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Questions and Answers
What does a higher cardio-thoracic (CT) ratio indicate?
What does a higher cardio-thoracic (CT) ratio indicate?
Children should have a CT ratio of less than 0.55.
Children should have a CT ratio of less than 0.55.
True
What medical management is prescribed for congestive cardiac failure (CCF)?
What medical management is prescribed for congestive cardiac failure (CCF)?
Digoxin and Furosemide
Increased blood flow to the ___________ leads to pulmonary congestion and recurrent pneumonia.
Increased blood flow to the ___________ leads to pulmonary congestion and recurrent pneumonia.
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Match the following complications with their descriptions:
Match the following complications with their descriptions:
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What is an indication for elective surgical closure of an ASD?
What is an indication for elective surgical closure of an ASD?
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Patent Ductus Arteriosus is more common in term babies than preterm babies.
Patent Ductus Arteriosus is more common in term babies than preterm babies.
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What can cause a left fascicular block in relation to ASD?
What can cause a left fascicular block in relation to ASD?
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Eisenmenger syndrome is characterized by the reversal of the ______.
Eisenmenger syndrome is characterized by the reversal of the ______.
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Match the following ASD types with their descriptions:
Match the following ASD types with their descriptions:
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What is the most common location for coarctation of the aorta?
What is the most common location for coarctation of the aorta?
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Prostaglandin production is curative in term babies with cyanotic congenital heart defects.
Prostaglandin production is curative in term babies with cyanotic congenital heart defects.
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Name two medications used in the medical management of congestive heart failure in neonates.
Name two medications used in the medical management of congestive heart failure in neonates.
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A significant feature of partial narrowing in coarctation of the aorta is systemic hypertension due to increased ______.
A significant feature of partial narrowing in coarctation of the aorta is systemic hypertension due to increased ______.
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Match the following features of coarctation of the aorta with their descriptions:
Match the following features of coarctation of the aorta with their descriptions:
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Which syndrome is associated with TOF and conotruncal defects?
Which syndrome is associated with TOF and conotruncal defects?
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Central cyanosis with improvement upon 02 inhalation is a major criterion for diagnosing Congenital Heart Disease.
Central cyanosis with improvement upon 02 inhalation is a major criterion for diagnosing Congenital Heart Disease.
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What type of genetic defect is associated with Williams Syndrome?
What type of genetic defect is associated with Williams Syndrome?
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The major criteria for diagnosing Congenital Heart Disease include a systolic murmur graded at or above ______.
The major criteria for diagnosing Congenital Heart Disease include a systolic murmur graded at or above ______.
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Match the following syndromes with their associated conditions:
Match the following syndromes with their associated conditions:
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What is a confirmed laboratory evidence for Cystic Fibrosis?
What is a confirmed laboratory evidence for Cystic Fibrosis?
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Antibiotics are not part of the treatment plan for Cystic Fibrosis.
Antibiotics are not part of the treatment plan for Cystic Fibrosis.
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What type of diet is recommended for patients with Cystic Fibrosis?
What type of diet is recommended for patients with Cystic Fibrosis?
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The sweat chloride test uses __________ to measure chloride levels in sweat.
The sweat chloride test uses __________ to measure chloride levels in sweat.
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Match the following treatments with their respective descriptions:
Match the following treatments with their respective descriptions:
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What is a common compensatory change noted in older children with coarctation of the aorta?
What is a common compensatory change noted in older children with coarctation of the aorta?
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Inferior rib notching is commonly associated with coarctation of the aorta.
Inferior rib notching is commonly associated with coarctation of the aorta.
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What is the primary treatment method for coarctation of the aorta?
What is the primary treatment method for coarctation of the aorta?
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Blood is diverted to the __________ arteries as a compensatory mechanism in coarctation of the aorta.
Blood is diverted to the __________ arteries as a compensatory mechanism in coarctation of the aorta.
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Match the following characteristics with their respective effects related to coarctation of the aorta:
Match the following characteristics with their respective effects related to coarctation of the aorta:
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What is the primary function of the ductus arteriosus in fetal circulation?
What is the primary function of the ductus arteriosus in fetal circulation?
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The umbilical vein carries deoxygenated blood from the placenta to the fetus.
The umbilical vein carries deoxygenated blood from the placenta to the fetus.
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What is the oxygen saturation (SaO2) percentage in the umbilical vein?
What is the oxygen saturation (SaO2) percentage in the umbilical vein?
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The ___________ allows blood to flow directly from the placenta to the inferior vena cava.
The ___________ allows blood to flow directly from the placenta to the inferior vena cava.
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Match the structure with its oxygen saturation level:
Match the structure with its oxygen saturation level:
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Which of the following vessels primarily carries deoxygenated blood to the placenta?
Which of the following vessels primarily carries deoxygenated blood to the placenta?
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What is the role of the foramen ovale in fetal circulation?
What is the role of the foramen ovale in fetal circulation?
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In a fetus, the pulmonary vascular resistance is low due to functional lungs.
In a fetus, the pulmonary vascular resistance is low due to functional lungs.
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What is the most common congenital heart disease?
What is the most common congenital heart disease?
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Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease.
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease.
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What anatomical remnant remains after the closure of the ductus arteriosus?
What anatomical remnant remains after the closure of the ductus arteriosus?
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The closure of the foramen ovale occurs within __________ months after birth.
The closure of the foramen ovale occurs within __________ months after birth.
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Match the following syndromes with their associated congenital heart defects:
Match the following syndromes with their associated congenital heart defects:
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What is indicated for surgical management of atrial septal defect (ASD)?
What is indicated for surgical management of atrial septal defect (ASD)?
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The ostium secundum ASD is the most common type of atrial septal defect.
The ostium secundum ASD is the most common type of atrial septal defect.
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Name one surgical option for the closure of an atrial septal defect.
Name one surgical option for the closure of an atrial septal defect.
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The _____ is associated with valve defects such as mitral insufficiency.
The _____ is associated with valve defects such as mitral insufficiency.
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Match the following types of atrial septal defects with their characteristics:
Match the following types of atrial septal defects with their characteristics:
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Which murmurs are commonly associated with the pathophysiology of atrial septal defect?
Which murmurs are commonly associated with the pathophysiology of atrial septal defect?
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Sinus venosus ASD is a common type of atrial septal defect.
Sinus venosus ASD is a common type of atrial septal defect.
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What does a pulmonary blood flow to systemic blood flow ratio greater than 2:1 indicate?
What does a pulmonary blood flow to systemic blood flow ratio greater than 2:1 indicate?
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Which type of Ventricular Septal Defect (VSD) is most common?
Which type of Ventricular Septal Defect (VSD) is most common?
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An Inlet VSD can close spontaneously.
An Inlet VSD can close spontaneously.
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What type of murmur is associated with turbulent flow in Ventricular Septal Defects?
What type of murmur is associated with turbulent flow in Ventricular Septal Defects?
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The defect in context of an Outlet VSD can lead to an increased risk of ______.
The defect in context of an Outlet VSD can lead to an increased risk of ______.
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Match each type of VSD with its description:
Match each type of VSD with its description:
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What is the typical location for the pansystolic murmur produced by VSDs?
What is the typical location for the pansystolic murmur produced by VSDs?
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Flow murmurs are considered diagnostic.
Flow murmurs are considered diagnostic.
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Identify one key feature of muscular VSDs.
Identify one key feature of muscular VSDs.
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Study Notes
Acyanotic Congenital Heart Defects
- Acyanotic congenital heart defects (ACHDs) are characterized by a lack of cyanosis because oxygenated blood mixes with deoxygenated blood.
- Management of ACHDs is categorized into medical and surgical treatment options.
- Medical treatment can include supportive care for congestive cardiac failure (CCF), pneumonia, and prostaglandin production through the use of NSAIDs.
- Ibuprofen is favored over indomethacin due to its lower risk of nephrotoxicity and necrotizing enterocolitis (NEC).
- Surgical treatment is indicated for preterm babies and all term babies.
- Surgical techniques include ligation and percutaneous catheterization using occluders or coils.
- Indications for surgical intervention include unresponsive medical management, platelet counts of 1.8 mg/dl, or active bleeding.
- Coarctation of the aorta is a narrowing of the aorta, typically in the juxtaductal region (near ductus arteriosus).
- Severity of coarctation affects the degree of blood flow restriction, leading to complications based on the level of narrowing.
- Near complete coarctation is uncommon, resulting in severely restricted blood flow and potential shock in neonates.
- Partial narrowing is the more common form, causing reduced blood flow to the descending aorta, leading to systemic hypertension (due to increased resistance).
- Hypertenstion can lead to hypertensive encephalopathy, presenting with seizures.
- Cardiothoracic (CT) ratio is a crucial X-ray finding used to assess cardiomegaly.
- CT ratio is calculated as (a + b)/ c, where a + b is the sum of the widest lengths on each side of the heart, and c is the widest length of the thorax.
- Normal CT ratio values are less than 0.55 for children and less than 0.6 for infants.
- Elevated CT ratio indicates cardiomegaly.
- Ventricular Septal Defect (VSD) is a common ACHD that causes increased blood flow to the right ventricle (RV) due to a hole in the interventricular septum.
- VSDs can lead to early complications such as congestive cardiac failure (CCF) and late complications such as Eisenmenger's syndrome.
- CCF occurs due to increased blood flow to the left ventricle (LV), leading to LV enlargement and a more prominent cardiac outline.
- Eisenmenger's syndrome is a late complication characterized by increased right ventricular pressure, leading to RV hypertrophy and a reversal of the shunt, resulting in cyanosis.
- Treatment of VSDs includes medical management for CCF and supportive care, as well as elective or early surgical closure.
- Patent Ductus Arteriosus (PDA) is a condition where the ductus arteriosus, a blood vessel connecting the pulmonary artery and aorta, remains open after birth.
- PDA is more common in preterm babies and can be associated with congenital rubella.
- Complications of PDA include congestion in the lungs and heart, leading to recurrent pneumonia and CCF, as well as Eisenmenger's syndrome.
- Treatment for PDA includes surgical or medical management based on the severity of the condition.
- DiGeorge Syndrome (CATCH 22) is associated with various CHDs, including tetralogy of Fallot (TOF), conotruncal defects, and supravalvular aortic stenosis.
- Williams Syndrome is characterized by an elfin facies and is associated with ASD and skeletal anomalies, such as an absent radius and thumb.
- Holt-Oram Syndrome is an autosomal dominant condition associated with pulmonary stenosis (PS) and a triangular face.
- NADA's Criteria are used to diagnose Congenital Heart Disease (CHD).
- Major criteria include systolic murmurs, diastolic murmurs, central cyanosis, and features of CCF.
- Minor criteria include systolic murmurs of less than grade 3, abnormal blood pressure, abnormal ECG, abnormal X-ray, and abnormal SaO2.
- Fetal circulation differs from postnatal circulation due to the presence of fetal structures that shunt blood around the lungs.
- Fetal structures include the ductus arteriosus, ductus venosus, and foramen ovale.
- Circulation before birth involves selective shunting of deoxygenated blood to the left atrium through the foramen ovale.
- The placenta plays a vital role in providing oxygen and nutrients to the fetus.
- Oxygen saturation (SaO2) levels are different in different parts of the fetal circulatory system.
- Compensatory changes in coarctation of the aorta include collateral formation, blood diversion to intercostal arteries, and inferior rib notching due to pressure from dilated and tortuous intercostal arteries.
- Treatment for coarctation includes balloon dilatation and stenting.
- Cystic Fibrosis (CF) is a genetic disorder characterized by a deficiency in the cystic fibrosis transmembrane conductance regulator (CFTR) protein.
- Laboratory evidence confirming CF include two sweat chloride concentrations of at least 60 mEq/L on separate days, identification of two CF mutations, and abnormal trans epithelial nasal potential difference.
- Treatment for CF includes respiratory care, dietary measures, and CFTR modulators.
- Respiratory care focuses on airway clearance through physiotherapy, mucolytics, and antibiotics.
- Dietary measures include a high-fat, high-protein, high-calorie diet, salt supplementation, pancreatic enzyme supplements, and fat-soluble vitamin supplementation.
- CFTR modulators aim to enhance the activity of the CFTR channel and are used to treat specific mutations.
Atrial Septal Defect (ASD)
- ASD is a type of ACHD characterized by a hole in the atrial septum.
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Types of ASD:
- Sinus venosus ASD: Rare, located at the superior or inferior vena cava opening.
- Primum ASD: Located at the lower part of the septum, often associated with valve defects and conduction problems.
- Ostium secundum ASD: Most common type, located in the upper part of the septum.
- Coronary sinus ASD: Rare, between the coronary sinus and the right atrium.
- Pathophysiology of ASD is characterized by minimal pressure difference, smooth flow, and no shunt murmurs.
- Murmurs associated with ASD are often flow murmurs, including delayed diastolic murmurs and ejection systolic murmurs.
- Wide, fixed splitting of the second heart sound (S2) is a diagnostic feature of ASD.
- Treatment of ASD may involve elective or early surgical closure depending on the severity of the defect.
- Indications for surgical closure include CCF, Qp:Qs ratio greater than 2:1, and associated valve defects, particularly aortic regurgitation (AR).
- Surgical options include closure with a dacron patch or percutaneous device closure.
Fetal Circulation & Introduction to Congenital Heart Disease
- Circulatory changes at birth involve transitions in breathing, pulmonary vascular resistance, and pressure dynamics in the heart chambers.
- Clamping of the umbilical cord and removal of the low-resistance placental circulation lead to increased systemic vascular resistance, aortic pressure, and left ventricle pressure.
- Closure of fetal structures after birth includes ductus venosus, foramen ovale, and ductus arteriosus.
- Prevalence of CHD is 6 to 8 per 1000 live births, and inheritance is often multifactorial.
- Ventricular Septal Defect (VSD) is the most common type of CHD, while Tetralogy of Fallot (TOF) is the most common cyanotic CHD.
- Associations with syndromes can increase the risk of specific CHDs.
- Down Syndrome is associated with atrioventricular septal defects and VSDs.
- Turner Syndrome is linked to bicuspid aortic valve, coarctation of the aorta, pulmonary stenosis, and transposition of great arteries.
- Noonan Syndrome is associated with ASD, PS, and VSD, and also with maternal conditions like SLE.
- Heart block can also be a risk factor for CHD, particularly in cases of anti-Ro (SS-A) and anti-La (SS-B) antibodies.
Ventricular Septal Defect (VSD)
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Different types of VSDs:
- Outlet/Subarterial VSD: Defect near the aorta and pulmonary arteries, associated with aortic defects, particularly AR.
- Perimembranous VSD: Most common type, located in the upper membranous part of the interventricular septum (IVS).
- Inlet VSD: Located near the valves, rarely closes spontaneously, and associated with valve defects, especially mitral valve insufficiency.
- Muscular VSD: Located in the lower muscular part of the IVS, has a higher rate of spontaneous closure.
- Swiss-cheese pattern: A variant with multiple defects.
- Pathophysiology of VSD involves turbulent blood flow, resulting in a pansystolic murmur (shunt murmur) at the left parasternal area.
- Flow murmurs occur due to increased blood flow to the pulmonary arteries, creating a systolic murmur.
- Systemic circulation involves blood flow through the atrium, ventricles, and major arteries.
- Pulmonary veins carry oxygenated blood from the lungs to the left atrium.
- Mitral valve insufficiency due to increased blood flow causes a delayed diastolic murmur (flow murmur) at the mitral area.
- Shunt murmurs are diagnostic of VSD, while flow murmurs are not.
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Description
Test your knowledge on Acyanotic Congenital Heart Defects (ACHDs). This quiz covers medical and surgical management strategies, treatment options, and key conditions like coarctation of the aorta. Understand the differences in treatment approaches and their indications for intervention.