Diffuse Myocardial Fibrosis in Repaired Tetralogy of Fallot
Linking Pathophysiology and Clinical Outcomes
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The success of surgical management of tetralogy of Fallot (TOF) early in life has dramatically changed the demographics and natural history of this once nearly uniformly lethal congenital heart disease. As early mortality decreased from 50% in the 1950s and 1960s to <2% in the modern era,1,2 the population of repaired TOF (rTOF) survivors steadily grew, and by 2010, the number of adults with rTOF exceeded the number of children with rTOF.3,4 Currently, during the first 2 decades of life, not only is survival excellent, but also morbidity is rare. However, beginning in the third decade of life, rates of morbidity and mortality increase.5 In response to these trends, clinicians and researchers have turned their attention to the pathophysiology and management of adolescent and adult survivors of rTOF in an effort to reduce morbidity and prolong life.
See Article by Yim et al
Despite many refinements in the surgical management of TOF, the majority of patients continue to have residual hemodynamic and electrophysiological abnormalities, resulting in a complex pathophysiological cascade that ultimately leads to ventricular dysfunction, arrhythmias, exercise intolerance, heart failure symptoms, and premature death.6–9 In light of these observations, investigators have focused on measurements of biventricular volumes and function to guide management decisions, such as timing of pulmonary valve replacement.8 However, these parameters provide only limited insight into the pathophysiology of rTOF and ignore the composition and health of the myocardium. Given that patients with rTOF generally compensate well during the first 20 to 30 years of life and then exhibit biventricular dysfunction and heart failure symptoms, there is …