BackgroundTo examine two different methods for treating patients with a total anomalous pulmonary venous connection (TAPVC) who need emergency surgery and to summarize the effects of the two strategies by retrospectively reviewing the data of 54 patients.MethodsA retrospective review of 54 patients with TAPVC who underwent emergency operations between December 2010 and July 2019 at a single institution was conducted. All patients exhibited respiratory and hemodynamic instability that required mechanical ventilation and inotropic support. Forty-four patients received emergency operations within 24 to 72 hours due to stabilization of the patient’s condition. Stable hemodynamics were achieved, and a constant milieu interne was maintained before the operation. These patients comprised the subemergency operation (SEO) group. Rather than being subjected to efforts to obtain stable hemodynamics and maintain a constant milieu interne, ten patients received emergency operations immediately within 24 hours of diagnosis or an emergency operation is performed immediately due to uncorrectable acidosis or progressive cardiovascular collapse. These patients comprised the emergency operation (EO) group. The hospital course, operative data, and outpatient records were reviewed.ResultsThe median weight, median age at surgery, mean cardiopulmonary bypass (CPB) duration and mean aortic cross-clamp (ACC) duration were not significantly different between the two groups. The median durations of ventilator support were 8.1±4.6 (2-13) days in the SEO group and 4.9±2.1 (2-18) days in the EO group, resulting in a significant difference (p=0.008). There were 12 operative mortalities (27.3%) in the SEO group and 2 operative mortalities (20%) in the EO group, resulting in no significant difference in mortality (p=0.636). Postoperative complications, such as low cardiac output and arrhythmia, were not significantly different between the two groups. The actuarial survival rates in the EO and SEO groups at 5 years were 87.5% and 89.9%, respectively. There was no difference in actuarial survival between the two groups at the latest follow-up (SEO group 89.9% versus EO group 87.5%, p=0.8115).ConclusionRather than making efforts to achieve stable hemodynamics and maintain a constant milieu interne, an emergency operation should be performed immediately, and some efforts to address metabolic acidosis do not reduce mortality.