Fetal Circulation and Congenital Heart Disease
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Questions and Answers

What happens to pulmonary blood flow after the first breaths?

  • It increases sixfold. (correct)
  • It remains unchanged.
  • It decreases by half.
  • It doubles.

    • What primarily causes the closure of the foramen ovale after birth?

  • Close of the ductus arteriosus.
  • Increase in right atrial pressure.
  • Decrease in left atrial pressure.
  • Rise in left atrial pressure. (correct)

    • Which is a common method of diagnosing congenital heart disease before birth?

  • Postnatal echocardiography.
  • Physical examination.
  • Fetal anomaly scan. (correct)
  • Heart catheterization.

    • What percentage of ventricular septal defects (VSDs) are perimembranous in nature?

    <p>67-80%</p> Signup and view all the answers

    In which type of congenital heart disease is cyanosis most likely to occur?

    <p>Tetralogy of Fallot.</p> Signup and view all the answers

    Which of the following is NOT classified as a congenital heart defect?

    <p>Pulmonary embolism.</p> Signup and view all the answers

    What is a risk factor that may lead to increased prenatal monitoring for congenital heart disease?

    <p>Family history of congenital heart disease.</p> Signup and view all the answers

    What percentage of congenital heart defects do ventricular septal defects (VSDs) account for?

    <p>25-30%</p> Signup and view all the answers

    What is the typical gender distribution for ventricular septal defects (VSDs)?

    <p>More common in females</p> Signup and view all the answers

    What determines the direction of shunting in a VSD?

    <p>The ratio of pulmonary to systemic vascular resistance</p> Signup and view all the answers

    What condition can develop due to chronic left-to-right shunting in VSDs?

    <p>Eisenmenger's syndrome</p> Signup and view all the answers

    Which symptom is typically associated with small VSDs?

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

    What is a common physical finding in patients with VSD?

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

    What ECG finding is expected with a moderate VSD?

    <p>Left ventricular hypertrophy</p> Signup and view all the answers

    What clinical manifestation is associated with Eisenmenger syndrome?

    <p>Cyanosis due to right to left shunting</p> Signup and view all the answers

    What might be seen on an X-ray in a patient with large VSD?

    <p>Cardiomegaly with left atrial enlargement</p> Signup and view all the answers

    What is the most common type of atrial septal defect (ASD)?

    <p>Ostium secundum defect</p> Signup and view all the answers

    What is the typical female to male ratio for children with atrial septal defects?

    <p>2:1</p> Signup and view all the answers

    Which of the following findings can suggest an ostium primum ASD on an ECG?

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

    What is the ideal age for performing surgery on children with asymptomatic ASDs?

    <p>2-4 years</p> Signup and view all the answers

    What is a common clinical sign that may be detected during a routine examination of a child with ASD?

    <p>Systolic ejection murmur</p> Signup and view all the answers

    What is the prognosis for a child with an atrial septal defect after surgical intervention?

    <p>Good, with surgical mortality less than 1%</p> Signup and view all the answers

    What is a characteristic finding in the plain radiographic exam of a child with ASD?

    <p>Pulmonary artery dilatation</p> Signup and view all the answers

    Which type of atrial septal defect is commonly associated with a cleft in the anterior mitral valve leaflet?

    <p>Ostium primum defect</p> Signup and view all the answers

    What is a major clinical manifestation of coarctation of the aorta in infants?

    <p>Poor feeding and respiratory distress</p> Signup and view all the answers

    Which diagnostic feature helps identify coarctation of the aorta?

    <p>Pressure difference of 20 mmHg between upper and lower limbs</p> Signup and view all the answers

    What is the primary indicator of central cyanosis?

    <p>Presence of deoxygenated hemoglobin over 5 gm/100 ml</p> Signup and view all the answers

    What is the typical treatment for moderate to severe pulmonary stenosis?

    <p>Balloon valvuloplasty</p> Signup and view all the answers

    Which cyanotic congenital heart disease is noted for presenting in the neonatal period?

    <p>Tricuspid atresia</p> Signup and view all the answers

    What is the common age of presentation for older children with coarctation of the aorta?

    <p>Early school age</p> Signup and view all the answers

    What is a common pulmonary cause of respiratory issues during the neonatal period?

    <p>Congenital pneumonia</p> Signup and view all the answers

    What is a characteristic ECG finding in pulmonary stenosis?

    <p>Right axis deviation with RVH</p> Signup and view all the answers

    Which of the following best describes the disorder 'methemoglobinemia'?

    <p>Decreased oxygen saturation despite normal PaO2</p> Signup and view all the answers

    Which of the following statements regarding pulmonary stenosis is true?

    <p>Post-stenotic pulmonary dilatation leads to normal vascularity.</p> Signup and view all the answers

    What factor does NOT contribute to central nervous system depression in infants?

    <p>Increased physical activity</p> Signup and view all the answers

    What is the typical gender ratio for coarctation of the aorta?

    <p>2:1 male to female</p> Signup and view all the answers

    Which condition is described as persistent fetal circulation in the newborn?

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

    What is a common echo finding in diagnosing pulmonary stenosis?

    <p>Color Doppler display indicating PS</p> Signup and view all the answers

    What is a significant cause of intrinsic airway obstruction?

    <p>Choanal atresia</p> Signup and view all the answers

    Which heart condition typically presents after the neonatal period?

    <p>Tetralogy of Fallot</p> Signup and view all the answers

    Study Notes

    Fetal Circulation

    • Resistance to pulmonary blood flow decreases at birth
    • The volume of blood flowing through the lungs increases sixfold after first breaths
    • This increases left atrial pressure
    • The volume of blood returning to the right atrium decreases as the placenta is excluded from circulation
    • The change in pressure causes the foramen ovale to close
    • The ductus arteriosus typically closes within a few hours or days after birth.

    Congenital Heart Disease (CHD) Presentation

    • Antenatal cardiac ultrasound diagnosis
    • Detection of a heart murmur
    • Cyanosis
    • Heart failure
    • Shock
    • Antenatal diagnosis includes fetal anomaly scan performed between 18 and 20 weeks gestation
    • If an abnormality is detected, detailed fetal echocardiography is performed by a paediatric cardiologist
    • Fetal echocardiography is also performed for fetuses at increased risk, such as those with Down syndrome, previous siblings diagnosed with CHD, or mothers with CHD

    Classification of CHD

    • Non-cyanotic (Acyanotic)
      • Ventricular septal defect (VSD)
      • Atrial septal defect (ASD)
      • Patent ductus arteriosus (PDA)
      • Aortic stenosis
      • Pulmonic stenosis
      • Coarctation of the aorta
      • Atrioventricular canal (endocardial cushion defect)
    • Cyanotic
      • Tetralogy of Fallot
      • Transposition of the great vessels
      • Tricuspid atresia
      • Total anomalous pulmonary venous return
      • Truncus arteriosus
      • Hypoplastic left heart
      • Hypoplastic right heart
      • Ebstein's anomaly

    Cyanotic Heart Disease

    • Pulmonary Blood Flow Decreased
      • Tetralogy of Fallot
      • Pulmonary atresia
      • Tricuspid atresia
    • Pulmonary Blood Flow Increased
      • Transposition of great arteries
      • Truncus arteriosus
      • Single ventricle
      • Hypoplastic left heart syndrome

    Non-Cyanotic (Acyanotic) CHD

    • Ventricular Septal Defect (VSD)
      • A communication between the right and left ventricles
      • The most common type of congenital heart defect
      • Types
        • Perimembranous (infracristal) VSD: 67-80% of defects
        • Supracristal defects: 5-8% of isolated VSDs
        • Muscular VSDs (trabecular)
      • Slight female predominance (56% female, 44% male)
      • Pathophysiology
        • Amount of blood flow across the VSD depends on size of the defect and pulmonary vascular resistance
        • Large VSDs are not symptomatic at birth due to elevated pulmonary vascular resistance
        • As pulmonary vascular resistance decreases, shunt increases and symptoms develop over the first 6-8 weeks of life
        • When a small communication is present, right and left ventricular pressure is equalized
        • Direction of shunt and magnitude are determined by the ratio of pulmonary to systemic vascular resistance
      • Clinical Manifestations
        • Small VSDs are often asymptomatic, with a loud murmur
        • Moderate to large VSDs result in pulmonary overcirculation and CHF, presenting as tachypnea with increased respiratory effort, fatigue, diaphoresis with feedings, and poor growth
        • Eisenmenger syndrome (VSD with severe pulmonary vascular obstruction): Symptoms include exertional dyspnea, chest pain, syncope, and hemoptysis
        • Cyanosis may develop due to right to left shunting
      • Physical Findings
        • Pansystolic murmur at the lower left sternal border
        • A thrill may be present
      • Investigations
        • Electrocardiography (ECG): Normal with small VSD, left ventricular hypertrophy (LVH) with moderate VSD, biventricular hypertrophy (BVH) with large VSD, RVH only with pulmonary vascular obstruction
        • X-ray Studies: Cardiomegaly of varying degrees involving the LA, LV, and sometimes RV, increased pulmonary vascular markings, degree of cardiomegaly and increased markings relate to magnitude of left to right shunt
      • Closure
        • Muscular defects: Closure is earlier and more frequent (80%)
        • Perimembranous defects: Close in the first few years of life (35-40%)

    Atrial Septal Defect (ASD)

    • A communication between the right and left atria
    • Types
      • Ostium secundum defect: Most common type
      • Ostium Primum defect: Typically associated with a cleft in the anterior mitral valve leaflet
      • Sinus venosus
      • Coronary sinus
    • ASD occurs in approximately 7% of children with CHD
    • Female to male ratio is approximately 2:1
    • History
      • Infants and young children are typically asymptomatic
      • Most ASDs are diagnosed after a suspicious murmur is detected during a routine health maintenance examination
    • Physical Findings
      • Midsystolic pulmonary ejection murmur
      • Fixed splitting of S2
      • Large shunts increase flow across the tricuspid valve, resulting in a middiastolic rumble at the left sternal border
    • Diagnosis
      • X-ray: Nonspecific findings include right atrial and right ventricular dilatation, pulmonary artery dilatation, and increased pulmonary vascular markings
      • Echocardiography
      • ECG: Right axis deviation, right ventricular hypertrophy, left axis deviation suggests an ostium primum-type ASD
      • Prolonged PR intervals may occur with all types of ASDs
      • Cardiac catheterization is rarely needed preoperatively
    • Treatment
      • Medical therapy is not beneficial in asymptomatic patients
      • Surgery is ideally performed in children aged 2-4 years to prevent adult complications like atrial arrhythmias
      • Closure through cardiac catheterization
    • Prognosis
      • Good prognosis with low surgical mortality rates (less than 1%)
      • Ostium secundum defects may close spontaneously (15%)
      • Spontaneous closure does not occur in other types of ASDs
      • Patients with ostium primum ASD and valve abnormalities may require a second operation later in life for mitral valve dysfunction

    Patent Ductus Arteriosus (PDA)

    • A channel connecting the pulmonary artery to the descending aorta (isthmus part)
    • Results from the persistence of the fetal ductus arteriosus after birth
    • Persistent PDA
    • Clinical Manifestations
      • Bounding peripheral pulses
      • Hyperdynamic precordium
      • Wide pulse pressure
      • Continuous murmur over the left second intercostal space
      • Enlargement of the left ventricle
      • Systolic and diastolic murmurs
      • Bounding peripheral pulses are more characteristic of infants with PDA.

    Pulmonary Stenosis (PS)

    • CXR
      • Prominent pulmonary conus due to poststenotic pulmonary dilatation
      • Normal pulmonary vascularity
    • ECG
      • Right-axis deviation and RVH
      • Peaked P-wave
    • Echo
      • Performed for diagnosis and severity determination
      • Level of stenosis (supra, valvular, or sub)

    Coarctation of the Aorta (CoA)

    • A localized narrowing of the thoracic aorta, occurring just below the origin of the left subclavian artery at the origin of the ductus arteriosus.
    • Etiology and Epidemiology - During aortic arch development, the area near the insertion of the ductus arteriosus fails to develop correctly, resulting in narrowing of the lumen. - Constitutes 5-10% of all congenital heart defects. - Occurs more commonly in males with a male to female ratio of 2:1. - Most common cardiac lesion in Turner syndrome.
    • Clinical Manifestations
      • Infants may present with congestive heart failure (CHF) due to severe narrowing, with symptoms such as poor feeding, respiratory distress, and shock.
      • Hypoplastic aortic arch with VSD may be present in infants.
      • Older children may be asymptomatic or present with systemic hypertension symptoms like leg discomfort while exercising, headache, or epistaxis.
      • Physical Examination
        • Decreased or absent lower extremity pulses
        • Pressure difference between upper and lower limbs of 20 mmHg
        • Upper extremity hypertension
        • Peripheral vascular disease (e.g., polyarteritis nodosa)
    • Investigations - Echocardiogram: Shows the coarctation and its severity - ECG: May show signs of left ventricular hypertrophy (LVH) - Chest X-ray: May show a narrow aorta with dilatation of the aortic arch and the descending aorta

    Central Cyanosis

    • Best observed on the tongue, can be obvious at rest or after exertion like feeding or crying
    • Detectable if there is deoxygenated hemoglobin of more than 5 gm/100 ml (arterial saturation of 75%)
    • Differential Diagnosis
      • Cardiac (Many start with "T")
        • Transposition of the great arteries
        • Tricuspid atresia
        • Total anomalous pulmonary venous drainage
        • Truncus arteriosus
        • Tricuspid regurgitation with Ebstein's anomaly
        • Pulmonary atresia
        • Complex congenital heart disease (e.g., single ventricle with critical pulmonary stenosis)
      • Respiratory Disease
        • Airway
          • Choanal atresia/stenosis
          • Pierre Robin syndrome
          • Intrinsic airway obstruction (laryngeal/bronchial/tracheal stenosis)
          • Extrinsic airway obstruction (bronchogenic cyst, duplication cyst, vascular compression)
        • Pulmonary
          • Neonatal period
            • Respiratory distress syndrome
            • Aspiration pneumonia (meconium, milk)
            • Congenital pneumonia
            • Transient tachypnea
            • Diaphragmatic hernia
            • Lung hypoplasia
          • After neonatal period
            • Pneumonia
            • Bronchiolitis
            • Bronchial asthma
            • Cystic fibrosis
            • Bronchiectasis
      • Nervous System and Neuromuscular Causes
        • Central nervous system lesions (e.g., birth asphyxia, head trauma, intracranial hypertension, hemorrhage)
        • Central nervous system depression by drugs (direct or through maternal route) (e.g., phenobarbitone)
        • Intercostal muscle weakness (e.g., spinal muscular atrophy)
        • Diaphragmatic weakness (e.g., phrenic nerve palsy)
      • Other Causes
        • Persistent pulmonary hypertension of the newborn (persistent fetal circulation)
        • Methemoglobinemia (very rare)
        • Polycythemia.

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    Description

    This quiz explores the critical changes in fetal circulation at birth, including the effects of decreased pulmonary resistance and the closure of the foramen ovale and ductus arteriosus. It also covers the presentation and diagnosis of congenital heart disease, including antenatal ultrasound and fetal echocardiography. Test your knowledge on these vital topics related to fetal and neonatal cardiology.

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