TETRALOGY OF FALLOT

Fetal echocardiography findings:

1. Small or absent of pulmonary valve
2. Aorta to pulmonary flow in the DA
3. Reversed orientation of the DA (inferior angle <90°)
4. Pulmonary valve z-score value less than 3, after 16 weeks
5. Left to right atrial flow across the foramen ovale and distal aortic arc (ductal dependent systemic circulation)

Delivery room preparations and early treatment:

1. Specialized cardiac care team in the DR
2. Specialized ventilation (prone)
3. Peripheral IV or umbilical access
4. Intubation with mechanical ventilation if needed
5. Consider 100% oxygen and inhaled nitric oxide to
6. decrease pulmonary resistance
7. Consider ECMO
COARCTATION OF THE AORTA
Fetal echocardiography findings:
1. Left/right heart size discrepancy with MV/TV and AoV/PV ratios <0.6
2. Distal arch in 3rd trimester <3 mm
3. AoI/DA in 3VV <0.7553
4. Abnormal Doppler flow in isthmus
5. Posterior shelf

Delivery room preparations and early treatments:

1. Initiation of prostaglandin infusion through peripheral IV or umbilical line
2. Intubation with mechanical ventilation only if clinically indicated
3. Transfer to cardiac center
HYPOPLASTIC LEFT HEART SYNDROME
Fetal echocardiography findings:
1. Ductal dependent lessions:
a. Small or absent of pulmonary valve
b. Aorta to pulmonary flow in the DA
c. Reversed orientation of the DA (inferior angle <90°)
d. Pulmonary valve z-score value less than 3, after 16 weeks
e. Left to right atrial flow across the foramen ovale and distal aortic arc (ductal dependent systemic circulation)
2. Pulmonary vein Doppler:
a. Moderate obstruction: PV f/r <5 and >3
b. Severe obstruction: PV f/r <3

Delivery room preparations and early treatments:

1. Initiation of prostaglandin infusion through peripheral
2. IV or umbilical line
3. Intubation with mechanical ventilation
4. OR or cath lab on standby
5. Plan for immediate intervention to decompress left atrium
6. ECMO available
TRANSPOSITION OF GREAT ARTERIES
Fetal echocardiography findings: Delivery room preparations and early
1. Foramen ovale findings: treatment:
a. Hypermobile septum
b. Angle of septum primum <30° 1. Initiation of prostaglandin infusion through peripheral
IV or umbilical line
c. Lack of swinging motion of septum or
“tethered” septum 2. Intubation with mechanical ventilation
d. Bowing of atrial septum >50%
e. Intact 3. Cath lab on standby

2. Abnormal ductus arteriosus: 4. Plan for immediate balloon atrial septostomy

a. With additional RFO 5. If ductal flow abnormal, consider pulmonary
b. Small with moderate/severe restriction hypertension therapy including intubation, 100%
c. Reversed, bidirectional or accelerated flow oxygen, inhaled nitric oxide
3. Pulmonary vein Doppler, proximal to the
left atrium:
a. Max velocity “s” wave >41 cm/s
EBSTEIN ANOMALY
Fetal echocardiography findings:
1. PV regurgitation
2. Large TV annulus
3. Pericardial effusion
4. Large cardiothoracic ratio
5. Lower TV velocity
6. Absence of anterograde flow across PV

Delivery room preparations and early treatmen:

1. Specialized cardiac care team in the DR
2. Specialized ventilation (prone)
3. Peripheral IV or umbilical access
4. Intubation with mechanical ventilation if needed
5. Consider 100% oxygen and inhaled nitric oxide to decrease pulmonary resistance
6. Consider ECMO cardioversion or medical therapy in DR as indicated for arrhythmia
TOTAL ABNORMAL PULMONARY VENOUS
RETURN
Fetal echocardiography findings:
1. Lack of identification of at least 2 pulmonary veins connecting to the left atrium
2. Right-left heart size discrepancy
3. Presence of the “twig” sign on the four-chamber view, defined as an abnormally wide space in the retro-atrial
4. Abnormal three-vessel view, with the presence of either an enlarged superior vena cava, or abnormal ascending vessel
5. Abnormal pulmonary vein Doppler waveform:
a. Monophasic nonpulsatile pulmonary
b. Infradiaphragmatic TAPVR

Delivery room preparations and early treatment:

1. Intubation with mechanical ventilation
2. Peripheral IV and/or umbilical line
3. OR team on standby
4. Initiation of prostaglandin infusion (may relax the ductus venosus smooth muscle for infradiaphragmatic TAPVR)
5. Plan for immediate surgical intervention