O0346 Oct.3: 4:30pm - 20 year Surgical Outcomes for Turner Syndrome Patients: Data from the Pediatric Cardiac Care Consortium

Background: Turner syndrome (TS) is a sex chromosome anomaly (monosomy-X) associated with congenital heart disease (CHD) with need for surgical intervention in childhood. A previous report from the Pediatric Cardiac Care Consortium (PCCC), a multi-institutional US-based registry of interventions for pediatric heart diseases, identified differential risk for perioperative mortality only for selected CHD among patients with congenital heart surgery (CHS). TS patients are at increased risk for later cardiovascular conditions including aortic aneurysms, hypertension and ischemic heart disease. The long-term survival outcomes in TS children with history of CHS has not been adequately studied. Here, we describe the long-term survival of TS patients undergoing CHS at ≤21 years of age with a focus on left heart obstructive lesions (LHOLs), the most common CHD among TS patients.

Methods: This is a retrospective cohort study from the PCCC. We queried the PCCC registry for patients with TS undergoing their first CHS in the PCCC between 1982 and 2003. We abstracted CHD diagnoses, year and age at first surgery, type of surgical intervention and major extracardiac anomalies. Outcomes were obtained from the PCCC records and from prospectively collected linkage data with the US National Death Index (NDI) through 2014. Kaplan Meier survival plots were created from the date of index procedure (excluding in-hospital deaths) and compared with the euploid population of children operated for comparable lesions without genetic syndromes during the same era in the PCCC.

Results: We identified 173 children with TS that survived CHS at ≤21 years of age (Table). Among patients with TS, 153 (88%) children had left heart obstructive lesions (LHOL): isolated mitral (n=1) or aortic (n=9) stenosis (6%), coarctation or interrupted arch (n=137; 79%) in isolation or with associated lesions, hypoplastic left heart syndrome (HLHS) (n=6; 3%). Partial anomalous pulmonary venous return was associated with LHOL in 10 patients (5%). Over 20 years of follow up, 9 deaths (6.2%) occurred among TS patients with operated two-ventricle LHOL. The survival at 20 years showed no significant difference for two-ventricle LHOL between TS and their female, male and overall grouped euploid cohorts (Figure). Various other CHDs were encountered among the remaining 12% of the cohort, but their numbers were too small for meaningful analysis.

Conclusions: This study is one of the largest long-term survival analyses in the TS pediatric population. The results suggest that TS children surviving congenital heart surgery for a LHOL have similar long-term survival as euploid patients with similar CHD. Ongoing monitoring of TS patients remains important to assess the interaction between the TS-associated pre-disposition for adult type cardiovascular conditions when superimposed on their background of repaired CHD.