Paediatrics Marrow Pg 141-150 (CVS)
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What is the most common type of Total Anomalous Pulmonary Venous Connection (TAPVC)?

  • Supracardiac (correct)
  • Non-obstructive
  • Infracardiac
  • Cardiac

    • In the obstructive type of TAPVC, there is pulmonary venous obstruction leading to severe cyanosis.

    True

    What appearance is associated with the X-ray findings in Total Anomalous Pulmonary Venous Connections?

    Snowman appearance

    The primary connection between the pulmonary veins and the right atrium in TAPVC is termed the ______.

    <p>abnormal connection</p> Signup and view all the answers

    Match the following types of Total Anomalous Pulmonary Venous Connections with their characteristics:

    <p>Supracardiac = Most common type Cardiac = Connections at the heart level Infracardiac = Connections below the heart Non-obstructive = Equal O2 saturation in all heart chambers</p> Signup and view all the answers

    What is a characteristic ECG finding in Ebstein's Anomaly?

    <p>Left-axis deviation</p> Signup and view all the answers

    All congenital heart defects (CHD) can be diagnosed solely through physical examination.

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

    What is the sequence of shunt surgeries performed in Ebstein's Anomaly treatment?

    <p>Modified B-T shunt, Glenn shunt, Fontan shunt</p> Signup and view all the answers

    In Ebstein's Anomaly, there is an __________ of the tricuspid valve.

    <p>inferior/apical displacement</p> Signup and view all the answers

    Match the following types of shunt surgeries with their descriptions:

    <p>Modified B-T shunt = Performed immediately after diagnosis Glenn shunt = Connects superior vena cava to pulmonary artery Fontan shunt = Connects inferior vena cava to pulmonary artery All shunt surgeries = Performed in stages</p> Signup and view all the answers

    What is the preferred investigation for diagnosing acute rheumatic fever?

    <p>Elevated ASO titer</p> Signup and view all the answers

    Arthritis in acute rheumatic fever typically results in permanent joint damage.

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

    List one common clinical finding in carditis associated with acute rheumatic fever.

    <p>Chest pain or frictional rub</p> Signup and view all the answers

    The _____ murmur is characterized as a delayed diastolic murmur associated with valvular involvement in acute rheumatic fever.

    <p>Carey coombs</p> Signup and view all the answers

    Match the following features of acute rheumatic fever with their descriptions:

    <p>Carditis = Permanent damage of heart valves can occur Arthritis = Migratory arthritis affecting large joints Mitral regurgitation = Soft S and pan systolic murmur Carey coombs murmur = Delayed diastolic murmur</p> Signup and view all the answers

    What is a key management step during a cyanotic spell in cases of Ventricular Septal Defect?

    <p>Oxygen inhalation</p> Signup and view all the answers

    Increased cyanosis is a sign of improving oxygen levels in the bloodstream.

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

    What is the most notable change observed in the X-ray findings of a patient with Tetralogy of Fallot?

    <p>Boot-shaped heart with right ventricular hypertrophy</p> Signup and view all the answers

    A non-restrictive Ventricular Septal Defect is characterized by ______ flow and the absence of a pansystolic murmur.

    <p>no turbulent</p> Signup and view all the answers

    Match the following management strategies with their purposes:

    <p>Oxygen inhalation = Improve oxygen levels Sodium bicarbonate = Counteract acidosis Morphine = Depress respiratory center Beta-blockers = Reduce heart workload</p> Signup and view all the answers

    What is the primary goal of palliative shunt surgery in cyanotic congenital heart defects?

    <p>To improve pulmonary blood flow</p> Signup and view all the answers

    Tricuspid atresia is characterized by an increase in blood flow to the right ventricle.

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

    What is the most common type of palliative shunt surgery?

    <p>Modified Blalock-Taussing shunt</p> Signup and view all the answers

    Tricuspid atresia leads to decreased blood flow to the right ventricle and subsequently __________ flow to the pulmonary artery.

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

    What is one of the four components of Tetralogy of Fallot (TOF)?

    <p>Ventricular Septal Defect (VSD)</p> Signup and view all the answers

    Match the following types of shunts with their corresponding major arterial connections:

    <p>Classic Blalock-Taussing = Subclavian artery Waterston shunt = Ascending aorta Potts shunt = Descending aorta Modified Blalock-Taussing = Subclavian artery or other</p> Signup and view all the answers

    Cyanotic CHD are more commonly seen than acyanotic CHD.

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

    Name one clinical feature of Tetralogy of Fallot (TOF).

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

    The aorta is shifted towards the __________ in Tetralogy of Fallot.

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

    Match the following cyanotic congenital heart defects with their classifications of pulmonary blood flow:

    <p>Tetralogy of Fallot = Low Hypoplastic Left Heart Syndrome = High Truncus Arteriosus = High Tricuspid Atresia = Low</p> Signup and view all the answers

    What is the characteristic early symptom of Transposition of Great Arteries (TGA) with intact ventricular septum?

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

    In Transposition of Great Arteries with VSD, the blood primarily flows in a single circuit.

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

    What is the definitive treatment for Transposition of Great Arteries?

    <p>Jatene's arterial switch repair</p> Signup and view all the answers

    The X-ray appearance of TGA is described as an _______ on a string.

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

    Match the following characteristics with the types of Transposition of Great Arteries:

    <p>TGA with intact ventricular septum = Early cyanosis with parallel circulation TGA with VSD = Less intense cyanosis and pansystolic murmur X-ray findings = Egg-on-string appearance Initial treatment = PGE-1 infusion to keep ductus arteriosus open</p> Signup and view all the answers

    Which of the following is a characteristic of Erythema Marginatum?

    <p>Erythematous with a pale center</p> Signup and view all the answers

    What is a characteristic treatment method for Lithium exposure causing accessory pathways on the right side of the heart?

    <p>Cone repair of tricuspid valve</p> Signup and view all the answers

    Subcutaneous nodules are typically found early in the presentation of the disease.

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

    What is the treatment provided if Sydenham's Chorea is not resolving?

    <p>Diazepam or Haloperidol</p> Signup and view all the answers

    Hypoplastic left heart syndrome is characterized by a normal-sized aorta.

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

    What cardiac anomaly is commonly associated with increased blood flow to the right atrium and right ventricle in hypoplastic left heart syndrome?

    <p>Atrial Septal Defect (ASD)</p> Signup and view all the answers

    The milkmaid grip sign is characterized by alternating __________ and __________ of grip.

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

    In hypoplastic left heart syndrome, severe reduction of blood flow to systemic circulation can lead to ______ in neonates.

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

    Match the following types of treatment with their descriptions:

    <p>Penicillin G = Single dose for treatment Oral Penicillin V = 250 mg QID for 10 days Erythromycin = Alternative for patients allergic to Penicillin Anti-inflammatory drugs = Includes steroids or aspirin</p> Signup and view all the answers

    Match the following conditions with their associated features:

    <p>Wolff-Parkinson-White syndrome = Abnormal electrical communication between RA and RV Tricuspid valve repair = Treatment for Lithium exposure related defects Hypoplastic left heart syndrome = Severely reduced blood flow to systemic circulation ASD = Common in hypoplastic left heart syndrome</p> Signup and view all the answers

    Which age group is primarily affected by acute rheumatic fever?

    <p>School-going children (5-15 years)</p> Signup and view all the answers

    Erythema marginatum is considered a minor criterion for the diagnosis of acute rheumatic fever.

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

    What is the probability of acute rheumatic fever occurring in a low-risk population?

    <p>0.3%</p> Signup and view all the answers

    Anti-streptococcal antibodies form as a result of the body reacting to ______ streptococci.

    <p>Group A β hemolytic</p> Signup and view all the answers

    Match the following major criteria for acute rheumatic fever with their descriptions:

    <p>Pancarditis = Inflammation of the heart layers Arthritis = Joint pain or inflammation Subcutaneous nodules = Painless lumps under the skin Sydenham's chorea = Involuntary movements and muscle contractions</p> Signup and view all the answers

    Study Notes

    Total Anomalous Pulmonary Venous Connections (TAPVC)

    • Abnormal connections between pulmonary veins (PV) and right atrium (RA)
    • Classified based on blood flow route: supracardiac (most common), cardiac, infracardiac
    • Obstructive type: pulmonary venous obstruction, lung congestion, severe cyanosis
    • Non-obstructive type (most common): equal oxygen saturation in all heart chambers
    • X-ray findings: snowman appearance/figure of 8 appearance (due to enlarged superior vena cava)

    Acute Rheumatic Fever (ARF)

    • Essential criteria (evidence of preceding streptococcal infection): elevated ASO titer (preferred), throat swab culture (not preferred), history of preceding sore throat
    • Diagnosis: first episode - 2 major criteria OR 1 major + 2 minor criteria
    • Recurrence: 2 major criteria OR 1 major + 2 minor criteria OR 3 minor criteria + essential criteria

    Major Jones Criteria

    • Carditis: most common feature of ARF (90%), early finding (within 2 weeks), all 3 layers affected (pancarditis)
      • Clinical diagnosis: pericarditis (chest pain + friction rub), valvular involvement (mitral valve > aortic valve)
      • Mitral valve abnormalities: valvulitis, regurgitation (soft S1, pan-systolic murmur, hemodynamic overload leading to left ventricular failure)
      • Carey Coombs murmur: delayed diastolic murmur
    • Arthritis: large joints (knee, ankle, elbow), migratory, early finding (30-50%), excellent response to aspirin, complete recovery without residual damage

    Ebstein's Anomaly

    • Increased right atrial size
    • Inferior/apical displacement of septal leaflet of tricuspid valve
    • Atrialization of right ventricle
    • Diagram 1: shows various types of shunts, blood flow directions, and left heart hypertrophy
    • Diagram 2: shows normal and abnormal positions of tricuspid valve, blood flow to pulmonary artery, right ventricle size and volume
    • Diagram 3: X-ray image of chest with enlarged right atrium
    • X-ray findings: box-shaped heart (enlarged right atrium)
    • ECG findings: left-axis deviation
    • Treatment: shunt surgery (modified B-T shunt, Glenn shunt, Fontan shunt)

    Cyanotic Congenital Heart Defects (CHD)

    • Rarely seen compared to acyanatic CHD
    • Classified by pulmonary blood flow: low (TOF, tricuspid atresia, Ebstein's anomaly) and high (HLHS, truncus arteriosus, TGA, TAPVC)

    Tetralogy of Fallot (TOF)

    • 4 components: overriding aorta, right ventricular hypertrophy, ventricular septal defect, sub-pulmonary stenosis
    • Clinical features: cyanosis (onset within 4 months), single S1 heart sound, murmurs (ejection systolic murmur, disappears during cyanotic spell)
    • Physiological changes: infundibular spasm (narrowing) leading to increased venous return, systemic vascular resistance, and heart overload, deoxygenated blood flow from RV to LV, cyanosis
    • Hyperventilation: stimulates respiratory center in the brain
    • X-ray findings: boot-shaped heart, right ventricular hypertrophy (RVH), uplifting of apex

    Transposition of Great Arteries (TGA)

    • Pathophysiology:
      • TGA with intact ventricular septum: parallel circulation, early onset cyanosis, fatal
      • TGA with VSD: blood flow from LV to RV, mixing of oxygenated and deoxygenated blood, less intense cyanosis, pansystolic murmur, majority of blood flow through parallel pathways
    • X-ray findings: string appearance (narrow positioning of great arteries), egg-on-string appearance
    • Treatment: emergency (PGE-1 infusion at birth), atrial septotomy, TGA with VSD (elective)
    • Definitive treatment: Jatene's arterial switch repair
    • Diagram: shows heart chambers, vessels, blood flow, and X-ray findings

    Hypoplastic Left Heart Syndrome (HLHS)

    • Pathophysiology: hypoplastic left ventricle, mitral valve atresia, hypoplastic aorta, ASD (in majority)
    • Early onset congestive cardiac failure
    • Treatment: multiple shunt surgeries (modified B-T shunt, Glenn shunt, Fontan shunt)

    Erythema Marginatum

    • Rare, early stage finding
    • Erythematous lesions with pale center, serpiginous rash, non-pruritic
    • Starts in trunk and then involves limbs, face not involved
    • Induced by warmth/heat

    Subcutaneous Nodules

    • Late finding (6 weeks after onset)
    • Painless nodules
    • Site: around bony prominences
    • Associated with high risk of carditis

    Sydenham's Chorea

    • Last manifestation (3 months after onset)
    • Semi-purposeful, jerky movements of limbs, abnormal coordination and posturing
    • Associated manifestations: hypotonia, emotional lability, jerky speech
    • Signs: milkmaid grip sign, darting tongue sign, protrusion
    • Treatment (if not resolving): diazepam, haloperidol

    Management of ARF

    • Bed rest for 2 weeks
    • Penicillin: injectable benzathine, oral penicillin V, erythromycin (if penicillin allergic)
    • Anti-inflammatory drugs: steroids, aspirin

    Secondary Prophylaxis of ARF

    • Penicillin: >30 kg - 12 lakh IU every 3 weeks
    • Other options: sulfadiazine, monthly intramuscular benzathine penicillin

    Etiology of ARF

    • Post-streptococcal disorder (Group A β hemolytic streptococci)
    • Age: school-going children (5-15 years)
    • Incidence: equal in males and females
    • Pathogenesis: pharyngitis → molecular mimicry → formation of anti-streptococcal antibodies → cross-reaction with body tissues

    Risk Stratification of ARF

    • Low-risk population:
      • Acute rheumatic fever: lower rates per lakh of school-going children
      • Rheumatic heart disease: lower rates per 1000 population
    • Moderate-high risk population (like in India):
      • Acute rheumatic fever: higher rates per lakh of school-going children
      • Rheumatic heart disease: higher rates per 1000 population

    Modified Jones Criteria (2015) for ARF

    • Major criteria (moderate-high risk population): pancarditis, arthritis, subcutaneous nodules, erythema marginatum, Sydenham's chorea
    • Minor criteria: arthralgia, fever, elevated ESR and CRP, prolonged PR interval
    • Note: in low-risk population only polyarthritis is considered major criteria

    Lithium Exposure and Heart Defects

    • Lithium exposure associated with accessory pathways on the right side of the heart
    • Wolff-Parkinson-White syndrome: abnormal electrical communication between RA and RV, bundle of Kent, early/pre-excitation of RV
    • Treatment: slurring of QRS complex, cone repair of tricuspid valve, return valve to original position

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    Description

    Test your knowledge on Total Anomalous Pulmonary Venous Connections (TAPVC) and Acute Rheumatic Fever (ARF). This quiz covers the classifications, criteria, and diagnostic features of these conditions. Perfect for students in cardiology or medicine.

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