心脏磁共振成像在弯刀综合征评估和治疗中的应用

Alee N. Pettit BS, BA , Constantine D. Mavroudis MD, MSc , Christopher Penney MSc , Ari Gartenberg MD , Mark A. Fogel MD , Danish Vaiyani MD , Ryan Callahan MD , Kevin K. Whitehead MD, PhD , Matthew A. Harris MD
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引用次数: 0

摘要

背景:弯刀综合征(ScS)的特征是肺静脉与下腔静脉的部分异常连接,可能伴有室间隔缺损、右肺发育不全和主动脉肺侧支。我们假设心脏磁共振成像测量可以阐明ScS生理学和指导手术决策。方法本回顾性队列研究纳入了2011年至2022年在费城儿童医院诊断为ScS的26例患者。排除单心室生理的患者。回顾了患者的病历特征和心脏磁共振成像数据。结果手术组右心室舒张末容积指数(RVEDV) (P = 0.007)和总分流容积指数(P = 0.029)明显高于非手术组。总分流容积对手术的影响中等(0.43),RVEDV对手术的影响较大(0.55)。这些心脏磁共振成像衍生的测量结果表明,手术矫正的ScS患者具有更高的肺总血流量、全身总血流量、显著更高的rvedv和总分流容积。RVEDV对手术的影响最大。这些数据标志着使用心脏磁共振成像衍生测量方法开发ScS手术转诊诊断标准的第一步。需要进一步的研究来确定临床临界值。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Cardiac Magnetic Resonance Imaging in the Evaluation and Management of Scimitar Syndrome

Background

Scimitar syndrome (ScS) features a partial anomalous pulmonary venous connection to the inferior vena cava, with possible septal defects, right lung hypoplasia, and aortopulmonary collaterals. We hypothesize that cardiac magnetic resonance imaging measurements can elucidate ScS physiology and guide surgical decisions.

Methods

This retrospective cohort study included 26 patients diagnosed with ScS from 2011 to 2022 at Children’s Hospital of Philadelphia. Patients with single-ventricle physiology were excluded. Medical records were reviewed for patient characteristics and cardiac magnetic resonance imaging data.

Results

Indexed right ventricular end-diastolic volume (RVEDV) (P = .007) and indexed total shunt volume (P = .029) were significantly greater in the surgical group vs the nonsurgical group. Total shunt volume had a moderate effect (0.43), and RVEDV had a large effect (0.55) on surgery.

Conclusions

These cardiac magnetic resonance imaging-derived measurements demonstrate that patients with surgically corrected ScS had a higher total pulmonary blood flow, total systemic blood flow, and significantly greater RVEDVs and total shunt volumes. RVEDV had the largest effect on surgery. These data mark a primary step in developing diagnostic criteria for ScS surgery referral using cardiac magnetic resonance imaging-derived measurements. Further research is needed to determine clinical cutoffs.
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