Literature review

1. PREVALENCE OF CONGENITAL
HEART DISEASE WORLDWIDE
COMPARISON TO IRAQ
A Project Report Submitted to the Community Department
Hawler Medical University
BY
Raveen Ismael Abdullah
BSc in Nursing
1st
of April 2017

2. Table of Contents
List of Figures.......................................................................................................................... 3
List of Abbreviations and Acronyms........................................................................................... 3
Introduction............................................................................................................................ 1
Aim:................................................................................................................................... 2
Objectives........................................................................................................................... 2
Review of Literature................................................................................................................ 3
1.1. Historical Background of CHD........................................................................................ 3
1.2. Definition of CHD ......................................................................................................... 3
1.3. Etiology........................................................................................................................ 3
1.4.Classification of congenital heart disease:.......................................................................... 4
1.5. Different types of congenital heart disease ........................................................................ 4
1.5.1. Cyanotic: (Hugh et al, 2000)...................................................................................... 4
1.5.2. Acyanotic:............................................................................................................... 4
1.6. Pathophysiology of CHD:............................................................................................... 4
1.6.1. Left-to-Right Shunts:................................................................................................ 5
1.6.2. Obstructive Lesions:................................................................................................. 5
1.6.3. Cyanotic Heart Anomalies:........................................................................................ 5
1.7. Sign and Symptoms of CHD: .......................................................................................... 6
1.7. Medical Diagnosis of CHD ............................................................................................. 6
1.7.1. Physical Exam:......................................................................................................... 6
1.7.2. Newborn screening:................................................................................................. 6
1.7.3. Diagnostic Tests ...................................................................................................... 7
1.8. Complications of CHD................................................................................................... 8
1.8.1. Heart Failure........................................................................................................... 8
1.8.2. Retardation of Growth and Development:.................................................................. 8
1.8.3. Polycythemia: ......................................................................................................... 8
1.8.4. Endocarditis:........................................................................................................... 8
1.8.5 Arrhythmia: ............................................................................................................. 8
1.9. Treatment ..................................................................................................................... 8
1.9.2 Therapeutic Catheterization: ..................................................................................... 9
1.9.3. Implantable Heart Devices:....................................................................................... 9
1.9.4. Heart Transplant:..................................................................................................... 9

3. 1.9.5. Surgical Procedures: ................................................................................................ 9
1.9.6. Prevention Methods: ..............................................................................................10
Results and discussion.............................................................................................................11
Conclusion and Recommendation ............................................................................................16
3.1. Conclusion ...................................................................................................................16
3.2. Recommendation: ........................................................................................................16
List of References ...................................................................................................................17
List of Figures
Figure1 : Total CHD Birth Prevalence from 1930 until 2010........................................................11
Figure 2 : Prevalence of CHD per Continent and World Bank Income Group since 1970.................12
Figure 3 : Prevalence of the 8 most common CHD subtypes from 1945 until 2010. ........................13
Figure 4 : Birth Prevalence of CHD Subtypes. ...........................................................................14
List of Abbreviations and Acronyms
List Title
AoS
ASD
CHD
Coarc
PDA
PS
TGA
TOF
VSD
Aortic Stenosis
Atrial Septal Defect
Congenital Heart Disease
Coarctation
Patent Ductusarteriosus
Pulmonary Stenosis
Transposition of the great arteries
Tetralogy of Fallot
Ventricular Septal Defect.

4. 1
Introduction
Congenital heart diseases (CHDs) defined as any abnormalities of the heart's structure and
function caused by abnormal or disordered heart development before birth.(Al-Hyali,
2015)Congenital Heart Diseases is the most common group of structural malformations in
children (Kareema et al , 2014)Reported birth prevalence of CHD varies widely among
studies worldwide. The estimate of 8 per 1,000 live births is generally accepted as the best
approximation
Congenital heart defects are the second leading cause of death in infancy, result from
abnormal cardiovascular development during fetal life that obstruct or alter blood-flow
patterns (Kareema et al , 2014).The clinical presentations of CHD varies according to the
type and severity of the defect.(Hussein et al., 2013)
Congenital heart diseases are responsible for more deaths in the first year of life than any
other birth defects .(Al-Hyali, 2015) Congenital heart diseases (CHD) comprise the most
common group of congenital malformations. Despite recent developments in interventional
and surgical techniques, heart disease in children continues to be an important cause of
morbidity &mortality.(Dagsh et al., 2015)
The cause of most congenital heart defects is unknown most cases of CHD are thought to be
multi-factorial and result from a combination of genetic predisposition and environmental
stimuli.(Hussein et al., 2013)Only around 15% of CHDs can be attributed to a known cause
approximately 5 -10% are associated with a chromosomeabnormality, 3–5% can be linked to
defects in single genes, and about 2% are attributed to known environmental factors (Al-
Hyali, 2015).
Congenital heart disease is a serious and understudied public health problem in Iraq, where
tens of thousands of children are on waiting lists for surgery and thousands more are born
with the disease each year, where complex surgical treatment remains largely
unavailable..(Philips A, 2012)It is important to have reliable information about worldwide

5. 2
CHD birth prevalence comparison to Iraq because this may lead to better insight into its
etiology. In addition, dedicated care could be better planned and provided. Congenital heart
disease not onlycontribute to a significant morbidity and mortality but also cause a
tremendous psychological stress and economic burden to the whole family (Hussein et al.,
2013).
Aim:
Prevalence of congenital heart disease worldwide comparison to Iraq
Objectives
1. Prevalence of congenital heart disease in Low-income countries and High-
income countries.
2. Prevalence of congenital heart disease in Iraq.
3. Prevalence of congenital heart disease worldwide
4. Prevalence of type congenital heart disease worldwide comparison to Iraq.

6. 3
Review of Literature
1.1. Historical Background of CHD
The developing of the heart has two histories: the first is the 3.8billion years of evolution
from pulsating tube of insects to the complex four-chambered heart of humans, the second
history encompasses the relatively brief developmental period, and the human heart is
morphologically complete by 6 weeks post conception .most congenital cardiovascular
malformations (CCVMs) originate during the primary morphogenesis (Hugh et al, 2000).
Baillie (1797) first described Aortic Coarctation that represents 7% of CHD; Transposition of
Great Artery (TGA) affected about 5%of infants with CHD and was a lethal condition with
90%mortality within the first year.In1866, the first syndrome was described by John Landgon
Down, consisting of multiple abnormalities including CHD.(D. K. A. Hussein, 2014)
The refinement of this technology led to drastic changes in diagnostic methods of CHD from
invasive catheterization to the non-invasive (Echo) methods. This drastic change led to the
shift of cardiac catheterization from diagnostic to therapeutic purposes
1.2. Definition of CHD
A congenital heart defect is a defect in the structure of the heart and great vessels of a
newborn. Approximately 1 from 125 babies is born each year in the United States with a
congenital heart defect (Chandrasekaran et al, 2009).
1.3. Etiology
The causes of most CHD are unknown. In most cases, it is of a multifactor origin and is a
result of both genetic predisposition and environmental factors.Knowing genetic causes of
heart disease includes the chromosomal abnormalities such as trismy21,13,and18, as well as a
range of newly recognized genetic point mutations, point deletions, and familial atrial septal
defect (ASD)with heart block.Other genetic factors were suspected to play a role in CHD,
for example, certain types of ventricular septal defect VSDs(supracristal)are more common in
Asian children. The risk to siblings and offspring of affected individuals is similar and
generally in the order of 2% to 5% known as ante-natal environment factors that include
maternal infections (Rubella), Drugs (Alcohol, Hydantoin, Lithium, and Thalidomide) and
maternal illness (DIabetsMellitus, PhenylKetonuira, and Systemiclupuserythemtosus
(Bernstein et al, 2004).

7. 4
1.4.Classification of congenital heart disease:
1. Cyanotic.
2. Acyanotic(left to right shunt and obstructive lesions )
1.5. Different types of congenital heart disease
1.5.1. Cyanotic: (Hugh et al, 2000)
1. Tetralogy of Fallot
2. Transposition of the great arteries
3. Tricuspid atresia
4. Pulmonary atresia
5. Persistent truncusarteriosus
6. Total anomalous pulmonary venous return
1.5.2. Acyanotic:
1.5.2.1. Left to right shunt
1. Ventricular septal defect
2. Atrial septal defect
3. Patent ductusarteriosus
4. Atrioventricularseptal defect
1.5.2.2. ObstructiveLesions :
1. Pulmonic stenosis
2. Aortic stenosis
3. Aortic coarctation
4. Hypoplastic left heart syndrome
1.6. Pathophysiology of CHD:
The physiologic consequences of congenital heart anomalies vary greatly, ranging from an
asymptomatic heart murmur or abnormal pulses to severe cyanosis and heart failure
(Berhaman et al, 2007).

8. 5
1.6.1. Left-to-Right Shunts:
Oxygenated blood from the left heart (left atrium or left ventricle) or the aorta shunts to the
right heart (right atrium or right ventricle) or the pulmonary artery through an opening or
communication between the 2 sides. Immediately after birth, pulmonary vascular resistance is
high and flow through this communication may be minimal or bidirectional. Within the first
24 to 48 h of life, however, the pulmonary vascular resistance progressively falls, at which
point blood will increasingly flow from left to right. The additional blood flow to the right
side increases pulmonary blood flow and pulmonary artery pressure to a varying degree. The
greater the increase, the more severe the symptoms; a small left-to-right shunt typically does
not cause symptoms or signs.High-pressure shunts (those at the ventricular or great artery
level) become apparent several days to a few weeks after birth; low-pressure shunts (atrial
septal defects) become apparent considerably later. If untreated, elevated pulmonary blood
flow and pulmonary artery pressure may lead to pulmonary vascular disease and eventually
Eisenmenger syndrome. Large left-to-right shunts (eg, large ventricular septal defect [VSD],
patent ductusarteriosus [PDA]) cause excess pulmonary blood flow and volume overload,
which may lead to signs of HF and during infancy often result in failure to thrive. A large
left-to-right shunt also decreases lung compliance, leading to frequent lower respiratory tract
infections (Bernstein et al, 2004).
1.6.2. Obstructive Lesions:
Blood flow is obstructed, causing a pressure gradient across the obstruction. The resulting
pressure overload proximal to the obstruction may cause ventricular hypertrophy and HF.
The most obvious manifestation is a heart murmur, which results from turbulent flow through
the obstructed (stenotic) point. Examples are congenital aortic stenosis, which accounts for 3
to 6% of congenital heart anomalies, and congenital pulmonic stenosis, which accounts for 8
to 12%(“Overview of Congenital Cardiovascular Anomalies - Pediatrics,” n.d.)
1.6.3. Cyanotic Heart Anomalies:
Varying amounts of deoxygenated venous blood are shunted to the left heart (right-to-left
shunt), reducing systemic arterial oxygen saturation. If there is > 5 g/dL of deoxygenated Hb,
cyanosis results. Detection of cyanosis may be delayed in infants with dark pigmentation.
Complications of persistent cyanosis include polycythemia, clubbing, thromboembolism

9. 6
(including stroke), bleeding disorders, brain abscess, and hyperuricemia. Hypercyanotic
spells can occur in infants with unrepaired tetralogy of Fallot(Hugh et al, 2000).
1.7. Sign and Symptoms of CHD:
Sign and symptoms are related the type and severity of the heart defect .some children have
no signs while others may have exhibit shortness of breath ,(cyanosis);A bluish tinge or color
known as cyanosis to the skin around mouth, lips and tongue ,chest pain, syncope,fatigue or
tiredness ,sweating, recurrent lung infections ,diminished strength of pulse in the baby, under
developing of limbs and muscles poor feeding, or poor growth, buildup of blood and fluid in
lungs, feet, ankles and legs. The congenital heart defects and abnormal heart structure
resulting in the production of certain sounds called heart murmur, and not all the heart
murmurs are caused by the congenital heart defects.The CHD symptoms are frequently
present early in life, but it's possible for some CHDs to go undetected throughout the life span
(Freedom and Nykanen,2002);(Thom et al,2000).
1.7. Medical Diagnosis of CHD
1.7.1. Physical Exam:
In physical examinations, the examiner Listens to child's heart and lungs with a stethoscope,
look for signs of a heart defect, such as cyanosis (a bluish tint to the skin, lips, or fingernails),
shortness of breath, rapid breathing, delayed growth, or signs of heart failure.
1.7.2. Newborn screening:
Manifestations of congenital heart disease may be subtle or absent in neonates, and failure or
delay in detecting CHD,The screening is done when infants are ≥ 24 h old and is considered
positive if ≥ 1 of the following is present:
1.7.2.1.Any oxygen saturation measurement is < 90%.
1.7.2.2.The oxygen saturation measurements in both the right hand and foot are < 95% on 3
separate measurements taken 1 h apart.
1.7.2.3.There is > 3% absolute difference between the oxygen saturation in the right hand
(preductal) and foot (postductal) on 3 separate measurements taken 1 h apart.
All neonates with a positive screen should undergo a comprehensive evaluation for CHD and
other causes of hypoxemia (e.g., various respiratory disorders, CNS depression, sepsis)

10. 7
typically including a chest x-ray, ECG, echocardiography, and often blood testing. Sensitivity
of pulse oximetry screening is slightly > 75%; the CHD lesions most often missed are left
heart obstructive lesions (Freedom and Nykanen,2002).
1.7.3. Diagnostic Tests
1.7.3.1. Echocardiography:
Echocardiography (echo) is a painless test that uses sound waves to create a moving picture
of the heart. During the test, the sound waves (called ultrasound) bounce off the structures of
the heart. A computer converts the sound waves into pictures on a screen. Echo is an
Important test for both diagnosing a heart problem and following the problem over time. The
test can show problems with the heart's structure and how the heart is reacting to those
problems. Echo will help your child's cardiologist decide if and when treatment is needed.
Fetal echo usually is done at about 18 to 22 weeks of pregnancy.(“Overview of Congenital
Cardiovascular Anomalies - Pediatrics,” n.d.)
1.7.3.2. ECG (Electrocardiogram):
An ECG is a simple test that records the electrical activity of the heart. This test shows how
fast the heart is beating and its rhythm (steady or irregular). An ECG also records the strength
and timing of electrical signals as they pass through the heart(Vishnu,2009).
1.7.3.3. Chest X- ray:
A chest x- ray is a test that creates pictures of the structures in the chest, such as the heart and
lungs. This test can show whether the heart is enlarged. It also can show whether the lungs
have extra blood flow or extra fluid, a sign of heart failure.
1.7.3.4. PulseOximetry:
A small sensor is attached to a finger or toe (like an adhesive bandage). The sensor gives an
estimate of how much oxygen is in the blood.
1.7.3.5. Cardiac Catheterization:
During cardiac catheterization, a thin, flexible tube called a catheter is put into a vein in the
arm, groin (upper thigh), or neck. The tube is threaded to the heart. Cardiac catheterization
with angiocardiography is occasionally needed to confirm the diagnosis or to assess severity

11. 8
of the anomaly; it is done more often for therapeutic purposes.(“Overview of Congenital
Cardiovascular Anomalies - Pediatrics,” n.d.)
1.7.3.6. Magnetic Resonance Imagining (MRI):
An MRI shows the detailed structure of the heart beat and assesses the weaker or damaged
parts of the Heart (Hugh et al,2000)
1.7.3.7. Positron Emission Tomography (PET):
This test determines the level of chemical activity in different areas of the heart. A decreased
blood flow caused by the disease or damaged muscles which can be detected by this scanning
method . (Hugh et al, 2000)
1.8. Complications of CHD
Thom et al (2000) Identified the most common complications of CHD which includes the
followings:
1.8.1. Heart Failure
This is a major complication of congenital heart defects. Heart failure may develop years
after the defect is diagnosed.
1.8.2. Retardation of Growth and Development:
Children with severe cardiac malformation frequently exhibit retardation of growth and
development, with height and Wight near or below the third percentile or weight 20
percentile points below the mean percentile for height.
1.8.3. Polycythemia:
This is an abnormal increase in the number of red blood cells .This may increase a Person's
risk for blood clots that can cause heart attacks or strokes (Robin,2007).
1.8.4. Endocarditis:
It is a significant complication seen in congenital heart patients before and after the surgical
repair (Gray, 2007).
1.8.5 Arrhythmia:
It is a group of conditions in which the heartbeat is irregular, too fast, or too slow.
1.9. Treatment
The treatment depends on the type and severity of heart defect. Other factors include child's
age, size, and general health interfere with treatment of CHD (Bernstein et al,2002).

12. 9
Some medications are most commonly prescribed and include diuretics and digoxin.The
diuretics aid the infant in excreting water and salts.Digoxin strengthens the contraction of the
heart, slows the heart beat and removes fluid from tissues.
1.9.2 Therapeutic Catheterization:
1.9.2. 1. Atrial Septal Defect:
In this procedure, a catheter is guided through a vein and threaded into the heart to the
septum. At this point, a tiny umbrella –like device folded up inside, the catheter is positioned
so as to plug the opening in the septum and the device is secured before the catheter is
withdrawn from the body.
1.9.2. 2. Pulmonary Valve Stenosis:
In this method, a catheter is inserted into a vein and threaded into the heart to the pulmonary
valve.Then, a tiny balloon at the end of the catheter is inflated to push apart the leaflets of the
valve and the balloon is withdrawn along with the catheter after deflation..
1.9.3. Implantable Heart Devices:
Some of the complications associated with congenital heart defects can be prevented with the
use of certain devices, including pacemakers and implantable cardioverter defibrillators
(ICDs). A pacemaker can help regulate an abnormal heart rate, and an ICD may correct life-
threatening irregular heartbeats.
1.9.4. Heart Transplant:
In the rare cases in which a congenital heart defect is too complex to fix, a heart transplant
may be needed. During this procedure, the child's heart is replaced with a healthy heart from
a donor.(“Congenital Heart Disease,” n.d.)
1.9.5. Surgical Procedures:
A child may need open-heart surgery if his or her heart defect can't be fixed using a catheter
procedure. Sometimes one surgery can repair the defect completely. If that's not possible, the
child may need more surgeries over months or years to fix the problem.The open heart
surgery is performed in order to:
1.9.5.1. Close holes in the heart with stitches or a patch.
1.9.5.2. Repair or replace heart valves.

13. 10
1.9.5.3. Widen arteries or openings to heart valves.
1.9.5.4. Repair complex defects, such as problems with the location of blood vessels.
1.9.6. Prevention Methods:
Congenital heart defects cannot be prevented, but certain measures are taken before and
during pregnancy to reduce the risk of having a baby with congenital heart defects .the steps
need to be taken include(Hugh et tal,2000)
1.9.6.2. A pre-conception visit could help in testing for immunity from rubella and
vaccination administered if the woman is not immune.
1.9.6.3. Woman suffering from diabetes and phenylketonuria,(PKU)must be adjusted with the
necessary medication and eating habits to keep these conditions under control before and
during the pregnancy.
1.9.6.4. Pregnant women must avoid people suffering from flu or other viral infections.
1.9.6.5. Avoid alcohol and other drugs during pregnancy.

14. 11
Results and discussion
Congenital heart disease (CHD) accounts for nearly one-third of all major congenital
anomalies(Linde et al., 2011)the prevalence congenital heart disease worldwide vary ,In a
meta-analysis study for identifying the prevalence of congenital heart disease worldwide
114 studies were included , In that systematic review and meta-analysis prevalence of total
CHD and the 8 most common subtypes of CHD from 1930 until 2010 been identified .
Figure1: Total CHD Birth Prevalence from 1930 until 2010
(Linde et al., 2011)
The prevalence of congenital heart disease increased substantially as it shows in( figure 1)
from 0.6 per 1,000 live birthsin 1930 to 1934 to 9.1 per 1,000 live births after 1995. (Linde et
al., 2011)

15. 12
Figure 1 : Prevalence of CHD per Continent and World Bank Income Group since 1970.
(Linde et al., 2011)
Figure 2.A.Shows that the prevalence of CHD in Europe was significantly higher than in
North America, South America, Oceania, and Africa. While the prevalence of CHD in Asia
was significantly higher than in Europe, North America, South America, Oceania, and Africa.

16. 13
Figure 2.B. Shows that prevalence of CHD per World Bank in upper-middle income
countries was significantly higher than in lower-middle-income. No data were available for
low-income countries. Prevalenceof CHD in high-income countries was significantly higher
than in upper- and lower- middle-income countries.
Significant differences between World Bank income groups were found, with the highest
reported total CHD birth prevalence in high-income countries (8.0 per 1,000 live births.
Reported total CHD birth prevalence in upper- middle-income countries was 7.3 per 1,000
live births and 6.9 per 1,000 live births in lower-middle-income countries, No data from low-
income countries were available.
Figure 2 : Prevalence of the8 most common CHD subtypes from 1945 until 2010.
(Linde et al., 2011)

17. 14
The above figure shows the Worldwide reported birth prevalence of the CHD subtypes (per
1,000 live births) was: 2.62VSD, 1.64ASD, 0.87 PDA, 0.50 PS, 0.34 TOF, 0.34 Coarc,
0.31TGA, and 0.22 AoS.
Figure 3 : Birth Prevalence of CHD Subtypes.
In( figure4) Significant geographical differences in the reported birth prevalence of the 8
most common CHD subtypes were detected, Asia reported relatively more pulmonary
outflow obstructions (PS and TOF) and fewer left ventricular outflow tract obstructions
(Coarc and AoS). Furthermore, Asia reported a lower TGA birth prevalence compared with
Europe, North America, South America, and Oceania.(Linde et al., 2011)
While, A Pilot Study of Congenital Anomaly Rates at Birth in Fallujah, Iraq in 2010 showed
the most common subtypes of CHD in Fallujah ( VSD 68% , ASD 63.92%, TOF
12.24%).(Alaani et al., 2012)

18. 15
In a study about Impact Of Congenital Heart Disease Upon Children Physical Growth Pattern
In Erbil/ Kurdistan Region/Iraq during 2013 showed that 51.5% of children with low socio-
economic status had CHD,while2.5% of children with high socio-economic status had CHD
Comparison to worldwide is different maybe the difference due to the limitation of data
resources on low-income countries , the most common subtypes of congenital heart disease
based on mentioned study were as follow : VSD37.5%, TOF 21.5, ASD7.5%, PS
8%.(Kareema et al, 2014)
A prospective study conducted in 2008 in maternity and children hospital which is the only
main hospital in AL-Diwaniyah governorate /Iraq, The conducted study showed that the
ventricular septal defect (VSD ) is themost common type of all CHD it account about 35% of
casesfollowed by PDA and ASD (26.6%and 8.3%) respectively , TOF is themost common
form of cyanotic CHD (8.3%).Other form of CHDwhether cyanotic or a cyanotic have the
same results(1.6%-3.3%).(Hussein et al., 2013)
In another study conducted in Mosul/Iraq about Prevalence and Risk Factors for Congenital
Heart Anomalies among Hospital Attendees in 2015 the result showed that 43.47%of
children who attended hospital been diagnosed with ASD, 30.43% with VSD and 9.13% with
PDA.(Al-Hyali, 2015)
A study about Prevalence of Congenital Heart Disease in Fallujah / Iraq from 2007to 2011,
the results of that study showed that The overall prevalence of CHD live births during this 5-
year period was 19.7 per 1000 live births. The rate of CHD was 24.44 per 1000 in 2007, 9.25
per 1000 in 2008, 8.82 per 1000in 2009, 23.81 in 2010 & 29.23 per 1000 in 2011. In 2007 &
2008 VSD was the commonest CHD & PS lowest rate in 2008. While ASD was the
commonest CHD in 2010 & 2011.(Dagsh et al., 2015)

19. 16
Conclusion and Recommendation
3.1. Conclusion
Congenital heart disease worldwide is in increase including Iraq.The most common subtypes
of congenital heart disease worldwide compared to Iraq were VSD, ASD, PDA, PS, and
TOF. The prevalence of CHD in Asia was significantly higher than in Europe, North
America, South America, Oceania, and Africa. Prevalence of CHD worldwide in high-
income countries was significantly higher than in upper- and lower- middle-income countries.
While in low- income countries no data were available. This showed that prevalence of CHD
varies based on geographical area; economic status might be affected by environmental
factors.
3.2. Recommendation:
3.2.1. Further studies are necessary to be conducted in low-income countries.
3.2.2. Finance Support for the family with children of congenital heart disease by
Government.
3.2.4. Conducting awareness programs regarding risk factors, prevention of CHD and home
care for child with CHD.

20. 17
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