This document discusses congenital disorders, including their definition, causes, manifestations, and management. It begins by defining a congenital disorder as an abnormality present from birth that can be clinically obvious or diagnosed later in life. Common causes are discussed as well as the types of structural disorders including malformations and constraints. Several specific congenital anomalies are then described in more detail, including congenital heart diseases such as ventricular septal defects, gastrointestinal malformations like gastroschisis, and atresias. Nursing and medical management options are also addressed.
Transposition of the great arteries is a serious but rare heart defect present at birth (congenital), in which the two main arteries leaving the heart are reversed (transposed). The condition is also called dextro-transposition of the great arteries.
Congenital heart disease refers to abnormalities of the heart present from birth that arise during embryonic development. The abnormalities fall into two categories: shunts, which are abnormal connections that allow blood to flow between chambers in the wrong direction, and obstructions, which are narrowings that block blood flow. Shunts can cause cyanosis if deoxygenated blood enters the systemic circulation from the right side of the heart. Over time, increased blood flow or pressure from shunts can lead to pulmonary hypertension and structural changes that are irreversible. Common types of congenital heart defects include atrial and ventricular septal defects, patent ductus arteriosus, tetralogy of Fallot, and transposition of the great
Congenital heart disease is an abnormality present at birth that affects the structure or function of the heart. The most common types are acyanotic conditions like atrial septal defects, ventricular septal defects, and patent ductus arteriosus which allow blood to flow from the left to the right side of the heart. Cyanotic conditions like tetralogy of Fallot and transposition of the great arteries prevent oxygenated blood from reaching the body. Abnormal development during embryogenesis can disrupt the normal partitioning of the heart, leading to these defects.
1. The document discusses various types of acyanotic congenital heart disease including ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA), and coarctation of aorta.
2. It provides details on the classification, symptoms, diagnosis, and treatment options for each condition.
3. The conditions are characterized by shunting of blood between the left and right sides of the heart without cyanosis, and can cause heart failure if left untreated.
This document discusses congenital heart disease (CHD), including atrial septal defects (ASD) and tetralogy of Fallot (TOF). It defines the conditions, describes their signs and symptoms, and outlines their treatment and nursing care. Key points include the definition of ASD as an abnormal opening between the atria, and TOF as having four structural defects including pulmonary stenosis and a ventricular septal defect. Nursing focuses on decreasing cardiac workload, preventing infection, and supporting nutrition and development.
The document discusses various types of congenital heart defects including cyanotic and acyanotic defects, summarizing key features such as symptoms, physical exam findings, treatments, and anesthetic considerations for septal defects, atrial septal defects, patent ductus arteriosus, interrupted aortic arch, tetralogy of Fallot, and single ventricle physiology. It provides an overview of important congenital heart conditions from an anesthesiology perspective.
This document provides information on congenital heart disease (CHD), including:
- CHD occurs during the first 8 weeks of fetal development in approximately 5-8 per 1000 live births.
- Causes are usually multifactorial involving both genetic and environmental factors.
- Diagnosis involves physical examination, echocardiogram, chest x-ray, fetal echocardiography, and cardiac catheterization.
- Common types of CHD discussed include atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), and atrioventricular septal defect (AVSD). Signs, symptoms, pathophysiology, and treatment are described for
Transposition of the great arteries is a serious but rare heart defect present at birth (congenital), in which the two main arteries leaving the heart are reversed (transposed). The condition is also called dextro-transposition of the great arteries.
Congenital heart disease refers to abnormalities of the heart present from birth that arise during embryonic development. The abnormalities fall into two categories: shunts, which are abnormal connections that allow blood to flow between chambers in the wrong direction, and obstructions, which are narrowings that block blood flow. Shunts can cause cyanosis if deoxygenated blood enters the systemic circulation from the right side of the heart. Over time, increased blood flow or pressure from shunts can lead to pulmonary hypertension and structural changes that are irreversible. Common types of congenital heart defects include atrial and ventricular septal defects, patent ductus arteriosus, tetralogy of Fallot, and transposition of the great
Congenital heart disease is an abnormality present at birth that affects the structure or function of the heart. The most common types are acyanotic conditions like atrial septal defects, ventricular septal defects, and patent ductus arteriosus which allow blood to flow from the left to the right side of the heart. Cyanotic conditions like tetralogy of Fallot and transposition of the great arteries prevent oxygenated blood from reaching the body. Abnormal development during embryogenesis can disrupt the normal partitioning of the heart, leading to these defects.
1. The document discusses various types of acyanotic congenital heart disease including ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA), and coarctation of aorta.
2. It provides details on the classification, symptoms, diagnosis, and treatment options for each condition.
3. The conditions are characterized by shunting of blood between the left and right sides of the heart without cyanosis, and can cause heart failure if left untreated.
This document discusses congenital heart disease (CHD), including atrial septal defects (ASD) and tetralogy of Fallot (TOF). It defines the conditions, describes their signs and symptoms, and outlines their treatment and nursing care. Key points include the definition of ASD as an abnormal opening between the atria, and TOF as having four structural defects including pulmonary stenosis and a ventricular septal defect. Nursing focuses on decreasing cardiac workload, preventing infection, and supporting nutrition and development.
The document discusses various types of congenital heart defects including cyanotic and acyanotic defects, summarizing key features such as symptoms, physical exam findings, treatments, and anesthetic considerations for septal defects, atrial septal defects, patent ductus arteriosus, interrupted aortic arch, tetralogy of Fallot, and single ventricle physiology. It provides an overview of important congenital heart conditions from an anesthesiology perspective.
This document provides information on congenital heart disease (CHD), including:
- CHD occurs during the first 8 weeks of fetal development in approximately 5-8 per 1000 live births.
- Causes are usually multifactorial involving both genetic and environmental factors.
- Diagnosis involves physical examination, echocardiogram, chest x-ray, fetal echocardiography, and cardiac catheterization.
- Common types of CHD discussed include atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), and atrioventricular septal defect (AVSD). Signs, symptoms, pathophysiology, and treatment are described for
This document provides information on congenital heart disease (CHD), including:
- CHD occurs during the first 8 weeks of fetal development in approximately 5-8 per 1000 live births.
- Causes are usually multifactorial involving both genetic and environmental factors.
- Diagnosis involves physical examination, echocardiogram, chest x-ray, fetal echocardiography and cardiac catheterization.
- Common types of CHD discussed include atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), and atrioventricular septal defect (AVSD). Signs, symptoms, pathophysiology, and treatment are described for each
Congenital heart disease can result from abnormalities present at birth during embryonic development of the heart and vessels. Causes are often unknown but may include genetic and environmental factors. Defects are grouped as those causing right-to-left shunts, left-to-right shunts, or obstructions. Common left-to-right shunt defects include atrial septal defects (ASD), ventricular septal defects (VSD), patent ductus arteriosus (PDA), and atrioventricular septal defects. Right-to-left shunt defects include tetralogy of Fallot. Infective endocarditis is a bacterial infection of heart valves or tissues, characterized by vegetations
This document discusses coarctation of the aorta, including:
1. The definition and history of coarctation as a congenital narrowing of the upper descending thoracic aorta.
2. Theories on the pathogenesis of coarctation related to reduced blood flow through the left side of the heart or abnormal ductal tissue.
3. Types of coarctation including preductal and postductal, and surgical techniques for repair such as patch aortoplasty or bypass grafting.
4. Presentation varies from heart failure in neonates to hypertension in older children and adults, with complications including aneurysm and rupture.
The document discusses congenital heart diseases, which occur in approximately 1% of live births. It describes several types of congenital heart defects including atrial septal defect (ASD), ventricular septal defect (VSD), atrioventricular canal defect, and patent ductus arteriosus - all of which involve increased pulmonary blood flow. It also discusses obstructive defects like aortic stenosis and pulmonary stenosis. The document provides details on the pathophysiology, clinical manifestations, diagnosis, and treatment of these various congenital heart conditions.
The document discusses cyanotic and acyanotic heart disease. It begins with an overview of heart development during embryogenesis and the fetal circulation. It then covers topics like patent ductus arteriosus, ventricular septal defect, atrial septal defect, coarctation of aorta, tetralogy of Fallot, and tricuspid atresia. For each condition, it discusses incidence, etiology, pathophysiology, clinical features, diagnostic evaluation, and therapeutic management. The document provides a comprehensive review of important congenital heart defects.
An atrial septal defect (ASD) is a hole in the wall between the two upper chambers of the heart that allows blood to leak from the left to the right side. There are four main types of ASDs. A ventricular septal defect (VSD) is an abnormal opening in the wall between the two lower chambers, allowing blood to flow between the right and left ventricles. Patent ductus arteriosus (PDA) occurs when the fetal blood vessel between the aorta and pulmonary artery fails to close after birth, allowing blood to flow back to the lungs. These three types of congenital heart defects are considered non-cyanotic as they do not result in low blood oxygen.
The document discusses acyanotic heart disease, specifically defining acyanotic heart defects as congenital disorders with left to right shunting and obstructive lesions that are not always apparent at birth. It provides details on atrial septal defects (ASD) and ventricular septal defects (VSD), including their incidence, pathophysiology, clinical manifestations, diagnostic testing, and treatment options. The objectives are to define acyanotic heart defects, describe different types of defects, and discuss their etiology, clinical presentation, diagnosis, management, and relevant nursing care.
Congenital heart disease (CHD) occurs in 1 in 125 live births and is the most common birth defect. The majority of cases have no known cause and are thought to be due to a combination of genetic and environmental factors. Common types of CHD include atrial septal defects, ventricular septal defects, patent ductus arteriosus, coarctation of the aorta, and tetralogy of Fallot. Treatment options include medical management, interventional procedures such as catheterization, and surgical repair. Without treatment, CHD can lead to heart failure, lung problems, and other issues.
The document discusses congenital heart defects, specifically ventricular septal defects (VSD). A VSD is an opening in the ventricular septum that allows blood to pass between the left and right ventricles. VSDs are the most common type of congenital heart defect. While small VSDs may cause no symptoms, larger VSDs can lead to issues like congestive heart failure in infants. Physical exam may reveal murmurs. Treatment options include medical management or surgical closure of the defect.
This document discusses congenital heart defects, specifically atrial septal defects (ASD). It describes the types of ASDs, including ostium secundum, patent foramen ovale, and ostium primum. Clinical manifestations are typically asymptomatic for infants and children, with possible findings of a heart murmur, EKG changes, and enlarged heart on xray. Echocardiogram can diagnose and show the defect. Small defects may close on their own, while larger defects are treated with devices or surgery if causing heart failure or pulmonary hypertension. Surgical repair involves closing the defect with sutures or a patch.
Thoracic coarctation of the aorta is a congenital narrowing of the aorta adjacent to the site of attachment of the ductus arteriosus. It results from abnormal development of the fourth and sixth aortic arches during embryogenesis. There are two main theories for its pathogenesis: decreased blood flow in the aortic isthmus causes hypoplasia, or migration of ductal smooth muscle cells into the aorta causes constriction. Coarctation presents in infancy as congestive heart failure if the ductus closes abruptly, or later in life with hypertension, reduced femoral pulses, and complications like aneurysm or rupture. Treatment is surgical repair or catheter-based procedures to relieve the obstruction.
A congenital heart defect is a problem with the structure of the heart. It is present at birth. Congenital heart defects are the most common type of birth defect. The defects can involve the walls of the heart, the valves of the heart, and the arteries and veins near the heart. They can disrupt the normal flow of blood through the heart. The blood flow can slow down, go in the wrong direction or to the wrong place, or be blocked completely.
Doctors use a physical exam and special heart tests to diagnose congenital heart defects. They often find severe defects during pregnancy or soon after birth. Signs and symptoms of severe defects in newborns include
Rapid breathing
Cyanosis - a bluish tint to the skin, lips, and fingernails
Fatigue
Poor blood circulation
Many congenital heart defects cause few or no signs and symptoms. They are often not diagnosed until children are older.
Many children with congenital heart defects don't need treatment, but others do. Treatment can include medicines, catheter procedures, surgery, and heart transplants. The treatment depends on the type of the defect, how severe it is, and a child's age, size, and general health.
The document discusses various types of congenital heart disease, including their definitions, classifications, clinical presentations, and treatments. It covers topics such as ventricular septal defects, atrial septal defects, fetal circulation, dextrocardia, and grown-up congenital heart disease. Specific congenital defects are explained in detail with regards to their symptoms, diagnostic evaluations, potential complications, and management approaches.
The document discusses several congenital heart diseases including ventricular septal defects (VSD), atrial septal defects (ASD), patent ductus arteriosus (PDA), pulmonary stenosis, aortic stenosis, and coarctation of the aorta. It describes the pathophysiology, clinical presentation, investigations, and management of each condition. Cyanotic heart diseases are defined as those involving a right-to-left or left-to-right shunt leading to low oxygen saturation. The document provides classification, epidemiology, etiology and detailed information about specific lesions causing cyanosis.
Congenital heart defects are problems with the heart's structure that are present at birth. They can involve the interior walls, valves, or arteries and veins around the heart. Signs and symptoms depend on the type and severity of defect and may include shortness of breath, cyanosis, fainting, or murmurs. Congenital heart defects are caused by genetic and environmental factors and occur during embryonic development when heart structures are forming.
The document provides information on congenital heart defects, including their causes, types, signs and symptoms, diagnosis, and treatment. It discusses several specific defects in detail, including aortic stenosis, coarctation of the aorta, pulmonary stenosis, and patent ductus arteriosus. The key points are:
1. Congenital heart defects can involve the heart's chambers, valves, or vessels and have various causes including genetic syndromes.
2. Specific defects like aortic stenosis and pulmonary stenosis can cause obstruction to blood flow while others like patent ductus arteriosus allow extra blood flow to the lungs.
3. Symptoms depend on the severity of the defect but may include heart failure,
This document discusses pediatric cardiac disorders, including:
1. Congenital heart defects (CHDs) are the most common birth defects and cause of infant mortality. CHDs can be acyanotic (left-to-right shunts) or cyanotic (right-to-left shunts). Common defects include atrial and ventricular septal defects, patent ductus arteriosus, tetralogy of Fallot, and transposition of the great arteries.
2. Assessment of suspected CHD involves history, physical exam including pulse oximetry, chest x-ray, EKG, and echocardiogram. Major signs are systolic murmurs, diastolic murmurs, cyan
There are two main types of congenital heart failures in children - cyanotic and non-cyanotic. Some common types include ventricular septal defects, atrial septal defects, patent ductus arteriosus, tetralogy of Fallot, transposition of the great arteries, and hypoplastic left heart syndrome. Many of these defects can be repaired through surgical procedures like closing holes or reconstructing heart structures. Without treatment, congenital heart failures can lead to complications like heart failure, pulmonary hypertension, or developmental delays.
Congenital heart defects are abnormalities in the heart's structure that are present at birth. They can affect the heart's chambers, valves, or blood vessels. Common defects include ventricular septal defects (holes in the wall between the ventricles), atrial septal defects, patent ductus arteriosus, tetralogy of Fallot, and transposition of the great arteries. Many defects cause no symptoms, but some can lead to heart failure, lung problems, or other serious issues if not treated with medication, catheterization, or surgery. Diagnosis involves medical history, physical exam, chest X-ray, electrocardiogram, and echocardiogram.
1. Congenital heart defects are abnormalities in the heart's structure that are present at birth. They can affect the heart's chambers, valves, or blood vessels.
2. The majority of congenital heart diseases have an unknown cause, resulting from defective development of the fetal heart between 3-8 weeks of gestation.
3. Common defects include ventricular and atrial septal defects, patent ductus arteriosus, tetralogy of Fallot, transposition of the great arteries, and truncus arteriosus. Diagnosis relies on clinical history, examination, and investigations like echocardiography.
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This document provides information on congenital heart disease (CHD), including:
- CHD occurs during the first 8 weeks of fetal development in approximately 5-8 per 1000 live births.
- Causes are usually multifactorial involving both genetic and environmental factors.
- Diagnosis involves physical examination, echocardiogram, chest x-ray, fetal echocardiography and cardiac catheterization.
- Common types of CHD discussed include atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), and atrioventricular septal defect (AVSD). Signs, symptoms, pathophysiology, and treatment are described for each
Congenital heart disease can result from abnormalities present at birth during embryonic development of the heart and vessels. Causes are often unknown but may include genetic and environmental factors. Defects are grouped as those causing right-to-left shunts, left-to-right shunts, or obstructions. Common left-to-right shunt defects include atrial septal defects (ASD), ventricular septal defects (VSD), patent ductus arteriosus (PDA), and atrioventricular septal defects. Right-to-left shunt defects include tetralogy of Fallot. Infective endocarditis is a bacterial infection of heart valves or tissues, characterized by vegetations
This document discusses coarctation of the aorta, including:
1. The definition and history of coarctation as a congenital narrowing of the upper descending thoracic aorta.
2. Theories on the pathogenesis of coarctation related to reduced blood flow through the left side of the heart or abnormal ductal tissue.
3. Types of coarctation including preductal and postductal, and surgical techniques for repair such as patch aortoplasty or bypass grafting.
4. Presentation varies from heart failure in neonates to hypertension in older children and adults, with complications including aneurysm and rupture.
The document discusses congenital heart diseases, which occur in approximately 1% of live births. It describes several types of congenital heart defects including atrial septal defect (ASD), ventricular septal defect (VSD), atrioventricular canal defect, and patent ductus arteriosus - all of which involve increased pulmonary blood flow. It also discusses obstructive defects like aortic stenosis and pulmonary stenosis. The document provides details on the pathophysiology, clinical manifestations, diagnosis, and treatment of these various congenital heart conditions.
The document discusses cyanotic and acyanotic heart disease. It begins with an overview of heart development during embryogenesis and the fetal circulation. It then covers topics like patent ductus arteriosus, ventricular septal defect, atrial septal defect, coarctation of aorta, tetralogy of Fallot, and tricuspid atresia. For each condition, it discusses incidence, etiology, pathophysiology, clinical features, diagnostic evaluation, and therapeutic management. The document provides a comprehensive review of important congenital heart defects.
An atrial septal defect (ASD) is a hole in the wall between the two upper chambers of the heart that allows blood to leak from the left to the right side. There are four main types of ASDs. A ventricular septal defect (VSD) is an abnormal opening in the wall between the two lower chambers, allowing blood to flow between the right and left ventricles. Patent ductus arteriosus (PDA) occurs when the fetal blood vessel between the aorta and pulmonary artery fails to close after birth, allowing blood to flow back to the lungs. These three types of congenital heart defects are considered non-cyanotic as they do not result in low blood oxygen.
The document discusses acyanotic heart disease, specifically defining acyanotic heart defects as congenital disorders with left to right shunting and obstructive lesions that are not always apparent at birth. It provides details on atrial septal defects (ASD) and ventricular septal defects (VSD), including their incidence, pathophysiology, clinical manifestations, diagnostic testing, and treatment options. The objectives are to define acyanotic heart defects, describe different types of defects, and discuss their etiology, clinical presentation, diagnosis, management, and relevant nursing care.
Congenital heart disease (CHD) occurs in 1 in 125 live births and is the most common birth defect. The majority of cases have no known cause and are thought to be due to a combination of genetic and environmental factors. Common types of CHD include atrial septal defects, ventricular septal defects, patent ductus arteriosus, coarctation of the aorta, and tetralogy of Fallot. Treatment options include medical management, interventional procedures such as catheterization, and surgical repair. Without treatment, CHD can lead to heart failure, lung problems, and other issues.
The document discusses congenital heart defects, specifically ventricular septal defects (VSD). A VSD is an opening in the ventricular septum that allows blood to pass between the left and right ventricles. VSDs are the most common type of congenital heart defect. While small VSDs may cause no symptoms, larger VSDs can lead to issues like congestive heart failure in infants. Physical exam may reveal murmurs. Treatment options include medical management or surgical closure of the defect.
This document discusses congenital heart defects, specifically atrial septal defects (ASD). It describes the types of ASDs, including ostium secundum, patent foramen ovale, and ostium primum. Clinical manifestations are typically asymptomatic for infants and children, with possible findings of a heart murmur, EKG changes, and enlarged heart on xray. Echocardiogram can diagnose and show the defect. Small defects may close on their own, while larger defects are treated with devices or surgery if causing heart failure or pulmonary hypertension. Surgical repair involves closing the defect with sutures or a patch.
Thoracic coarctation of the aorta is a congenital narrowing of the aorta adjacent to the site of attachment of the ductus arteriosus. It results from abnormal development of the fourth and sixth aortic arches during embryogenesis. There are two main theories for its pathogenesis: decreased blood flow in the aortic isthmus causes hypoplasia, or migration of ductal smooth muscle cells into the aorta causes constriction. Coarctation presents in infancy as congestive heart failure if the ductus closes abruptly, or later in life with hypertension, reduced femoral pulses, and complications like aneurysm or rupture. Treatment is surgical repair or catheter-based procedures to relieve the obstruction.
A congenital heart defect is a problem with the structure of the heart. It is present at birth. Congenital heart defects are the most common type of birth defect. The defects can involve the walls of the heart, the valves of the heart, and the arteries and veins near the heart. They can disrupt the normal flow of blood through the heart. The blood flow can slow down, go in the wrong direction or to the wrong place, or be blocked completely.
Doctors use a physical exam and special heart tests to diagnose congenital heart defects. They often find severe defects during pregnancy or soon after birth. Signs and symptoms of severe defects in newborns include
Rapid breathing
Cyanosis - a bluish tint to the skin, lips, and fingernails
Fatigue
Poor blood circulation
Many congenital heart defects cause few or no signs and symptoms. They are often not diagnosed until children are older.
Many children with congenital heart defects don't need treatment, but others do. Treatment can include medicines, catheter procedures, surgery, and heart transplants. The treatment depends on the type of the defect, how severe it is, and a child's age, size, and general health.
The document discusses various types of congenital heart disease, including their definitions, classifications, clinical presentations, and treatments. It covers topics such as ventricular septal defects, atrial septal defects, fetal circulation, dextrocardia, and grown-up congenital heart disease. Specific congenital defects are explained in detail with regards to their symptoms, diagnostic evaluations, potential complications, and management approaches.
The document discusses several congenital heart diseases including ventricular septal defects (VSD), atrial septal defects (ASD), patent ductus arteriosus (PDA), pulmonary stenosis, aortic stenosis, and coarctation of the aorta. It describes the pathophysiology, clinical presentation, investigations, and management of each condition. Cyanotic heart diseases are defined as those involving a right-to-left or left-to-right shunt leading to low oxygen saturation. The document provides classification, epidemiology, etiology and detailed information about specific lesions causing cyanosis.
Congenital heart defects are problems with the heart's structure that are present at birth. They can involve the interior walls, valves, or arteries and veins around the heart. Signs and symptoms depend on the type and severity of defect and may include shortness of breath, cyanosis, fainting, or murmurs. Congenital heart defects are caused by genetic and environmental factors and occur during embryonic development when heart structures are forming.
The document provides information on congenital heart defects, including their causes, types, signs and symptoms, diagnosis, and treatment. It discusses several specific defects in detail, including aortic stenosis, coarctation of the aorta, pulmonary stenosis, and patent ductus arteriosus. The key points are:
1. Congenital heart defects can involve the heart's chambers, valves, or vessels and have various causes including genetic syndromes.
2. Specific defects like aortic stenosis and pulmonary stenosis can cause obstruction to blood flow while others like patent ductus arteriosus allow extra blood flow to the lungs.
3. Symptoms depend on the severity of the defect but may include heart failure,
This document discusses pediatric cardiac disorders, including:
1. Congenital heart defects (CHDs) are the most common birth defects and cause of infant mortality. CHDs can be acyanotic (left-to-right shunts) or cyanotic (right-to-left shunts). Common defects include atrial and ventricular septal defects, patent ductus arteriosus, tetralogy of Fallot, and transposition of the great arteries.
2. Assessment of suspected CHD involves history, physical exam including pulse oximetry, chest x-ray, EKG, and echocardiogram. Major signs are systolic murmurs, diastolic murmurs, cyan
There are two main types of congenital heart failures in children - cyanotic and non-cyanotic. Some common types include ventricular septal defects, atrial septal defects, patent ductus arteriosus, tetralogy of Fallot, transposition of the great arteries, and hypoplastic left heart syndrome. Many of these defects can be repaired through surgical procedures like closing holes or reconstructing heart structures. Without treatment, congenital heart failures can lead to complications like heart failure, pulmonary hypertension, or developmental delays.
Congenital heart defects are abnormalities in the heart's structure that are present at birth. They can affect the heart's chambers, valves, or blood vessels. Common defects include ventricular septal defects (holes in the wall between the ventricles), atrial septal defects, patent ductus arteriosus, tetralogy of Fallot, and transposition of the great arteries. Many defects cause no symptoms, but some can lead to heart failure, lung problems, or other serious issues if not treated with medication, catheterization, or surgery. Diagnosis involves medical history, physical exam, chest X-ray, electrocardiogram, and echocardiogram.
1. Congenital heart defects are abnormalities in the heart's structure that are present at birth. They can affect the heart's chambers, valves, or blood vessels.
2. The majority of congenital heart diseases have an unknown cause, resulting from defective development of the fetal heart between 3-8 weeks of gestation.
3. Common defects include ventricular and atrial septal defects, patent ductus arteriosus, tetralogy of Fallot, transposition of the great arteries, and truncus arteriosus. Diagnosis relies on clinical history, examination, and investigations like echocardiography.
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2. Objectives
At the end of this chapter you will be able to ;
• Define a congenital disorder.
• List the causes of congenital disorders.
• Describe manifestations of d/t congenital disorder
• Describe nursing & medical management options of
d/t congenital disorder
10/1/2023 2
3. Congenital disorder
It is an abnormality of structure or function in
a person, which is present from birth.
It may be clinically obvious at birth, or may
only be diagnosed sometime later in life.
10/1/2023 3
4. Congenital disorder…
Congenital malformation -only the structural
defects present at the birth.
Congenital anomaly- Includes all the
biochemical, structural & functional disorders
present at the birth
10/1/2023 4
5. Con…
Structural congenital disorders can be divided
into two large groups.
• Malformations
• Constraint.
A malformation is a structural congenital
disorder which develops during the first
trimester and is caused by failure of the
embryo to develop normally.
10/1/2023 5
6. Con…
Constraint is external forces can result in
congenital disorders after the fetus is already
normally formed. That is after the first
trimester.
There are two types of congenital disorders
due to constraint:
• Deformities: normally formed fetus is pushed
out of shape by a mechanical force in the
uterus
• Disruptions
10/1/2023 6
8. 1. Congenital heart diseases
Objectives
At the end of this session you will be able to ;
–Overview normal fetal circulation
–Define CHD
–Discuss the types of CHD
–Describe manifestations of d/t CHDs
–Describe management options of d/t CHDs
10/1/2023 8
9. Overview of fetal circulation
Knowledge about fetal circulation is absolutely
necessary for proper understanding of congenital
heart diseases.
Umbilical cord:
2 umbilical arteries: return de-oxygenated blood, fetal
waste, CO2 to placenta
1umbilical vein: brings oxygenated blood and nutrients to
the fetus(from placenta)
10/1/2023 9
10. Con…
Three shunts are present in fetal life:
Ductus venosus: connects the umbilical vein to the
inferior vena cava
Ductus arteriosus: connects the main pulmonary artery
to the aorta
Foramen ovale: anatomic opening between the right and
left atrium.
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11. Con…
Fetus receives oxygenated blood from the placenta by umbilical
vein, which enters the fetus at the umbilicus.
The umbilical vein carries blood to the liver & given off
branches to the left lobe to supply the oxygenated blood &
receives the deoxygenated blood from portal vein
Most of the umbilical venous blood by passes the liver though
the ductus venosus & enters in the inferior vena cava (also
contains the deoxygenated blood from lower extremities), then
to the right atrium
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12. Con…
From right atrium(RA),1/3rd of return blood enters the
left atrium(LA)though the foramen ovale & the rest 2/3rd
flows to the right ventricle(RV)
In the LA, there is mixing of blood received from right
atrium with the small amount of venous blood returning
from the lungs through the pulmonary veins.
From LA, blood flows to the LV which is then pumped in to
ascending aorta & arch of aorta to supply heart, head, neck
& upper extremities.
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13. Fetal circulation con…
The right ventricular blood is pumped in to the pulmonary
trunk & a small amount of it enters the pulmonary circulation.
The major portion the blood by passes the non functioning
lungs through the ductus arteriosus in to the descending aorta
&mixed with the small amount of blood from aortic arch which
then supply to lower extremities & other structures below the
diaphragm
The deoxygenated blood leaves the blood by two umbilical
arteries(branches of internal iliac arteries)
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15. CONGENITAL HEART DISEASES(CHD)
A problem in the structure of the heart or great vessels, present
at birth.
Symptoms can vary from non to life-threatening.
Causes of CHD
Idiopathic in most of the cases
Chromosomal abnormality
Adverse maternal conditions (environmental)Congenital infections:
Rubella (PDA), Substance abuse: Alcohol (VSD), Drugs – valproate
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16. Types of congenital heart disease
1. Acyanotic congenital heart disease
Increased pulmonary blood flow due to left to right shunt
Ventricular septal defect
Atrial septal defect
Patent ductus arteriosus
Coarctation of aorta
2. Cyanotic congenital heart disease (4T’S)
Decreased pulmonary blood flow due to right to left shunt
Tetralogy of fallot
Truncus arteriosus.
Transposition of great arteries
Tricuspid atresia
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18. A cyanotic CHD
Ventricular septal defect
The commonest congenital heart defect
40% of all congenital heart diseases
Small defects (< 5mm) close spontaneously
Blood shunts from left to right at ventricular level with
excess flow to the lung
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23. Diagnosis
Clinical
CXR - Right. V & A enlargement
- Large pulmonary artery
- ↑ed pulmonary vascularity
Echocardiography
Complications - pulmonary Hypertension, Eismenger
syndrome
Treatment
Surgery-for all symptomatic
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24. Patent ductus arteriosus(PDA)
Defect range from few mm to large cm
Left to right shunt at arterial level
Excess blood flow to the lung
Enlarged pulmonary artery and left atrium related to blood
volume
Uncorrected defect leads to pulmonary vascular disease and
flow reversal
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26. Patent Ductus Arteriosus…
• Symptoms:
– May be asymptomatic if small
– continuous, often machinery-sounding murmur best
heard over the upper left chest below the clavicle
– Dyspnea, tachypena, tachycardia
– Frequent respiratory infections
– Poor feeding , fatigue,
– No wt gain,
– Irritability
– If PDA is large size ,child may go for congestive heart
failure
10/1/2023 26
27. Diagnosis
- Clinical
- Chest X-ray
- Echocardiography
Prognosis
- Small PDA - normal life
- Large PDA - CHF
Treatment - Medical-indomethacin( Prostaglandin synthase
inhibitor)
- Surgical closure
10/1/2023 27
28. Acyanotic CHD…
2.3 Coarctation of the Aorta
• Occur at any site from the arch of aorta to iliac
bifurcation
10/1/2023
Coarctation of the Aorta
Occur at any site from the arch of aorta to iliac
bifurcation
28
29. Con …
Classic signs
1- Disparty in pulse & BP
2 - Radio-femoral delay
3- Systolic M at inter-scapular area
Treatment
- Medical - IV PGE1 in neonatal age
- Surgery
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30. Cyanotic CHD (right – to left shunt)
Develop symptoms early
Cyanosis is the main feature
Respiratory distress
Signs of CHF if there is severe obstruction or excess flow
to the lung
The presence of VSD and PDA is life saving by mixing
more oxygenated blood
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31. Tetralogy of fallot
Tetralogy of fallot comprises:
1.Right Ventricular hypertrophy,
2.Plumonary stenosis
3.VSD and
4. Dextroposition of aorta.
Deoxygenated blood mixes through
VSD.
The degree of severity is determined by
the size of pulmonary stenosis.
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32. Presenting Symptoms of TOF
Diagnosed with first few weeks of life
Loud murmur
Cyanosis
Respiratory distress
“Tet Spells”
Infant assume Squatting position(knee chest)
CXR - Narrow base & uplifted apex
- A boot or wooden shoe heart
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34. Transposition of great arteries
Aorta arise from RV and
pulmonary artery from LV.
Deoxygenated blood from RV
circulates to the body
While oxygenated blood goes to
the lungs.
Patient dies soon unless there is
mixing of the two parallel
circulation via ASD, VSD or PDA.
10/1/2023 34
35. Clinical manifestations
- Cyanosis, hypoxemia, respiratory distress
- Soft systolic ejection murmur at the lt middle sternal border
or murmur may be absent
Diagnosis
- CXR – mild cardiomegaly with marrow mediastinum (egg-
shaped heart)
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39. Gastroschisis
In the womb, fetal intestines develop outside
of the abdomen for a brief time.
In normal cases, the intestines return to
the abdominal cavity,andthe baby’s abdomen
closes before birth.
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40. Gastroschisis…
It is an abdominal—wall defect that occurs
on the side of the umbilical cord (umbilicus).
The baby is born with intestines
protruding through this defect, and no
protective sac is present.
It is a life—threatening defect, requiring
immediate intervention
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43. EXOMPHALOS(OMPHALOCOELE)
Exomphalos is a weak of the baby’s abdominal
wall where the umbilical cord joins it.
This weakness allows the abdominal contents,
mainly the bowel and the liver protrude
outside the abdominal cavity where they are
contained in a loose sac
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44. EXOMPHALOS(OMPHALOCOELE)
It is a defect in which the bowel or other viscera
protrude through the umbilicus where they are
contained in a loose sac
10/1/2023 44
46. MANAGEMENT
After birth the cord should be clamped well
away from the exomphalos.
The abnormality should be covered with sterile
gauze or plastic wrapping.
Whether the exomphalos is big or small, all
these infants must be urgently Referred
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47. ATRESIAS
It is defined as a congenital absence or
abnormal narrowing of a body opening.
It can affect different sites in the GI tract
:Esophageal, Duodenal, Small bowel, Colonic
or Anorectal.
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48. Esophageal Atresia
It is an obstruction of the esophagus due to a section of
the esophagus which is missing.
It is usually associated with a connection (fistula)
between the lower oesophagus and the bronchi
The feed, which cannot be swallowed, is inhaled into the
lungs. Gastric acid passes from the stomach into the
bronchi, via a fistula, especially when the infants lie
down.
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49. Esophageal Atresia…
Both inhaled feeds and the reflux of gastric acid
result in respiratory distress.
Do not feed any infant that you suspect of
having an esophageal atresia.
The diagnosis is may evidenced by the inability
to pass a nasogastric tube.
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50. DUODENAL ATRESIA
It is an obstruction of the duodenum.
Soon after delivery the infant starts vomiting.
The vomit is often bile stained
The diagnosis is easily confirmed by an abdominal
X—ray
Theseinfants must bekept nil per mouth, the stomach
should be emptied via a nasogastric tube.
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51. Cleft lip & cleft palate
• Cleft lip
The congenital deformity of a cleft the upper lip, on one
or both sides.
• Cleft palate
A fissure in the midline of the palate due to failure of the
two sides to fuse in embryonic development.
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53. MANAGEMENT
Cleft lip repair. The edges of the cleft between the lip and nose are cuf (A and B). The
bottom ol the nostril is formed with sufure (C). The upper part of the lip tissue is
closed (D), and the stitches are extended down to close the opening entirely (E).
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56. Club foot…
• It is one of the most common orthopedic
defect
• It occurs approximately 1 in 1000 to 1 in 700
live births
• Boys are affected twice as often as girls
10/1/2023 56
57. Risk factors
• Sex. Clubfoot is more common in males.
• Family history. If either one of the parents or their other
children have had clubfoot, the baby is more likely to
have it as well.
• Smoking during pregnancy.
• Not enough amniotic fluid during pregnancy.
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58. Pathophysiology
• Club foot is a foot that has been twisted out of
its normal shape or position in utero and is
fixed.
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59. Types of club foot
• Various types of club foot , it is the combination of
10/1/2023 59
Term Meaning
1.Talipes Foot and ankle
2.Varus Bending inward
3.Valgus Bending outward
4.Equinus Toes are lower than heel
5.Calcaneus Toes are higher than the heel
61. Types of club foot
• Talipes equinovarus ;
– It is the type of clubfoot that
occurs 95% of cases
– The foot is fixed in planter flexion
and deviate medially, i.e heel is
elevated.
– If the condition is not corrected
the child walks on the toe and
outer border of foot.
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62. Types of club foot…
• Talipescalcaneus
valgus:
– The foot is dorsiflexed
and deviate laterally. That
is heel turned outward
from the midline of the
body.
– If it is not corrected the
child walks on outwardly
turned heel and the inner
border of the foot.
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63. Types of club foot…
• Talipes varus:
– It is due to the heels being turned inward from
the midline if the leg only outer portion of the
sole rest on the floor.
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64. Types of club foot…
• Talipes valgus:
– It due to heel is being turned outward from the
midline of the leg only inner side of the sole rest
on the floor.
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65. Types of club foot…
• Talipes calcaneovarus:
– It is due to the heel being turned
towards the midline of the body
and the anterior part of the foot
being elevated. Only heel rest on
the floor.
• The whole foot bends internal to the
knee.
• The changes affect the muscles,
tendons, ligaments, bone skeleton
and skin of the deformed foot.
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66. Clinical manifestation
• Painless
• Change in shape of the foot
o Either Inward and down ward twistin
– The calf muscles in the affected leg are usually
underdeveloped.
– The affected foot may be up to 1/2 inch (about 1
centimeter) shorter than the other foot.
Diagnosis
Observation
Imaging(x-ray,CT-scan,MRI)
10/1/2023 66
67. Therapeutic management
The treatment include
• Manipulation of the foot
– It is manipulation or exercise program to
be done several times a day.
10/1/2023 67
68. Therapeutic management…
• Care of infant in a Denis Browne splint
– This appliance is made of two foot plates
attached to a cross bar. When splint is fitted
to the feet various positions of angulations
can be maintained.
– As the infants kicks the foot are
automatically moved in to correct position.
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70. Therapeutic management…
• Care of infant or child in caste
– Use of caste provide gradual stretching of tight
muscle and contraction of previously relaxed
muscle until a position of overcorrection has
been reached
– Caste has to be replaced every 1-2 weeks
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72. DDH…
• Developmental dysplasia of the hip, occurs
when a child is born with an unstable hip
due to abnormal formation of the hip joint
during early stages of fetal development.
• This instability worsens as the child grows
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73. Causes
It is unknown, but certain factors such as
family history,
intrauterine position,
delivery type and
postnatal positioning are believed to affect the
risk of DDH.
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74. Pathophysiology
• Developmental dysplasia of the hip (DDH)
involves abnormal growth of the hip.
• Ligamentous laxity is also believed to be
associated with hip dysplasia, although this
association is less clear.
– Ligamentous laxity is a term given to describe
"loose ligaments”
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75. Clinical manifestation
• In Infants,
o Shortening of limb on affected side
o Restricted abduction of hip on affected side
o Unequal gluteal folds
o Positive ortolani test
o Positive Barlow test
Older infant and child
– Affected leg shorter than the other
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76. Therapeutic management
• Treatment varies with the child's age and extent of
dysplasia.
• The goal of treatment is to obtain and maintain a safe,
congruent position of the hip joint to promote normal
hip joint development.
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77. Therapeutic management
• Newborn to age 6 months:
– Pavli harness is used to maintain a safe position of
hip joint . Harness is worn continuously until the hip
is proved stable on clinical examination, usually
about 3- 5 months .
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78. Therapeutic management
Age 6 to 18 months:
– If child has shortening of limb and contracture of hip
adductor and flexor muscle became evident , the
reduction by traction is used for 3 weeks.
– Child under go a closed reduction of hip using
general anesthesia, if the hip is not reducible, an open
reduction is performed. After reduction child is
placed in hip spica cast for 2-4 months until hip is
stable.
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79. Therapeutic management
Older children
– Operative reduction which involve preoperative
traction , tenotomy of contracted muscle .
– When open reduction is performed, the patient wears
a Spica cast for 6 weeks
– Range of motion exercise for restore movement.
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80. Nursing management
• Teaching the parents to apply and maintain the reduction
device
Skin care include
– Check frequently the red areas under the stripes and
clothing
– Gently massage the healthy skin under the stripes once a
daily to stimulate circulation
– Place diaper under the stripes
– Assess for irritation, and maintain cleanliness of child in
cast
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83. CONGENITAL GENITO URINARY
DISORDER
These problems usually required surgical
correction.
Some of them are producing no clinical
symptoms.
10/1/2023 83
84. Renal agenesis
• Definition :It is the absence of kidney due to failure of
ureteric bud formation.
• It may be Bilateral or Unilateral
10/1/2023 84
85. Exstrophy of bladder
It is a congenital malformation in which lower portion of the
abdominal wall and the anterior wall of the bladder are missing so
that bladder is everted through the opening and may found on the
lower abdomen with continuous passage of urine to the outside.
Male are more commonly affected
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86. Clinical manifestations:
Urinary dribbling
Skin excoriation
Infection and ulceration
UTI
Diagnosis :
Cystoscopic examination
X-ray
US
10/1/2023 86
87. Management :
Surgical closure of the bladder within 48 hours
Supportive nursing care
Pre operative care
Post operative care
Follow up
10/1/2023 87
88. Hypospadias
it is the congenital abnormal urethral opening on
the ventral aspect( under surface ) of the penis.
Classification :
Anterior hypospadias(65 to 70%) : it may found as
glandular or coronal or on distal penile shaft
Middle (10-15%) penile shaft hypospadias.
Posterior hypospadias(20%) : it may be found on
proximal penile shaft or as penoscrotal,scortal or
perineal type.
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89. Problems related to hypospadias :
1.Painful downward curvature of penis
2.Deflected stream of urine
3.Inability void urine while standing
Management :
surgical reconstruction
Meatotomy
Chordee correction
urethroplasty
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90. Phimosis
Narrow opening of the prepuce that prevents it being drawn back
over the glans penis.
Management:
Circumcision
apply Betamethasone cream
10/1/2023 90
91. Paraphimosis
It is the retraction of a phimotic foreskin, behind coronal sulcus.
It may develop phimotic child which also need for surgical
management by circumcision or reduction with application of
lubricant under deep sedation.
Clinical features :
edematous
Severe pain
10/1/2023 91
92. Nursing management of the child with
urologic surgery
Preparing for diagnostic procedures
Involving the parents in child care
Monitoring intake and output
Preventing infections
Providing comfort
Providing adequate nutrition
Teaching the parents about related care
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95. Classification
1. Obstructive / non communicating : caused by a
block in the passage of fluid.
2. Communicating/ extra ventricular fluid passes
between the ventricles and spinal cord
10/1/2023 95
98. Microcephaly
Disorder in which brain growth is so slow that it fails
more than three standard deviations below normal on
growth charts.
Causes:
Intrauterine infection (rubella, cytomegalovirus,
toxoplasmosis)
Severe malnutrition or anoxia in
infancy
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99. Spinal bifida
A developmental birth defect involving the neural
tube:
Incomplete closure of the embryonic neural tube
results in an incompletely formed spinal cord.
The vertebrae overlying the open portion of the spinal
cord do not fully form and remain unfused and open
10/1/2023 99
100. Con…
Types:
1. Spinal bifida occulata
2. Myelomeningocele
3. Meningocele
The most common location of the malformations is
the lumbar and sacral areas of the spinal cord
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101. Con…
Spinal bifida occulata
no opening of the back, but the outer
part of some of the vertebrae are not
completely closed
The skin at the site of the lesion may
be normal, or it may have some hair
growing from it
10/1/2023 101
102. Con…
Myelomeningocele
Most common
Portion of the spinal column allows the spinal cord to
protrude through an opening in the overlying
vertebrae
10/1/2023 102
103. Con…
Meningocele
Meninges covering the spinal cord herniate through
the unformed vertebrae
Sacks does not contain part of spinal cord
10/1/2023 103
104. Management
No cure for nerve damage
Closure ofthe opening onthe back
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