Christiane Mhanna, Katerina Kourpas, Takeshi Tsuda
BACKGROUND: Severe right ventricular (RV) dilatation and exercise intolerance are considered indications for pulmonary valve replacement in surgically repaired tetralogy of Fallot (rTOF). METHODS AND RESULTS: , respectively. RV and left ventricular (LV) ejection fractions were 50.3±7.8% and 59.1±6.1%, respectively. A strong positive correlation was noted between RV end-diastolic volume and RVSV (P<0.0001), RVSV and LVSV (P<0.0001), and RVEDVi and peak OP/BSA (P=0.0004). Larger RVEDVi was correlated with better exercise performance, whereas smaller RVEDVi was correlated with worse exercise performance. RVEDVi was larger in male than female rTOF patients despite comparable PRF, RVSVi, LVEDVi, and BSA-indexed LVSV. CONCLUSIONS: A smaller RV was associated with worse exercise performance, indicating exercise intolerance is independent of RV dilatation in young rTOF with PI and represents a unique pathological entity responsible for reduced exercise performance.