Published online Mar 26, 2020. doi: 10.4330/wjc.v12.i3.97
Peer-review started: December 18, 2019
First decision: January 6, 2020
Revised: January 8, 2020
Accepted: January 28, 2020
Article in press: January 28, 2020
Published online: March 26, 2020
Turner syndrome (TS) is a sex chromosome disorder involving partial or complete loss of the X chromosome. Multiorgan involvement has been observed, with cardiovascular disease recognized as a principle cause of mortality. Congenital and acquired cardiovascular disease, including aortic aneurysms, aortic dissection, and coronary artery disease, occur with increased frequency in this population, and cardiovascular surgery is not uncommonly required. The surgical outcomes of population have not been well characterized.
The topic of the study is the outcomes of cardiovascular surgery in TS. Due to the relatively small size of this patient population, outcomes of cardiovascular surgery have not been well described. As patients with TS live longer, and their cardiovascular disease is better recognized, understanding how to optimally manage these patients in the perioperative period is important to ensure the best possible surgical outcomes.
The objectives of this study were to describe the types of cardiovascular surgery these patients require and to characterize their operative morbidity and mortality. By identifying surgical mortality rate and long-term survival, we have highlighted the importance of ongoing focus on this cohort of patients. Further research is needed to improve early surgical outcomes and minimize reoperation.
A retrospective case series was assembled of patients undergoing cardiovascular surgery at Mayo Clinic from 1977-2017. Data pertaining to the patients’ baseline demographics, congenital heart disease history, medical comorbidities, and baseline echocardiographic imaging was compiled. Their operative course and medical follow up were then reviewed to establish survival rates using the Kaplan Meier method.
Fifty-one patients had at least one cardiovascular surgery at Mayo Clinic during the study period. Almost half of these patients were under the age of 18, and a third had already undergone a cardiovascular surgery at a prior institution. Aortic surgeries were most commonly performed, including aortic coarctation repair and composite aortic root/ascending aortic replacement. Aortic valve replacement was also common. After the initial Mayo Clinic surgery, 6 patients required a subsequent cardiovascular surgery. The early post-operative mortality rate was 8%. Freedom from death was 97% at 10 years and 89% at 20 years. After 20 years, 81% of patients remained free of reoperation.
We demonstrate that TS patients have a relatively high early post-operative mortality rate, and propose that this is related to the greater level of medical complexity present in this patient population. In patients that survive to hospital dismissal, however, long-term survival is good despite the prevalence of medical comorbidities. Many patients require reoperation in their lifetime. These findings suggest that more research is needed into the perioperative management of TS patients, so that their early mortality and reoperation rates are minimized.
Future research should be directed at perioperative management of TS patients, as well as pre-anesthesia medical examination to identify any confounding factors that may raise an individual patient’s operative risk.