BACKGROUND: Infants with univentricular hearts often require intervention, such as a systemic to pulmonary shunt, as part of a staged surgical palliation. This physiology is inherently unstable, and interim mortality ranges from 4% to 15%. Heterotaxy syndrome confers a high incidence of intestinal rotation and fixation abnormalities. Controversy persists as to the need for elective Ladd's procedure. The purpose of this study is to review our experience in children with heterotaxy syndrome and functionally univentricular hearts who underwent Ladd's procedure. METHODS: A retrospective chart review (2005 to 2010) from our institutional database identified patients with heterotaxy syndrome who underwent a Ladd's procedure. RESULTS: Nine patients (3 boys and 6 girls) underwent Ladd's procedure. Patient demographics were as follows: median gestational age, 38 weeks (range, 37 to 39); median birth weight, 2.7 kg (range, 2 to 3.4 kg); and median age at Ladd's procedure, 180 days (range, 7 to 1,080). Four patients (44%) exhibited feeding intolerance with documented intestinal rotation and fixation abnormalities. Two of these patients underwent Ladd's procedure before cardiac surgery, 1 of whom had subsequent pulmonary artery banding, had recurrent necrotizing enterocolitis, and died. Two children had Ladd's procedure after initial cardiac palliation; both had shunt thrombosis during abdominal surgery, with a mortality of 50%. Five patients who were asymptomatic underwent Ladd's procedure after second-stage palliation. Mortality in this group was 0%. CONCLUSIONS: Ladd's procedure in single ventricle heterotaxy patients who are tolerating enteral feeding should be performed after physiologic palliation to a more balanced circulation, ideally after cavopulmonary connection. Symptomatic neonates pose management challenges. Timing of abdominal surgery should be carefully considered, given the significant risk of mortality in these patients.
BACKGROUND:Infants with univentricular hearts often require intervention, such as a systemic to pulmonary shunt, as part of a staged surgical palliation. This physiology is inherently unstable, and interim mortality ranges from 4% to 15%. Heterotaxy syndrome confers a high incidence of intestinal rotation and fixation abnormalities. Controversy persists as to the need for elective Ladd's procedure. The purpose of this study is to review our experience in children with heterotaxy syndrome and functionally univentricular hearts who underwent Ladd's procedure. METHODS: A retrospective chart review (2005 to 2010) from our institutional database identified patients with heterotaxy syndrome who underwent a Ladd's procedure. RESULTS: Nine patients (3 boys and 6 girls) underwent Ladd's procedure. Patient demographics were as follows: median gestational age, 38 weeks (range, 37 to 39); median birth weight, 2.7 kg (range, 2 to 3.4 kg); and median age at Ladd's procedure, 180 days (range, 7 to 1,080). Four patients (44%) exhibited feeding intolerance with documented intestinal rotation and fixation abnormalities. Two of these patients underwent Ladd's procedure before cardiac surgery, 1 of whom had subsequent pulmonary artery banding, had recurrent necrotizing enterocolitis, and died. Two children had Ladd's procedure after initial cardiac palliation; both had shunt thrombosis during abdominal surgery, with a mortality of 50%. Five patients who were asymptomatic underwent Ladd's procedure after second-stage palliation. Mortality in this group was 0%. CONCLUSIONS: Ladd's procedure in single ventricle heterotaxy patients who are tolerating enteral feeding should be performed after physiologic palliation to a more balanced circulation, ideally after cavopulmonary connection. Symptomatic neonates pose management challenges. Timing of abdominal surgery should be carefully considered, given the significant risk of mortality in these patients.
Authors: Kurt D Piggott; Grace George; Harun Fakioglu; Carlos Blanco; Sukumar Saguna Narasimhulu; Kamal Pourmoghadam; Hamish Munroe; William Decampli Journal: World J Clin Pediatr Date: 2016-08-08
Authors: Thomas G Saba; Gabrielle C Geddes; Stephanie M Ware; David N Schidlow; Pedro J Del Nido; Nathan S Rubalcava; Samir K Gadepalli; Terri Stillwell; Anne Griffiths; Laura M Bennett Murphy; Andrew T Barber; Margaret W Leigh; Necia Sabin; Adam J Shapiro Journal: Orphanet J Rare Dis Date: 2022-09-09 Impact factor: 4.303