Ladd's procedure in functional single ventricle and heterotaxy syndrome

Does timing affect outcome?

Mahesh S. Sharma*, Kristine J. Guleserian, Joseph M. Forbess

*Corresponding author for this work

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1403-1408
Number of pages6
JournalAnnals of Thoracic Surgery
Volume95
Issue number4
DOIs
StatePublished - Apr 1 2013

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Heterotaxy Syndrome
Mortality
Necrotizing Enterocolitis
Enteral Nutrition
Birth Weight
Pulmonary Artery
Thoracic Surgery
Gestational Age
Thrombosis

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery
  • Pulmonary and Respiratory Medicine

Cite this

@article{c016000b1d7d46b7b8112ebbaf9b5520,
title = "Ladd's procedure in functional single ventricle and heterotaxy syndrome: Does timing affect outcome?",
abstract = "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.",
author = "Sharma, {Mahesh S.} and Guleserian, {Kristine J.} and Forbess, {Joseph M.}",
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Ladd's procedure in functional single ventricle and heterotaxy syndrome : Does timing affect outcome? / Sharma, Mahesh S.; Guleserian, Kristine J.; Forbess, Joseph M.

In: Annals of Thoracic Surgery, Vol. 95, No. 4, 01.04.2013, p. 1403-1408.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Ladd's procedure in functional single ventricle and heterotaxy syndrome

T2 - Does timing affect outcome?

AU - Sharma, Mahesh S.

AU - Guleserian, Kristine J.

AU - Forbess, Joseph M.

PY - 2013/4/1

Y1 - 2013/4/1

N2 - 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.

AB - 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.

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EP - 1408

JO - Annals of Thoracic Surgery

JF - Annals of Thoracic Surgery

SN - 0003-4975

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