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The success of cardiac surgery over the past six decades has led to a paradigm shift in the management of patients with repaired tetralogy of Fallot (rTOF) towards addressing late sequelae in adulthood. Although surgical techniques have evolved, late morbidity and mortality have not been abolished in this patient cohort. Significant knowledge gaps remain in mediating adverse outcomes of arrhythmias, heart failure and sudden cardiac death.
Surgical repair of TOF almost inevitably results in pulmonary regurgitation (PR) during the relief of RV outflow tract obstruction. While pulmonary valve replacement (PVR) represents the current standard of care for treating the haemodynamic consequences of pulmonary dysfunction, its survival benefit and impact on long-term morbidity remains largely unproven. Contemporary guidelines advocate PVR in the presence of symptoms, significant or progressive RV dilatation or dysfunction.1 However, decision-making favouring intervention is less straightforward in the asymptomatic cohort that forms the majority of patients with rTOF with significant PR. Irreversible ventricular impairment may ensue when intervening too late with chronic volume loading, while operating prematurely may increase the lifetime cumulative number of procedures and confer surgical risk, which may increase with successive sternotomies. Ascertaining the optimal timing of PVR therefore remains elusive.
Outcomes in patients with rTOF following PVR are …
Contributors ELH: Drafting and revision of manuscript. All authors contributed to critical and intellectual revision of the manuscript. All authors have given approval of manuscript for publication.
Competing interests None declared.
Provenance and peer review Commissioned; internally peer reviewed.