1830
The most common cyanotic congenital heart disease.
Consists of ventricular septal defect, right ventricular outflow tract obstruction, aortic override, and right ventricular hypertrophy.
In about 50% of cases the dilated aorta overides the septum.
Aortic dilation may be significan so that aortic regurgitation is present.
Pulmonary valve stenosis may be present as a result of a biscuspid pulmonary valve.
25% of cases associated with a right sided aortic arch.
An anomalous left anterior anterior descending coronary artery from the right cusp may be present, and during correction of the right ventricular outflow obstruction can be injured and cause an anterior myocardial infarction.
Early primary repair is recommended.
Hemodynamically significant pulmonary valve regurgitation is well tolerated for many years but has right sided heart camber complications.
Chronic hypoxia may cause cerebral complications due to the right-to-left shunt, myocardial dysfunction and increased incidence of ventricle and atrial arrhythmias.
Operative mortality for intracardiac repair is less than 1%.
Operative mortality is age dependent and is nearly 0% for patients 12 months or younger and 4.4% for patients older than 12 months (Van Arsdell GS et al).
For patients greater than 40 years or older repair was associated with a 30 day mortality of 6% (Attenhoffer CH et al).