Introduction: Inbreeding increases homozygotes for autosomal recessive disorders resulting in high prevalence of congenital heart disease (CHD). This cross-section looks whether consanguineous marriage (CM) makes CHDs more complex. Subjects and Methods: This was an observational study which looked at the cross-section of a cohort of consecutive 113 patients having different CHD who visited Nizam Institute of Medical Sciences, Andhra Pradesh, during the year 2016 for surgery or intervention. The definitive diagnosis of CHD was supported by clinical profile, chest X-ray, echo, ultrasound abdomen, and cardiac catheterization which is appropriate. The study population was divided into CM group and distance marriage (DM) group. The categorical variables were analyzed using descriptive statistics. Results: Out of 113 patients with CHD, 61 (54%) were females. A majority 104 (94%) were Hindu. About 74% of patients were from below poverty line category. Approximately 27% and 73% patients were born out of consanguineous and DM, respectively. Most of the patients were from a rural background, and 63.7% had an education level of ≤10th class. Approximately, a total of 50 (44.2%) patients had complex CHDs (CCHDs). CCHD had significant association with CM (odds ratio: 2.60 95% confidence interval: 1.11–6.09, and P = 0.02). Conclusion: As there was a significant association of CCHD with inbreed marriage, this observation discourages CM.

1. Official Publication of Nigeria Cardiac Society
NIGERIANJOURNALOF
CARDIOLOGY
Volume 15 | Issue 2 | July-December 2018
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http://www.nigjcardiol.org
NigerianJournalofCardiologylVolume15lIssue1lJanuary-June2018lPages1-70
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2. 98 © 2019 Nigerian Journal of Cardiology | Published by Wolters Kluwer - Medknow
Original Article
INTRODUCTION
Consanguineous marriage (CM) is associated with
an increased susceptibility to congenital heart
disease (CHD).[1]
Children born with CHD suffer from high
mortality, morbidity, high health‑care costs, nonavailability
of advanced care, and the need for lifelong care.[2,3]
There
are several recognized risk factors for CHD such as family
history of CHD, inbreed marriage, genetic causes, vitamin
deficiency, use of certain medications, maternal diabetes,
and age of parents.[4‑7]
This study aimed to find whether CM is a potential risk
factor for complex congenital heart disease(CCHD) among
the patients with CHD attending a tertiary care hospital
in South India.
Consanguineous marriage makes congenital heart diseases
more complex
Ramachandra Barik, Prafulla Kumar Swain1
, Mimansa Barik2
Department of Cardiology, All India Institute of Medical Sciences, 1
Department of Statistics, Utkal University,
2
Mother's Public School, Bhubaneswar, Odisha, India
Introduction: Inbreeding increases homozygotes for autosomal recessive disorders resulting in high
prevalence of congenital heart disease (CHD). This cross‑section looks whether consanguineous
marriage (CM) makes CHDs more complex.
Subjects and Methods: This was an observational study which looked at the cross‑section of a cohort
of consecutive 113 patients having different CHD who visited Nizam Institute of Medical Sciences,
Andhra Pradesh, during the year 2016 for surgery or intervention. The definitive diagnosis of CHD was
supported by clinical profile, chest X‑ray, echo, ultrasound abdomen, and cardiac catheterization which
is appropriate. The study population was divided into CM group and distance marriage (DM) group. The
categorical variables were analyzed using descriptive statistics.
Results: Out of 113 patients with CHD, 61 (54%) were females. A majority 104 (94%) were Hindu. About 74%
of patients were from below poverty line category. Approximately 27% and 73% patients were born out of
consanguineous and DM, respectively. Most of the patients were from a rural background, and 63.7% had
an education level of ≤10th
class. Approximately, a total of 50 (44.2%) patients had complex CHDs (CCHDs).
CCHD had significant association with CM (odds ratio: 2.60 95% confidence interval: 1.11–6.09, and P = 0.02).
Conclusion: As there was a significant association of CCHD with inbreed marriage, this observation
discourages CM.
Keywords: Complex congenital heart disease, consanguineous marriage, distance marriage, South India
Abstract
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DOI:
10.4103/njc.njc_40_17
Address for correspondence: Dr. Ramachandra Barik, All India Institute of Medical Sciences, Bhubaneswar ‑ 751 019, Odisha, India.
E‑mail: cardioramachandra@gmail.com
How to cite this article: Barik R, Swain PK, Barik M. Consanguineous
marriage makes congenital heart diseases more complex. Nig J Cardiol
2018;15:98-101.
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3. Barik, et al.: Consanguineous marriage makes congenital heart diseases more complex
Nigerian Journal of Cardiology | Volume 15 | Issue 2 | July‑December 2018 99
SUBJECTS AND METHODS
A total of 113 consecutive patients with CHD attending
cardiology clinic of our institute were enrolled in the
study during 2015–2016. This government hospital is the
main referral center for cardiac intervention and cardiac
surgery for most of the socioeconomically challenged
population in the state of Andhra Pradesh. The diagnosis
of CHD was made using clinical profile, two‑dimensional
echocardiography, and chest X‑ray. Ultrasound abdomen
was done in most of the cases to rule out associated
abdominal viscera malformation. CHD among the siblings
was also looked for in all the studied patients. Referral
slips were scrutinized for already accomplished genetic
evaluation. The demographic characteristics such as parental
age, consanguinity, gravida, religion, parental education,
occupation, socioeconomic status, maternal health during
pregnancy, and history of CHDs in first‑degree relation of
the patients and maternal smoking or alcohol intake or any
other significant illness in mother were documented. CM in
this study indicates a marriage between first‑cousins (father
had married his uncle’s daughter and parents are the
children of brother and sister in relation to each other).
Even though, the marriage between nice and uncle is
quite common, we have not included in this study. Simple
CHDs included mild pulmonary valve stenosis, a small,
uncomplicated atrial septal defect or ventricular septal
defect, patent ductus arteriosus, and successfully repaired
atrial septal defect, ventricular septal defect, patent ductus
arteriosus, and anomalous pulmonary venous connection.
Complex CHD, on the other hand, includes any complex
anatomical or physiological lesion as defined by the
Bethesda conference (Warnes CA, Liberthson R, Danielson
GK, Dore A, Harris L, Hoffman JI, Somerville J, Williams
RG, Webb GD. Task force 1: The changing profile of CHD
in adult life. J Am Coll Cardiol. 2001;37:1170‑75).
Statistical analysis
Statistical analysis was performed using test of proportion
online calculator (http://www.socscistatistics.com/tests/)
and https://www. medcalc.org/index.php. Differences
between groups were determined by the Chi‑square test
for categorical variables. The α error (P) was considered
significant when it was 0.05 at the β error of 0.20. The
odds ratio (OR), its standard error and 95% confidence
interval are calculated according to Altman, 1991. Ratio
and proportion were used for descriptive observations.
RESULTS
This study included a total of 113 patients having 19
different types of CHD [Figure 1]. Female accounted
DM CM Total
ALCAPA 0 1 1
ASD 18 5 23
AVCD 3 1 4
CCTGA 0 1 1
COA 5 0 5
DCRV 4 1 5
DORV 3 3 6
DTGA 0 1 1
GERBODE 1 1 2
PDA 2 4 6
PS 2 0 2
SI VSD 1 0 1
SMR 1 0 1
SUPRAAS 0 1 1
TAPVC 3 0 3
TOF 14 8 22
TRIA 0 1 1
TRUNCUS 1 0 1
VSD 24 3 27
0
5
10
15
20
25
30
AXISTITLE
A total 19 types CHD in DM and CM groups
Figure 1: The profile of simple congenital heart disease versus complex
congenital heart disease in the consanguineous marriage and distance
marriage group
54%. Only 29 patients (25.6%) belonged to the age
group of ≤1 year. Most of the patients were Hindu,
104 (92%) and were from rural areas. Of 113 patients,
31 (27.4%) were born out of CM [Table 1]. The level
of education was ≤10th
constituted 73 (65%). A total
of 27.4% of patients had the paternal age of ≥30 years
had no significant relation with the incidence of
CCHD [Table 1]. Maternal age of 25 years was seen
in 26 (23%). Most patients (n = 83, 74%) belonged to
below poverty line. A total of 50 (44.2%) had CCHD. The
CM had significant association with CCHD (OR: 2.83,
95% CI: 0.1.21–6.60, P = 0.01) [Figure 2 and Table 2]
and level of education (OR: 3.13, CI: 1.163–8.44 and
P = 0.02) [Figure 3]. Tetralogy of Fallot (TOF) was the
most common CCHD among the CM. Only patient
with dextrocardia with VSD had situs in versus in the
ultrasound abdomen.

4. Barik, et al.: Consanguineous marriage makes congenital heart diseases more complex
100 Nigerian Journal of Cardiology | Volume 15 | Issue 2 | July‑December 2018
DISCUSSION
In our study, out of 113 cases with CHD, consanguinity was
observed in 31 patients (28.3%) which is significantly quite
high in comparison to previously reported researches.[7‑9]
This kind of higher incidences of CHD in CM may be
due to the higher prevalence of inbreeding marriage in
Andhra Pradesh, lack of awareness and low‑socioeconomic
status. As we have mentioned earlier, most of the patients
who attend this hospital are financially challenged, which
could be an apparent bias for this observation. This kind
of observation is also further supported by only 25% of
people who had attended the dedicated cardiac center for
definitive care ≤1 year of age. Both simple CHD (SCHD)
and CCHD had the higher prevalence in CM group with
TOF being the most common CCHD in our study.[10]
Limitation
Small sample size and limited genetic testing for recessive
trait analysis were the major limitations in this study.
CONCLUSION
As there was a significant association of CHD with
inbreed marriage, this observation discourages CM to
reduce CHDs. We hope a larger observation to consider
causal association would further provide strength to this
finding.
Financial support and sponsorship
Nil.
Conflicts of interest
Dr. Ramachandra Barik has collected data and had prepared
the manuscript, Dr. Prafulla Kumar Swain has provided
12
51
19
31
0
10
20
30
40
50
60
70
80
90
Consanguineous Distance marriage
Number
Types of CHD
Complex
Simple
Figure2:Thedistributionofcongenitalheartdiseaseinconsanguineousand
distance marriages (odds ratio = 2.60, 95% confidence interval: 1.11–6.09)
24
48
7
34
0
10
20
30
40
50
60
70
80
90
Consanguineous Distance marriage
Number
LEVEL OF EDUCATION
10th
class
10th
Class or less
Figure 3: The patients having ≤10th
class versus higher education
had (odds ratio = 2.43, 95% confidence interval: 1.01–6.23) times odds
of consanguinity marriage than distance marriage
Table 1: Epidemiological, clinical profile of study population
Characteristics Category Frequency (%)
Gender Male 52 (46)
Female 61 (54)
Community Hindu 104 (92)
Muslim 7 (6.2)
Christian 2 (1.8)
Marital status CM 31 (27.4)
DM 82 (72.6)
Area of residence Rural 84 (74.3)
Urban 29 (25.7)
SES BPL 83 (73.5)
APL 30 (26.5)
Education level ≤10th
class 72 (63.7)
≥10th
class 41 (36.2)
Types of CHD Simple CHD 63 (55.8)
Complex CHD 50 (44.2)
Paternal age ≤30 years 82 (72.6)
30 years 31 (27.4)
Maternal age ≤25 years 87 (77)
25 years 26 (23)
CHDs – Congenital heart diseases; CM – Consanguineous marriage;
DM – Distance marriage; SES – Socioeconomic status; BPL – Below
poverty line; APL – Above poverty line
Table 2: Relative strength of association of congenital heart
disease with various congenital heart disease
Characteristics Category CM DM χ2
P
Types of CHD Simple 12 (19.05) 51 (80.95) 5.03 0.021*
Complex 19 (31.0) 31 (62.0)
Community Hindu 29 (27.88) 75 (72.12) 0.132 0.53
Others 2 (22.22) 7 (77.78)
Residence Rural 22 (26.19) 62 (73.81) 0.254 0.39
Urban 9 (31.03) 20 (68.97)
SES BPL 23 (27.71) 60 (72.29) 0.012 0.55
APL 8 (26.67) 22 (73.33)
Education level ≤10th
class 24 (33.33) 48 (66.67) 3.47 0.047*
10th
class 7 (17.07) 34 (82.93)
Parental age ≤30 years 24 (29.27) 58 (70.73) 0.505 0.322
30 years 7 (22.58) 24 (77.42)
Maternal age ≤25 years 26 (29.88) 61 (70.12) 1.141 0.209
25 years 5 (19.23) 21 (80.77)
CM – Consanguineous marriage; DM – Distance marriage;
SES – Socioeconomic status; BPL – Below poverty line; APL – Above
poverty line; CHD – Congenital heart disease

5. Barik, et al.: Consanguineous marriage makes congenital heart diseases more complex
Nigerian Journal of Cardiology | Volume 15 | Issue 2 | July‑December 2018 101
statistical analysis support and Miss Mimansa Barik has
supported the language editing for the manuscript.
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