Fuchs Margaret M, Attenhofer Jost Christine Helena, Said Sameh M, Hagler Donald J, Connolly Heidi M, Dearani Joseph A, Egbe Alexander C
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, United States.
Division of Pediatric Cardiothoracic Surgery, Masonic Children's Hospital, University of Minnesota, Minneapolis, MN 55905, United States.
World J Cardiol. 2020 Mar 26;12(3):97-106. doi: 10.4330/wjc.v12.i3.97.
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.
To describe the cardiovascular surgical outcomes of patients with TS.
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.
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.
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.
心血管疾病是特纳综合征（TS）患者的主要死因，这些患者常需进行心血管手术治疗。TS常伴有多种合并症，这可能使该患者群体的护理变得复杂。
描述TS患者的心血管手术结局。
对1977年至2017年在罗切斯特梅奥诊所接受至少一次心血管手术的51例连续TS患者进行回顾性病例系列研究。回顾了这些患者的基线临床数据，包括人口统计学、合并症、先天性心脏病史和用药情况。详细分析了超声心动图报告。查阅了手术报告和手术住院过程。使用病历和社会保障死亡指数确定长期死亡率。采用Kaplan-Meier方法进行生存分析。
该队列包括51例TS患者，在梅奥诊所手术时的平均年龄为28（8 - 41）岁，23例（45%）患者年龄在18岁以下。在梅奥诊所首次手术时，18例（35%）患者此前曾在其他机构接受过心脏手术。最常见的手术是14例（28%）患者进行主动脉缩窄修复，6例（12%）患者进行主动脉瓣置换，7例（14%）患者进行主动脉根部/升主动脉复合置换，7例患者接受了不止一处病变的修复。5例患者因主动脉夹层需要手术干预。在梅奥诊所首次手术后，6例（13%）患者需要进行后续手术。平均住院时间为6±2天。有4例（8%）早期手术死亡。10年和20年时的无死亡生存率分别为97%和89%，10年和20年时的无再次手术生存率分别为93%和81%。
鉴于TS患者的病情复杂性，心血管手术的早期死亡率为8%。存活出院的患者生存率良好。后期心血管再次手术并不罕见。
