Overview/Definition
Definition:
• Patent Ductus Arteriosus (PDA) is persistence of the fetal ductus arteriosus beyond 72 hours in term infants or beyond several weeks in preterm infants
- Medical closure uses NSAIDs (indomethacin or ibuprofen) to promote ductal constriction and closure
- Device closure involves transcatheter placement of occluder devices for PDAs unsuitable for medical therapy
- Choice between medical and device closure depends on patient age, PDA size, contraindications, and clinical presentation.
Epidemiology:
• PDA occurs in 5-10% of all congenital heart defects, higher in females (2:1 ratio)
- Incidence in preterm infants inversely related to gestational age: 80% at 24-28 weeks, 25% at 32-36 weeks
- In term infants, isolated PDA occurs in 1 in 2000-2500 births
- In India, higher altitude areas show increased PDA incidence due to chronic hypoxia.
Age Distribution:
• Preterm infants: Usually diagnosed within first few days to weeks of life during NICU stay
- Term infants: May be diagnosed anytime from newborn period to adulthood if asymptomatic
- Medical closure most effective: <3 days of age in preterm infants, limited efficacy beyond 1 week
- Device closure: Typically performed after 6 months of age when PDA diameter adequate for device placement.
Clinical Significance:
• Essential topic for DNB Pediatrics and NEET SS, frequently tested in cardiology and neonatology sections
- Early recognition and appropriate treatment prevent complications like pulmonary hypertension and heart failure
- Understanding indications for medical vs
device closure crucial for optimal patient outcomes
- Cost-effectiveness considerations important in resource-limited settings like India.
Age-Specific Considerations
Newborn:
• Extremely preterm (<28 weeks): High incidence, often hemodynamically significant requiring early intervention
- Very preterm (28-31 weeks): Medical closure most effective in first 3 days of life
- Late preterm (32-36 weeks): Lower incidence but still higher than term infants
- Term newborns: PDA present at birth should close within 72 hours, persistence requires evaluation.
Infant:
• Early infancy (1-6 months): Large PDAs may cause congestive heart failure, poor feeding, failure to thrive
- Medical closure: Limited efficacy beyond neonatal period, contraindicated after certain age
- Device closure: Usually not performed before 6 months due to small vessel size
- Monitoring period: Some moderate PDAs may close spontaneously during first 6-12 months.
Child:
• School age (2-12 years): Ideal age for device closure in appropriate candidates
- Medical closure: Generally not effective beyond infancy, contraindicated in older children
- Surgical closure: May be preferred for large PDAs or those with unfavorable anatomy
- Asymptomatic PDAs: May be discovered incidentally, require evaluation for closure indications.
Adolescent:
• Device closure: Preferred method for suitable anatomy, excellent success rates and low morbidity
- Medical closure: Contraindicated due to premature closure of other arteries
- Surgical closure: Reserved for complex anatomy, very large PDAs, or failed device closure
- Pulmonary hypertension: May develop if large PDA left untreated, affects closure options.
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Clinical Presentation
Symptoms:
• Asymptomatic: Small PDAs may have no symptoms, discovered on routine examination
- Heart failure symptoms: Poor feeding, tachypnea, diaphoresis, failure to thrive in infants
- Respiratory symptoms: Recurrent pneumonia, difficulty breathing, exercise intolerance
- Growth issues: Poor weight gain, delayed milestones due to increased metabolic demands.
Physical Signs:
• Continuous murmur: Classic "machinery" murmur heard best at left upper sternal border
- Precordial activity: Hyperdynamic precordium with prominent apical impulse
- Pulse characteristics: Wide pulse pressure, bounding peripheral pulses
- Signs of heart failure: Hepatomegaly, gallop rhythm, pulmonary rales.
Severity Assessment:
• Small PDA: Continuous murmur, normal cardiac silhouette, no symptoms
- Moderate PDA: Cardiomegaly, increased pulmonary markings, mild symptoms
- Large PDA: Significant cardiomegaly, pulmonary edema, congestive heart failure
- Eisenmenger syndrome: Cyanosis, clubbing, pulmonary hypertension with reversed shunt.
Differential Diagnosis:
• Venous hum: Continuous murmur, disappears with neck compression or supine position
- Arteriovenous malformation: Usually in head/neck, different location from typical PDA murmur
- Pulmonary artery stenosis: Systolic murmur only, different timing from PDA
- Aortic regurgitation: Diastolic murmur, different characteristics from continuous PDA murmur.
Diagnostic Approach
History Taking:
• Perinatal history: Prematurity, birth weight, maternal rubella, high altitude residence
- Symptoms: Feeding difficulties, poor weight gain, respiratory infections, exercise intolerance
- Family history: Congenital heart disease, genetic syndromes, consanguinity
- Associated conditions: CHARGE syndrome, Down syndrome, DiGeorge syndrome.
Investigations:
• Echocardiography: Gold standard for diagnosis, assesses size, flow direction, left heart volume overload
- Chest X-ray: Cardiomegaly, increased pulmonary vascular markings in significant PDAs
- Electrocardiogram: Left atrial enlargement, left ventricular hypertrophy in large PDAs
- Cardiac catheterization: Usually not needed for diagnosis, may be done for device closure.
Normal Values:
• Echocardiography: No flow across ductus arteriosus, normal left heart dimensions
- PDA diameter: <1.5 mm considered small, 1.5-3 mm moderate, >3 mm large
- Left atrial to aortic root ratio: Normal <1.4, elevated suggests significant left-to-right shunt
- Pulmonary artery pressure: Should be normal in absence of significant PDA.
Interpretation:
• Hemodynamic significance: Determined by left heart volume overload on echo, not just PDA size
- Shunt direction: Left-to-right normal, bidirectional concerning, right-to-left indicates Eisenmenger
- Closure suitability: Device closure suitable for most PDAs >2 mm diameter with adequate anatomy
- Timing considerations: Medical closure most effective within 72 hours in preterm infants.
Management/Treatment
Acute Management:
• Hemodynamically significant PDA in preterm: Consider medical closure with indomethacin or ibuprofen
- Congestive heart failure: Diuretics (furosemide), ACE inhibitors, digoxin for symptomatic relief
- Fluid restriction: Limit fluids to 120-150 mL/kg/day to reduce volume overload
- Respiratory support: May need CPAP or mechanical ventilation for severe heart failure.
Chronic Management:
• Medical closure: Indomethacin 3 doses (0.1-0.3 mg/kg) or ibuprofen 3 doses (5-10 mg/kg)
- Device closure: Transcatheter placement of Amplatzer or similar occluder device
- Surgical closure: Video-assisted thoracoscopic or open surgical ligation
- Conservative management: Monitor asymptomatic small PDAs for spontaneous closure.
Lifestyle Modifications:
• Activity restrictions: Limit strenuous activity in symptomatic patients before closure
- Nutrition optimization: High-calorie feeds, nutritional supplementation if failure to thrive
- Infection prevention: Pneumococcal and influenza vaccination, RSV prophylaxis if indicated
- Endocarditis prophylaxis: Required for 6 months after device closure or indefinitely if residual shunt.
Follow Up:
• Medical closure: Echo at 24-48 hours to assess response, repeat if unsuccessful
- Device closure: Echo at 1 day, 1 month, 6 months, then annually
- Conservative management: Serial echos every 6-12 months to monitor for spontaneous closure
- Long-term: Annual cardiology follow-up after successful closure.
Age-Specific Dosing
Medications:
• Indomethacin: 3 doses IV - 1st dose 0.1-0.3 mg/kg, 2nd and 3rd doses 0.1-0.25 mg/kg
- Ibuprofen: 3 doses IV - 10 mg/kg, then 5 mg/kg, then 5 mg/kg every 24 hours
- Furosemide: 1-2 mg/kg IV/PO every 12-24 hours for heart failure
- Captopril: 0.01-0.05 mg/kg PO TID, maximum 2 mg/kg/day for afterload reduction.
Formulations:
• Indomethacin: 1 mg/mL IV solution, reconstitute with preservative-free water or saline
- Ibuprofen: 10 mg/mL IV solution (Pedea), give over 15 minutes via dedicated line
- Furosemide: 10 mg/mL injection, 8 mg/mL oral solution for chronic use
- Captopril: 1 mg/mL suspension (extemporaneous), crush tablets for smaller doses.
Safety Considerations:
• NSAID contraindications: Renal dysfunction, severe heart failure, bleeding, necrotizing enterocolitis
- Indomethacin side effects: Decreased urine output, hyperkalemia, bleeding, platelet dysfunction
- Ibuprofen monitoring: Similar to indomethacin but may have less renal toxicity
- Drug interactions: Avoid concurrent aminoglycosides, which increase nephrotoxicity.
Monitoring:
• Pre-treatment: Renal function, platelet count, bleeding time, echocardiogram
- During treatment: Daily weights, urine output, electrolytes, creatinine
- Post-treatment: Echo at 24-48 hours, repeat course if unsuccessful and no contraindications
- Long-term: Monitor for recurrence, residual shunt, pulmonary hypertension.
Prevention & Follow-up
Prevention Strategies:
• Prenatal: Avoid maternal rubella infection, control maternal diabetes, adequate nutrition
- Delivery: Avoid unnecessary oxygen exposure, maintain appropriate oxygen saturation targets
- NICU care: Judicious fluid management, early nutrition, avoid prolonged mechanical ventilation
- High altitude: Supplemental oxygen may help promote ductal closure in some cases.
Vaccination Considerations:
• Standard immunizations: Follow routine schedule, especially important for cardiac patients
- Pneumococcal vaccine: 13-valent conjugate vaccine plus 23-valent polysaccharide after age 2
- Influenza vaccine: Annual vaccination strongly recommended for all cardiac patients
- RSV prophylaxis: Palivizumab for high-risk infants with significant cardiovascular disease.
Follow Up Schedule:
• Post-medical closure: Echo at 48 hours, 1 week, 1 month to confirm sustained closure
- Post-device closure: Echo at 1 day, 1 month, 6 months, then annually
- Unsuccessful closure: Repeat attempt or alternative therapy based on clinical status
- Long-term: Annual cardiology visits, periodic echo, exercise testing if indicated.
Monitoring Parameters:
• Cardiac function: Left ventricular dimensions, ejection fraction, mitral regurgitation
- Pulmonary pressures: Monitor for development of pulmonary hypertension
- Growth: Weight gain, developmental milestones, exercise tolerance
- Complications: Arrhythmias, endocarditis, device-related issues if applicable.
Complications
Acute Complications:
• Congestive heart failure: Volume overload, poor feeding, respiratory distress requiring diuretics
- Pulmonary edema: Severe left-to-right shunt causing respiratory failure
- Arrhythmias: Atrial fibrillation or flutter due to left atrial enlargement
- NSAID toxicity: Renal dysfunction, bleeding, necrotizing enterocolitis, platelet dysfunction.
Chronic Complications:
• Eisenmenger syndrome: Irreversible pulmonary hypertension with shunt reversal
- Left heart failure: Progressive cardiomyopathy from chronic volume overload
- Infective endocarditis: Risk highest with moderate-sized PDAs creating turbulent flow
- Device-related: Embolization, residual shunt, arrhythmias, hemolysis rare with modern devices.
Warning Signs:
• Heart failure: Tachypnea, poor feeding, excessive sweating, failure to thrive
- Cyanosis: May indicate shunt reversal or severe heart failure
- Arrhythmias: Irregular heart rate, palpitations, syncope
- Device complications: Chest pain, new murmur, hemolysis after device closure.
Emergency Referral:
• Cardiothoracic surgery: Failed medical/device closure, complex anatomy, surgical complications
- Intensive care: Severe heart failure, pulmonary hypertension crisis, post-procedural complications
- Interventional cardiology: Device embolization, vascular complications during catheterization
- Emergency cardiology: New arrhythmias, signs of endocarditis, unexplained deterioration.
Parent Education Points
Counseling Points:
• PDA explanation: Connection between aorta and pulmonary artery that should close after birth
- Treatment options: Medication, device, or surgery depending on age and PDA characteristics
- Prognosis: Excellent outcomes with appropriate treatment, normal life expectancy expected
- Timing: Early intervention prevents complications, but not all PDAs require immediate closure.
Home Care:
• Feeding: High-calorie formula or breast milk, frequent small feeds if heart failure present
- Activity: Normal activity as tolerated, avoid overexertion if symptomatic
- Weight monitoring: Daily weights if heart failure, weekly weights for growth monitoring
- Medication compliance: Give medications as prescribed, especially diuretics and cardiac medications.
Medication Administration:
• Diuretics: Give in morning to avoid nighttime urination, monitor for dehydration
- Cardiac medications: Exact dosing important, use oral syringe for accuracy
- Side effects: Watch for poor feeding, lethargy, changes in urination pattern
- Storage: Keep medications in original containers, check expiration dates regularly.
When To Seek Help:
• Signs of heart failure: Rapid breathing, poor feeding, excessive sweating, irritability
- Growth concerns: Poor weight gain, failure to meet developmental milestones
- Respiratory symptoms: Frequent infections, difficulty breathing, wheezing
- Emergency signs: Blue color around lips, severe difficulty breathing, loss of consciousness.