Surgical Palliation Strategy Does Not Affect Interstage Ventricular Dysfunction or Atrioventricular Valve Regurgitation in Children With Hypoplastic Left Heart Syndrome and Variants

Abstract

Background-All 3 palliation strategies, Norwood, Sano, and Hybrid, currently used for hypoplastic left heart syndrome pose a risk of myocardial injury at different times and through different mechanisms. We sought to compare these strategies to understand longitudinal differences in interstage ventricular dysfunction and their subsequent impact on transplant-free survival and atrioventricular valve regurgitation (AVVR) as well as the relationship between adverse events and ventricular function.

Almanac 2014: congenital heart disease

“…Surgical procedures include the 'Hybrid approach', Norwood and the Sano procedure, all of which serve to ultimately transition the patient to a complete Fontan anatomy. 9 While it has been reported that the choice of palliation strategy may not affect mortality in this patient group, [10][11][12][13] the optimal approach remains elusive with differences seen in specific subgroups. As an example, comparison of early and mid-term results of the 'hybrid approach' demonstrated no mortality difference despite significantly higher surgical (Aristotle) risk scores in the 'hybrid group' (see figure 1).…”

Risk Factors for Death or Heart Transplant for Patients with Right Ventricular Dysfunction After the Norwood Procedure: A Secondary Analysis of the Single Ventricle Reconstruction Trial

“…They found that greater indexed atrioventricular valve annular size, increased cardiopulmonary bypass time and decreased post-repair ventricular function were all independent predictors of mortality. Interestingly, another analysis from the same group found that shunt types were not associated with the risk of developing AVVR (Chetan et al 2013). In our analysis, either the presence or severity of tricuspid valve regurgitation was not associated with death or heart transplant without achieving the stage II procedure.…”

“…The spike in moderate ventricular dysfunction incidence seen at three to six months post-Norwood was shown to coincides temporally with the early hazard for death or transplant using the Congenital Heart Surgeons' Society cohort (Wilder, McCrindle, et al 2015b). In a single center series of 138 infants with HLHS, freedom from death or heart transplant was significantly reduced in patients who develop ventricular dysfunction in the early post-operative period after the first stage surgery both at 3 months (47 % vs. 84 %, p<0.001) and 6 months (27 % vs. 73 %, p< 0.001) (Chetan et al 2013). In a cohort study comparing 22 HLHS patients with persistent HLHS patients who experienced interstage deaths compared to 99 HLHS controls (Hehir et al 2008) and showed that the incidence of ventricular dysfunction did not differ between the two groups.…”

Impact of Norwood versus hybrid palliation on cardiac size and function in hypoplastic left heart syndrome

“…This allowed us to compare the impact on mortality and morbidity of both approaches, demonstrating very similar overall mortality and transplant-free survival between both groups 1 3. Qualitative assessment of RV function after both procedures was reported to be similar,1 3 but no detailed quantitative comparison of echocardiographic RV remodelling and function was included.…”

“…This allowed us to compare the impact on mortality and morbidity of both approaches, demonstrating very similar overall mortality and transplant-free survival between both groups 1 3. Qualitative assessment of RV function after both procedures was reported to be similar,1 3 but no detailed quantitative comparison of echocardiographic RV remodelling and function was included. The aim of the current study was therefore to evaluate the impact of stage I palliation on RV remodelling and performance, by comparing RV and tricuspid valve (TV) function in HLHS survivors managed with Norwood and hybrid strategies throughout all stages of palliation by use of serial echocardiography with a standardised imaging protocol.…”