart disease is only found in 5.2% of infants with jejunal
children with major CHD in Sweden, and found extra- or ileal atresia.44,45 This difference may result from the
cardiac malformation was identified in 25.7% of patients varying causes of the lesions. Similarly, Gallo et al found
after excluding 15% of the children with chromosomal 14 cases of esophageal atresia in 1354 cases of congenital

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 Jan M et al. Int J Contemp Pediatr. 2023 Jun;10(6):806-812

heart disease (1.0%) but also did not report the frequency 4. Working group on management of congenital heart
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International Journal of Contemporary Pediatrics | June 2023 | Vol 10 | Issue 6 Page 812