Ductus-Dependent Circulation

Ductus-Dependent Circulation

Q1. Which of the following is an example of ductus-dependent systemic circulation?

Hypoplastic Left Heart Syndrome
Pulmonary Atresia
Tricuspid Atresia
Tetralogy of Fallot
In HLHS, systemic perfusion depends on the ductus arteriosus supplying the aorta.

Q2. Closure of ductus arteriosus in a pulmonary atresia baby leads to:

Systemic hypertension
Severe cyanosis
Bradyarrhythmia
Polycythemia
Pulmonary atresia is ductus-dependent for pulmonary blood flow; closure causes cyanosis.

Q3. Which drug is lifesaving in ductus-dependent CHD?

Prostaglandin E1 (Alprostadil)
Indomethacin
Furosemide
Dobutamine
PGE1 keeps the ductus open; indomethacin closes it.

Q4. Ductus-dependent systemic circulation lesions typically present with:

Shock and weak pulses
Isolated cyanosis
Hypertension
Polyarthritis
Systemic ductus-dependent lesions cause systemic hypoperfusion → shock.

Q5. Transposition of great arteries with intact septum is ductus-dependent for:

Systemic circulation
Pulmonary circulation
Mixing circulation
Coronary perfusion
TGA with intact septum needs ductus for mixing of oxygenated and deoxygenated blood.

Q6. Which of the following is NOT ductus-dependent?

Hypoplastic Left Heart Syndrome
Atrial Septal Defect
Critical Pulmonary Stenosis
Interrupted Aortic Arch
ASD is not ductus-dependent; others need ductal patency.

Q7. Clinical deterioration in ductus-dependent lesions usually occurs:

Immediately after birth
Within 24–48 hours
After 2 weeks
Only during adolescence
Symptoms appear after ductal closure, usually 1–2 days after birth.

Q8. Which prostaglandin side effect is most important in neonates?

Bradycardia
Apnea
Renal failure
Hypoglycemia
Apnea is a common complication; neonates may require intubation.

Q9. Which lesion is ductus-dependent for pulmonary circulation?

Tricuspid Atresia
Coarctation of Aorta
Hypoplastic Left Heart
Interrupted Aortic Arch
Tricuspid atresia requires ductus for pulmonary blood flow.

Q10. In HLHS, ductal closure causes:

Systemic circulatory collapse
Pulmonary embolism
Arrhythmia
Pulmonary hypertension
HLHS needs ductus for systemic output; closure causes collapse.

Q11. First-line stabilization in suspected ductus-dependent CHD is:

Start PGE1 infusion
Start IV fluids only
Immediate surgery
High-flow oxygen alone
PGE1 is started while definitive surgery is planned.

Q12. Which condition is ductus-dependent for systemic flow?

Pulmonary Atresia
Critical Coarctation
Tricuspid Atresia
TOF
Critical coarctation relies on ductus to perfuse descending aorta.

Q13. Which finding suggests ductus-dependent systemic lesion?

Differential cyanosis & weak femoral pulses
Loud P2
Polycythemia
Fixed splitting of S2
Weak femoral pulses & systemic hypoperfusion suggest systemic ductus dependence.

Q14. Which CHD may present with severe cyanosis soon after ductal closure?

Pulmonary Atresia
ASD
VSD
PDA
Pulmonary atresia requires ductus to perfuse lungs.

Q15. Which drug closes ductus arteriosus and must be avoided here?

Indomethacin
Prostaglandin E1
Dopamine
Milrinone
Indomethacin closes ductus; contraindicated in ductus-dependent lesions.

Q16. Which of the following is a ductus-dependent mixing lesion?

HLHS
Pulmonary Atresia
TGA with intact septum
Critical Coarctation
TGA intact septum requires ductus for mixing of blood.

Q17. Prostaglandin infusion is started at:

0.05–0.1 µg/kg/min
0.5–1 mg/kg
5–10 mg/kg
1 U/kg/min
The typical starting dose of PGE1 is 0.05–0.1 µg/kg/min.

Q18. Ductus-dependent pulmonary lesions present mainly with:

Shock
Cyanosis
Bradycardia
Polyuria
Pulmonary ductus-dependent lesions cause cyanosis when ductus closes.

Q19. Which of the following is TRUE?

Indomethacin closes the ductus
Prostaglandin closes the ductus
Oxygen keeps ductus open
All cyanotic CHDs are ductus-dependent
Indomethacin (NSAID) closes ductus; prostaglandin keeps it open.

Q20. A neonate collapses 2 days after birth with severe shock, mottling, and absent femoral pulses. Most likely diagnosis?

Critical coarctation of aorta
Tricuspid Atresia
TGA with intact septum
Pulmonary Atresia
Critical coarctation depends on ductus for systemic flow; closure causes collapse.

The ductus arteriosus is a vital fetal vascular connection between the pulmonary artery and descending aorta.
In utero, it shunts blood away from the lungs (high resistance) toward systemic circulation.

After birth, as pulmonary vascular resistance falls and oxygen rises, the ductus normally constricts within hours to days.
However, in certain congenital heart diseases (CHDs), survival depends on ductal patency.


🔹 Types of Ductus-Dependent Circulations

1. Ductus-Dependent Systemic Circulation

  • The ductus provides systemic blood flow (aorta supplied from pulmonary artery).
  • Closure leads to shock, acidosis, weak pulses, collapse.
  • Examples:
    • Hypoplastic Left Heart Syndrome (HLHS)
    • Critical Aortic Stenosis
    • Interrupted Aortic Arch
    • Coarctation of Aorta (critical)

2. Ductus-Dependent Pulmonary Circulation

  • The ductus provides pulmonary blood flow (pulmonary artery supplied from aorta).
  • Closure leads to severe cyanosis, hypoxemia, acidosis.
  • Examples:
    • Pulmonary Atresia
    • Critical Pulmonary Stenosis
    • Tricuspid Atresia
    • Tetralogy of Fallot with severe RVOTO

3. Ductus-Dependent Mixing Circulation

  • Ductus is required for adequate mixing of systemic & pulmonary blood.
  • Examples:
    • Transposition of Great Arteries (TGA) with intact septum

⚠️ Clinical Presentation

  • Severe cyanosis (if pulmonary flow dependent)
  • Cardiogenic shock, poor perfusion (if systemic flow dependent)
  • Collapsed infant after ductal closure (24–48 hrs after birth)
  • Differential diagnosis: cyanotic vs shock presentations

💊 Management

  • Prostaglandin E1 (PGE1, alprostadil) infusion → keeps ductus open
    • Dose: 0.05–0.1 µg/kg/min IV (then titrate to lowest effective dose)
    • Side effects: apnea, hypotension, fever, flushing, seizures
  • Supportive care: ventilation, fluids, correction of acidosis
  • Definitive therapy: surgery or catheter-based palliation

📊 Quick Comparison Table

Type of dependenceExamplesEffect of ductal closureClinical clue
SystemicHLHS, critical AS, IAA, CoAShock, poor pulsesGray, mottled baby
PulmonaryPA, critical PS, TOF, TASevere cyanosis“Blue” baby
MixingTGA with intact septumSevere cyanosisCyanosis refractory to O₂

✅ In summary: Ductus-dependent circulation = CHDs where neonatal survival depends on keeping the ductus arteriosus patent until definitive repair or palliation.

#Key PointExplanation
1DefinitionCHDs where neonatal survival depends on keeping the ductus arteriosus open.
2Ductus roleConnects pulmonary artery to descending aorta in fetal life.
3Closure timingUsually begins within 24–48 hrs after birth → critical for presentation.
4Systemic dependent lesionsHLHS, critical AS, interrupted aortic arch, critical coarctation.
5Pulmonary dependent lesionsPulmonary atresia, critical PS, tricuspid atresia, severe TOF.
6Mixing dependent lesionsTGA with intact septum.
7Systemic closure effectShock, weak/absent pulses, metabolic acidosis, collapse.
8Pulmonary closure effectSevere cyanosis, hypoxemia refractory to O₂.
9Mixing closure effectInadequate mixing → profound cyanosis.
10Clinical onsetTypically 1–2 days after birth (as ductus closes).
11Presentation clueCyanosis → pulmonary/mixing; shock → systemic lesions.
12Physical exam systemicMottled skin, poor pulses, weak femorals, acidosis.
13Physical exam pulmonary“Blue baby,” severe hypoxemia, relatively preserved pulses.
14Diagnostic toolEchocardiography confirms ductus dependency and anatomy.
15Lifesaving therapyProstaglandin E1 infusion to reopen/maintain ductus.
16PGE1 doseStart at 0.05–0.1 µg/kg/min IV infusion.
17PGE1 side effectsApnea, hypotension, fever, flushing, seizures.
18Contraindicated drugIndomethacin → promotes ductal closure.
19Definitive therapySurgical or catheter-based palliation/repair once stabilized.
20Mnemonic“S.P.M.” → Systemic, Pulmonary, Mixing → 3 types of ductus dependency.

Q1. Ductus-dependent systemic circulation is seen in:

Hypoplastic Left Heart Syndrome
Pulmonary Atresia
Tricuspid Atresia
TOF
HLHS requires ductus for systemic blood supply.

Q2. A neonate presents with severe cyanosis 36 hours after birth. Which lesion is most likely?

Pulmonary Atresia
Atrial Septal Defect
VSD
PDA
Pulmonary atresia is ductus-dependent for pulmonary flow; closure → cyanosis.

Q3. Which drug keeps ductus arteriosus open?

Prostaglandin E1
Indomethacin
Ibuprofen
Aspirin
PGE1 maintains ductal patency; indomethacin/ibuprofen close it.

Q4. Critical coarctation of aorta is ductus-dependent for:

Systemic circulation
Pulmonary circulation
Mixing circulation
Coronary circulation
Systemic perfusion is dependent on PDA in critical coarctation.

Q5. TGA with intact septum is dependent on ductus for:

Systemic flow
Pulmonary flow
Mixing of blood
Coronary flow
In TGA with intact septum, ductus allows oxygenated and deoxygenated blood to mix.

Q6. Typical time of clinical deterioration in ductus-dependent CHD is:

24–48 hours after birth
Immediately at birth
After 1 week
At 6 months
Symptoms appear once ductus begins to close, usually 1–2 days after birth.

Q7. A ductus-dependent pulmonary lesion causes:

Cyanosis
Shock
Polycythemia
Hypertension
Pulmonary ductus-dependent CHD causes cyanosis when ductus closes.

Q8. Which lesion is NOT ductus-dependent?

HLHS
Critical PS
ASD
Interrupted Aortic Arch
ASD is not ductus-dependent, while others are.

Q9. The first-line emergency management in suspected ductus-dependent lesion is:

Start IV PGE1 infusion
High-dose oxygen only
Immediate surgery
IV fluids only
PGE1 infusion is the first step while preparing for surgery.

Q10. Which is a common complication of prostaglandin E1 infusion?

Apnea
Polyuria
Thrombosis
Renal failure
Apnea is a frequent complication; infants may require intubation.

Q11. Which lesion causes systemic collapse if ductus closes?

HLHS
TOF
Tricuspid Atresia
Pulmonary Atresia
HLHS is ductus-dependent for systemic circulation.

Q12. Which drug must be avoided in ductus-dependent CHD?

Indomethacin
Prostaglandin
Dopamine
Milrinone
Indomethacin closes ductus; contraindicated here.

Q13. Critical pulmonary stenosis is ductus-dependent for:

Pulmonary circulation
Systemic circulation
Mixing circulation
Coronary circulation
Pulmonary stenosis relies on ductus to provide pulmonary flow.

Q14. Which lesion is ductus-dependent mixing lesion?

TGA with intact septum
HLHS
Critical PS
Interrupted Aortic Arch
TGA intact septum requires ductus for mixing blood.

Q15. Dose of PGE1 infusion in neonates:

0.05–0.1 µg/kg/min
0.5–1 mg/kg
5–10 mg/kg
1 U/kg/min
Usual starting dose: 0.05–0.1 µg/kg/min IV infusion.

Q16. In systemic ductus-dependent lesions, which feature is most prominent?

Weak/absent femoral pulses
Polycythemia
Loud P2
Fixed splitting of S2
Systemic perfusion failure leads to weak femoral pulses.

Q17. What is the hallmark presentation of ductus-dependent pulmonary lesions?

Severe cyanosis refractory to oxygen
Shock
Hypertension
Arrhythmia
Cyanosis refractory to O₂ is typical for pulmonary ductus-dependent lesions.

Q18. Which of the following is a systemic ductus-dependent lesion?

Tricuspid Atresia
TOF
Interrupted Aortic Arch
Interrupted aortic arch requires ductus for systemic perfusion.

Q19. Which therapy is definitive for ductus-dependent lesions?

Surgery or catheter-based palliation
Long-term PGE1 infusion
High-dose oxygen
IV fluids only
PGE1 is temporary; definitive correction requires surgery or cath intervention.

Q20. A neonate collapses 2 days after birth with mottling, shock, absent femoral pulses. Likely lesion?

Critical Coarctation of Aorta
Tricuspid Atresia
Pulmonary Atresia
ASD
Critical coarctation causes systemic collapse once ductus closes.

Short Q&A on Ductus-Dependent Circulation

Q1. What is ductus-dependent circulation?
A congenital heart disease condition in which systemic or pulmonary circulation depends on a patent ductus arteriosus for survival.


Q2. Which drug is used to keep the ductus arteriosus open?
Prostaglandin E1 (Alprostadil).


Q3. What happens when the ductus closes in systemic-dependent lesions?
Severe shock, metabolic acidosis, and collapse due to loss of systemic blood flow.


Q4. What happens when the ductus closes in pulmonary-dependent lesions?
Severe cyanosis due to loss of pulmonary blood flow.


Q5. Give two examples of systemic ductus-dependent lesions.

  • Hypoplastic left heart syndrome (HLHS)
  • Critical coarctation of the aorta

Q6. Give two examples of pulmonary ductus-dependent lesions.

  • Pulmonary atresia
  • Critical pulmonary stenosis

Q7. Give an example of a mixing ductus-dependent lesion.
Transposition of the Great Arteries (TGA).


Q8. What is the initial emergency management of suspected ductus-dependent CHD?
Start Prostaglandin E1 infusion immediately.


Q9. What is the starting dose of Prostaglandin E1 (PGE1)?
0.05–0.1 µg/kg/min IV infusion.


Q10. Name a common side effect of PGE1 infusion.
Apnea.


Q11. Which drug is used to close the ductus arteriosus?
Indomethacin or Ibuprofen.


Q12. What clinical clue suggests systemic ductus dependence?
Weak pulses, shock, and metabolic acidosis.


Q13. What clinical clue suggests pulmonary ductus dependence?
Profound cyanosis with minimal improvement in oxygen.


Q14. How does oxygen therapy affect pulmonary ductus-dependent lesions?
Minimal effect on cyanosis (does not improve significantly).


Q15. What is the definitive treatment for ductus-dependent CHD?
Surgical or catheter-based correction/palliation.


Q16. Why does ductus closure typically occur after birth?
Falling prostaglandin levels and rising oxygen tension.


Q17. What is the usual age of presentation for ductus-dependent CHD?
Within the first few days after birth, often after ductus closure (24–72 hours).


Q18. Which investigation confirms ductus-dependent circulation?
Echocardiography.


Q19. What is the mnemonic “SPM” for ductus-dependent lesions?
Systemic, Pulmonary, Mixing types.


Q20. Why is early recognition of ductus-dependent lesions critical?
Delay can result in shock, metabolic acidosis, multiorgan failure, and death.


Duct Dependent Congenital Heart Disease
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