Risk Factors of Total Anomalous Pulmonary Venous Connection Surgery

Background: Total anomalous pulmonary venous connection is a rare congenital heart disease that needs to be surgically corrected. In our study, we retrospectively evaluated the factors associated with mortality and morbidity in patients undergoing total anomalous pulmonary venous connection surgery. Patients and methods: Retrospective data were collected on 34 patients with isolated total anomalous pulmonary venous connection seen from 1990 to 2015. The median age at time of repair was 36 days (range, 1 to 2520 days) with median weight of 3.465 kg (range, 1.4 to 20.7 kg). Pulmonary venous obstruction, reintervention, associated factors for mortality and overall mortality were recorded and analyzed. Results: There were 16 deaths (13 surgical deaths and 3 late deaths due to pulmonary venous restenosis and reintervention). Preoperative pulmonary venous obstruction was presented in 21 patients (21/34, 61.8%) and was found to be associated with overall mortality (p=0.0052). Postoperative pulmonary venous restenosis was also a significant risk factor for mortality (p=0.0421). Emergent surgery is a risk factor for mortality (p=0.004). The associated risk factors, such as pre-operation ventilator FiO2, pre-operation inotropic use, postoperation ventilator FiO2 and post-operation epinephrine use were also associated with patient mortality. The initial SpO2 when arriving at our hospital was a protective factor for mortality. Conclusion: The overall mortality of total anomalous pulmonary venous connection patients has improved in recent years. However, preoperative pulmonary venous obstruction was still an important risk factor for mortality. Postoperative pulmonary venous restenosis was also a risk factor for mortality. Heart failure and emergent surgery were associated with higher mortality rates.