Department of Medicine - HeartOtago, Dunedin School of Medicine, University of Otago, Dunedin, New Zealand.
Pacific Radiology Group, Christchurch, New Zealand; Department of Radiology, University of Otago, Christchurch, New Zealand.
Heart Lung Circ. 2020 Nov;29(11):1613-1620. doi: 10.1016/j.hlc.2020.04.017. Epub 2020 Jul 3.
Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease with a growing population of adult survivors. Late pulmonary outflow tract and pulmonary valve postoperative complications are frequent, leading to long-term risks such as right heart failure and sudden death secondary to arrhythmias. Cardiac magnetic resonance imaging (CMR) is the gold standard for assessment of cardiac function in patients with repaired ToF. We aimed to determine the most useful CMR predictors of disease progression and the optimal frequency of CMR.
We systematically reviewed PubMed from inception until 29 April 2019 for longitudinal studies assessing the relationship between CMR features and disease progression in repaired ToF. Fourteen (14) studies were identified. Multiple studies showed that impaired right and left ventricular function predict subsequent disease progression. Right ventricular end diastolic volume, while being associated with disease progression when analysed alone, was generally not associated with disease progression on multivariate analysis. Severity of tricuspid regurgitation and pulmonary regurgitation likewise did not show a consistent association with subsequent events. A number of non-CMR factors were also identified as being associated with disease progression, in particular QRS duration and older age at repair. Restrictive right ventricular physiology was not consistently an independent predictor of events.
Impaired right and left ventricular function are the most consistent independent predictors of disease progression in repaired ToF. The optimal timing of repeat cardiac imaging remains controversial. Large scale prospective studies will provide important information to guide clinical decision making in this area.
法洛四联症（ToF）是最常见的发绀型先天性心脏病，其成年幸存者人数不断增加。晚期肺动脉流出道和肺动脉瓣术后并发症频繁发生，导致右心衰竭和心律失常导致的猝死等长期风险。心脏磁共振成像（CMR）是评估修复后 ToF 患者心功能的金标准。我们旨在确定最有用的 CMR 预测疾病进展的指标以及 CMR 的最佳频率。
我们系统地检索了从创建到 2019 年 4 月 29 日期间在 PubMed 上发表的关于评估修复后 ToF 中 CMR 特征与疾病进展之间关系的纵向研究。确定了 14 项研究。多项研究表明，右心室和左心室功能受损可预测随后的疾病进展。右心室舒张末期容积虽然单独分析时与疾病进展相关，但在多变量分析中通常与疾病进展无关。三尖瓣反流和肺动脉瓣反流的严重程度也与随后的事件没有一致的关联。还确定了一些非 CMR 因素与疾病进展有关，特别是 QRS 持续时间和修复时的年龄较大。限制性右心室生理功能并不始终是事件的独立预测指标。
右心室和左心室功能受损是修复后 ToF 疾病进展的最一致的独立预测指标。重复心脏成像的最佳时机仍存在争议。大规模前瞻性研究将为该领域的临床决策提供重要信息。
