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Background and Objectives: Chronic pulmonic regurgitation (PR) following repair of congenital heart disease (CHD) impairs right ventricular function that impacts peak exercise cardiac index (pCI). We aimed to estimate in a non-invasive way pCI and peak oxygen consumption (pVO2) and to evaluate predictors of low pCI in patients with significant residual pulmonic regurgitation after CHD repair.Method: We included 82 patients (median age 19 years (range 10-54 years)) with residual pulmonic regurgitation fraction >40%. All underwent cardiac MRI and cardiopulmonary testing with measurement of pCI by thoracic impedancemetry. Low pCI was defined <7 L/min/m2.Results: Low pCI was found in 18/82 patients. Peak indexed stroke volume (pSVi) tended to compensate chronotropic insufficiency only in patients with normal pCI (r=-0.31, p=0.01). Below 20 years of age, only 5/45 patients had low pCI but near-normal (≥6.5 L/min/m2). pVO2 (mL/kg/min) was correlated with pCI (r=0.58, p=0.0002) only in patients aged >20 years. Left ventricular stroke volume in MRI correlated with pSVi only in the group of patients with low pCI (r=0.54, p=0.02). No MRI measurements predicted low pCI. In multivariable analysis, only age predicted a low pCI (OR=1.082, 95% CI 1.035 to 1.131, p=0.001) with continuous increase of risk with age.Conclusions: In patients with severe PR, pVO2 is a partial reflection of pCI. Risk of low pCI increases with age. No resting MRI measurement predicts low haemodynamic response to exercise. Probably more suitable to detect ventricular dysfunction, pCI measurement could be an additional parameter to take into account when considering pulmonic valve replacement. [ABSTRACT FROM AUTHOR]

Objective: Pulmonary valve replacement (PVR) is often required in patients with congenital heart disease. We aimed to describe temporal trends in PVR in a nationwide English cohort between 1997 and 2014, survival and the need for re-PVR.Methods: Patients were identified in the Hospital Episode Statistics Database. Survival data were retrieved from the UK Office for National Statistics.Results: A total of 2733 patients underwent PVR (2845 procedures) over the study period. Median age at first procedure increased from 20.1 years in 1997-2005 to 24.7 years in 2006-2014. The annual number of PVRs increased from 23 in 1997 to 251 in 2014. Homografts were the most common choice in the early years, but the use of xenografts increased after 2005. During a median follow-up of 5.8 years, 176 patients died and 108 required redo PVR. Early (30 day) survival was 98% for all PVRs and was similar for all types of prostheses but longer-term mortality dropped to 92% at 10 years and 90% at 15 years. Age >16 years and percutaneous PVR were risk factors for death. The cumulative incidence for re-PVR at 10 years was 8% for all PVRs and 11% at 15 years. Risk factors for re-PVR were complex diagnosis, male gender and black ethnicity.Conclusion: There was a significant increase in the number of PVRs performed in England over the last two decades and a significant change in the type of prosthesis employed. While early mortality was low across the board, longer-term mortality was not negligible in this young population. [ABSTRACT FROM AUTHOR]

Objectives: Treatment of infants with tetralogy of Fallot (ToF) has evolved in the last two decades with increasing use of primary surgical repair (PrR) and transcatheter right ventricular outflow tract palliation (RVOTd), and fewer systemic-to-pulmonary shunts (SPS). We aim to report contemporary results using these treatment options in a comparative study.Methods: This a retrospective study using data from the UK National Congenital Heart Disease Audit. All infants (n=1662, median age 181 days) with ToF and no other complex defects undergoing repair or palliation between 2000 and 2013 were considered. Matching algorithms were used to minimise confounding due to lower age and weight in those palliated.Results: Patients underwent PrR (n=1244), SPS (n=311) or RVOTd (n=107). Mortality at 12 years was higher when repair or palliation was performed before the age of 60 days rather than after, most significantly for primary repair (18.7% vs 2.2%, P<0.001), less so for RVOTd (10.8% vs 0%, P=0.06) or SPS (12.4% vs 8.3%, P=0.2). In the matched groups of patients, RVOTd was associated with more right ventricular outflow tract (RVOT) reinterventions (HR=2.3, P=0.05 vs PrR, HR=7.2, P=0.001 vs SPS) and fewer pulmonary valve replacements (PVR) (HR=0.3 vs PrR, P=0.05) at 12 years, with lower mortality after complete repair (HR=0.2 versus PrR, P=0.09).Conclusions: We found that RVOTd was associated with more RVOT reinterventions, fewer PVR and fewer deaths when compared with PrR in comparable, young infants, especially so in those under 60 days at the time of the first procedure. [ABSTRACT FROM AUTHOR]