OBJECTIVES This study was performed to determine the clinical and haemodynamic variables associated with early adverse outcomes after the neonatal Norwood procedure. METHODS Patients who underwent the neonatal Norwood procedure between 2001 and 2019 were included. The patient diagnosis, morphological characteristics and haemodynamic parameters were analysed to identify factors associated with length of stay (LOS) in the intensive care unit (ICU) and mortality during the stay. RESULTS A total of 322 patients were depicted. The median age and weight at the Norwood procedure were 9 days and 3.2 kg, respectively. Certain morphological and preoperative parameters, such as birth weight below 2.5 kg, restrictive atrial septal defect, extracardiac anomalies and the diameter of the ascending aorta, were found to be associated with the LOS in the ICU. Analysis using early postoperative haemodynamic variables revealed that systolic arterial pressure, diastolic arterial pressure, serum lactate levels and reduced ventricular function at 2 days postoperatively were associated with the LOS in the ICU. Birth weight <2.5 kg (P = 0.010), a restrictive atrial septal defect (P = 0.001) and smaller ascending aorta (P = 0.039) were associated with death in the ICU. Reduced ventricular function, lower systolic aortic pressure and higher lactate levels at various time points (P < 0.05) were also associated with ICU deaths. The LOS in the ICU was significantly associated with late mortality (P < 0.001, Hazard Ratio (HR) = 1.015). CONCLUSIONS The LOS in the ICU after the Norwood procedure was predicted by early postoperative haemodynamic variables, suggesting that good early postoperative haemodynamics determine early recovery. A prolonged stay in the ICU after the Norwood procedure was associated with late mortality.

Objective This study aimed to determine the longitudinal change of systemic ventricular function and atrioventricular valve regurgitation after total cavopulmonary connection. Methods In 620 patients who underwent total cavopulmonary connection between 1994 and 2021, 4219 longitudinal echocardiographic examinations of systemic ventricular function and atrioventricular valve regurgitation were evaluated retrospectively. Results The most frequent primary diagnosis was hypoplastic left heart syndrome in 172, followed by single ventricle in 131, tricuspid atresia in 95, and double inlet left ventricle in 91 patients. Dominant right ventricle was observed in 329 (53%) and dominant left ventricle in 291 (47%). Median age at total cavopulmonary connection was 2.3 (1.8–3.4) years. Transplant-free survival at 5, 10, and 15 years after total cavopulmonary connection was 96.3, 94.7, and 93.6%, respectively in patients with dominant right ventricle and 97.3, 94.6, and 94.6%, respectively in those with dominant left ventricle (p = 0.987). Longitudinal analysis of systemic ventricular function was similar in both groups during the first 10 years postoperatively. Thereafter, systemic ventricular function worsened significantly in patients with dominant right ventricle, compared with those with dominant left ventricle (15 years: p = 0.007, 20 years: p = 0.03). Atrioventricular valve regurgitation was more frequent after total cavopulmonary connection in patients with dominant right ventricle compared with those with dominant left ventricle (p < 0.001 at 3 months, 3 years, 5 years, 10 years, and 15 years, p = 0.023 at 20 years). There was a significant correlation between postoperative systemic ventricular dysfunction and atrioventricular valve regurgitation (p < 0.001). Conclusions There was no transplant-free survival difference and no difference in ventricular function between dominant right ventricle and dominant left ventricle for the first 10 years after total cavopulmonary connection. Thereafter, ventricular function in dominant right ventricle was inferior to that in dominant left ventricle. The degree of atrioventricular valve regurgitation was significantly higher in dominant right ventricle, compared with dominant left ventricle, and it was positively associated with ventricular dysfunction, especially in dominant right ventricle.