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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
Related
truncus arteriosus
General
congenital heart disease; congenital cyanotic heart disease
References
- Langman, Medical Embryology, Williams & Wilkins 1975
- McElhinney DB and Berger S
eMedicine: Truncus Arteriosus
medicine.medscape.com/article/892489-overview
- Wikipedia: Persistent truncus arteriosus
http://en.wikipedia.org/wiki/Persistent_truncus_arteriosus