Preprocedural Imaging Evaluation of Pulmonary Valve Replacement After Repair of Tetralogy of Fallot: What the...

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.