Background “Swiss Cheese” ventricular septal defects (VSDs) is a kind of rare and complex congenital heart defects and the surgical management remains controversial and a challenge. We reviewed our preliminary clinical experience on biventricular surgical repair of “Swiss Cheese” VSDs with two-patch and right ventricle apex excluding technique in 10 cases. Methods From May 2014 to December 2019, a series of 10 patients (M/F=3/7) were admitted in our center. 9 cases underwent one-stage surgical repair with two-patch and right ventricle apex excluding technique and 1 case received a second-stage surgical repair with the same technique. Surgical repair was done with cardiopulmonary bypass (CPB) in all cases. 2 fresh autologous pericardium patches were used to close defects of the outflow tract area and the apex trabecular area respectively and as a result, the right ventricular apex was excluded from the right ventricular inflow tract. Results All the operations were successful. Median CPB time and aortic clamping time were 96 min and 68 min respectively. Delayed chest closure were done in 2 cases within 48-72 hours postoperatively. The Median time of mechanical ventilation and ICU stay were 131.3 hours and 8 days respectively. Median length of stay after operation was 11 （9-42）days. There was no mortality and major complication except for 2 cases of ventilator associated pneumonia. There was no death and major complication during the median follow-up time of 3.2 years.. The latest echocardiography results showed the left and right heart function was normal in all the cases. Conclusions Biventricular surgical repair of “Swiss Cheese” VSDs with two-patch of fresh autologous pericardium and right ventricle apex excluding technique in infants is safe and feasible with favorable early and mid-term results. Long term results need to be evaluated with more cases.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease.Highly penetrant copy number variants (CNVs) and genes related to the etiology of TOF likely exist with differences among populations. We aimed to identify CNV contributions to sporadic TOF cases in Han Chinese. Genomic DNA was extracted from peripheral blood in 605 subjects (303 sporadic TOF and 302 unaffected Han Chinese[Control] from cardiac centers in China) and analyzed by genome-wide association study (GWAS). The GWAS results were compared with existing Database of Genetic Variants. These CNVs were further validated by qPCR. Bioinformatics analyses were performed with protein-protein interaction (PPI) network and KEGG pathway enrichment. Across all chromosomes 119 novel "TOF-specific CNVs" were identified with prevalence of CNVs of 21.5% in chromosomes 1-20 and 37.0% including Chr21/22.In chromosomes 1-20, CNVs on 11q25 (encompasses genes ACAD8,

Background “Swiss Cheese” ventricular septal defects (VSDs) is a kind of rare and complex congenital heart defects and the surgical management remains controversial and a challenge. We reviewed our preliminary clinical experience on biventricular surgical repair of “Swiss Cheese” VSDs with two-patch and right ventricle apex excluding technique in 10 cases.Methods From May 2014 to December 2019, a series of 10 patients (M/F=3/7) were admitted in our center. 9 cases underwent one-stage surgical repair with two-patch and right ventricle apex excluding technique and 1 case received two-stage surgical repair with the same technique. Surgical repair was done with cardiopulmonary bypass (CPB) in all cases. 2 fresh autologous pericardium patches were used to close defects of the outflow tract area and the apex trabecular area respectively and as a result, the right ventricular apex was excluded from the right ventricular inflow tract.Results All operations were successful. Median CPB time and aortic clamping time were 96 min and 68 min respectively. Delayed chest closure was performed in 2 cases within 48-72 hours postoperatively. The Median time of mechanical ventilation and ICU stay were 131.3 hours and 8 days respectively. Median length of hospital stay after operation was 11 （9-42）days. There was no mortality and major complication except for 2 cases of ventilator associated pneumonia. There was no death and major complication during a median follow-up time of 3.2 years.. The latest echocardiography results showed the left and right heart function was normal in all the cases.Conclusions Biventricular surgical repair of “Swiss Cheese” VSDs with two-patch of fresh autologous pericardium and right ventricle apex excluding technique in infants is safe and feasible with favorable early and mid-term results. Long term results need to be evaluated with more cases.