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Critical Congenital Heart Defects (CCHD) (Five T's (Others…
Critical Congenital Heart Defects (CCHD)
Structural abnormality in the circulatory system of an infant that is apparent at birth
Pathophysiology
Right-to-Left Shunt with cyanosis and hypoxemia
Five T's
Tetralogy of Fallot
Total anomalous pulmonary venous return
Transposition of the great arteries
Tricuspid atresia
Truncus arteriosus
Others
Hypoplastic left heart syndrome
Pulmonary atresia with intact septum
Physical examination
Central cyanosis or persistent pallor
Abnormal cardiovascular findings
Abnormal HR
Murmurs
Weak/absent peripheral pulses
Abnormal respiratory findings
Tachypnea
Labored breathing at rest
Coughing
Wheezing
Increased distress when feeding
Cyanotic infants
Assess Pa02 after 10 mins of breathing 100% (Hyperoxia test)
Pa02 < 150 = Intracardiac R-L shunt/CCHD likely
Pa02 > 150 but < 200 = ambiguous results
Pa02 > 200 Pulmonary R-L shunt (RDS, PPHN)
Recommend
Chest X-ray
Total anomalous pulmonary venous return
"Snowman"
Pulmonary atresia/Tetralogy of Fallot/Tricuspid atresia
"Boot"
Transposition of the great arteries
"Egg on string"
ECG
Detect axis deviation
Right axis deviation
Right ventricular hypertrophy
Transposition of the great arteries
Total anomalous pulmonary venous return
Tetralogy of Fallot
Left axis deviation
Left ventricular or biventricular hypertrophy
Trucus arteriosus
Transposition of the great arteries
Tricuspid atresia
2D and Doppler echocardiography
Infants who test positive for CCHD on pulse ox screening or hyperoxia test
Treatment
Surgical correction
Ductal dependent defect
IV prostaglandin E1 to dilate the ductus arteriosis
Heart failure/systemic hypotension/low CO
Inotropic agent (Dopamine)
Mechanical Ventilation