Clinical spectrum of congenital
heart diseases in a tertiary care hospital
Phuljhele S.1, Dewangan
S.2, Thombre P 3
1Dr. Sharja Phuljhele, Professor, 2Dr. Shashikant Dewangan, Assistant
Professor, 3Dr. Pranali Thombre, Resident; all authors are affiliated
with Department of Paediatrics, Pt J.N.M. Medical College, Raipur, CG,
India
Address for
correspondence: Dr. Shashikant Dewangan, Email:
drshashikantdewangan@gmail.com
Abstract
Introduction-
In developing countries congenital heart diseases (CHDs) causes deaths
of thousands of children. A study is necessary to understand incidence
and clinical spectrum of CHDs so that early diagnosis and intervention
can be done. Material
and Methods – Newborns and children up to the
age of 14 years attending pediatric OPD as well as admitted in ward
with CHDs enrolled for study. Data were analyzed using SPSS system
version 21. Results-
A total of 400 children with CHDs included for final analysis. Male to
Female ratio is 1.78:1.Most common CHDs was Ventricular septal defect
followed by Tetralogy of fallot. Major symptoms were cough, difficulty
in breathing, poor weight gain and suck rest suck cycle. Maternal risk
factors found were consanguinity, maternal diabetes mellitus, maternal
hypertension, maternal seizure disorder, maternal obesity, systematic
lupus erythematosus etc. Conclusion-
VSD is most common acyanotic CHD. Males are more affected with CHDs.
Consanguinity and Maternal Diabetes are important risk factors for
CHDs.
Key words:
Congenital heart diseases, Echocardiography, clinical features,
Maternal risk factors
Manuscript received 11th
September 2016, Reviewed:
24th September 2016
Author Corrected:
5th October 2016,
Accepted for Publication: 18th October 2016
Introduction
Congenital Heart Diseases (CHDs) is commonest of all congenital lesions
and most common type of heart disease in children [1]. Congenital Heart
Diseases (CHDs), in a definition proposed by Mitchell et al is, a gross
structural abnormality of the heart or intrathorasic great vessels that
is actually or potentially of functional significance [2].
The reported incidence of CHDs is 8-10\1000 according to various
studies from different parts of world, with higher rates in stillbirth,
spontaneous abortions and prematurity [3]. Various studies reported
incidence of CHDs in India from 0.8 to 4.2 per 1000 live births [4-9].
Approximately 10 % of infant mortality in our country is due to CHDs.
In developing countries CHDs causes deaths of thousands of children
[10]. Due to improved and more available diagnostic facilities many
CHDs are diagnosed in initial years of life. In spite of early
diagnosis overall prognosis is still poor because of lack of centres
expert in correction of CHDs.
Early, accurate diagnosis and timely intervention is the key for better
prognosis in CHD. Emergence of cross-sectional echocardiography and
then colour flow mapping in 1980s has provided a unique tool to study
noninvasively the change in form and function of congenitally malformed
hearts also the response and sequelae of interventions. Elder and Hertz
of Lund university in Sweden received the Lasker award in 1977 for
their pioneering work in echocardiography during 1950s [11].
Echocardiography is a cheap and non-invasive modality with the
advantage of reproducibility of results, instantaneous images and
reliable level of accuracy. Now a day’s 2D echocardiography
with colour doppler provides comprehensive means for evaluating
virtually all forms of CHDs both in adults and children [12]. In India
pediatric echocardiography is not widely available even further optimal
use of this technology is hampered by factors such as cost, lack of
skilled persons and the absence of appropriate probes. The few studies
carried out in India were either community or school based study they
did not indicate clinical profile of CHD. A good reflection of
incidence and pattern of CHD can be obtained by study of congenital
heart disease in general hospital which serves majority of population
around. Hence we decided to describe the spectrum of CHDs seen in a
tertiary care hospital.
Material
and Methods
This prospective observational study was carried over 4 year from June
2011 to June 2015 on children from newborn to 14 years of age admitted
in paediatric ward and attending OPD with cardiovascular and
respiratory problems in Dr. BRAM hospital Raipur. After enrolment
detailed history of patients was taken to know there clinical
presentation and complete clinical examination was also done. Diagnosis
of CHDs were established by detailed 2-D Echocardiography and colour
doppler. Relevant investigations like Xray, ECG, complete blood count ,
serum investigations were also carried out. All the data related to
history, examination and investigations was filled in pro forma.
Datasheet was prepared and analysed by using SPSS system version 21.
Two dimensional and colour doppler echocardiography were done with
Neonatal (12MHz) and Pediatric (8MHz) sector transducer.
Echocardiographic examinations were completed according to
recommendation of American society of Echocardiography (ASE)
[13].Complete doppler examination was according to the recommendations
of ASE.
Children with cardiovascular and respiratory problem in whom diagnosis
was confirmed by echocardiography were included in study. Operated
cases of CHDs and those with acquired heart diseases were excluded from
study.
Result
A total of 486 children from newborn to 14 years of age with heart
diseases were examined over a period of 4 years. Out of which 35 cases
were already operated and 51 were acquired heart diseases hence
excluded from study. Total 400 children with CHDs were included in
study for final analysis.
Table 1: Age and sex
distribution of children with congenital heart diseases
Types
of CHD
|
Females
|
Males
|
Total
(%)
|
Newborns
|
16
|
32
|
48 (12%)
|
<
6 months
|
56
|
72
|
128 (32%)
|
6-12
months
|
16
|
40
|
56 (14%)
|
1-2
yrs
|
16
|
32
|
48 (12%)
|
>
2 yrs
|
40
|
80
|
120 (30%)
|
Total
(%)
|
144(36%)
|
256 (64%)
|
400
(100%)
|
In our study higher prevalence of CHDs in males (64%) as
compared to female (36%)
Table 2: Distribution of
various congenital heart diseases
Types
of CHD
|
Males
|
Females
|
Total
(%)
|
VSD
|
70
|
46
|
116
(29%)
|
PDA
|
46
|
29
|
75((18.7%)
|
ASD
|
24
|
22
|
46(11.5%)
|
COA
|
10
|
9
|
19(4.7%)
|
TOF
|
58
|
22
|
80(20%)
|
d-TGA
|
5
|
2
|
7(1.75%)
|
Tricuspid
atresia
|
5
|
1
|
6(1.5%)
|
TAPVC
|
4
|
2
|
6(1.5%)
|
Others
|
34
|
11
|
45(11.25%)
|
Total
|
256
(64%)
|
144
(36%)
|
400
(100%)
|
In this study most common CHD is Ventricular septal defect
(29%) followed by TOF (20%). This study found TOF as most common
cyanotic CHDs amounting to 64.5% of total cyanotic CHDs.
Table-3: Distribution of
different presenting symptoms
Symptoms
|
No
of patients
|
Percent
|
Cough
|
30
|
60.0
|
Difficulty
in breathing
|
31
|
62.0
|
Fever
|
22
|
44.0
|
SRS
cycle
|
23
|
46.0
|
Chest
inf
|
31
|
62.0
|
Cyanosis
|
18
|
36.0
|
Squating
|
6
|
12.0
|
Chest
pain
|
1
|
2.0
|
Poor
wt
|
36
|
72.0
|
Fatigability
|
30
|
60.0
|
Total
|
50
|
100.0
|
Major symptoms are cough (60%), difficulty in breathing
(60%), Poor wt gain (72%) and Suck rest suck cycle (46%) and Bluish
discolouration of body (36%).
Table-4: Association
with different maternal risk factors
Risk
factors
|
No.
Of Patients
|
Percent
|
Maternal
DM
|
5
|
1.25%
|
Maternal
Hypertension
|
4
|
1%
|
Consaguinity
|
10
|
2.5%
|
Primary
infertility
|
2
|
0.5%
|
Radiation
exposure
|
0
|
0
|
Fever
with rash
|
3
|
0.75%
|
Seizure
disorder
|
4
|
1%
|
Maternal
obesity
|
1
|
0.25%
|
SLE
|
2
|
0.5%
|
NO
risk factor
|
369
|
92.25%
|
In 92.25% cases no maternal risk factor found. In 10 (2.5%)
cases consanguinity was found. Other maternal risk factors were
maternal diabetes mellitus (1.25%), maternal hypertension (1%), seizure
disorder (1%) etc.
Discussion
In our study 70% children were diagnosed by the age of 2 years. In an
other Indian study 82.6% children were diagnosed between the ages of
0-3 years [4]. Delayed diagnosis of CHDs probably related to lack of
diagnostic facilities, parental unawareness, social taboos and
financial constraints. Similar findings were described in other studies
[14]. Delay in recognition of CHDs has serious problems in long term
outcome [15]. There may be increased morbidity and mortility associated
with delayed diagnosis [16].
In our study male to female ratio is 1.78:1. This finding is similar to
observation of other studies [6, 17,18 ]. Slightly higher prevalence of
males in our study may be related to gender bias towards males. In our
study most common CHD is VSD (29%) .VSD is most common CHD across the
globe with average incidence of around 34% [19]. Other studies found
incidence of VSD ranging from 34.6% -45% [4-9, 17, 18]. In many studies
that included adult population found ASD as most common CHD [20-21].
This variation is due to the fact that small VSDs close during
childhood and those with large VSDs die before adulthood.
We found higher incidence of TOF (20%) against the global average of
around 5% [19]. Our study shows incidence of PDA and ASD 18.7% and
11.5% respectively. This is in contrast to average incidence of 10 %
and 13 % of PDA and ASD respectively [19]. In a study from Kanpur
reported incidence of PDA and ASD were 14.6% and 18.9% respectively
[4]. Males have outnumbered female in TOF (2.63:1), VSD (1.52:1) and
PDA (1.58:1) while there is approximately equal distribution of ASD and
COA cases among males and females. In a study from bohemia there were
significantly more girls than boys with PDA (1:1.66) and TOF (1:1.12)
while a higher proportion of males were found with COA [22]. In an
Indian study that included 10641 children up to 15 years of age boys to
girls ratio in ASD was (4.3:1), in VSD was (1.6:1) and in PDA was
(1.5:1) [4]. No girl child with TOF was found.
Cough, difficulty in breathing, poor weight gain and recurrent chest
infection were major presenting symptoms in our study.
Poor weight gain and malnutrition is common in children with congenital
heart disease [23]. Other clinical observations are similar to findings
in other studies [24-25].
We found important clinical correlation of CHDs with consanguinity,
maternal diabetes mellitus, maternal hypertension and seizure disorder.
In a study from Saudi Arabia first cousin consanguinity was
significantly associated with VSD, ASD, Pulmonary stenosis and
Pulmonary atresia while there is no association found in TOF, PDA, COA,
Tricuspid atresia and Aortic stenosis [26]. In a study from South India
findings revealed that first cousin marriages and uncle-niece marriages
are equally significant in increasing CHDs [27]. Other studies also
found consanguinity as important risk factor for CHDs [28, 29, 30].
Materanal diabetes and maternal use of antiepileptics are well known
noninherited risk factor for congenital heart disease [31].
Preconceptional maternal diabetes was strongly associated with
cardiovascular malformation of early embryonic origin [32].
Epidemiological studies of congenital heart diseases (CHDs) are
essential for early detection, evaluation, proper management and to
understand natural course of disease. This study demonstrates
epidemiology of congenital heart diseases presenting to a tertiary care
hospital. Thus it is hoped that this study more or less represents the
spectrum of CHD in general population of Chhattisgarh region where Bhim
Rao Ambedkar Memorial hospital is major general hospital serving
population surrounding it.
Conclusion
In this part of world acyanotic CHDs are more common. VSD is most
common acyanotic CHD while TOF is most common cyanotic CHD. Higher
number of males are affected with CHDs. Around 44% of children are
symptomatic by the age of 6 months. VSD, PDA and TOF are much more
common in Males while ASD and COA have almost equal distribution among
males and females. Majority of them were present to clinician with poor
weight gain, difficulty in breathing, chest infection and fatigability.
Consanguinity and maternal diabetes mellitus are important risk factor
for CHDs.
Funding:
Nil, Conflict of
interest: None initiated.
Permission from IRB:
Yes
References
1. Schoen FJ. The Heart. In: Cortan RS, Kumar V, Robins SL, 6th ed.
Robins Pathologic Basis of Disease. Philadelphia: W.B. Saunders
Company, 1999; 543-600. [PubMed]
2. Hoffman JI, Kaplan S, Liberthson RR. Prevalence of congenital heart
disease. Am Heart J. 2004 Mar; 147(3):425-39. [PubMed]
3. Alabdulgader AA. Congenital heart disease in 740 subjects:
epidemiological aspects. Ann Trop Paediatr. 2001 Jun;21(2):111-8. [PubMed]
4. Kapoor R, Gupta S. Prevalence of congenital heart disease, Kanpur,
India. Indian Pediatr. 2008 Apr;45(4):309-11. [PubMed]
5. Mohd Ashraf, J Chowdhary, K Khajuria and AM Reyaz. Spectrum of
Congenital Heart Diseases in Kashmir, India. Indian Pediatr. 2009
Dec;46(12):1107-8. [PubMed]
6. Vaidyanathan B1, Sathish G, Mohanan ST, Sundaram KR, Warrier KK,
Kumar RK. Clinical screening for congenital heart disease at birth: a
prospective study in a community hospital in Kerala. Indian Pediatr.
2011 Jan;48(1):25-30. [PubMed]
7. Khali A, Aggarwal R, Thirupuram S, Arora R. Incidence of congenital
heart disease among hospital live births in India. Indian Pediatr. 1994
May; 31(5):519-27. [PubMed]
8. Thakur JS, Negi PC, Ahluwalia SK, Sharma R, Bhardwaj R. Congenital
heart disease among school children in shimla hills. Indian Heart J.
1995 May-Jun;47(3):232-5. [PubMed]
9. Chadha SL, Singh N, Shukla DK. Epidemiological study of congenital
heart disease. Indian J Pediatr. 2001 Jun;68(6):507-10. [PubMed]
10. Cohen AJ, Tamir A, Houri S, Abegaz B, Gilad E,
Omohkdion S, Zabeeda D, Khazin V, Ciubotaru A,
Schachner A. Save a child’s heart: we can and we should. Ann
Thorac Surg. 2001 Feb;71(2):462-8. [PubMed]
11. Allen HD , Lange LW, Sahn DJ ,Goldberg SJ, ultrasound cardiac
diagnosis. Pediatr Clin North Am. 1978 Nov;25(4):677-706. [PubMed]
12. Chung KJ, Simpson IA, Newman R, David JS, Frederick SS, Hesselink
JR. Cine magnetic resonance imaging for evaluation of congenital heart
disease: Role in pediatric cardiology compared with echocardiography
and angiography. J Pediatr. 1988 Dec;113(6):1028-35.
13. Campbell RM, Douglas PS, Eidem BW, Lai WW, Lopez L, Sachdeva R.
ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE 2014 appropriate use criteria
for initial transthoracic echocardiography in outpatient pediatric
cardiology. J Am Coll Cardiol. 2014 Nov 11;64(19):2039-60.
14. Rashid U, Qureshi AU, Hyder SN, Sadiq M. Pattern of congenital
heart disease in a developing country tertiary care center: Factors
associated with delayed diagnosis. Ann Pediatr Card 2016;9:210-5.
15. Pfammatter JP1, Stocker FP. Delayed recognition of haemodynamically
relevant congenital heart disease. Eur J Pediatr. 2001
Apr;160(4):231-4. [PubMed]
16. Massin MM, Dessy H. Delayed recognition of congenital heart
disease. Postgraduate Medical Journal. 2006;82(969):468-70. [PubMed]
17. Shah GS, Singh MK, Pandey TR, Kalakheti BK, Bhandari GP . Incidence
of congenital heart disease in tertiary care hospital. Kathmandu Univ
Med J (KUMJ). 2008 Jan-Mar;6(1):33-6. [PubMed]
18. L. Kasturi, A.V. Kulkarni, A. Amin and V.A. Mashankar.
Congenital heart disease: Clinical spectrum. Indian Pediatr. 1999 Sep;
36(9):953. [PubMed]
19. van der Linde D, Konings EE, Slager MA, Witsenburg M, Helbing WA,
Takkenberg JJ, Roos-Hesselink JW. Birth prevalence of congenital heart
disease worldwide: a systematic review and meta-analysis. J Am Coll
Cardiol. 2011 Nov 15;58(21):2241-7. doi: 10.1016/j.jacc.2011.08.025.
20. Rahman F, Salman M, Akhter N, Patwary SR, Anam K, Rahman MM, Hasan
Z, Uddin MJ, Khalil MM, Hafiiz MG, Zaman SM, Fatema N, Rashid MA,
Banerjee SK, Haque SS, Chowdhury NA. Pattern of congenital heart
diseases. Mymensingh Med J. 2012 Apr;21(2):246-50.
21. Shrestha B. Nepal's noble echocardiography-database with video
clips and color still images: a single individual's 6 years' experience
at the Echocardiography Lab of Nepal Medical College, Teaching
Hospital. Nepal Med Coll J. 2012 Sep;14(3):180-6. [PubMed]
22. Samánek M. Boy:girl ratio in children born with
different forms of cardiac malformation: a population-based study.
Pediatr Cardiol. 1994 Mar-Apr;15(2):53-7. [PubMed]
23. Cameron JW, Rosenthal A, Olsan AD. Malnutrition in hospitalized
children with congenital heart disease. Arch Pediatr Adolesc Med. 1995
Oct;149(10):1098-102. [PubMed]
24. Silove ED. Assessment and management of
congenital heart disease in the newborn by district pediatrician. Arch
Dis Child Fetal Neonatal Ed. 1994 Jan;70(1):F71-4. [PubMed]
25. el Hag AI. Pattern of Congenital Heart Disease in Sudanese
Children. East Afr Med J. 1994 Sep;71(9):580-6. [PubMed]
26. Becker SM, Al Halees Z, Molina C, Paterson RM. Consanguinity and
congenital heart disease in Saudi Arabia. Am J Med Genet. 2001 Feb
15;99(1):8-13. [PubMed]
27. Ramegowda S, Ramachandra NB. Parental consanguinity increases
congenital heart diseases in South India. Ann Hum Biol.
2006;33:519–28. [PubMed]
28. El Mouzan MI, Al Salloum AA, Al Herbish AS, Qurachi MM, Al Omar AA.
Consanguinity and major genetic disorders in Saudi children: a
community-based cross-sectional study. Ann Saudi
Med.2008;28:169–173. [PubMed]
29. Shieh JT, Srivastava D. Heart malformation: what are the chances it
could happen again? Circulation. 2009Jul 28;120(4):269-71. doi:
10.1161/CIRCULATIONAHA.109.878637. Epub 2009 Jul 13. [PubMed]
30. Bittles AH. A community genetics perspective on consanguineous
marriage. Community Genet. 2008;11(6):324-30. doi: 10.1159/000133304.
Epub 2008 Aug 5. [PubMed]
31. Patel SS, Burns TL. Nongenetic risk factors and congenital heart
defects. Pediatr Cardiol. 2013 Oct;34(7):1535-55. doi:
10.1007/s00246-013-0775-4. Epub 2013 Aug 21. [PubMed]
32. LoffredoCA, WilsonPD, FerenczC.Maternal diabetes: an independent
risk factor for major cardiovascular malformations with
increased mortality of affected infants. Teratology. 2001 Aug;
64(2):98-106.
How to cite this article?
Phuljhele S, Dewangan S, Thombre P. Clinical spectrum of congenital
heart diseases in a tertiary care hospital. Int J Med Res Rev
2016;4(12):2114-2119.doi:10.17511 /ijmrr. 2016.i12.06.