Abstract
BACKGROUND: Patients with palliated pulmonary valve stenosis (PVS) have less cardiac remodeling and symptoms as compared to patients with repaired tetralogy of Fallot (TOF) presenting with similar severity of right ventricular outflow tract (RVOT) disease. What is not known is whether patients with PVS versus TOF presenting with similar severity of RVOT disease at baseline, would have similar (or different) pace of cardiac remodeling and disease progression over time. The study objective was to compare temporal changes in clinical and cardiac function indices between adults with palliated PVS and repaired TOF presenting with moderate/severe RVOT disease. METHODS: Cardiac function indices (based on strain imaging) and clinical indices (N-terminal pro-B-type natriuretic peptide [NT-proBNP], model for end-stage liver disease excluding international normalized ratio [MELD-XI], peak oxygen consumption [VO(2)]), were assessed at baseline, 3 years, and 5 years. Temporal changes were calculated as relative changes from baseline (Δ). Cardiovascular adverse event was assessed as time-to-event outcome. RESULTS: Compared to TOF group (n = 173), the PVS group (n = 173) had less temporal change in right atrial reservoir strain (-9±4% versus -21 ± 6%, p < 0.001), RV free wall strain (-8±4% versus -20 ± 5%, p < 0.001), NT-proBNP (8 ± 5% versus 17 ± 6 %, p < 0.001), MELD-XI (6 ± 4% versus 19 ± 4%, p = 0.008), and peak VO(2) (-7±3% versus -12 ± 7%, p < 0.001) at 5 years. The 5-year freedom from cardiovascular adverse event was higher in the PVS group (76% versus 54%, p = 0.01). CONCLUSIONS: These data suggest that a less frequent clinical and imaging follow-up may be appropriate in patients with PVS (as compared to patients with TOF).