To explored the surgical treatment strategy for complete type endocardial cushion defect (TECD) combined with tetralogy of Fallot (TOF) or double outlet of right ventricular (DORV) by summarizing the data of patients with these malformations in single-center.
Methods
The clinical data of patients with TECD combined with TOF or DORV from January 2001 to December 2021 in Fuwai Hospital were retrospectively collected. The patients were divided into single ventricular appliance group and double ventricular appliance group. The single ventricular correction group included superior vena-pulmonary artery connection subgroup (Glenn subgroup) and total vena-pulmonary artery connection subgroup. The preoperative baseline data, intraoperative cardiopulmonary bypass time, aortic cross-clamp time, differences in postoperative mitral regurgitation, tricuspid regurgitation, and mortality differences between the single ventricular correction group and the double ventricular correction group were compared. Additionally, the differences in severe atrioventricular valve regurgitation, other complications, and mortality between the single ventricular and double ventricular patients in the two time periods from 2001 to 2010 (the first 10 years subgroup) and from 2011 to 2021 (the second 10 years subgroup) were compared.
Results
From January 2001 to December 2021, there were a total of 184 patients with TECD combined with TOF or DORV [median age 45 (16-84) months, male 64.1% (118/184)] in our center. There was no significantly difference was found between the biventricular correction group (26.6%, 49/184) and the total cava subgroup (26.6%, 49/184) and Glenn subgroup (46.8%, 86/184) (38 months vs. 60 months vs. 35 months, P=0.058). There were 109 patients in the first 10 years subgroup, including 22 patients (20.2%, 22/109) in the biventricular correction group and 87 patients (79.8%, 87/109) in the single ventricular correction group. There were 75 patients in the second 10 years subgroup, including 27 patients (36.0%, 27/75) in the biventricular correction group and 48 patients (64.0%, 48/75) in the single ventricular correction group. Compared with the first and second 10 years subgroup, there was a statistically significant difference in the proportion of severe atrioventricular regurgitation in the biventricular correction group [27.3% (6/22) vs. 14.8% (4/27), P=0.01], the operative mortality rate of biventricular correction and single ventricular correction in the first 10 years subgroup was higher than that in the second 10 years subgroup [biventricular correction operative mortality rate:13.6% (3/22) vs. 3.7% (1/27), P=0.02; single-ventricular corrective operative mortality rate: 5.7% (5/87) vs. 2.1% (1/48), P=0.06].
Conclusion
TECD combined with TOF or DORV is a rare congenital complex malformation. With the advancement of technology and the selection of individualized surgical strategies, patients with TECD combined with TOF or DORV can obtain a good prognosis.
