Natural History of Kawashima Palliation in Single-Ventricle and Interrupted Inferior Vena Cava Heart Disease in China 11 Years Result

Background: The long-term outcomes of patients treated with a Kawashima procedure and keeping the antegrade pulmonary blood flow (AnPBF) in single-ventricle (SV) and interrupted inferior vena cava (IVC) heart disease is still uncertain as yet. Methods: We investigated 18 patients who underwent the Kawashima procedure with SV physiology and an interrupted IVC between January 2009 and June 2018, perioperative, operative and postoperative characteristics were recorded. Results: A total of 18 patients underwent the Kawashima procedure at a median age of 2.7 years (range 0.5-24.7 years), of which 12 (66.7%) were male and 6 (33.3%) were female. The mean saturation was 76.2 +/- 8.5% in preoperative period and 94.2 +/- 2.2% in postoperative period. All patients had kept AnPBF. The median duration of mechanical ventilation was 12 h (range 2.5-22.5 h) and the median duration of pleural drainage was 5 days (range 2-27 days). The median hospital stay was 9 days (range 6-70 days). There was no operative death and no mortality was seen in early postoperative period. Follow-up was 100% completed, with an average follow-up period of 6.1 +/- 2.7 years (range 1-11 years). 4 patients died during the follow-up. The overall 5 and 10 years' survival rates estimated by Kaplan-Meier method were 88% and 68%, respectively. Although there were no significant differences in the duration of postoperative follow-up between the death group and the survival group (p > 0.05), the major systemic ventricular end-diastolic diameter (SVEDD) (p = 0.018) and the degree of AVVR (p = 0.001) showed significant difference between the two groups. The diameters of main pulmonary artery showed significant growth in both the death group (p = 0.015) and the survival group (p = 0.012) over time. SVEDD had no significant increase in the survival group (p = 0.665) but was significantly larger in the death group (p = 0.014). Multivariable risk factors of late mortality in patients treated with Kawashima procedure were follow-up AVVR (p = 0.044; HR: 3.124; 95%CI: 1.030-9.473) and SVEDD (p = 0.031; HR: 9.766; 95%CI: 1.226-77.8). 14 patients (100%) were all in New York Heart Association (NYHA) functional class I and the mean saturation was 93 +/- 2% at last follow-up. Only one patient finished Fontan completion. Conclusions: The Kawashima procedure with AnPBF can be safely performed with acceptable early and long outcomes. Although some previous studies have shown the risk of pulmonary arteriovenous malformations (PAVMS) after Kawashima procedure in the mid- and long-term, our findings are in contradiction with it. No PAVMs occurred in all the survivors. Kawashima procedure with open AnPBF may be a good option for unsuitable Fontan candidates.