Right-to-Left Shunt

Genetic syndromes, e.g. Trisomy 21, 18 and 13, DiGeorge

Maternal risk factors, e.g. diabetes, retinoic acid or trimethadione ingestion

If present later, then increasing cyanosis, murmur and hypercyanotic episodes (crying with deep rapid breathing and increased cyanosis).

Echocardiogram (visualisation of pulmonary stenosis, ventricular septal defect, right ventricular hypertrophy and an overriding aorta).

ECG (may show signs of right ventricular hypertrophy).

Chest X-ray (“boot” shaped heart)

Hyper-oxygenation Testing (no significant increase noted in PaO2)

1. Right ventricular hypertrophy
2. Overriding aorta
3. Right ventricular outflow tract obstruction
4. Ventricular Septal Defect

1. Manoeuvres to increase systemic venous return
2. Supportive measures, e.g. IV fluids, blood products, oxygenation
3. Beta-blocker

Non-remitting severe cyanosis (due to severe pulmonary obstruction)

1. Surgical shunt
2. Emergency Extracorporeal Membrane Oxygenation (ECMO)

Severe cyanosis and limited pulmonary blood flow

1. Prostaglandin E1 (maintain ductus arteriosus patency)

Ongoing

Complete Surgical Repair

• Technique varies and is patient-specific, dependent on their anatomy

Monitoring and potential pulmonary valve replacement

• The patient is monitored for right ventricular dilatation secondary to pulmonary valve regurgitation

Antimicrobial Prophylaxis

• Prevention of infective endocarditis in patients with unrepaired cyanotic CHD and repaired CHD but with residual defects.
• Prior to dental, respiratory tract and skin procedures

Pre-conception counselling, including monitoring during pregnancy.

Complications include progressive right ventricular failure and pulmonary regurgitation, arrhythmias (ventricular and atrial), sudden cardiac death and heart failure.

After surgical repair, the prognosis is individual, based on the patient’s anatomy and the occurrence of any complications. Overall, long-term survival rates are improving.