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
Abstract
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.

Keywords: Turner syndrome; Cardiac surgery; Congenital heart disease; Aortic dissection; Outcome; Reoperation

Core tip: Patients with Turner syndrome have relatively high early mortality after cardiovascular surgery, likely related to their high burden of medical comorbidities including congenital heart disease and vascular disease. Those patients that survive to hospital dismissal have good long-term survival. Many patients require multiple cardiovascular surgeries over their lifetime.