Retrospective Study
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Cardiol. Mar 26, 2020; 12(3): 97-106
Published online Mar 26, 2020. doi: 10.4330/wjc.v12.i3.97
Cardiovascular surgery in Turner syndrome - early outcome and long-term follow-up
Margaret M Fuchs, Christine Helena Attenhofer Jost, Sameh M Said, Donald J Hagler, Heidi M Connolly, Joseph A Dearani, Alexander C Egbe
Margaret M Fuchs, Christine Helena Attenhofer Jost, Donald J Hagler, Heidi M Connolly, Alexander C Egbe, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, United States
Sameh M Said, Division of Pediatric Cardiothoracic Surgery, Masonic Children’s Hospital, University of Minnesota, Minneapolis, MN 55905, United States
Donald J Hagler, Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN 55905, United States
Joseph A Dearani, Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905, United States
Author contributions: Fuchs MM, Jost CH, Egbe AC designed the research; Fuchs MM, Jost CH performed the research; Fuchs MM, Egbe AC analyzed the data; Fuchs MM, Egbe AC wrote the paper; Jost CH, Said SM, Hagler D, Connolly HM, and Dearani JA reviewed and revised the paper.
Institutional review board statement: The study was reviewed and approved by the Mayo Clinic Institutional Review Board.
Informed consent statement: The Mayo Clinic institutional review board waived informed consent for patients that provided research authorization.
Conflict-of-interest statement: No authors have a conflict of interest.
Data sharing statement: Statistical code and dataset available from the corresponding author at egbe.alexander@mayo.edu.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Margaret M Fuchs, MD, Doctor, Department of Cardiovascular Medicine, Mayo Clinic, 200 First Street SW, Rochester, Rochester, MN 55905, United States. fuchs.margaret@mayo.edu
Received: December 18, 2019
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
Processing time: 89 Days and 13 Hours
ARTICLE HIGHLIGHTS
Research background

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.

Research motivation

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.

Research objectives

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.

Research methods

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.

Research results

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.

Research conclusions

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.

Research perspectives

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.