Duct dependent circulation.ppt2
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Transcript of Duct dependent circulation.ppt2
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Management of a Patient with
duct dependent circulation
Dr Anil S.R
Consultant Pediatric Cardiologist
Apollo Hyderabad
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Duct dependent Physiology
• Pulmonary Circulation
• Systemic Circulation
• Both Circulation- Intercirculatory Mixing
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Duct dependent Pulmonary
circulation
• Pulmonary atresia- VSD or Intact Septum
• Severe TOF
• Neonatal Ebsteins
• Critical PS- Isolated or with complex CHD
• Severe Tricuspid Valve regurgitation
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Duct dependent systemic circulation
• Left heart: Hypoplastic left heart
• Critical AS
• CoA
• Interrupted arch
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Pulmonary Vs Systemic
Pulmonary
Cyanosis predominant- Hypoxia dominating the clinical picture
Relatively well at least initially- present in 24-48 hours of life
Systemic
Poor Perfusion- Decreased cardiac output
Sick infants- present in the first 2 weeks of life.
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Suspect and Rule out
• Any baby with shock or collapse after 24
hours- “Duct Dependent systemic
circulation”
– Poor perfusion, absent pulses, pallor, shock
• Cyanosis
• Differential cyanosis
• Respiratory symptoms but lungs clear
• No response to oxygen or worsening with
oxygen
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What to DO???
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Prenatal Physiology
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Prenatal Physiology
• Severe left or right sided lesions do not
affect fetal growth and development.
• Factors that mimic fetal circulation will
stabilize the neonate.
• Ductus-dependent fetal cardiac defects are
contraindications to the maternal use of
prostaglandin inhibitors during pregnancy.
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Neonatal Cardiac Evaluation
•General Evaluation
Airway,Breathing,Circulation
History and Examination
•Inspection and Palpation
Activity
Cyanosis
Peripheral Perfusion
Dysmorphism and System Review
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Neonatal Cardiac Evaluation
•Auscultation
Single S2
Murmurs +/-
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Neonatal Cardiac Evaluation
•Saturations
•4limb BP
•Liver Size
•Auscultation
•CXR
•Hyperoxia Test
•ABG- pH, PCO2, pO2, HCO3,Blood Sugar
•Septic Workup
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Hyperoxia Test
100% Oxygen (or as close as possible) for 10
minutes -by Blow by Mask, intubation
-Repeat pO2 assessments by ABG
-Pulseoximeter is Unacceptable
Measure right arm pO2
pO2 > 250 – Unlikely to be Cyanotic CHD
pO2 100-250 – possible cyanotic CHD
pO2 <100 – Cyanotic CHD
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Pulmonary Oligemia
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Diagnosis of duct dependence
Echo is the gold standard
-Delineate the anatomy
-Assess the size of PDA, degree of
restriction/constriction
-Assess PVR/SVR ratio on the flow pattern
across the duct
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Management Principles
Back to Basics
A , B, C and
Drugs (Prostaglandin E1)
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Management of Sick Neonate
• Ensure Airways
• Optimise Oxygen
• Breathing - Good Now- Will it Sustain?- Fatigue an issue- When to ventilate?
• Ventilate : Off load work of breathingEnsure good OxygenationSecure LinesSecure airwaysConcomitant respiratory issues
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When in doubt
• Blue baby
• Baby in shock
Start………………Prostaglandin
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PGE1 Preparation
• Available as PROSTIN VR from Upjohn
pharmacia as 1 ml ampoules containing
500 mcg/ml.
• We dilute 0.1 ml (50 mcg) in 50 cc 5%
Dextrose under laminar air flow to avoid sepsis.
• The remaining PGE1 is stored in a fridge at 2-8o C.
• Dilute 0.1 ml (50 mcg) in 50 ml 5%D; in this
dilution, each ml equals 1 mcg of PGE1
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PGE1 infusion protocol
• PGE1 infusion is started at 0.05 - 0.1 mcg/kg/min
• Depending on clinical response ( Saturations, femoral Pulse, urine output) infusion rates can be increased upto 0.4 mcg/kg/min
• Once a desired response is obtained, (stable sats of 70 - 80% ) maintain patency by continuing infusions at 0.01 - 0.05 mcg/kg/min
• It is often possible to bring down the rate to low levels to maintain effect
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0.01mcg/kg
0.025mcg/kg
0.05mcg/kg
0.1mcg/kg
2 kg 17 days 7 days 3.5 days 2 days
3 kg 12 days 5 days 3 days 1.5 days
4 kg 8 days 3.5 days 2 days 1 day
How long does one Ampoule last?
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Monitoring PGE1
Hypotension : Manifests as cold extremities, delayed
capillary refill > 3 min tachycardia, low
urine
Rx : IV fluid bolus 5-10 ml/kg N.Saline in
Apnea : Often noted in first hours, noted by apnea
monitor or a watchful nurse (ideal).
common in < 2 kg, preterms
Rx : Stimulation, Hand Bagging, Ventilation
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Fever : often in first 24 hours, needs tepid sponging
Rx : Ensure that insensible fluid losses of a febrile
neonate are met adequately
Fluid retention:
Often a common problem
Seizures:
Rare, may need cessation of PGE1 infusion
Rx : Rule out other metabolic and infective causes in a sick
neonate
Monitoring PGE1
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Monitoring PGE1
Bradycardia : if severe stop infusion
Flushing : often noted in first few hours
Others : Diarrhea, sepsis, DIC, Low K+.
> 7 DAYS USE : Long bone hyperosteosis
Gastric outlet (antral)obstruction
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PDA patency
• Oral/IV PGE
• Atropine
• PDA balloon/Stent
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Surgical alternative: BT shunt
• Shunt blockage:Acute thrombosis
• Shunt overflow: CHF, pleural effusions
• Chylothorax
• Diaphragmatic paralysis
• Late: Pulmonary artery distortion or
stenosis
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Keeping the PDA patent
• Formalin infiltration of PDA at
thoracotomy-invasive
• Early experience of PDA stenting
– Perforation of PA’s/chambers
– PDA’s longer, tortuous, vertical in cyanotic
CHD-axillary cut down needed
– Inability to enter the duct, ductal spasm,
incomplete stenting
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PDA stenting
• Current coronary equipment:better
flexibility, lower profile
• Heparin coating and drug elution may
prolong palliation
• 4F long Cook sheaths; cut pig (u) for
vertical ducts or Judkins right
• Extra support choice PT wire
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PDA stenting
• Transfemoral or transvenous via the VSD
• Need to cover complete length of PDA esp
distal end near PA
• Measure length once PDA fully
straightened by extra stiff wire
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Blue babies: Other Non surgical
Rx
• Critical PS/AS/TOF with valvar stenosis
Emergent non-surgical Rx:
– Valvuloplasty
• Atresia:
– Valve perforation with straight wire or 2F RF
ablation
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D-TGA: Non surgical Rx
Key is to ensure mixing
Fluid boluses
PGE1
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Life saving in TGA where inadequate
mixing can lead to cyanosis
and low cardiac output Use umbilical or
femoral vein
Balloon atrial septostomy
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• Keep calm
• Knee chest
• IV fluid bolus
• Bicarbonate
• Morphine IM or SC
• Metoprolol (0.1 mg/kg)
• Phenylephrine
• If all else fails:
• General anesthesia
• or
Spells
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Conclusion
• The key to a successful outcome in duct
dependent cardiac lesions is…
to realise the baby IS duct dependent!!
Thank you!