pediatrics
Persistent Truncus Arteriosus
Key Points
In persistent truncus arteriosus, the primitive truncus does not divide into the pulmonary artery and aorta, resulting in a single large arterial trunk that overlies a large ventricular septal defect (VSD). Different types are distinguished based on the origin of the pulmonary arteries and associated defects. Patients present with mild cyanosis, significant pulmonary overcirculation, and heart failure; a grade 2 to 4/6 systolic murmur is audible along the left sternal border and a mid-diastolic mitral flow murmur may be audible at the apex, and bounding peripheral pulses. Treat heart failure with diuretics,digoxin, and ACE inhibitors; prostaglandin infusion is beneficial to maintain duct patency only in patients with type A4 truncus with interrupted aortic arch or coarctation. Do surgical repair early; one or more revisions are usually needed as children grow. 