Congenital Heart Surgery Outcomes in Turner Syndrome: The Society of Thoracic Surgeons Database Analysis

Abstract

Background: Turner syndrome (TS) is a genetic syndrome characterized by monosomy X (45,XO) in phenotypic females and is commonly associated with congenital heart disease. We sought to describe the distribution, mortality, and morbidity of congenital heart surgery in TS and compare outcomes to individuals without genetic syndromes.

Methods: The Society of Thoracic Surgeons Congenital Heart Surgery Database was used to evaluate index cardiovascular operations performed from 2000 to 2017 in pediatric patients (aged 0-18 years) with and without TS. Analyses were stratified by the most common operations, including coarctation repair, aortic arch repair, partial anomalous pulmonary venous return repair, Norwood, superior cavopulmonary anastomosis (Glenn), and Fontan.

Results: Included were 780 operations in TS and 62,659 operations in controls. The most common TS operations were coarctation repair in 274 (35%), aortic arch repair in 116 (15%), and Norwood in 59 (8%). Compared with controls, TS patients had lower weight-for-age Z-scores across all operations (P < .01 for all); however, operative mortality rates did not differ significantly. The chylothorax rate was higher in TS after coarctation repair (8.8% vs 2.8%, P < .001) and Norwood (22% vs 8.1%, P < .001). The median (interquartile range) postoperative length of stay was longer in TS for coarctation repair (6.5 [5.0-15.5] days vs 5.0 [4.0-9.0] days, P < .001), aortic arch repair (15.0 [8.0-27.5] days vs 11.0 [7.0-21.0] days, P = .004), and Glenn (9.0 [6.0-16.0] days vs 6.0 [5.0-11.0] days, P = .013).

Conclusions: Turner syndrome patients most commonly underwent operations for left-sided obstructive lesions. Despite increased morbidity for select operations, TS was not associated with increased operative mortality.