Jurnal Ilmiah Mahasiswa Kedokteran Universitas Airlangga

ORIGINAL ARTICLE
2019 August, X (02)

Profile of Acyanotic Congenital Heart Defect in Children at Dr. Soetomo

General Hospital Surabaya Period of January – December 2016

Katherine Fedora1, I Ketut Alit Utamayasa2*, Sri Purwaningsih3

1 Facultyof Medicine, Universitas Airlangga – Dr. Soetomo General Hospital, Surabaya, Indonesia
2 Department of Paediatrics, Faculty of Medicine, Universitas Airlangga – Dr. Soetomo General Hospital, Surabaya, Indonesia
3 Department of Pharmacology, Faculty of Medicine, Universitas Airlangga – Dr. Soetomo General Hospital, Surabaya,

Indonesia

ABSTRACT ARTICLE INFO

Introduction: Acyanotic congenital heart defect is a congenital structural Article history:

abnormality arising from incomplete formation of the heart or major blood vessels
which generally do not interfere with the amount of oxygen or blood that reaches the Received 05 August 2019
body's tissues so that cyanosis are rarely found in these patients. This research aims
to evaluate the profile of acyanotic congenital heart defect patients in Paediatric Received in revised form 16
August 2019
Cardiology Outpatient Clinic Dr. Soetomo General Hospital Surabaya period of
January – December 2016 based on 4 variables; gender, age, nutritional status, type
Accepted 20 August 2019
of congenital heart disease and symptoms. Until 2017, the profile of acyanotic
congenital heart defect in children in Outpatient Unit of Paediatrics Department Dr.
Soetomo General Hospital Surabaya has not been much researched, so this
Keywords:
research is expected to be used as learning reference for practitioners and other Acyanotic,
readers, and also as a reference in developing management strategies for children Congenital heart defect,
with acyanotic congenital heart defect in the future. Children,
Paediatrics,
Methods: This was a descriptive non-experimental study using cross-sectional Growth.
design and was performed by evaluating medical record of acyanotic congenital
heart defect patients in Department of Paediatrics Dr. Soetomo General Hospital
Surabaya period of January – December 2016 and analysed using Microsoft Excel.

Results: The results of this study revealed that majority of acyanotic congenital heart
defect patients were female (53.85%) with age range between 0 – <5 years old. Atrial
septal defect (ASD) was the most common type of acyanotic congenital heart defect
(49.8%), with sign and symptom that is commonly found in patients were failure to
thrive (61.94%). Majority of patients also suffered from growth disruption with most
of patients aged 0 – <5 years old are severely underweight (42.46%) and most of
patients aged 5 – 18 years old are having malnutrition (38.23%).

© 2019 Jurnal Ilmiah Mahasiswa Kedokteran Universitas Airlangga. All rights reserved.

* Correspondence: [email protected]

© Jurnal Ilmiah Mahasiswa Kedokteran Universitas Airlangga. All rights reserved.

Available at https://e-journal.unair.ac.id/juxta
 Jurnal Ilmiah Mahasiswa Kedokteran Universitas Airlangga 2019 August, X (02) Page 80

Introduction weight, and 1.1% above the normal range. For children aged
5–18 years old, nutritional status is categorized based on the
Congenital heart defect is a structural disorder that classification by Indonesian Ministry of Health 1974 and
arises from the formation of the heart or the main blood reveals that 22.1% were categorized as malnutrition,
vessels present at birth. This disorder can involve the walls 38.23% as undernutrition, 26.5% in the normal range, and
of the heart, heart valves, or arteries and veins near the 13.2% above the normal range. Atrial septal defect was the
heart. Disruption of the compartment can interfere with most frequent type, followed by ventricular septal defect,
blood flow through the heart. Blood flow can be slowed patent ductus arteriosus, pulmonary valve stenosis, aortic
down, flow to the wrong direction, or be completely valve stenosis, and coarctation of aorta, with the number of
blocked.1, 2 49.8%, 45.7%, 26.3%, 12.5%, 1.6%, and 0.4% respectively.
Until now, the etiology of the cause of congenital heart There was not any patient with atrioventricular septal defect
defect is still unknown. However, there are several factors found in this study. The most frequent sign and symptom
that are thought to increase the risk of developing that was found in this study is failure to thrive (61.9%), heart
congenital heart defect in children, such as heredity, murmurs (55.1%), cough (25.1%), shortness breath
genetic disorders, and smoking habits. Parents who have (17.4%), and runny nose (16.2%).
congenital heart abnormalities are more likely to have
children with congenital heart defect. Children who have Table 1. General characteristic of the patients.
genetic disorders, such as Down syndrome, often have
congenital heart defects. Smoking during pregnancy has Number of
Characteristics
also been linked to several congenital heart disorders, patients
including cardiac septal defects.1,2 Gender
Congenital heart defect can be classified into cyanotic Male 133(53.85%)
or acyanotic congenital heart defect. Cyanotic congenital
Female 114(46.15%)
heart defect is abnormalities that allow blood with low
oxygen levels to be distributed to body tissues, this causes Age
bluish color (cyanosis) on the skin, lips and nails. 0 - <5 years old 179(72.47%)
Meanwhile acyanotic congenital heart defect usually does 5 - <11 years old 47(19.03%)
not interfere with the amount of oxygen or blood that
11 - 18 years old 21(8.50%)
reaches the body's tissues, so cyanosis symptoms are
Growth status
rarely found in these patients.2
This study was intended to determine the profile of Age <5 years old
acyanotic congenital heart defect patients in Paediatric Severely underweight 76(42.46%)
Cardiology Outpatient Clinic Dr. Soetomo General Underweight 36(20.11%)
Hospital Surabaya period of January – December 2016.
Normal 65(36.31%)
Above the normal range 2(1.12%)
Age 5 - 18 years old
Methods
This was a descriptive non-experimental study using Malnutrition 15(22.06%)
cross-sectional design. The study was conducted at the Undernutrition 26(38.23%)
Paediatric Cardiology Outpatient Clinic, Dr. Soetomo Normal 18(26.50%)
General Hospital Surabaya, from January to April 2018.
Above the normal range 9(13.23%)
The population studied were all patients with acyanotic
congenital heart defect who were treated at Paediatric Source: research data, processed
Cardiology Outpatient Clinic, Dr. Soetomo General
Hospital Surabaya. This study obtained data from clinical Table 2. Types of acyanotic congenital heart defect of the
history and medical records of patients with acyanotic patients.
congenital heart defect. Data were analysed with Number of
descriptive statistics and presented in frequency Types of defect
patients
distribution tables.
Atrial septal defect (ASD) 123(49.80%)
Ventricular septal defect (VSD) 113(45.75%)
Results
Patent ductus arteriosus (PDA) 65(26.31%)
A total of 247 patients in outpatient unit of paediatric Pulmonary valve stenosis 31(12.55%)
department was included in this study. There were 46.1% Aortic valve stenosis 4(1.62%)
males and 53.8% were females, with a male to female ratio
Coarctation of aorta 1(0.40%)
of 1:1.2. In this study, 72.5% were diagnosed by 5 years
of age, 19% by 5–<11 years, and 8.5% by 11–18 years. Atrioventricular septal defect 0(0%)
Based on WHO growth standard for children aged below Source: research data, processed
5 years old, 42.5% were categorized as severely
underweight, 20.1% as underweight, 36.3% as normal
 Jurnal Ilmiah Mahasiswa Kedokteran Universitas Airlangga 2019 August, X (02) Page 81

Table 3. Sign and symptoms of the patients. Age Distribution in Acyanotic Congenital Heart Defect
Patients

Sign and symptoms Number of patients The difference in the age of diagnosis in children with
acyanotic congenital heart defect depends on the type and
Failure to thrive 153(61.94%)
complexity of the defects.5 Some patients may show typical
Heart murmur 136(55.06%)
symptoms in early life, while others may only be showing
Cough 62(25.10%)
minimal symptoms such as recurrent respiratory infections
Dyspnea 42(17.40%)
or difficulty in gaining weight, that makes it rarely detected
Runny nose 40(16.19%)
until adulthood.5 The high number of patients under 5 year
Fever 30(12.14%) of age shows a very good referral system and early
Cyanosis 26(10.53%) diagnosis.5 Several studies that have been done before, one
Edema 25(10.12%) of them by Ain et al. (2015), revealed that majority of patients
Chest pain 6(2.43%) with acyanotic congenital heart defect in RSUP M. Djamil
Vomit 6(2.43%) Padang were under 1 year of age with the frequency of
Clubbing finger 5(2.02%) 56.4%, while the remaining 43.6% were above 1 year old.
Icterus 5(2.02%) The result is also in accordance with previous study by Didik
Palpitation 4(1.62%) Hariyanto (2012) in Padang, that 62.2% of patients with
Sweating 3(1.21%) acyanotic congenital heart defect were under 1 year of age,
Seizure 2(0.81%) while the remaining 37.8% were over 1 year old.
Regurgitation 2(0.81%)
Lethargic 2(0.81%) Growth Status of Acyanotic Congenital Heart Defect
Liquid stool 2(0.81%) Patients
Headache 1(0.40%)
Retraction 1(0.40%) The presence of congenital defect in the heart structure
Syncope 1(0.40%) increase the metabolic demand and total energy
Hemoptoe 1(0.40%) expenditure of the body, while reduced body mass
Decreased appetite 1(0.40%) presentation tends to increase the basal metabolic rate,
Nausea 1(0.40%) which if not treated properly will worsen the child’s health
Sore throat 1(0.40%) condition.6
Melena 1(0.40%) Acyanotic congenital heart defect also tends to cause
Restless 1(0.40%) congestive heart failure, that leads to decrease in cardiac
Source: research data, processed output and water and salt retention.7 Therefore, the tissue
oxygen demand is not met optimally and causes the child
growth to be hampered.7
Discussion Other than that, symptoms experienced by children with
congestive heart failure such as dyspnea and tachypnea
Gender Difference in Acyanotic Congenital Heart that appear during eating cause children to feel tired, loss of
Defect Patients appetite, and lead to a decrease in the quantity of food
consumed by children.8
Females are predicted to have higher number of
Decrease of ghrelin in blood is also noted in acyanotic
incidents compared to males. This can be affected by the
congenital heart defect patients.6 Ghrelin causes inhibition
presence of biological differences in the structure of blood
of leptin, so it can be concluded that if there is a decrease in
vessels, in which the diameter of arteries in female is
ghrelin levels, it can be predicted there will be an increase in
smaller than male.3 This condition increases the risk of
leptin levels in blood.6 Both of these peptides play a role in
vascular endothelial dysfunction, thereby contributing to
the development of anorexia-cachexia syndrome in children,
the occurrence of pulmonary hypertension.3 Spontaneous
metabolic conditions characterized by anorexia, negative
septal defect closure is more common in males than
energy balance, weight loss, and muscle atrophy. 6
females.4 This supports the results of this study that shows
Increased levels of leptin in the blood also causes activation
55.75% patients with ventricular septal defect were
of the melanocortin system, which then increases the body's
females and 44.25% were males, while 53.66% patients
energy expenditure and decreases appetite.7
with atrial septal defect were females and 46.34% were
The presence of concomitant infections also plays an
males. Several studies are in accordance with the results
important role through release of proinflammatory cytokines
of this study, which showed that female patients had a
such as TNF-α which induces cell apoptosis, tissue proteins
higher number rather than males. Previous study
breakdown, and increases leptin secretion into the blood.7
conducted by Didik Hariyanto (2012) in Padang showed
that among patients with acyanotic congenital heart defect, This mechanism causes the body's protein reserves to
51% were females and 49% males. Other study by Ain et decrease, decreased appetite, and increased body’s energy
al. (2015) in Padang revealed that number of female expenditure.7
There was also an association between chronic
patients were 61.8%, while 38.2% other were males.
hypoxemia and endocrine function in patients with
 Jurnal Ilmiah Mahasiswa Kedokteran Universitas Airlangga 2019 August, X (02) Page 82

congenital heart defect.8 Chronic hypoxemia can cause a Types of Acyanotic Congenital Heart Defect
decrease in IGF-1 levels in the blood, a peptide that plays
in important role in growth, so that the growth of body Congenital heart defect is strongly influenced by genetic
tissue cannot be achieved optimally.8 Although chronic factors, although it is also influenced by environmental
hypoxemia is more common in cyanotic congenital heart factors. According to research conducted by Fahed et al. in
defect compared to acyanotic, this mechanism still can be 2002, there were 25 genes that affect the formation of
one of the causes of failure to thrive in children with congenital heart defects, especially related to acyanotic
acyanotic congenital heart defect.8 congenital heart defect. Out of the 25 genes, 13 of them
Growth pattern in acyanotic congenital heart defect induced the formation of atrial septal defects (ASD), namely
patients influenced by several factors such as type of mutations from the genes CITED2, GATA4, GATA6,
congenital heart defect, presence of gastrointestinal NKX2.5, TBX5, TBX20, ZIC3, CFC1, CRELD1, GJA1,
disorder, and presence of chromosomal abnormalities.6 ACTC, MYH6, and MYH7. Meanwhile 10 of the 25 genes
The degree of failure to thrive in children is directly induced the formation of ventricular septal defects (VSD),
proportional to the size of the shunt, therefore children with namely mutations from the genes CITED2, GATA4, GATA6,
congenital heart defect without a shunt may show normal IRX4, NKX2.5, TBX5, TBX20, ZIC3, CFC1, and TOGF1.
pattern of growth.6 Therefore, based on the theory, the chance of ASD
formation is higher than VSD.9
However, ASD is usually presented asymptomatic or
with very smooth murmur, this defect often does not lead to
early diagnosis.10 The high number of patients with ASD
shows a very good early diagnosis system.10

Table 4. Comparative study of lesions with other studies.

Ain et al., Bhardwaj et Didik H., American

Types of acyanotic Gupta et al.,
2015 al., 2015 2012 Heart
congenital heart defect 2016 (India)
(Indonesia) (India) (Indonesia) Association

Atrial septal defect 20.34% 22.73% 29.10% 30.97% 28.16%

Ventricular septal defect 52.41% 50% 50.81% 30.97% 30.06%
Patent ductus arteriosus 13.10% 18.18% 6.23% 29.20% 21.69%
Pulmonary valve stenosis 6.00% 4.54% 2.31% 8.85% 20.18%
Aortic valve stenosis 0.33% – 0.92% – –
Coarctation of aorta 0.33% – 0.92% – –
Atrioventricular septal defect 1% 4.54% 9.70% – –

Sign and Symptoms Distribution in Acyanotic fluid in the lungs tries to be excreted by the patient by
Congenital Heart Defect Patients coughing.13
Abnormal blood flow also causes a decrease in cardiac
The most common symptom found in acyanotic
output and develop congestive heart failure which is often
congenital heart defect patients is failure to thrive that is
characterized by edema.14 Decreased cardiac output
already described above. Heart murmurs also found in
causes the volume of blood distributed throughout the
majority of the patients because the presence of heart
body decrease, so that the blood pressure decreases and
valve structure abnormalities or shunt in heart septum
induces the activation of baroreceptor reflexes which aim
makes the blood flow becomes abnormal and causes
to increase blood vessel pressure to return to normal. The
turbulence that is heard as heart murmurs. 11 This
presence of baroreceptor stimulation activates the
abnormal blood flow allows blood from the left side of the
sympathetic nervous system which then causes an
heart to flow to the right side of the heart due to the
increase in heart rate that is experienced by patients as
pressure difference between the two parts of the heart. 11
palpitation.15, 16 Not only affects the heart, the sympathetic
Therefore, right ventricular blood volume increases, and
nervous system also activates the skin effectors including
the flow of blood to the lungs through pulmonary artery
sweat glands that has the effect of increasing sweat
also increases.12 An increase in blood volume increases
secretion.17, 18
pulmonary artery pressure in which develops further into
The risk of respiratory infection is increased in patients
pulmonary hypertension.12 If the volume exceeds its
with acyanotic congenital heart defects, this may
maximum capacity then fluid enter the interstitial space
contribute to the onset of runny nose as a sign of upper
and accumulate in the alveoli, it can cause symptoms of
respiratory tract infections and coughing as a sign of lower
breathing difficulties in patient due to thickening of the
respiratory tract infections.19 Malnutrition also affects the
barrier for oxygen exchange.12 Besides, the accumulated
immune system, therefore increasing the risk of infection
and slowing down the healing process.20
 Jurnal Ilmiah Mahasiswa Kedokteran Universitas Airlangga 2019 August, X (02) Page 83

6.Argent AC, Balachandran R, Vaidyanathan B, Khan A and

Kumar RK. Management of undernutrition and failure to
Conclusion thrive in children with congenital heart disease in low- and
middle-income countries. Cardiology in the young. 2017;
Distribution of acyanotic congenital heart defect patients 27: S22-s30.
in this study were dominated by female (53.85%) between 7.Mangili G, Garzoli E and Sadou Y. Feeding dysfunctions
0 - <5 years old (48.58%). Majority of patients under 5 years and failure to thrive in neonates with congenital heart
diseases. La Pediatria medica e chirurgica : Medical and
old were having malnutrition (42.46%), while patients above
surgical pediatrics. 2018; 40.
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septal defect (49.8%), with symptoms that are often Genetics of Congenital Heart Disease. Circulation
complained by patients were failure to thrive (61.94%). This Research. 2013; 112: 707-20.
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Cardiology. 2002; 39: 1890-900.
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Paediatrics Department Dr. Soetomo General Hospital Department of Pediatrics, University of Texas Medical
Surabaya in the future. If the same research is carried out Branch, 2017.
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