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Persistent truncus arteriosus associated with interrupted aortic arch: report of two cases
1Department of Pediatrics, National Cheng-Kung University Hospital, Tainan, Taiwan, R.O.C.
Zhonghua Minguo Xiao Er Ke Yi Xue Hui Za Zhi [Journal]. Zhonghua Minguo Xiao Er Ke Yi Xue Hui
|May 1, 1993
Summary
This report details two neonates with a rare combination of truncus arteriosus and interrupted aortic arch (Type A4). These complex congenital heart defects present significant surgical challenges and potential genetic links like Di-George syndrome.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Neonatal Medicine
Background:
- Truncus arteriosus and interruption of the aortic arch are severe congenital heart defects.
- Type A4, according to the Van Praagh classification, represents a specific anatomical subtype.
- These conditions require complex surgical management in neonates.
Observation:
- Two neonates presented with tachypnea, poor appetite, and progressive cyanosis within the first week of life.
- Both infants had a combination of truncus arteriosus and interruption of the aortic arch (Type A4).
- One neonate was diagnosed with Di-George syndrome, exhibiting hypocalcemia and T cell dysfunction.
Findings:
- The co-occurrence of truncus arteriosus and interrupted aortic arch (Type A4) poses a high surgical risk.
- Di-George syndrome was confirmed post-mortem in one patient, highlighting a potential genetic association.
- Clinical presentation included critical symptoms within the neonatal period.
Implications:
- Early diagnosis and surgical intervention are crucial for neonates with Type A4 defects.
- Genetic screening for conditions like Di-George syndrome should be considered in affected infants.
- Understanding these complex defects is vital for improving surgical outcomes and patient management.