Transcript Diagnosis and Early Management of the Infant with Suspected Congenital Heart Disease

Diagnosis and Early Management
of the Infant with Suspected
Congenital Heart Disease
Introduction
• Congenital heart disease occurs in 1% of liveborn infants
• Almost 1/2 of all cases of congenital heart
disease are diagnosed during the 1st week of
life
• The most frequently occuring anomalies seen
during the 1st week are: PDA, D-transposition
of the great arteries, hypoplastic left heart
syndrome, TOF, and pulmonary atresia
Indications for Fetal
Echocardiography
Maternal Risk Factors Associated
With Congenital Heart Disease
• Congenital heart disease
• Cardiac teratogen exposure
– Lithium
– Amphetamines
– Alcohol
– Anticonvulsants: phenytoin, valproic acid,
carbamazepine, and trimethadione
– Isotretinoin
Maternal Metabolic Disorders
or Infection
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Diabetes mellitus
PKU
Hyperthyroidism
Lupus, collagen vascular disease
Rubella, CMV, Coxsackie, Parvovirus
Fetal Risk Factors Associated
With Congenital Heart Disease
• Trisomies, Turner’s syndrome, abnormal
karyotype
• Congenital malformations: duodenal
atresia, TEF, omphalocele, diaphragmatic
hernia, renal dysgenesis, and hydrocephalus
• Fetal arrhythmias
• IUGR
• Nonimmune hydrops
• ?2 vessel cord
Cyanosis
• Etiology: CV, pulmonary, airway obstruction,
neurological, neuromuscular, or hematological
(methemoglobinemia or polycythemia)
• Infants can appear cyanotic when the
deoxygenated Hgb concentration is at least
3g/dL; it is not related to the percent saturated
• 2 babies with sats of 80%: one with a hgb of
20g/dL and 4g/dL of desaturated hgb will be
cyanotic, but an anemic infant with 10g/dL
with 2g/dL deoxygenated hgb will not be
cyanotic
Evaluation
• ABC’s
• PE: murmur, pulses, precordium,
respiratory status, HSM, color, capillary
refill
• 4 ext BPs: if SBP >10mmHg in right hand
compared to lower ext, concerning for arch
anomaly (though if normal may not rule it
out)
• Pre/post ductal saturations: if see a
difference >5%, concerning for PPHN or
left heart abnormalities
Evaluation (Continued)
• Hyperoxia test: baseline pre-ductal ABG
when infant in room air, then repeat on
100% FiO2
• Reason for ABG and not just sats: with a
saturation of 100%, you can have a PaO2 of
80 or 300; very different
• CXR: cardiomegaly; normal, increased, or
decreased pulmonary vascularity
• EKG
• Echo
Interpretation of hyperoxia test: From Harriet Lane Handbook
FiO2= 0.21 PaO2
(%sats)
FiO2 =1.00 PaO2
(%sats)
PaCO2
Normal
70 (95%)
>200 (100%)
35
Pulmonary Dz
50 (85%)
>150 (100%)
50
Neurologic Dz
50 (85%)
>150 (100%)
50
Methemoglobinemia
70 (95%)
>200 (100%)
35
Cardiac Dz
Separate circulation
(T GA no VSD)
Restricted PBF
( TA +PS, PA, PS + no VSD, TOF)
<40 (<75%)
<50 (85%)
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< 40 (<75%)
<50 (<85)
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<150 (<100%)
35
Complete mix no restricted PBF 50 (85%)
(Truncus, TAPVR, Single Vent,
T GA +VSD, TA no PA or PS)
PPHN
PFO no R->L shunt
PFO + R->L shunt
Preductal Post ductal
70 (95%)
<40 (<75%)
<40 (<75%) <40 (75%)
Variable
Variable
35-50
35-50
Specific Heart Disease
Abnormalities
Cyanotic With Decreased
Pulmonary Blood Flow
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Tetrology of Fallot
Ebsteins Anomaly
Tricuspid Atresia with PA or PS
Pulmonary atresia with intact septum
Critical pulmonic stenosis
PPHN
Right Sided Obstructive
Lesions
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Cyanosis
No respiratory distress
Normal pulses and perfusion
Single second heart sound
Murmur
Moderate to marked hypoxemia
CXR: normal to large sized heart, decreased
pulmonary blood flow (PBF)
Tetralogy of Fallot
Tetrology of Fallot
Ebstein’s Anomaly
Ebstein’s Anomaly
Tricuspid Atresia
Tricuspid Atresia
EKG : QRS axis
•Tricuspid atresia with PS or PA with intact
ventricular septum: superior (0— -90)
•Critical PS or PA : 0 to 90 degree quadrant
•TOF and TOF with PA: 90-180 degree
quadrant
Cyanotic With Increased
Pulmonary Blood Flow
• d-Transposition of the great vessels
• Truncus arteriosus
• Total anomalous pulmonary venous return,
above diaphragm
• Single ventricle
• Endocardial cushion defect
Inadequate Mixing Lesions
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Cyanosis
Mild tachypnea
Normal pulses
Single heart sound
Murmur
ABG: marked hypoxemia, + acidosis
CXR: cardiomegaly, normal or increased
PBF
Transposition of the Great
Arteries
d - Transposition of the Great
Vessels
Truncus Arteriosus
Truncus Arteriosus
Lesions with Poor Gas
Exchange
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Cyanosis
Marked tachypnea
Fair perfusion, normal pulses
May or may not have a single heart sound
May or may not have a murmur
CXR: normal heart size, pulmonary
congestion
Total Anomalous Pulmonary
Venous Return
Total Anomalous Pulmonary
Venous Return
Left Sided Obstructive
Lesions
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Coarctation of aorta, interrupted aortic arch
Hypoplastic left heart syndrome
Aortic stenosis
Mitral stenosis
Total anomalous pulmonary venous return,
below diaphragm
Left Sided Obstructive
Lesions
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Grey or ashen color
Tachypnea
Poor perfusion
Decreased pulses/differential pulses
Single second heart sound
Murmur + gallop
Hepatomegaly
ABG: metabolic acidosis
CXR: cardiomegaly with increased PBF
Coarctation of the Aorta
Hypoplastic Left Heart
Syndrome
Hypoplastic Left Heart
Syndrome
Aortic Stenosis
Acyanotic With Increased
Pulmonary Blood Flow
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VSD
ASD
PDA
Endocardial cushion defect
Ventricular Septal Defect
Ventricular Septal Defect
Atrial Septal Defect
Atrioventricular Canal
Patent Ductus Arteriosus
Initial Stabilization
• ABC’s: Volume resuscitation, ionotorpic support,
correction of metabolic acidosis, r/o sepsis
• Intubate if needed, titrate Fi02 to keep Sp02 80%85% to prevent pulmonary overcirculation
• Placement of umbilical lines
• Infants who present in shock within the first 3
weeks of life, consider ductal dependent lesions
• Use of PGE1 (0.025 to 0.1mcg/kg/min)
Stabilization for Transport
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Reliable vascular access
Intubation if on PGE1, OG placement
Oxygen delivery, Sp02
Monitor HR, tissue perfusion, blood
pressure, and acid-base status
• Calcium and glucose status (increased risk
for DiGeorge)
Prostaglandin E1
• Failure to respond: diagnosis incorrect,
older infant with unresponsive ductus,
ductus absent, obstructed pulmonary venous
return
• Clinical deterioration after PGE1:
obstructed blood flow out of pulmonary
veins or left atrium, HLHS with restrictive
FO, TGA with intact ventricular septum and
restrictive FO, obstructed TAPVR, mitral
atresia with restrictive FO
PGE 1 - Side Effects
• Common: Apnea, fever, leukocytosis,
cutaneous flushing, and bradycardia.
• Uncommon: seizures, hypoventilation,
hypotension, tachycardia, cardiac arrest,
sepsis, diarrhea, DIC, fever
• Rare: urticaria, bronchospasm, hemorrhage*,
hypoglycemia, and hypocalcemia
*inhibits platelet aggregation