Ladd's procedure in functional single ventricle and heterotaxy syndrome: does timing affect outcome?

Mahesh S Sharma, Kristine J Guleserian, Joseph M Forbess
Annals of Thoracic Surgery 2013, 95 (4): 1403-7; discussion 1407-8

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.

Full Text Links

Find Full Text Links for this Article


You are not logged in. Sign Up or Log In to join the discussion.

Related Papers

Remove bar
Read by QxMD icon Read

Save your favorite articles in one place with a free QxMD account.


Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"