Abstract

<b>Background:</b> This retrospective cohort study aimed to explore the causes of death in children with congenital heart disease (CHD) after cardiac surgery in one of the biggest cardiac centers for children with CHD in China. <b>Methods:</b> A total of 26,856 children undergoing cardiac surgery from January 1, 2012 to December 31, 2019 were included. Based on the clinical data, the causes of death were divided into ten categories and further compared among different periods, types of CHD and surgical procedures. <b>Results:</b> Of all patients, 513 (1.9%) died (median age 162 d, median weight 5.6 kg). The mortality in 2016–2019 was lower than that in 2012–2015 (1.4 ± 0.3% <i>vs</i>. 2.5 ± 0.3%, <i>p</i> = 0.005). A total of 42.5% of children died of heart failure, and 32.9% died of residual anatomic defects. Patients with transposition of the great arteries tended to die from residual anatomic defects (21.9%), while those with double-outlet right ventricle (20%) and single ventricle (20%) tended to die from pulmonary hypertension (PH) (<i>p</i> = 0.006). After biventricular repair, children tended to die from heart failure (90.4%), while after single-ventricle repair, children tended to die from PH (50%) (<i>p</i> < 0.0001). There is a negative correlation between mortality and the ECMO implantation rate (<i>r</i> = −0.898, <i>p</i> = 0.002). <b>Conclusions:</b> Heart failure and residual anatomic defects were the main causes of death after cardiac surgery. The cause of death patterns differed among CHD types and surgical strategies. ECMO may be a life-saving tool when other conventional therapies do not work.

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