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大动脉转位心房改道术患者的32年随访

32 year follow up of patients following atrial redirection surgery for transposition of the great arteries.

作者信息

Morrison M Louise, Grant Brian, McCrossan Brian A, Sands Andrew J, Owens Colum G, Spence Mark S, Casey Frank A, Craig Brian G, Lockhart Christopher J

机构信息

Department of Paediatric Cardiology, The Royal Belfast Hospital for Sick Children, Belfast Health & Social Care Trust, Belfast, UK.

Department of Adult Congenital Heart Disease, The Royal Victoria Hospital, Belfast Health & Social Care Trust, Belfast, UK.

出版信息

Congenit Heart Dis. 2019 Sep;14(5):846-853. doi: 10.1111/chd.12822. Epub 2019 Sep 6.

Abstract

OBJECTIVE

A significant body of patients who have undergone Mustard or Senning procedure require lifelong follow up. In this retrospective review, we examined the cohort of such patients currently attending our center.

DESIGN

Patients who had undergone either Mustard or Senning procedure were identified. We retrospectively reviewed medical records, recorded demographic information and data regarding the clinical state, NHYA class, cardiopulmonary exercise testing, NT-proBNP measurement, and recent cardiac MRI findings.

RESULTS

Forty-six patients were identified, the mean age was 32.2 years (± 6.1 years), 67.4% were male. Thirty-two patients (69.6%) had undergone a Senning procedure. The median length of the follow-up was 32 years. Thirty-two patients (69.6%) were NHYA class 1. The mean VO achieved was 24.2 ± 5.8 mL/min/kg. The mean NT-proBNP was 266.4 pg/mL (± 259.9 pg/mL). The mean right ventricular end-diastolic volume (RVEDV) was 212.4 mL ± 73.1 mL (indexed 114.2 mL/m  ± 34.4 mL/m ). The mean right ventricular ejection fraction (RVEF) was 53.7% ± 7.9%. The mean left ventricular end-diastolic volume (LVEDV) was 161.5 mL ± 73.7 mL (indexed 87.8 mL/m  ± 41.1 mL/m ). The mean left ventricular ejection fraction (LVEF) was 59.8% ± 5.7%. There was a significant correlation between right ventricular (RV) size on MRI and NT-proBNP level.

CONCLUSIONS

We present a relatively well cohort of patients with overall favorable long-term outcome. The majority of patients are NHYA class 1 and the systemic right ventricular function appears to be well preserved as assessed by MRI. The exercise tolerance is reduced, with the majority of patients achieving around 60% of the estimated VO . Regular specialist follow-up and assessment with advanced imaging at regular intervals remain important for this group.

摘要

目的

大量接受Mustard或Senning手术的患者需要终身随访。在这项回顾性研究中,我们对目前在我们中心就诊的此类患者队列进行了检查。

设计

确定接受过Mustard或Senning手术的患者。我们回顾性地查阅了病历,记录了人口统计学信息以及有关临床状态、纽约心脏协会(NYHA)分级、心肺运动试验、NT-脑钠肽(NT-proBNP)测量值和近期心脏磁共振成像(MRI)结果的数据。

结果

共确定了46例患者,平均年龄为32.2岁(±6.1岁),67.4%为男性。32例患者(69.6%)接受了Senning手术。随访的中位时间为32年。32例患者(69.6%)为NYHA 1级。平均达到的最大摄氧量(VO)为24.2±5.8毫升/分钟/千克。平均NT-proBNP为266.4皮克/毫升(±259.9皮克/毫升)。平均右心室舒张末期容积(RVEDV)为212.4毫升±73.1毫升(指数化后为114.2毫升/平方米±34.4毫升/平方米)。平均右心室射血分数(RVEF)为53.7%±7.9%。平均左心室舒张末期容积(LVEDV)为161.5毫升±73.7毫升(指数化后为87.8毫升/平方米±41.1毫升/平方米)。平均左心室射血分数(LVEF)为59.8%±5.7%。MRI上右心室(RV)大小与NT-proBNP水平之间存在显著相关性。

结论

我们展示了一组长期总体预后相对良好的患者。大多数患者为NYHA 1级,并且通过MRI评估,系统性右心室功能似乎保存良好。运动耐量降低,大多数患者达到预计VO的约60%。对于该组患者,定期专科随访以及定期进行高级成像评估仍然很重要。

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