Cardiovascular surgery in Turner syndrome - early outcome and long-term follow-up

BACKGROUND Cardiovascular disease is the leading cause of death in patients with Turner syndrome (TS), and cardiovascular surgery is frequently required for management of these patients. TS is associated with medical comorbidities than can complicate the care of this patient population. AIM To describe the cardiovascular surgical outcomes of patients with TS. METHODS A retrospective case series was compiled of 51 consecutive TS patients who had at least one cardiovascular surgery at Mayo Clinic Rochester from 1977-2017. The baseline clinical data of these patients were reviewed including demographics, medical comorbidities, congenital heart disease history, and medications. Echocardiographic reports were analyzed in detail. Operative reports and surgical hospital courses were reviewed. Long-term mortality was determined using medical records and the Social Security Death Index. Survival analysis was performed with the Kaplan Meier method. RESULTS The cohort comprised 51 TS patients, average age at the time of surgery at Mayo Clinic was 28 (8-41) years, and 23 (45%) patients were under the age of 18. At the time of first Mayo Clinic surgery, 18 (35%) patients had previously undergone cardiac surgery at another institution. The most common procedures were repair of aortic coarctation in 14 (28%) patients, aortic valve replacement in 6 (12%) patients, and composite aortic root/ascending aorta replacement in 7 (14%) patients, with 7 patients undergoing repair of more than one lesion. Aortic dissection required operative intervention in 5 patients. After initial Mayo Clinic surgery, subsequent operations were required in 6 (13%) patients. Average hospital length of stay was 6 ± 2 d. There were 4 (8%) early surgical deaths. Freedom from death was 97% and 89% at 10 and 20 years, and the freedom from reoperation was 93% and 81% at 10 and 20 years. CONCLUSION Cardiovascular surgery is associated with 8% early mortality given the medical complexity of TS patients. Those who survive to dismissal have good survival. Later cardiovascular reoperations are not rare.