Abstract
Surgical management of tetralogy of Fallot (TOF) results in anatomic and functional abnormalities in the majority of patients. Although right ventricular volume load due to severe pulmonary regurgitation can be tolerated for many years, there is now evidence that the compensatory mechanisms of the right ventricular myocardium ultimately fail and that if the volume load is not eliminated or reduced by pulmonary valve replacement the dysfunction might be irreversible. Cardiovascular magnetic resonance (CMR) has evolved during the last 2 decades as the reference standard imaging modality to assess the anatomic and functional sequelae in patients with repaired TOF. This article reviews the pathophysiology of chronic right ventricular volume load after TOF repair and the risks and benefits of pulmonary valve replacement. The CMR techniques used to comprehensively evaluate the patient with repaired TOF are reviewed and the role of CMR in supporting clinical decisions regarding pulmonary valve replacement is discussed.
Highlights
Introduction the management of tetralogy of Fallot (TOF) has evolved considerably since Blalock and Taussig described the first systemic artery-to-pulmonary artery shunt in 1945 and Lillehei and Varco reported the first repair by an open-heart procedure in 1954 [1,2,3], optimal surgical repair has remained elusive
Right ventricular (RV) dilation from pulmonary regurgitation (PR), residual atrial and/or ventricular septal defect, tricuspid regurgitation, right ventricular outflow tract (RVOT) aneurysm, pulmonary artery stenosis, and tachyarrhythmias are some of the abnormalities frequently encountered in patients with repaired TOF
Cardiovascular magnetic resonance (CMR) has emerged as an essential diagnostic tool in this patient population because it overcomes many of the limitations of echocardiography, cardiac CT, and cardiac catheterization, while providing unique quantitative data as well as prognostic information
Summary
The management of tetralogy of Fallot (TOF) has evolved considerably since Blalock and Taussig described the first systemic artery-to-pulmonary artery shunt in 1945 and Lillehei and Varco reported the first repair by an open-heart procedure in 1954 [1,2,3], optimal surgical repair has remained elusive. CMR in Clinical Decision Making CMR supports clinical decision making in patients with repaired TOF by providing comprehensive anatomic and functional information on postoperative cardiovascular abnormalities Important in these patients is quantitative information on RV size, global and regional RV function, LV size and function, myocardial scar, RVOT aneurysm or obstruction, valve regurgitation (especially helpful when quantified), residual intracardiac shunts, and anatomic abnormalities of the pulmonary arteries and aorta. The decision to insert a pulmonary valve relies on clinical assessment of symptoms and signs attributable to the cardiovascular system and on measurements of PR, biventricular size and function, shunt ratio, and several morphologic criteria (e.g., RVOT aneurysm, branch pulmonary artery stenosis, severe aortic dilatation). CMR will be an essential component of any such study
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