Paediatrics Marrow Pg 131-140 (CVS)
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Questions and Answers

What does a higher cardio-thoracic (CT) ratio indicate?

  • Pulmonary embolism
  • Normal heart size
  • Cardiomegaly (correct)
  • Decreased lung capacity
  • Children should have a CT ratio of less than 0.55.

    True

    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.

    <p>Right Ventricle</p> Signup and view all the answers

    Match the following complications with their descriptions:

    <p>Congestive cardiac failure = Increased blood flow leads to left ventricle enlargement Eisenmenger's Syndrome = Reversal of right to left shunt Infective endocarditis = Increased risk due to forceful blood flow Pulmonary congestion = Result of increased right ventricle pressure</p> Signup and view all the answers

    What is an indication for elective surgical closure of an ASD?

    <p>ASD size &gt; 8mm</p> Signup and view all the answers

    Patent Ductus Arteriosus is more common in term babies than preterm babies.

    <p>False</p> Signup and view all the answers

    What can cause a left fascicular block in relation to ASD?

    <p>Left chamber overload from mitral valve insufficiency</p> Signup and view all the answers

    Eisenmenger syndrome is characterized by the reversal of the ______.

    <p>shunt</p> Signup and view all the answers

    Match the following ASD types with their descriptions:

    <p>Ostium secundum ASD = Most common type of ASD Ostium primum ASD = Often associated with congenital defects Right-axis deviation = ECG finding in ASD Left-axis deviation = Due to left fascicular block</p> Signup and view all the answers

    What is the most common location for coarctation of the aorta?

    <p>Juxtaductal near the ductus arteriosus</p> Signup and view all the answers

    Prostaglandin production is curative in term babies with cyanotic congenital heart defects.

    <p>False</p> Signup and view all the answers

    Name two medications used in the medical management of congestive heart failure in neonates.

    <p>Digoxin and furosemide</p> Signup and view all the answers

    A significant feature of partial narrowing in coarctation of the aorta is systemic hypertension due to increased ______.

    <p>resistance</p> Signup and view all the answers

    Match the following features of coarctation of the aorta with their descriptions:

    <p>Near complete = Severely restricted blood flow leading to shock in neonates Partial narrowing = Decreased blood flow to descending aorta leading to systemic hypertension Physical signs = Claudication pain and feeble femoral pulses Complications = Hypertensive encephalopathy presenting with seizures</p> Signup and view all the answers

    Which syndrome is associated with TOF and conotruncal defects?

    <p>DiGeorge Syndrome</p> Signup and view all the answers

    Central cyanosis with improvement upon 02 inhalation is a major criterion for diagnosing Congenital Heart Disease.

    <p>False</p> Signup and view all the answers

    What type of genetic defect is associated with Williams Syndrome?

    <p>microdeletion</p> Signup and view all the answers

    The major criteria for diagnosing Congenital Heart Disease include a systolic murmur graded at or above ______.

    <p>3</p> Signup and view all the answers

    Match the following syndromes with their associated conditions:

    <p>DiGeorge Syndrome = TOF, Conotruncal defects Williams Syndrome = ASD Holt-Oram Syndrome = Pulmonary stenosis Alagille Syndrome = Not specified</p> Signup and view all the answers

    What is a confirmed laboratory evidence for Cystic Fibrosis?

    <p>Two sweat chloride concentrations of ≥60 mEq/L on separate days</p> Signup and view all the answers

    Antibiotics are not part of the treatment plan for Cystic Fibrosis.

    <p>False</p> Signup and view all the answers

    What type of diet is recommended for patients with Cystic Fibrosis?

    <p>High-fat, high-protein, and high-calorie diet</p> Signup and view all the answers

    The sweat chloride test uses __________ to measure chloride levels in sweat.

    <p>Pilocarpine</p> Signup and view all the answers

    Match the following treatments with their respective descriptions:

    <p>Mucolytics = Helps to thin mucus in the airways Salt supplementation = Increases intake during dehydration CFTR Modulators = Enhances CFTR channel activity Antibiotics = Fights bacterial infections in lungs</p> Signup and view all the answers

    What is a common compensatory change noted in older children with coarctation of the aorta?

    <p>Formation of collaterals</p> Signup and view all the answers

    Inferior rib notching is commonly associated with coarctation of the aorta.

    <p>True</p> Signup and view all the answers

    What is the primary treatment method for coarctation of the aorta?

    <p>Balloon dilatation of coarctation ± Stenting</p> Signup and view all the answers

    Blood is diverted to the __________ arteries as a compensatory mechanism in coarctation of the aorta.

    <p>intercostal</p> Signup and view all the answers

    Match the following characteristics with their respective effects related to coarctation of the aorta:

    <p>Pre-stenotic dilatation = Widening of the aorta before the narrowed segment Post-stenotic dilatation = Widening of the aorta after the narrowed segment Inferior rib notching = Bony changes in ribs due to altered blood flow Dilated intercostal arteries = Compensatory enlargement of arteries supplying the rib area</p> Signup and view all the answers

    What is the primary function of the ductus arteriosus in fetal circulation?

    <p>To shunt blood from the pulmonary artery to the aorta</p> Signup and view all the answers

    The umbilical vein carries deoxygenated blood from the placenta to the fetus.

    <p>False</p> Signup and view all the answers

    What is the oxygen saturation (SaO2) percentage in the umbilical vein?

    <p>80%</p> Signup and view all the answers

    The ___________ allows blood to flow directly from the placenta to the inferior vena cava.

    <p>ductus venosus</p> Signup and view all the answers

    Match the structure with its oxygen saturation level:

    <p>Superior vena cava = 70% Left ventricle = 65% Umbilical artery = 50-60% Right ventricle = 55%</p> Signup and view all the answers

    Which of the following vessels primarily carries deoxygenated blood to the placenta?

    <p>Umbilical artery</p> Signup and view all the answers

    What is the role of the foramen ovale in fetal circulation?

    <p>Selectively shunts deoxygenated blood to the left atrium</p> Signup and view all the answers

    In a fetus, the pulmonary vascular resistance is low due to functional lungs.

    <p>False</p> Signup and view all the answers

    What is the most common congenital heart disease?

    <p>Ventricular septal defect (VSD)</p> Signup and view all the answers

    Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease.

    <p>True</p> Signup and view all the answers

    What anatomical remnant remains after the closure of the ductus arteriosus?

    <p>Ligamentum arteriosum</p> Signup and view all the answers

    The closure of the foramen ovale occurs within __________ months after birth.

    <p>3</p> Signup and view all the answers

    Match the following syndromes with their associated congenital heart defects:

    <p>Down Syndrome = Ventricular septal defect (VSD) Turner Syndrome = Transposition of great arteries Noonan Syndrome = Atrial septal defects (ASD)</p> Signup and view all the answers

    What is indicated for surgical management of atrial septal defect (ASD)?

    <p>Cardiac output failure after supportive treatment</p> Signup and view all the answers

    The ostium secundum ASD is the most common type of atrial septal defect.

    <p>True</p> Signup and view all the answers

    Name one surgical option for the closure of an atrial septal defect.

    <p>Dacron patch closure</p> Signup and view all the answers

    The _____ is associated with valve defects such as mitral insufficiency.

    <p>Primum ASD</p> Signup and view all the answers

    Match the following types of atrial septal defects with their characteristics:

    <p>Sinus venosus ASD (SVC-type) = At the superior vena cava opening Primum ASD = Associated with valve defects Ostium secundum ASD = Most common type Coronary sinus ASD = Between coronary sinus and atrium</p> Signup and view all the answers

    Which murmurs are commonly associated with the pathophysiology of atrial septal defect?

    <p>Delayed diastolic and ejection systolic murmurs</p> Signup and view all the answers

    Sinus venosus ASD is a common type of atrial septal defect.

    <p>False</p> Signup and view all the answers

    What does a pulmonary blood flow to systemic blood flow ratio greater than 2:1 indicate?

    <p>Atrial septal defect (ASD)</p> Signup and view all the answers

    Which type of Ventricular Septal Defect (VSD) is most common?

    <p>Perimembranous VSD</p> Signup and view all the answers

    An Inlet VSD can close spontaneously.

    <p>False</p> Signup and view all the answers

    What type of murmur is associated with turbulent flow in Ventricular Septal Defects?

    <p>Pansystolic murmur</p> Signup and view all the answers

    The defect in context of an Outlet VSD can lead to an increased risk of ______.

    <p>Aortic Regurgitation</p> Signup and view all the answers

    Match each type of VSD with its description:

    <p>Outlet VSD = Defect in the area leading to aortic risk Perimembranous VSD = Defect in the upper membranous part of IVS Inlet VSD = Never closes spontaneously Muscular VSD = Defect in the lower muscular part of IVS</p> Signup and view all the answers

    What is the typical location for the pansystolic murmur produced by VSDs?

    <p>Left parasternal area of 4th intercostal space</p> Signup and view all the answers

    Flow murmurs are considered diagnostic.

    <p>False</p> Signup and view all the answers

    Identify one key feature of muscular VSDs.

    <p>Higher rate of spontaneous closure</p> Signup and view all the answers

    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.
    • 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)

    • 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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    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.

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