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 …