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Critical Congenital Heart Disease (Types of CCHD (HLHS, Pulmonary atresia,…
Critical Congenital Heart Disease
Screening
33% reduction of infant death
7 categories
primary targets
for pulse oximetry screening
TGA
HLHS
PA IVS
TOF
TAPVD
TA
Truncus arteriosus
Secondary target
Critical CoA
Atresia/Hypoplasia/interruption of Aortic arch
DORV
DORV
Ebstein anomaly
Severe AS
Severe PS
Single ventricle
Oxygen screening
Types of CCHD
HLHS
Pulmonary atresia
TOF
TAPVD
Critical pulmonary stenosis
Tricuspid atresia
Coarctation of Aorta/Interrupted aortic arch
TGA
Critical aortic stenosis
Top 2 reasons go to EMG
Cyanosis
Dyspnea
Congenital heart disease
Prevalence
Affect 7-9 every 1000 newborns (1% of births)
Leading cause of infant death
28% death first month of life
30-50% death during 2-12 mo
Often unrecognized
1 in 20.000 to 1 in 40.000 live births result in death from unrecognized CHD
Critical congenital heart disease 25% from CHD
Definition
Serious + life threatening symptoms
More death than other CHD
Associated with: Hypoxemia
Req interv in the 1st year of life
Early intervention reduce mortality 2-3/1000 --> 0.6-0.8/1000
Differentiation by Cyanosis
Immediately after birth
TGA-IVS
PA-IVS
TAPVD with obstruction
Within several hours - 2 weeks
Pulmonary atresia
Critical PS with R-L shunt
HLHS
2-3 months of age (Fall of PVR - increased L-R shu)
Common mixing lesion
Double outlet
Double inlet
Truncus arteriosus
Duct Dependent CHD
Types
Systemic circulation
A.k.a left side obstruction
Ex:
HLHS
Severe AS
Severe CoA
IAA
Purpose
Perfusion to
Whole body
Lower side of body
Closure of duct
Acidosis
Lef pulses weak
Oliguria
Pulmonary circulation
a.k.a right sided obstructed lesions
Ex
Critical TOF
PA and variants
Critical PS
Tricuspid atresia
PS/PA with or without VSD
Univentricular heart with PS/PA
Severe Ebstein anomaly
Adequate mixing
Ex
TGA IVS
Two parallel circulation
Mixing occur via ductus
Condition
Cyanosis at birth
Progressive cyanosis
Pulmonary vs Systemic
Pulmonary
Cyanosis predominant
Relatively well initially
Systemic
Poor perfusion - Decreased CO
Sick infant, 2 wks of life
Management
Balloon Atrial Septectomy
Classification
Optimal
Biventricular physiology - TGA
Target
Improvement of intercirculatory mixing
Stable Peripheral oxygen sat
Not pursuing wide ASD diameter
Maximal
Univentricular physyoilogy
Tricuspid atresia
Mitral Atresia
TAPVC
PA-IOVS with restrictive PFO/ASD
Main goal
Atrial decompression - Lowering resistance and gradient
Need maximum diameter