The progression of the developmental abnormality of the aortic valve from mild to severe is referred to as aortic valve stenosis. This stenosis results in a moderate to severe narrowing of the left ventricular outflow, and it may be associated with other conditions of developmental abnormality of the heart, such as autosomal dominant inherited diseases, different levels of left heart underdevelopment, or other forms of obstruction of the left heart.
Valvar AS in children can be present at birth, that is, inherited, or it can develop in childhood. Congenital valvar AS results from a developmental anomaly of the aortic valve mechanism during fetal development.
As indicated by extreme stenosis, approximately 10 to 15 percent of such patients are believed to develop the disorder before they are a year old. Since the blockage is gradual, the remaining patients may present themselves in later childhood or adulthood.
Epidemiology
Incidence and Prevalence: The incidence relates to the number of individuals affected by the disease at a given period, with the figures usually being standardized to 1,000 live births. The prevalence expresses the proportion of people in a population having a specific disorder at a given point in time. The reported incidence of valvar AS is not uniform in different investigations and populations but falls between 4–7 cases/10,000 live births.
Anatomy
Pathophysiology
Pure aortic valve stenosis results in progressive ventricular dilation in proportion to the degree of obstruction. Patients with comparatively low amounts of LVH, normal left ventricular function, and only mild to moderate stenoses of the AV valves are usually asymptomatic. Hypertrophy gradually increases as wall tension increases with stenosis, followed mainly by periods of intense somatic development.
Besides, the continuous demand for ejecting the pressure placed on the left ventricle leads to hypertrophy of the cardiac muscles to form LVH.
The severe blockage in utero leads to various degrees of the left-sided cardiac hypoplasia, endomyocardial fibroelastosis, reduced ventricular function and mitral regurgitation.
Etiology
The natural history is demonstrated by studies revealing that among children with congenital heart disease, aortic valve stenosis in the form of left ventricular outflow tract obstructive lesions has higher repeat rates. Children of an affected father have a prevalence rate of roughly 3% of having an occurrence, while such children of an affected mother have a prevalence rate of roughly 15%.
Left-sided CADL, including AS and other congenital obstructive heart diseases, are believed to be secondary to altered fetal blood flow.
Valvar AS may also develop after childhood because of acquired conditions, though the condition is not very rampant in children. Acquired causes of valvar AS may include:
Rheumatic Fever: Another disease which may necessitate the deformation of the aortic valve is rheumatic fever that results from the scaring of the valve due to untreated streptococcal infection. Eventually this results in the development of the valvar stenosis and leads to morbidity and mortality of the affected child.
Infective Endocarditis: Valvar stenosis can result from severe aortic valve infections, such as infective endocarditis, which can injure and scar the valve.
Genetics
Prognostic Factors
The outcome in Congenital Aortic Valve Stenosis depends on both anatomy of the valve and its reciprocal to intervention. The disease severity may range from asymptotic form in specific individuals with the bicuspid aortic valve and without stenosis to severe disease in newborns.
This risk has been reduced over the last twenty years; patients who develop severe or critical aortic valve stenosis in the first year of life, particularly in the neonatal period, have higher mortality rates.
Clinical History
Age Group:
Infants and Neonates: Valvar AS can be identified in infants immediately after birth during newborn screening or due to heart murmurs or poor feeding.
Adolescents and Young Adults: Valvar AS can persist or be diagnosed in adolescents and young adults.
Physical Examination
General Appearance
Cardiovascular Examination
Pulses
Age group
Associated comorbidity
Genetic Syndromes
Connective Tissue Disorders
Associated activity
Acuity of presentation
Sometimes, valvar AS may be an acute condition characterized by severe and sudden symptoms that need urgent intervention.
Critical Neonatal AS: Newborns with critical valvar AS may appear at birth or within the first hours or days of life and have severe symptoms that may include poor feeding, cyanosis, or signs of heart failure.
Acute Decompensation: An acute incident, such as an illness, dehydration, or other stresses, might cause a kid with underlying valvar AS to worsen rapidly.
Promoting physical activity according to the child’s capabilities and limitations is recommended by their doctor. Exercise is beneficial in increasing the cardiovascular endurance and muscle strength of the heart, as well as aiding in weight reduction.
Implementation of Stress Management:
Help maintain a stress free and supportive environment for the child. Stress is known to have possible effects on the heart, therefore there should be proper recommendations of stresses relieving activities including exercises and hobbies.
Use of Prostaglandin E1 in valvar aortic stenosis for children
Prostaglandin E1: It is also called alprostadil and may be given intravenously to Keep or reopen the ductus arteriosus, so that RV is able to sustain the Systemic circulation during the period of transition.
The primary goal is to promote an adequate alleviation of aortic valve obstruction but avoid the generation of critically insufficient valve damage, especially in neonates and infants.
Use of diuretic therapy to treat valvar aortic stenosis in children
Furosemide: Furosemide impacts the kidneys’ loop of Henle to increase urine formation and reduce fluid accumulation. It can be administered intravenously to provoke a desired and rapid urination.
Intravenous furosemide, a loop diuretic, may sometimes be administered to children with valvular AS in cases of congestive symptoms.
Use of inotropic drugs to treat valvar aortic stenosis in children
Dopamine: They act upon the adrenergic receptors in the heart and increase the myocardial contractility, thus improving the output of the cardiac muscle. This is known as a positive inotropic drug for heart muscles and improves the working of the heart muscles. Further, it has the characteristics of a vasodilator that enhances blood flow by reducing systemic vascular resistance.
Dobutamine: It is an example of an inotropic agent which is frequently employed. Furthermore, it improves the force of cardiac contraction and promotes vasodilation mainly through beta-1 adrenergic receptors. It can be administered parenteral and is usually applied in crisis intervention to increase the strength of heart muscularity force.
Epinephrine: They are normally recommended in symptomatic valvular AS where there is hemodynamic instability, poor output or reduced function of the cardiac muscles. It has properties that shrink blood vessels and that can raise the blood pressure.
Use of various Surgical therapies to treat valvar aortic stenosis in children
Aortic valvotomy: To enlarge the constricted region, a surgical incision is made in the aortic valve. This procedure is usually carried out in infants who have severe valvar AS and can immediately help in enhancing the blood flow of the valvular area. Aortic valvotomy can be done mainly as a palliative intervention.
Aortic Valve Replacement: This is done when the valve cannot be put to working order through repair mechanisms that may be available. The non-working valve is replaced, and this will be either a mechanical valve or a bioprosthetic (tissue) valve.
use-of-interventional-methods
Transcatheter Aortic Valve Implantation (TAVI): TAVI is however a not very frequently interventions that is used in children especially where surgical interventions are not feasible. It involves through the implantation of a bioprosthetic valve without using surgery and accomplishing the procedure using a catheter.
Balloon Aortic Valvuloplasty: Balloon dilation is a non-surgical procedure and therefore is comparatively safer and is used initially in managing significant valvar AS in children. During the process, a tube with an inflatable cuff is passed through the tube and inserted into the narrowed aortic valve. It is then inflated to expand the valve opening and thus increases the blood flow. The balloon is then deflated and taken away from the patient’s body.
This objective is often accomplished with a guarded balloon valvuloplasty whereby the peak-to-peak systolic gradient is reduced by 50%. Usually, the size of balloons is from eighty to one hundred percent of the size of the aortic valve anulus.
Diagnosis: This usually entails a history and physical assessment performed on the patient with a primary focus on auscultation of the heart to detect murmurs.
Assessment and Risk Identification: An evaluation of the severity of the AS and the child’s overall status follows the diagnosis to confirm the diagnosis, acuity of the valvar AS, and assess the child for any associated symptoms or problems.
assessment-and-risk-identification
After the diagnosis, a comprehensive assessment of the child’s condition is conducted to determine the severity of the valvar AS and assess its impact on the child’s overall health.
The progression of the developmental abnormality of the aortic valve from mild to severe is referred to as aortic valve stenosis. This stenosis results in a moderate to severe narrowing of the left ventricular outflow, and it may be associated with other conditions of developmental abnormality of the heart, such as autosomal dominant inherited diseases, different levels of left heart underdevelopment, or other forms of obstruction of the left heart.
Valvar AS in children can be present at birth, that is, inherited, or it can develop in childhood. Congenital valvar AS results from a developmental anomaly of the aortic valve mechanism during fetal development.
As indicated by extreme stenosis, approximately 10 to 15 percent of such patients are believed to develop the disorder before they are a year old. Since the blockage is gradual, the remaining patients may present themselves in later childhood or adulthood.
Incidence and Prevalence: The incidence relates to the number of individuals affected by the disease at a given period, with the figures usually being standardized to 1,000 live births. The prevalence expresses the proportion of people in a population having a specific disorder at a given point in time. The reported incidence of valvar AS is not uniform in different investigations and populations but falls between 4–7 cases/10,000 live births.
Pure aortic valve stenosis results in progressive ventricular dilation in proportion to the degree of obstruction. Patients with comparatively low amounts of LVH, normal left ventricular function, and only mild to moderate stenoses of the AV valves are usually asymptomatic. Hypertrophy gradually increases as wall tension increases with stenosis, followed mainly by periods of intense somatic development.
Besides, the continuous demand for ejecting the pressure placed on the left ventricle leads to hypertrophy of the cardiac muscles to form LVH.
The severe blockage in utero leads to various degrees of the left-sided cardiac hypoplasia, endomyocardial fibroelastosis, reduced ventricular function and mitral regurgitation.
The natural history is demonstrated by studies revealing that among children with congenital heart disease, aortic valve stenosis in the form of left ventricular outflow tract obstructive lesions has higher repeat rates. Children of an affected father have a prevalence rate of roughly 3% of having an occurrence, while such children of an affected mother have a prevalence rate of roughly 15%.
Left-sided CADL, including AS and other congenital obstructive heart diseases, are believed to be secondary to altered fetal blood flow.
Valvar AS may also develop after childhood because of acquired conditions, though the condition is not very rampant in children. Acquired causes of valvar AS may include:
Rheumatic Fever: Another disease which may necessitate the deformation of the aortic valve is rheumatic fever that results from the scaring of the valve due to untreated streptococcal infection. Eventually this results in the development of the valvar stenosis and leads to morbidity and mortality of the affected child.
Infective Endocarditis: Valvar stenosis can result from severe aortic valve infections, such as infective endocarditis, which can injure and scar the valve.
The outcome in Congenital Aortic Valve Stenosis depends on both anatomy of the valve and its reciprocal to intervention. The disease severity may range from asymptotic form in specific individuals with the bicuspid aortic valve and without stenosis to severe disease in newborns.
This risk has been reduced over the last twenty years; patients who develop severe or critical aortic valve stenosis in the first year of life, particularly in the neonatal period, have higher mortality rates.
Age Group:
Infants and Neonates: Valvar AS can be identified in infants immediately after birth during newborn screening or due to heart murmurs or poor feeding.
Adolescents and Young Adults: Valvar AS can persist or be diagnosed in adolescents and young adults.
General Appearance
Cardiovascular Examination
Pulses
Genetic Syndromes
Connective Tissue Disorders
Sometimes, valvar AS may be an acute condition characterized by severe and sudden symptoms that need urgent intervention.
Critical Neonatal AS: Newborns with critical valvar AS may appear at birth or within the first hours or days of life and have severe symptoms that may include poor feeding, cyanosis, or signs of heart failure.
Acute Decompensation: An acute incident, such as an illness, dehydration, or other stresses, might cause a kid with underlying valvar AS to worsen rapidly.
Supravalvar Aortic Stenosis
Subvalvar Aortic Stenosis
Aortic Regurgitation
Pediatrics, General
Daily physical activity:
Promoting physical activity according to the child’s capabilities and limitations is recommended by their doctor. Exercise is beneficial in increasing the cardiovascular endurance and muscle strength of the heart, as well as aiding in weight reduction.
Implementation of Stress Management:
Help maintain a stress free and supportive environment for the child. Stress is known to have possible effects on the heart, therefore there should be proper recommendations of stresses relieving activities including exercises and hobbies.
Pediatrics, Cardiology
Prostaglandin E1: It is also called alprostadil and may be given intravenously to Keep or reopen the ductus arteriosus, so that RV is able to sustain the Systemic circulation during the period of transition.
The primary goal is to promote an adequate alleviation of aortic valve obstruction but avoid the generation of critically insufficient valve damage, especially in neonates and infants.
Pediatrics, Cardiology
Furosemide: Furosemide impacts the kidneys’ loop of Henle to increase urine formation and reduce fluid accumulation. It can be administered intravenously to provoke a desired and rapid urination.
Intravenous furosemide, a loop diuretic, may sometimes be administered to children with valvular AS in cases of congestive symptoms.
Pediatrics, Cardiology
Dopamine: They act upon the adrenergic receptors in the heart and increase the myocardial contractility, thus improving the output of the cardiac muscle. This is known as a positive inotropic drug for heart muscles and improves the working of the heart muscles. Further, it has the characteristics of a vasodilator that enhances blood flow by reducing systemic vascular resistance.
Dobutamine: It is an example of an inotropic agent which is frequently employed. Furthermore, it improves the force of cardiac contraction and promotes vasodilation mainly through beta-1 adrenergic receptors. It can be administered parenteral and is usually applied in crisis intervention to increase the strength of heart muscularity force.
Epinephrine: They are normally recommended in symptomatic valvular AS where there is hemodynamic instability, poor output or reduced function of the cardiac muscles. It has properties that shrink blood vessels and that can raise the blood pressure.
Pediatrics, Cardiology
Aortic valvotomy: To enlarge the constricted region, a surgical incision is made in the aortic valve. This procedure is usually carried out in infants who have severe valvar AS and can immediately help in enhancing the blood flow of the valvular area. Aortic valvotomy can be done mainly as a palliative intervention.
Aortic Valve Replacement: This is done when the valve cannot be put to working order through repair mechanisms that may be available. The non-working valve is replaced, and this will be either a mechanical valve or a bioprosthetic (tissue) valve.
Surgery, Cardiothoracic
Transcatheter Aortic Valve Implantation (TAVI): TAVI is however a not very frequently interventions that is used in children especially where surgical interventions are not feasible. It involves through the implantation of a bioprosthetic valve without using surgery and accomplishing the procedure using a catheter.
Balloon Aortic Valvuloplasty: Balloon dilation is a non-surgical procedure and therefore is comparatively safer and is used initially in managing significant valvar AS in children. During the process, a tube with an inflatable cuff is passed through the tube and inserted into the narrowed aortic valve. It is then inflated to expand the valve opening and thus increases the blood flow. The balloon is then deflated and taken away from the patient’s body.
This objective is often accomplished with a guarded balloon valvuloplasty whereby the peak-to-peak systolic gradient is reduced by 50%. Usually, the size of balloons is from eighty to one hundred percent of the size of the aortic valve anulus.
Pediatrics, Cardiology
Diagnosis: This usually entails a history and physical assessment performed on the patient with a primary focus on auscultation of the heart to detect murmurs.
Assessment and Risk Identification: An evaluation of the severity of the AS and the child’s overall status follows the diagnosis to confirm the diagnosis, acuity of the valvar AS, and assess the child for any associated symptoms or problems.
Pediatrics, Cardiology
After the diagnosis, a comprehensive assessment of the child’s condition is conducted to determine the severity of the valvar AS and assess its impact on the child’s overall health.
The progression of the developmental abnormality of the aortic valve from mild to severe is referred to as aortic valve stenosis. This stenosis results in a moderate to severe narrowing of the left ventricular outflow, and it may be associated with other conditions of developmental abnormality of the heart, such as autosomal dominant inherited diseases, different levels of left heart underdevelopment, or other forms of obstruction of the left heart.
Valvar AS in children can be present at birth, that is, inherited, or it can develop in childhood. Congenital valvar AS results from a developmental anomaly of the aortic valve mechanism during fetal development.
As indicated by extreme stenosis, approximately 10 to 15 percent of such patients are believed to develop the disorder before they are a year old. Since the blockage is gradual, the remaining patients may present themselves in later childhood or adulthood.
Incidence and Prevalence: The incidence relates to the number of individuals affected by the disease at a given period, with the figures usually being standardized to 1,000 live births. The prevalence expresses the proportion of people in a population having a specific disorder at a given point in time. The reported incidence of valvar AS is not uniform in different investigations and populations but falls between 4–7 cases/10,000 live births.
Pure aortic valve stenosis results in progressive ventricular dilation in proportion to the degree of obstruction. Patients with comparatively low amounts of LVH, normal left ventricular function, and only mild to moderate stenoses of the AV valves are usually asymptomatic. Hypertrophy gradually increases as wall tension increases with stenosis, followed mainly by periods of intense somatic development.
Besides, the continuous demand for ejecting the pressure placed on the left ventricle leads to hypertrophy of the cardiac muscles to form LVH.
The severe blockage in utero leads to various degrees of the left-sided cardiac hypoplasia, endomyocardial fibroelastosis, reduced ventricular function and mitral regurgitation.
The natural history is demonstrated by studies revealing that among children with congenital heart disease, aortic valve stenosis in the form of left ventricular outflow tract obstructive lesions has higher repeat rates. Children of an affected father have a prevalence rate of roughly 3% of having an occurrence, while such children of an affected mother have a prevalence rate of roughly 15%.
Left-sided CADL, including AS and other congenital obstructive heart diseases, are believed to be secondary to altered fetal blood flow.
Valvar AS may also develop after childhood because of acquired conditions, though the condition is not very rampant in children. Acquired causes of valvar AS may include:
Rheumatic Fever: Another disease which may necessitate the deformation of the aortic valve is rheumatic fever that results from the scaring of the valve due to untreated streptococcal infection. Eventually this results in the development of the valvar stenosis and leads to morbidity and mortality of the affected child.
Infective Endocarditis: Valvar stenosis can result from severe aortic valve infections, such as infective endocarditis, which can injure and scar the valve.
The outcome in Congenital Aortic Valve Stenosis depends on both anatomy of the valve and its reciprocal to intervention. The disease severity may range from asymptotic form in specific individuals with the bicuspid aortic valve and without stenosis to severe disease in newborns.
This risk has been reduced over the last twenty years; patients who develop severe or critical aortic valve stenosis in the first year of life, particularly in the neonatal period, have higher mortality rates.
Age Group:
Infants and Neonates: Valvar AS can be identified in infants immediately after birth during newborn screening or due to heart murmurs or poor feeding.
Adolescents and Young Adults: Valvar AS can persist or be diagnosed in adolescents and young adults.
General Appearance
Cardiovascular Examination
Pulses
Genetic Syndromes
Connective Tissue Disorders
Sometimes, valvar AS may be an acute condition characterized by severe and sudden symptoms that need urgent intervention.
Critical Neonatal AS: Newborns with critical valvar AS may appear at birth or within the first hours or days of life and have severe symptoms that may include poor feeding, cyanosis, or signs of heart failure.
Acute Decompensation: An acute incident, such as an illness, dehydration, or other stresses, might cause a kid with underlying valvar AS to worsen rapidly.
Supravalvar Aortic Stenosis
Subvalvar Aortic Stenosis
Aortic Regurgitation
Pediatrics, General
Daily physical activity:
Promoting physical activity according to the child’s capabilities and limitations is recommended by their doctor. Exercise is beneficial in increasing the cardiovascular endurance and muscle strength of the heart, as well as aiding in weight reduction.
Implementation of Stress Management:
Help maintain a stress free and supportive environment for the child. Stress is known to have possible effects on the heart, therefore there should be proper recommendations of stresses relieving activities including exercises and hobbies.
Pediatrics, Cardiology
Prostaglandin E1: It is also called alprostadil and may be given intravenously to Keep or reopen the ductus arteriosus, so that RV is able to sustain the Systemic circulation during the period of transition.
The primary goal is to promote an adequate alleviation of aortic valve obstruction but avoid the generation of critically insufficient valve damage, especially in neonates and infants.
Pediatrics, Cardiology
Furosemide: Furosemide impacts the kidneys’ loop of Henle to increase urine formation and reduce fluid accumulation. It can be administered intravenously to provoke a desired and rapid urination.
Intravenous furosemide, a loop diuretic, may sometimes be administered to children with valvular AS in cases of congestive symptoms.
Pediatrics, Cardiology
Dopamine: They act upon the adrenergic receptors in the heart and increase the myocardial contractility, thus improving the output of the cardiac muscle. This is known as a positive inotropic drug for heart muscles and improves the working of the heart muscles. Further, it has the characteristics of a vasodilator that enhances blood flow by reducing systemic vascular resistance.
Dobutamine: It is an example of an inotropic agent which is frequently employed. Furthermore, it improves the force of cardiac contraction and promotes vasodilation mainly through beta-1 adrenergic receptors. It can be administered parenteral and is usually applied in crisis intervention to increase the strength of heart muscularity force.
Epinephrine: They are normally recommended in symptomatic valvular AS where there is hemodynamic instability, poor output or reduced function of the cardiac muscles. It has properties that shrink blood vessels and that can raise the blood pressure.
Pediatrics, Cardiology
Aortic valvotomy: To enlarge the constricted region, a surgical incision is made in the aortic valve. This procedure is usually carried out in infants who have severe valvar AS and can immediately help in enhancing the blood flow of the valvular area. Aortic valvotomy can be done mainly as a palliative intervention.
Aortic Valve Replacement: This is done when the valve cannot be put to working order through repair mechanisms that may be available. The non-working valve is replaced, and this will be either a mechanical valve or a bioprosthetic (tissue) valve.
Surgery, Cardiothoracic
Transcatheter Aortic Valve Implantation (TAVI): TAVI is however a not very frequently interventions that is used in children especially where surgical interventions are not feasible. It involves through the implantation of a bioprosthetic valve without using surgery and accomplishing the procedure using a catheter.
Balloon Aortic Valvuloplasty: Balloon dilation is a non-surgical procedure and therefore is comparatively safer and is used initially in managing significant valvar AS in children. During the process, a tube with an inflatable cuff is passed through the tube and inserted into the narrowed aortic valve. It is then inflated to expand the valve opening and thus increases the blood flow. The balloon is then deflated and taken away from the patient’s body.
This objective is often accomplished with a guarded balloon valvuloplasty whereby the peak-to-peak systolic gradient is reduced by 50%. Usually, the size of balloons is from eighty to one hundred percent of the size of the aortic valve anulus.
Pediatrics, Cardiology
Diagnosis: This usually entails a history and physical assessment performed on the patient with a primary focus on auscultation of the heart to detect murmurs.
Assessment and Risk Identification: An evaluation of the severity of the AS and the child’s overall status follows the diagnosis to confirm the diagnosis, acuity of the valvar AS, and assess the child for any associated symptoms or problems.
Pediatrics, Cardiology
After the diagnosis, a comprehensive assessment of the child’s condition is conducted to determine the severity of the valvar AS and assess its impact on the child’s overall health.
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