persistent truncus arteriosus

Etiology

• spontaneous
• 50% of cases associated with DiGeorge syndrome
• tetratogens

Pathology

• truncus arteriosus fails to divide into the pulmonary artery & aorta
• the pulmonary artery originates from a common trunk it shares with the aorta

Clinical manifestations

• congenital cyanosis
• heart failure may occur within weeks
• systolic ejection murmur at the left sternal border
• widened pulse pressure
• bounding arterial pulses
• loud second heart sound (S2)
• biventricular hypertrophy
• cardiomegaly
• increased pulmonary vascularity
• hypocalcemia (if associated with DiGeorge syndrome)

Management

• neonatal surgical repair
  • ventricular septal defect is closed with a patch
  • pulmonary arteries are detached from the truncus arteriosus & connected to the right ventricle using a tube

More general terms

• congenital heart disease; congenital cyanotic heart disease

Additional terms

• truncus arteriosus