Heterotaxy syndrome infants are at risk for early shunt failure after Ladd procedure

Abstract

Background: Cardiac-specific risks and complications after a Ladd procedure in patients with heterotaxy syndrome (HS) and intestinal rotational anomalies (IRA) are unknown. We sought to (1) describe rates of hospital mortality and early systemic-to-pulmonary (S-P) artery shunt failure after the Ladd procedure in patients with HS and (2) explore risk factors associated with early shunt failure in patients with HS with single ventricle (SV).

Methods: This retrospective study included all Ladd procedures performed from January 1999 to December 2012 in patients with HS at a single center. Risk factors investigated for early S-P artery shunt failure included birth weight, gestational age, sex, age at and timing of Ladd procedure relative to cardiac operations, and shunt type.

Results: Ladd procedure was performed on 54 infants with HS and congenital heart disease. Hospital mortality for the entire cohort was 5.6% (3 of 54 patients). Early shunt failure occurred in 19% (4 of 21) of HS infants with SV. Mean preoperative blood urea nitrogen (BUN) levels were higher in HS infants with early shunt failure (20 versus 12.5 mg/dL; p = 0.054).

Conclusions: Patients with SV and HS with S-P artery shunts are at risk for early shunt failure after a Ladd procedure. A higher mean preoperative BUN level is noted in patients with HS and early shunt failure. Careful risk-benefit analysis is indicated before recommending routine elective Ladd procedures in patients with HS.