Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support

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

Functional Health Status of Adults With Tetralogy of Fallot: Matched Comparison With Healthy Siblings

BACKGROUND: Survival prospects for adults with repaired tetralogy of Fallot (TOF) are now excellent. Attention should therefore shift to assessing and improving functional health status and quality of life. We aimed to assess late functional health status of adults surviving TOF repair by matched comparison to their healthy siblings. METHODS: All 1,693 TOF repairs performed at our institution between 1946 and 1990 were reviewed. A matched comparison was undertaken whereby presumed survivors and their healthy sibling were contacted and asked to complete the Ontario Health Survey 1990 and the 36-Item Short Form Health Survey (SF-36) questionnaire. RESULTS: Both questionnaires were completed by 224 adult survivors and their sibling closest in age. Adults with repaired TOF had lower scores for self-perceived general health status (p < 0.001), were less likely to rate their health as good or excellent (p < 0.001), and had lower SF-36 scores for physical functioning and general health (p = 0.001) than their siblings. However, patients reported similar satisfaction with their lives, similar levels of social participation and support, and were as likely to be in long-term partnerships. Worse physical and mental health scores were associated with older age at surgery and at time of questionnaire completion and recent requirement for noncardiac medication. CONCLUSIONS: Although reporting lower functional health status then their siblings, quality of life and life satisfaction for adults who underwent surgery for TOF during childhood is comparable to that of their siblings without heart defects. Follow-up of younger adults is required to understand current health outcomes attributable to improvements in the management of TOF