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Supracardiac total anomalous pulmonary venous connection is the most common subtype of total anomalous pulmonary venous connection. We aimed to describe the morphological spectrum of supracardiac total anomalous pulmonary venous connection and to identify risk factors for death and postoperative pulmonary venous obstruction. From February 2009 to June 2019, 241 patients diagnosed with supracardiac-Ia (left-sided vertical vein, n = 185) or supracardiac-Ib (right-sided connection directly to superior vena cava, n = 56) total anomalous pulmonary venous connection underwent initial surgical repair at our institute. Cases with functionally univentricular circulations or atrial isomerism were excluded. Patients' postoperative survival was described by Kaplan–Meier curves. Cox proportional hazards models and competing risk regression models were used to identify clinical risk factors for death and postoperative pulmonary venous obstruction. There were 8 early deaths and 4 late deaths. The overall survivals at 30 days, 1 year, and 10 years were 97.1%, 94.8%, and 94.8%, respectively, in the supracardiac-Ia group (2.7%, 5/185) (hazard ratio, 4.8; P =.003). Five patients required reoperation for pulmonary venous obstruction, including 2 patients who required reintervention for superior vena cava syndromes (all in the supracardiac-Ib group). One patient required superior vena cava balloon dilation for superior vena cava syndromes. Multivariable analysis showed that the supracardiac-Ib group (12.5%, 7/56) had a significantly higher mortality rate than the supracardiac-Ia group (adjusted hazard ratio, 8.5, P =.008). Surgical weight less than 2.5 kg (adjusted hazard ratio, 10.8, P =.023), longer duration of cardiopulmonary bypass (adjusted hazard ratio, 1.15 per 10 minutes, P =.012), and supracardiac-Ib subtype (adjusted hazard ratio, 4.7, P =.037) were independent risk factors associated with death. The supracardiac-Ib subtype (adjusted hazard ratio, 4.8, P =.003) was an incremental risk factor associated with postoperative pulmonary venous obstruction. Morphological features of supracardiac total anomalous pulmonary venous connection, especially the supracardiac-Ib subtype, were risk factors associated with postoperative pulmonary venous obstruction and survival. Patients with unique anatomic subtypes might require more individualized surgical planning. [Display omitted] [Display omitted] [ABSTRACT FROM AUTHOR]

Background Studies on the outcomes of surgical repair for supracardiac total anomalous pulmonary venous connection through the superior approach are uncommon. Methods From January 2004 to January 2016, 198 patients with supracardiac total anomalous pulmonary venous connection underwent side-to-side anastomosis between the common pulmonary vein and left atrium through the superior approach. Kaplan-Meier curve was used to demonstrate the survival estimates. Cox proportional hazard model and competing risk regression model were used to identify risk factors for death and postoperative pulmonary venous obstruction. Results There were six in-hospital deaths and no late deaths. The survival rates at 30 days, 1 year, and 12 years were 97%, 97%, and 97%, respectively. Follow-up was completed in 92.2% of the survivors. Median follow-up was 47 months (range: 0 to 136 months). Twenty-seven patients (14.1%, 27 of 192) required reoperation for pulmonary venous obstruction, residual atrial septal defect, or superior cava vena syndrome. Multivariable analysis showed that preoperative pulmonary venous obstruction ( p = 0.012), longer duration of ventilation ( p = 0.011), and emergency operation ( p = 0.010) were incremental risk factors for death. Aortic cross-clamp time ( p < 0.001) and preoperative pulmonary venous obstruction ( p = 0.002) were associated with postoperative pulmonary venous obstruction. Conclusions Side-to-side anastomosis through a superior approach in surgical repair of supracardiac total anomalous pulmonary venous connection can achieve satisfactory results. [ABSTRACT FROM AUTHOR]