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Pediatrics · Topic 28 of 42

Transposition of the Great Arteries (TGA)

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Cardiac · congenital defect

Transposition of the Great Arteries (TGA)

The aorta and pulmonary artery leave the wrong ventricles, so blood runs in two parallel loops. The newborn survives only while the loops can mix.

01 · The hallmarkCyanosis that 100% oxygen doesn't fix
02 · Keep it openAlprostadil IV keeps the ductus open
03 · Watch forApnea on alprostadil: airway at bedside
01

Why it happens

Two parallel circulations — survival depends on mixing

Aorta arises from the right ventricle
Pulmonary artery arises from the left ventricle
Two separate, parallel circulations form
Mixing keeps the infant alive: ductus, foramen ovale/ASD or VSD

TGA makes up about 5–7% of congenital heart defects and is more common in males. Untreated, most infants die within the first year.

02

What you'll see

A term, well-grown newborn turning blue within hours

Severe central cyanosis within hours of birth hallmark
Cyanosis does not improve with 100% oxygen
Term and well grown — yet deeply cyanotic
Murmur often absent — a quiet heart doesn't rule out TGA

With a large VSD, cyanosis may be mild or seen only with crying, and heart failure signs appear in the first weeks; care as for infant heart failure.

03

Labs & diagnostics

Often found before birth or on the newborn pulse-ox screen

Fetal ultrasound and echocardiogram often find it before birth
Newborn pulse oximetry screening for critical heart defects
Chest x-ray: egg-on-a-string heart shape

Hyperoxia test: on 100% oxygen, preductal PaO₂ typically stays below 50 mmHg in TGA, separating heart from lung causes of cyanosis.

04

Red flags

Apnea on alprostadil (prostaglandin E1) report now
Bradycardia or hypotension on alprostadil report now
Fever or flushing on alprostadil drug effect
Ischemic ECG changes or dysrhythmia after the switch coronary problem
Weak pulses, cool mottled skin after the switch low cardiac output
Low urine output or falling BP after the switch low cardiac output

A kinked or narrowed re-implanted coronary artery can cause myocardial ischemia after the arterial switch.

05

Treatment

Cyanotic newborn with TGA — right now
1
Alprostadil IV infusion as prescribed
→
2
Continuous breathing + SpO₂ monitoring
→
3
Airway and ventilation equipment at bedside
Prostaglandin E1keeps the ductus open
alprostadil

Continuous IV infusion so the two circulations mix

Can cause apnea — monitor breathing continuously
Septostomywhen prostaglandin isn't enough

Balloon atrial septostomy enlarges the atrial opening

Arterial switchthe definitive repair

Arteries moved to the correct ventricles; coronaries re-implanted

Continuous ECG afterward for ischemia
Timing, numbers and later life
Switch in the first 1–2 weeks, before the left ventricle weakens
Alprostadil apnea: about 10–12% of neonates, usually in the first hourmost often under 2 kg birth weight
Switch survival over 95%; most children have no symptoms afterward
Narrowing at the pulmonary artery repair: most common reoperation
06

Nursing priorities

Cyanosis unchanged on 100% oxygen: suspect a heart defect
Keep the alprostadil infusion running continuously
Monitor breathing and SpO₂ continuously; airway equipment ready
Monitor temperature, heart rate and BP on alprostadil
After the switch: continuous ECG; report low cardiac output
07

TGA or tetralogy of Fallot?

TGA presents in the first days; tetralogy is the most common cyanotic defect overall

TGATetralogy
Most common cyanotic defectIn the first days of lifeOverall
CyanosisSevere, within hours of birthSet by how tight the pulmonary stenosis is
MurmurOften absentHarsh systolic ejection murmur, left sternal border
Chest x-rayEgg on a stringBoot-shaped heart
RepairArterial switch, first 1–2 weeksComplete repair, usually in the first year
08

Teach the family

The infusion keeps the ductus open so blood mixes until surgery
The arterial switch is the definitive repair

Arterial switch survival is over 95% and most children have no symptoms afterward; lifelong cardiology follow-up is still needed.

Pearl

A blue newborn who stays blue on 100% oxygen has a heart problem, not a lung problem. In TGA, alprostadil keeps the ductus open — and keep airway equipment at the bedside for apnea.

Sources · Transposition of the Great Arteries (MSD Manual Professional) · Transposition of the great arteries (Orphanet J Rare Dis) · Transposition of the great vessels (MedlinePlus) · Prostin VR Pediatric (alprostadil) label (DailyMed) · Myocardial ischemia after arterial switch operation (Ann Card Anaesth) · Screening for Critical Congenital Heart Defects (CDC) · Prostaglandin Duration and Outcomes in TGA (Congenit Heart Dis) · Tetralogy of Fallot (MedlinePlus) · Tetralogy of Fallot (MSD Manual Professional) · Follow your facility's protocol for prostaglandin E1 infusions.

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