Summary

• Ventricular septal defect with over-riding aorta and right ventricular (RV) outflow tract obstruction and resulting RV hypertrophy. The common embryological cause for this constellation of findings is anterior and cephalad deviation of the muscular outlet of the ventricular septum.
• Usually presents in the neonatal period with a murmur, cyanosis, or both.
• Diagnosed by echocardiography.
• Treatment is by surgical repair. This usually consists of complete intracardiac repair typically during the neonatal or infant period. Occasionally, an aortopulmonary shunt is used palliatively before complete repair.
• The most common long-term complications of complete repair are progressive pulmonary regurgitation and RV failure, atrial arrhythmias, and ventricular arrhythmias.