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Cardiovascular surgery in Turner syndrome - early outcome and long-term follow-up
Margaret M Fuchs1, Christine Helena Attenhofer Jost2, Sameh M Said3
1Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, United States. fuchs.margaret@mayo.edu.
Insights
Cardiovascular surgery in Turner syndrome (TS) patients has an 8% early mortality risk due to complex medical conditions. Survivors experience good long-term survival, but reoperations are common.
Area of Science:
- Cardiology
- Genetics
- Surgical Outcomes
Background:
- Cardiovascular disease is a primary cause of mortality in Turner syndrome (TS).
- TS patients present with complex comorbidities complicating surgical care.
- Cardiovascular surgery is frequently necessary for TS management.
Purpose of the Study:
- To evaluate cardiovascular surgical outcomes in patients with Turner syndrome.
- To analyze mortality and reoperation rates following cardiovascular surgery in TS.
Main Methods:
- Retrospective case series of 51 TS patients undergoing cardiovascular surgery (1977-2017).
- Review of clinical data, echocardiograms, operative reports, and hospital courses.
- Kaplan-Meier survival analysis for long-term mortality and reoperation rates.
Main Results:
- Common procedures included aortic coarctation repair, aortic valve replacement, and aortic root/ascending aorta replacement.
- Early surgical mortality was 8% (4 patients).
- 10- and 20-year freedom from death was 97% and 89%; freedom from reoperation was 93% and 81%.
Conclusions:
- Cardiovascular surgery in TS patients carries an 8% early mortality risk due to medical complexity.
- Patients surviving initial surgery demonstrate favorable long-term survival.
- Late cardiovascular reoperations are frequently required in this population.
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.
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