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法洛四联症矫正患者的临床结局和疾病进展的长期评估。

Long-term assessment of clinical outcomes and disease progression in patients with corrected Tetralogy of Fallot.

机构信息

Institut of Clinical Physiology IFC-CNR, Massa-Pisa, Italy.

出版信息

Eur Rev Med Pharmacol Sci. 2021 Oct;25(20):6300-6310. doi: 10.26355/eurrev_202110_27000.

DOI:10.26355/eurrev_202110_27000
PMID:34730210
Abstract

OBJECTIVE

Understanding changes of right ventricular (RV) geometry and function in repaired Tetralogy of Fallot (rToF) patients can improve decision-making for pulmonary valve replacement. Therefore, we aimed to assess the magnitude and clinical correlations of RV changes in rToF patients.

PATIENTS AND METHODS

Clinical and MRI data of rToF patients who underwent repeated cardiac magnetic resonance imaging (MRI) at two centers between December 2003 and September 2020 were analyzed together with anatomical factors, including RV outflow tract obstruction, pulmonary artery branch stenosis, and tricuspid regurgitation. Adverse cardiac events and/or NYHA class worsening were documented and correlated with MRI changes. QRS length was reported at each MRI.

RESULTS

Two-hundred-and-nineteen rToF patients (53% males, aged 20.2 ± 10.1 years) were enrolled. An increase of ventricular dimensions, except LVEDVi, and worsening of right and left ejection fractions were found over an average period of 5 years of follow-up. These changes were statistically significant but within 10% of the initial value. No significant changes were reported on a year-to-year basis, except in a small group of patients (6%) in whom no predictive factors were identified. Despite similar RV dimensions at the first examination, younger patients had a higher RV ejection fraction and a different annual rate of change of ventricular dimensions compared to older ones. Patients with arrhythmias (20%) were more frequently older and had larger RV dimensions but showed no significant correlations with MRI changes/years.

CONCLUSIONS

Changes in RV dimensions and function occur rarely and very slowly in rToF patients. A small percentage of patients experience a significant worsening in a short time interval without any recognized risk factors. Arrhythmias appear to occur in a small percentage of cases in the late follow-up.

摘要

目的

了解修复性法洛四联症(rToF)患者右心室(RV)几何形状和功能的变化,可以改善肺动脉瓣置换的决策。因此,我们旨在评估 rToF 患者 RV 变化的幅度和临床相关性。

患者和方法

分析了 2003 年 12 月至 2020 年 9 月期间在两个中心接受重复心脏磁共振成像(MRI)的 rToF 患者的临床和 MRI 数据,包括 RV 流出道梗阻、肺动脉分支狭窄和三尖瓣反流等解剖因素。记录不良心脏事件和/或 NYHA 分级恶化,并与 MRI 变化相关联。每次 MRI 时都报告 QRS 长度。

结果

共纳入 219 例 rToF 患者(53%为男性,年龄 20.2±10.1 岁)。在平均 5 年的随访期间,发现心室尺寸(LVEDVi 除外)增加,右心室和左心室射血分数恶化。这些变化具有统计学意义,但在初始值的 10%以内。除了一小部分(6%)患者(未识别出任何预测因素)外,每年均未报告明显变化。尽管首次检查时 RV 尺寸相似,但较年轻的患者具有更高的 RV 射血分数,且心室尺寸的年变化率也不同。心律失常(20%)患者年龄较大,RV 尺寸较大,但与 MRI 变化/年无明显相关性。

结论

rToF 患者的 RV 尺寸和功能变化很少且非常缓慢。一小部分患者在短时间内出现明显恶化,且无明显的危险因素。心律失常在随访后期发生的比例较小。

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