Department of Radiology, University of Southern California, Los Angeles, CA.
Departments of Radiology and Medical Imaging.
J Thorac Imaging. 2020 May;35(3):153-166. doi: 10.1097/RTI.0000000000000478.
Tetrallogy of Fallot (TOF) is the most frequent form of cyanotic congenital heart disease. Despite advances in surgical and medical treatment, mortality remains high. Residual dysfunction of the pulmonary valve (PV) after correction of right ventricular outflow tract obstruction is an important cause of morbidity, leading to irreversible right ventricular dysfunction, arrhythmias, heart failure and occasionally, death. The strategies for PVR have evolved over the last decades, and the timing of the intervention remains the foundation of the decision-making process. Symptoms of heart failure are unreliable indicators for optimal timing of repair. Imaging plays an essential role in the assessment of PV integrity and dysfunction. The identification of the best timing for PVR requires a multimodality approach. Transthoracic echocardiography is the most commonly used imaging modality for the initial assessment and follow-up of TOF patients, although its utility has technical limitations, especially in adults. Cardiac computed tomography and magnetic resonance imaging are now routinely used for preoperative and postoperative evaluation of these patients, and provide highly valuable information about the anatomy and pathophysiology. Imaging evidence of disease progression is now part of the major guidelines to define the best timing for reintervention. The purpose of this article is to review the pathophysiology after TOF repair, identify the main imaging anatomic and physiologic features, describe the indications for PVR and recognize the role of imaging in the assessment of these patients to define the appropriate timing of PVR.
法洛四联症(TOF)是最常见的发绀性先天性心脏病。尽管在外科和内科治疗方面取得了进展,但死亡率仍然很高。右心室流出道梗阻矫正后肺动脉瓣(PV)残余功能障碍是发病率的重要原因,可导致不可逆的右心室功能障碍、心律失常、心力衰竭,偶尔还会导致死亡。过去几十年来,PVR 策略不断发展,干预时机仍然是决策过程的基础。心力衰竭的症状并不可靠,不能作为修复的最佳时机的指标。影像学在评估 PV 完整性和功能障碍方面发挥着重要作用。确定 PVR 的最佳时机需要采用多模态方法。经胸超声心动图是 TOF 患者初始评估和随访的最常用影像学方法,尽管其具有技术局限性,尤其是在成人中。心脏计算机断层扫描和磁共振成像现在常用于这些患者的术前和术后评估,可提供有关解剖结构和病理生理学的非常有价值的信息。疾病进展的影像学证据现已成为主要指南的一部分,用于定义再次干预的最佳时机。本文旨在回顾 TOF 修复后的病理生理学,确定主要的影像学解剖和生理特征,描述 PVR 的适应证,并认识到影像学在评估这些患者中的作用,以确定 PVR 的适当时机。