Description of 8 Symptomatic Infants With HS

Patient No.Heterotaxy TypePrimary Cardiac DiagnosisSymptoms Suggesting IRAAge at Symptoms, dUGI ResultsLadd Procedure PerformedAge at Ladd Procedure, dFindings at Ladd Procedure
A4RIUAVSD, DORV, PS, TAPVRNot tolerating feeds121PositiveNo
C1RIDextrocardia, UAVSD, DORV, PS, TAPVRVomiting61PositiveNo
D1LIUAVSD, hypoplastic arch, interrupted IVCVomiting171PositiveNo
D2LIDextrocardia, UAVSD, PA, interrupted IVCHematochezia38NegativeNo
E2RIUAVSD, PS, TAPVRAbdominal distention and tenderness5NonrotationYes5Nonrotation with wide mesentery and no volvulus; chylous ascites
E7RIAVSD, DORV, aorta anterior and to the right of pulmonary artery, PS, TAPVRNot tolerating feeds46NonrotationYes with PDC95Incomplete rotation without volvulus, inflammation, multiple jejunal perforations
E8RIDextrocardia, L-looped ventricles, UAVSD, DORV, PSNot tolerating feeds46NonrotationNo
S3LIDORV, LAVV atresia, interrupted IVCBilious vomiting23NonrotationYes64Nonrotation with Ladd’s bands adhering duodenum to cecum and right colon
  • AVSD, atrioventricular septal defect; DORV, double outlet right ventricle; IVC, inferior vena cava; LAVV, left atrioventricular valve; PA, pulmonary atresia; PDC, peritoneal dialysis catheter; PS, pulmonary stenosis; TAPVR, total anomalous pulmonary venous return; UAVSD, unbalanced atrioventricular septal defect; —, not applicable.