Download Congenital Heart Disease - East Bay Newborn Specialists

Congenital Heart Disease
Initial evaluation and stabilization
Priscilla Joe, MD
Children’s Hospital and Research Center
Oakland
Initial evaluation
 History
 Physical exam with 4 extremity blood
pressures
 Pre-ductal and post-ductal oxygen saturations
 Hyperoxia test
 CXR
 EKG
 ECHO
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Indications for fetal
echocardiography
Fetal risk factors associated with CHD:
 Trisomies, Turner’s syndrome, abnormal
karyotype
 Congenital malformations: duodenal atresia,
TEF, omphalocele, diaphragmatic hernia,
renal dysgenesis, and hydrocephalus
 Fetal arrhythmias
 IUGR
 Nonimmune hydrops
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Maternal metabolic disorders or
infection
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Diabetes mellitus
PKU
Hyperthyroidism
Lupus, collagen vascular disease
Rubella, CMV, Coxsackie, HIV
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Maternal risk factors associated
with congenital heart disease
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Congenital heart disease
Cardiac teratogen exposure
Lithium
Amphetamines
Alcohol
Anticonvulsants: phenytoin, valproic acid,
carbamazepine,and trimethadione
 Isotretinoin
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Lungs vs heart:
Differential cyanosis and the hyperoxia test
 PaO2 <50 and SpO2 <85% pre-ductal
despite 100% FiO2
-PPHN
-left-heart abnormalities
 Post-ductal saturation higher than preductal saturation
-TGA
-TAPVR above diaphragm with PDA
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Neonatal Heart Disease
 Ductal dependent lesions
 Congestive heart failure
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Right heart obstructive lesions
Left heart obstructive lesions
Mixing lesions
Inadequate gas exchange
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Normal heart
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Pulmonary Hypertension
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Pulmonary Hypertension
Preductal SpO2
Postductal SpO2
PA
Ao
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Transposition of great arteries
Ao
PA
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Transposition
Preductal
SpO2
Postductal
SpO2
Ao
PA
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TAPVR
Preductal SpO2
Postductal SpO2
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CXR
 Heart size
 Pulmonary blood flow
 Cardiac position
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Ebstein’s anomaly
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Cyanotic with decreased
pulmonary blood flow
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Right Sided Obstructive LesionsBlue, but comfortable
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Cyanosis
No respiratory distress
Normal pulses and perfusion
Single second heart sound (no closing sound
from abnormal pulm valve)
 Murmur
 Moderate to marked hypoxemia
 CXR: normal to large sized heart, decreased
PBF
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Tetrology of Fallot
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Tetrology of Fallot
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Tetrology of Fallot
Infundibular septum
angled anteriorly
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Tricuspid Atresia
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Tricuspid Atresia
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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
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Management
right sided obstructive lesions
 PGE
 Supplemental O2 is OK (may slightly
improve pulmonary vasodilatation)
 Surgical intervention
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Left sided obstructive lesions
Acute shock
Left sided obstructive lesions
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Grey or ashen color (may not be blue)
Tachypnea
Poor perfusion
Decreased pulses/differential pulses
Single second heart sound
Murmur + gallop
Hepatomegaly
ABG: metabolic acidosis
CXR: cardiomegaly with increased PBF
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Left sided obstructive lesions
 Coarctation of aorta, interrupted aortic
arch
 Hypoplastic left heart syndrome
 Aortic stenosis
 Mitral stenosis
 Total anomalous pulmonary venous
return, below diaphragm
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Hypoplastic left heart syndrome
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Aortic stenosis
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Hypoplastic Left Heart Syndrome
PDA supplies:
•body
•lungs
•head
•coronaries
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Coartation of aorta
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HLHS Treatment
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Fetal diagnosis is vital to prevent end organ failure
PGE
Balance perfusion to body/coronaries/head vs lungs
Avoid oxygen, hyperventilation, pressors to limit PBF
Control ventilation; paralyze and hypoventilate
Blend in nitrogen to raise PVR and limit PBF
Surgical intervention
Cyanotic with increased
pulmonary blood flow
Inadequate mixing
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Inadequate Mixing Lesions
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Cyanosis, often profound
Mild tachypnea
Normal pulses
Single heart sound
Murmur
ABG: marked hypoxemia, + acidosis
CXR: cardiomegaly, normal or increased PBF
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d - Transposition of the Great Vessels
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Transposition of Great Arteries
Mixing at PFO
and PDA
Truncus arteriosus
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Truncus arteriosus
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
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Treatment of mixing lesions: TGA
 PGE
 Avoid too much PBF, may worsen patient
 Balloon septostomy
 Supplemental O2 may be helpful
 Surgical repair
Lesions with poor gas exchange
Lesions with poor gas exchange
 Cyanosis
 Marked tachypnea (difficult to
differentiate from GBS pneumonia/MAS
 Perfusion fair, pulses normal
 Second heart sound may be single
 May or may not have a murmur
 CXR: normal heart size, pulmonary
congestion
Total anomalous pulmonary
venous return
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Supracardiac TAPVR
Management TAPVR
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Ventilation with PEEP
Diuretics
PGE may worsen patient
iNO will worsen patient
Surgical intervention
Initial stabilization
 Airway management: use of
neuromuscular blockade
 Titrate Fi02 to keep Sp02 80%85%.
 Use of PGE1 (0.02 to 0.05
mcg/kg/min)
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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)
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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
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Stabilization for transport
Reliable vascular access
Intubation if on PGE1
Oxygen delivery, Sp02
Monitor HR, tissue perfusion, blood
pressure, and acid-base status
 Calcium and glucose status
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EKG : QRS axis
 Tricuspid atresia with PS or PA : superior
 Critical PS or PA : 0 to 90 degree quadrant
 TOF and TOF with PA: 90-180 degree
quadrant
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Acyanotic with increased
pulmonary blood flow
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VSD
ASD
PDA
Endocardial cushion defect
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Ventriculo septal defect
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Cardiac malpositions and
heterotaxy
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Dextrocardia
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