Efficiency at Heart: Navigating the Hybrid Single-Ventricle Pathway.

Background: Single-ventricle cardiac defects (SVCDs) are among of the most health care resource-intensive congenital diseases. Although SVCDs are traditionally palliated using the Norwood pathway, in the last 2 decades select programs have used the hybrid strategy, which redistributes the operative and interstage risks. This study sought to characterize resource use for a cohort of patients with hybrid-palliated SVCD.

Increased physiologic dead space fraction is associated with mortality after comprehensive stage 2 operation.

Objective: Our objective was to assess the predictive value of physiologic dead space fraction for mortality in patients undergoing the comprehensive stage 2 operation.

Methods: This was a single-centre retrospective observational study conducted at a quaternary free-standing children's hospital specialising in hybrid palliation of single ventricle cardiac disease. 180 patients underwent the comprehensive stage 2 operation.

Outcomes After Hybrid Palliation for Infants With Critical Left Heart Obstruction.

Background: Hybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) is an initial management strategy for infants with critical left heart obstruction and serves as palliation until subsequent operations are pursued.

Objectives: This study sought to determine patient characteristics and factors associated with subsequent outcomes for infants who underwent hybrid palliation.

Methods: From 2005 to 2019, 214 of 1,236 prospectively enrolled infants within the Congenital Heart Surgeons' Society's critical left heart obstruction cohort underwent hybrid palliation across 24 institutions.

Novel pathogenic GATA6 variant associated with congenital heart disease, diabetes mellitus and necrotizing enterocolitis.

Background: Pathogenic GATA6 variants have been associated with congenital heart disease (CHD) and a spectrum of extracardiac abnormalities, including pancreatic agenesis, congenital diaphragmatic hernia, and developmental delay. However, the comprehensive genotype-phenotype correlation of pathogenic GATA6 variation in humans remains to be fully understood.

Methods: Exome sequencing was performed in a family where four members had CHD.

Comparing apples to apples: Exploring public reporting of congenital cardiac surgery outcomes based on common congenital heart operations.

Objective: We sought to simplify reporting of outcomes in congenital heart surgery that compares well-defined patient groups and accommodates multiple stakeholder needs while being easily understandable.

Methods: We selected 19 commonly performed congenital heart surgeries ranging in complexity from repair of atrial septal defects to the Norwood procedure. Strict inclusion/exclusion criteria ensured the creation of 19 well-defined diagnosis/procedure cohorts.