Abstract

The most prevalent congenital cardiac defect in children is congenital heart disease (CHD).  It is a major contributor to neonatal morbidity and mortality.  An outline of the epidemiology and aetiology of CHD is given in this article.  The WHO reports that the incidence of congenital cardiac disease in Bangladesh is 6%.  Eighty percent of congenital heart disease is caused by nine common abnormalities.  The remaining 20% of congenital cardiac disease is composed of rare or complex abnormalities.  The study intends to improve patient outcomes and influence public health policies by providing an overview of CHD.  In order to gather and examine the body of literature on children who may have congenital heart disease, this review study uses a systematic review methodology.The data primarily consists of a review of CHD from multiple studies including sample groups of diverse races; gender and age categories were assessed.  Fifty studies were included in the systematic review after predetermined inclusion/exclusion criteria were used.  Millions of women worldwide are affected by CHD, and children endure in silence without realising that there is a cure.  Epidemiology study reveals CHD, and with the aid of a successful transition program, it must acknowledge the need of frequent medical examinations.  In order to improve quality of life and stop the condition from getting worse, the most recent research on the pathogenesis, incidence, clinical symptoms, and treatment of congenital heart disease (CHD) was thoroughly reviewed.

Introduction

        Congenital heart disease is the most common type of heart disease in children and the most common congenital lesion. It has a major role in both neonatal mortality and morbidity. A severe heart or intrathoracic great vessel anatomical defect that is either currently or may become congenital cardiac disease is defined as one that is functionally significant. About 8 out of every 1000 live births are affected by congenital cardiac disease; the occurrences are higher in stillbirth, spontaneous abortion, and preterm. Even while improvements in surgery and medicine have raised childhood survival rates for congenital heart disease from 20% in 1950 to over 90% today, the disease's incidence has been steady over the last three decades, suggesting that our knowledge of its origin has not advanced much , demonstrating little progress in our understanding of the pathogenesis of CHD, even when surgical and medicinal improvements have raised childhood survival in CHD from 20% in 1950 to >90% today (Mantuan et al. 2017, Zimmerman et al. 2020, Wu et al. 2020). Mortality has also remained disproportionately higher in infants and at lower sociodemographic indices, indicating a persistent health disparity. Furthermore, some survivors claim to have a low quality of life and to have chronic illnesses including developmental impairments.

The World Health Organization (WHO) reports that the incidence of congenital heart disease is 6% in Bangladesh, 15% in India, 6% in Burma, and 10% in Sri Lanka, among other cardiovascular disorders (Malik 1976). This review provided a thorough analysis of congenital heart

disease (CHD) in children, including its causes, clinical signs, prognosis, diagnostic methods, and treatment modalities. By compiling and analyzing recent knowledge and advancements, this review seeks to elevate awareness and understanding among healthcare providers, facilitate early diagnosis and treatment, and ultimately improve the health outcomes for children with CHD.

Eighty percent of congenital heart disease is composed of nine common lesions, and the relative frequency of the most common lesions varies according to the source (Jackson et al. 1996). These consist of coarctation of the heart, pulmonary stenosis, atrial septal defect, patent ductus arteriosus, and tetralogy of fallout (4%, 9%, 5%, and 9%, respectively).

ventricular septal defect (36%), aorta (5%), and transposition of the great arteries (5%). Most of the uncommon or intricate abnormalities that comprise the remaining 20% of congenital cardiac disease were documented.
In 2008, Bernstein et al. Clinical results are improved and long-term consequences are decreased when children with suspected congenital heart disease are identified early and treated promptly. CHD is a frequent congenital defect
that places a heavy financial and emotional strain on the entire family. However, if the problems are discovered early in life, the chance of long-term consequences is reduced and the result is better. Because of better medical and surgical care, more children with congenital heart disease are living into adolescence and adulthood (Camm and Bunce 2005). The clinical pattern of congenital heart disease (CHD) in Bangladeshi children is little understood, with the exception of a few isolated cases (Sharmin et al. 2008). The project intends to enhance patient outcomes and inform public health policy by aiding in the better diagnosis, management, and treatment of congenital heart disease in children.

Methods

This is based on a thorough study of the literature, which included looking at a recent meta-analysis and examining the Cochrane database, Elsiver, PubMed, and the journal of cardiovascular surgery. The data mostly consists of a review of several cardiovascular disease research using sample populations from diverse ethnic backgrounds. By looking for cardiovascular disease epidemiology and symptomatology, we attempted to find the papers. This review study gathers and examines the body of literature on children with suspected congenital heart disease (CHD) using a systematic review and meta-analysis methodology. To guarantee thorough and open reporting of the review process, the study complies with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) criteria. Studies involving children (ages 0–18) who may have congenital cardiac disease are eligible to be included.

   Studies that make use of non-invasive diagnostic techniques like chest X-rays, magnetic resonance imaging (MRI), electrocardiograms (ECGs), and echocardiography. English-language articles published in peer-reviewed journals. Research on the sensitivity, specificity, and accuracy of diagnostic techniques, clinical outcomes, risk factors, and therapeutic options. Criteria for exclusion: research on congenital cardiac disease in adults. editorials, review pieces, and case reports. Research that isn't fully available in English or other languages .Sources of Information and Search Methods: The Cochrane Library, MEDLINE, and PubMed electronic databases will be used to do a thorough literature search. This study does not require ethical approval since it is a review of previously published research and does not include the gathering of primary data.

However, by guaranteeing truthful and objective reporting of findings, the review will comply with ethical norms in research. This research's possible drawbacks include the variation in study designs, diagnosis

criteria, and outcome measurements from the selected research, which might add variation and impact how broadly the results can be applied. There are several ways to classify congenital cardiac abnormalities; the following categories, such as (a) CHD with shunt between systemic and pulmonary circulation, (b) left heart CHD, (c) right heart CHD, (d) CHD with anomalous origin of great arteries, and (e) miscellanea, could be used to highlight the underlying anatomy and pathophysiology (Micheletti 2019). Below is some brief information based on the things mentioned:

CHD with a pulmonary and systemic circulatory shunt
  Atrial Septal Defect: Left-to-right shunting is made possible by an atrial septal defect (ASD), which is a connection between the atrial chambers (Fig. 1). It affects 7–10% of persons overall, making it the second most common kind of CHD. It is most commonly found in late childhood or adulthood and is more prevalent in women (2:1). Anderson et al. (2002) noted that ASD comes in a variety of forms.

ASD of Ostium Secundum:

   A deficiency in the flap valve of the oval fossa causes the most prevalent kind of ASD, which accounts for 70–80% of all instances. This defect might show up as numerous fenestrations, incomplete development, failed overlapping to the septum secundum, or even complete absence.

Ostium premium: 15% of all ASDs have this component of the atrioventricular septal defect, which is located at the crux.Defect of the sinus venosus: Complete anatomic closure happens in 70-75% of adults, but functional closure happens in 5-10% of new-borns’ close to the superior and inferior vena cava (SVC) or inferior and posterior to the inferior vena cava (IVC) entrances (Schneider et al. 1996). The patent foramen ovale (PFO), which describes the postnatal maintenance of this normal connection, assumes therapeutic significance in certain patients with cerebral vascular accident and congenital heart disease (CHD) because of paradoxical emboli (Furlan et al. 2012).

Diagnosis: A low-pitched systolic ejection murmur is present along with the second heart sound, which is widely divided and fixed from an early age

Treatment

The existence of a hemodynamically substantial L-R shunt (a pulmonary blood flow to systemic blood flow ratio of >1.5:1.0) and/or right chamber volume overload without significant PH (pulmonary hypertension) is the rationale for ASD closure; surgery or, in the event of secundum ASD and acceptable anatomic rims, device placement during interventional catheterization can be used to achieve closure (Butera et al. 2008). The most common late problem is atrial arrhythmia, and the risk increases as the child ages following treatment. The preferred diagnostic test is TTE. Diagnostic cardiac catheterization is necessary to assess pulmonary circulation vasoreactivity in the case of PH and determine pulmonary vascular resistance (Micheletti 2019)

A VSD, or ventricular septal defect, is a connection between the two ventricles (Fig. 2). It is the most common CHD and makes for over 20% of all defects. It may occur on its own or as part of a more complex cardiac anomaly (Hoffman et al. 2004). Various VSD kinds can be grouped according to the conduction system's anatomical location. Membranous septal aneurysms might eventually close completely or partially and are often connected.
Muscular VSD: Approximately 15–18% of all VSDs are entirely covered by muscle and can be found inside any muscle component of the IVS. Often, minor flaws resolve on their own; in rare cases, many flaws may form the so-called "Swiss-cheese" septum.Juxta-arterial VSD, which is double-committed, is more common in Asian countries and is limited to the region immediately beneath the artery valves, which are therefore in continuous fibrous tissue.  Located in the inflow portion of the septum, directly inferior to the atrioventricular valve system, an inlet defect is commonly observed in individuals with Down syndrome.  The direction and amount of the shunt are influenced by the extent of the defect, ventricular systolic and diastolic function, the presence of right ventricular outflow tract blockage (RVOT), and PVR.  Diagnosis: The normal pansystolic murmur, which is best heard at the left mid to lower sternal border, may not be heard for a few weeks after delivery until the shunt reaches its apex.  Generally speaking,As the defect size gets smaller, the murmur strength gets stronger.  Proper pulmonary pressure is indicated by the presence of a second sound that is physiologically distinct from respiration.  POVD linked to VSD may exhibit varying degrees of cyanosis.  The degree of cardiomegaly and the increase in pulmonary vascular markings on a chest x-ray are directly related to the size of the L-R shunt.  Position, size, number, connection to the tricuspid valve and its cordal apparatus, direction of the shunt, presence of aneurysmal tissue, distance between the VSD and aortic valve, and relationship to other congenital defects or aortic valve dysfunction are all evaluated by TTE.In certain carefully selected situations, a diagnostic cardiac catheterization may be required to measure PVR and the vasoreactivity of the pulmonary circulation.  Treatment: Self-closing small, restricted VSDs are possible.  During infancy, open cardiac surgery is frequently used to close moderate to large VSDs in order to prevent future problems (Nygren et al. 2005).  Although transcatheter closure is now a feasible alternative that should only be used in specific scenarios involving either adults or children, surgery is still the recommended course of treatment due to its low operative mortality.Result: According to Roos-Hesselink et al. (2004), a patient who has a successful surgically corrected VSD has a good long-term outlook and a life expectancy that is similar to that of the general population.  Acute and/or late-onset complete atrioventricular block (cAVB) due to conduction system injury following surgical or transcatheter closure of peri-membranous VSD remains a major concern; the incidence of cAVB following device closure that requires permanent pacemaker implantation is 2.6%, and the risk is higher in children younger than six (Holzer et al. 2006, Butera et al. 2007, Carminati et al. 2007).

Atrioventricular Septal Defect (AVSDs)

A spectrum of cardiac malformations known as atrioventricular septal defects are characterized by aberrant Development of the atrioventricular junction, which typically results from endocardial cushion tissue Diagnosis: It results in an ECG with a superior and rightward deviation of the QRS axis, which is frequently accompanied by an extended QRS duration and PR interval.  Eisenmenger syndrome is indicated by the presence of prominent central pulmonary arteries along with peripheral “pruning” and an enlarged right heart; cardiomegaly and increased pulmonary vascular markings on a chest x-ray indicate significant left-to-right intracardiac shunting and/or left AV valve regurgitation.  A comprehensive and sequential 2D and 3D TTE should be carried out in order to rule out unbalanced ventricles, outflow tract obstructions, and associated pathologies (Hlavacek et al. 2006).Treatment: Complete AVSD usually necessitates medical therapy with diuretics and ACE inhibitors while awaiting surgical repair, while the utility of afterload reduction with ACE inhibitors is still up for debate.  Most infants’ failure to thrive indicates the need for a repair, which is often made during the first three to six months of life.  Patients with Wiesenberger syndrome and unrepaired AVSD react to pulmonary vasodilator therapy following diagnostic catheterization (Micheletti 2019).  After AVSD correction, excellent short-term survival is now common.  Heart block, subaortic stenosis, persistent septal anomalies, and left AV valve failure are the most common causes of late morbidity and mortality in postoperative AVSD patients who need reoperation.

Patent Ductus Arteriosus

The descending aorta and the roof of the pulmonary arterial trunk are connected by a vascular structure Called the patent ductus arteriosus (PDA), which is located close to the origin of the left pulmonary artery

Diagnosis: Small to moderate PDA is accompanied by either left ventricular hypertrophy (LVH) or a normal ECG.  Large PDA and pulmonary hypertension are associated with biventricular hypertrophy.  When the relative pulmonary-to-systemic flow ratio is more than 2:1, a chest x-ray typically shows an enlarged cardiac profile; cardiomegaly is characterized by the enlargement of the left atrium (LA), left ventricle, and ascending aorta.  There is an increase in pulmonary vascular markings.  TTE examination is practically possible in nearly all infants, children, and many adults, and provides precise information about ductal anatomy and physiology (Micheletti 2019).

Treatment:

 Patients with PAH whose PAP or PVR is less than two thirds of their systemic pressure or two thirds of their systemic vascular resistance, respectively, should have their PDA closed, as should even asymptomatic patients with LV volume excess.  For individuals who meet specific body weight and ductal size parameters, percutaneous closure is an option. It is safe and practicable for children weighing more than 5 kg with a PDA diameter of 2.5–3.0 mm.  Although thoracotomy surgery is currently the best option for treating larger PDAs, this approach is still controversial for new-borns with lower birth weights.

Left-Heart Congenital Heart Disease

Cor Triatriatum Sinister A fibrous membrane separates the left atrium into two chambers in cor triatriatum sinister (CTS), a rare Congenital anomaly that affects the mitral valve and left atrial appendage. The proximal chamber Diagnosis: ECGs often show right ventricular hypertrophy (RVH) and right axis deviation.  The right heart appears enlarged to varying degrees on a chest x-ray, along with substantial PA and signs of pulmonary edema or pulmonary venous congestion.  TTE is necessary to assess the intra-atrial membrane characteristics and the existence of associated CHD.Treatment: Any age should be taken into consideration if there are any associated symptoms or Consequences because surgery is the only effective treatment.

Outcome: Following surgery, there is little chance of recurrence intra-atrial blockage.

Congenital Mitral Valve Stenosis

Congenital mitral stenosis (CMS) is a congenital heart malformation comprising a spectrum of Morphologically heterogeneous developmental anomalies that result in functional and anatomic obstruction of Inflow into the left ventricle of a patient

Diagnosis:

 A normal ECG throughout infancy may change as the condition advances to LA enlargement, RVH enlargement, and RA enlargement. Atrial arrhythmias could develop if the atrium continues to dilate. LA, PA, and RV are dilated on a chest x-ray, and Karley’s B lines might be visible in cases of severe disease. In terms of MV anatomical and functional aspects, TTE is diagnostic of the disease.

Treatment: To treat pulmonary venous congestions, patients may need diuretics. Surgery to repair the valve Or replace it with a prosthetic valve may be necessary in cases of severe obstruction, although doing so Bears risk of morbidity and mortality.

The heterotaxy  An abnormal arrangement of the internal thoracic and abdominal organs along the left-right axis of the body is known as heterotaxy, and it refers to a range of cardiac and systemic abnormalities that arise from an embryological failure to differentiate.  Mirror-image duplication of normally unilateral structures is the main feature of heterotaxy; some people seem to have bilateral left-sidedness, or left isomerism, while others seem to have bilateral right-sidedness, or right isomerism.  Atrial appendages may exhibit isomerism of the pectinate muscles at the cardiac level, and there is a strong correlation between the lungs, atrial appendages, and bronchial morphology.The appendages are the most consistent elements within the

Atrium; their shape and the unique morphology of their junction with the remainder of the atrium allow them to

Be identified as being morphologically right or left. CHDs are typically present in heterotaxy and range in

Complexity from mild to severe; some CHDs, like AVSD, can be observed in both forms of isomerism but are

More common in the right one (approximately two-thirds of cases) than the left (about half of cases). Other CHDs, like pulmonary stenosis, TGA, and TAPVC, are substantially more prevalent in right isomerism Children with Congenital Heart Disease and Their Quality of Life  According to a prior study, 52% of children who passed away between 1954 and 1979 from non-cardiac reasons had a congenital cardiac abnormality.  We supplemented this retrospective analysis with a prospective research in 1984.  We found that 39% of the children probably died for non-heart-related causes, and an additional 5% probably did too.  To put it another way, 44% of neonates with heart abnormalities who died most likely did not do so because of a cardiac condition. The TNO-AZL Child Quality of Life Questionnaire results of parents and children with congenital heart disease were compared to those of a reference group of children of the same age, according to a prior study.  Compared to reference peers, the entire group of ConHD children (n = 113, ages 8 to 15) had significantly lower mean scores on positive emotional functioning, motor functioning, and cognitive functioning, suggesting that they were doing worse.  When compared to reference peers, children with congenital heart disease (CHD) showed worse mean scores on five out of the seven TACQOL scales.  They also had lower positive emotional functioning scores than children with congenital heart disease aged 12 to 15.

The Viewpoint of Bangladesh on Congenital Heart Disease  Over the course of three years, from January 2006 to December 2008, a prospective hospital-based study was conducted in the pediatric cardiology department of the Combined Military Hospital (CMH) in Dhaka.  All 5,658 live newborns delivered at CMH Dhaka between January 2006 and December 2008 made up the study's data set.  At 25/1000 live births, the study’s reported incidence is much higher than any other study conducted to date (Fatema et al. 2008).Data were obtained from hospital records for a second study conducted at Dhaka Shishu Hospital over two years, from January 2008 to December 2009 prospectively and another two years, from January 1998 to December 1999 retrospectively.  Patients who were enrolled in the study ranged in age from birth to age twelve.  Between January 2008 and December 2009, 539 individuals were diagnosed with congenital heart disease.  Of the 312 individuals with CHD diagnosed between January 1998 and December 1999, only 11.9% were diagnosed during the neonatal period.  Nonetheless, 27.5% of CHD patients were identified in the newborn stage between January 2008 and December 2009 (Hussain et al. 2010).

Conclusion:

Public health officials can prioritize which regions need intervention and any new regulations they may implement by using the epidemiology and etiology of CHD.  The importance of routine medical examinations must be understood by patients and their parents of infants with congenital heart disease. This can be achieved with the support of a successful transition program and provider collaboration.  Improving outcomes for children with congenital heart disease (CHD) requires early identification and efficient treatment.  Pediatric cardiologists, surgeons, and other medical specialists must work together to provide comprehensive care.  Children with congenital heart disease have a better prognosis and a higher quality of life thanks to ongoing research and technological improvements.

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