Primary pulmonary vein stenosis is a rare congenital heart condition and carries a poor prognosis. Various surgical techniques have been introduced to treat primary pulmonary vein stenosis. However, there is no consensus on the optimal approach. This study aimed to evaluate the outcomes of surgical treatment of primary pulmonary vein stenosis, identify the risk factors for pulmonary vein restenosis and reintervention, and determine the optimal strategy for primary pulmonary vein stenosis repair.

From December 2008 to December 2023, 64 patients with primary pulmonary vein stenosis undergoing surgical repair in our institution were included [non-sutureless group, n = 42; sutureless group, n = 22]. The Cox proportional hazard model was used to identify risk factors for pulmonary vein restenosis and pulmonary vein restenosis-related reintervention.

There were three in-hospital deaths and one late death. The survival rates at 1, 5, and 15 years were 91.9%, 84.2%, and 69.8%, respectively, in the whole cohort. Compared with the non-sutureless group, the sutureless group did not significantly improve the long-term survival of primary pulmonary vein stenosis patients (P = 0.484). Pulmonary vein restenosis occurred in 15 patients. Multivariable analysis showed that Involvement of the left inferior pulmonary vein (P < 0.001) was the only independent risk factor for pulmonary vein restenosis, while sutureless repair (P = 0.037) was a protective factor. Pulmonary vein restenosis-related reintervention occurred in 12 patients. On multivariate analysis, three or four pulmonary veins involvement (P = 0.001) and preoperative severity score exceeding 5 (P = 0.050) were found to be independent risk factors associated with pulmonary vein restenosis-related reintervention; however, sutureless repair (P = 0.021) was a protective factor.

Management of infants with primary pulmonary vein stenosis is challenging. Surgical repair of primary pulmonary vein stenosis can be safely achieved using different techniques, with similar long-term mortality. Compared with non-sutureless repair, sutureless repair was significantly associated with decreased pulmonary vein restenosis and decreased pulmonary vein restenosis-related reintervention, respectively. Preoperative severity score exceeding 5, 3, or 4 pulmonary veins involvement, and left inferior pulmonary vein involvement are independent risk factors for adverse outcomes.

The aim of this study was to evaluate the association of bicuspid aortic valve on contemporary outcomes, including reoperation rates, after one-stage correction for interrupted aortic arch with ventricular septal defect or for aortic coarctation with hypoplastic aortic arch and ventricular septal defect.

Seventy-four consecutive patients (35 boys, 47% and 39 girls, 53%) with interrupted aortic arch (n = 41, 55%) or aortic coarctation with hypoplastic aortic arch (n = 33, 45%) with ventricular septal defect underwent early one-stage correction. Twenty (27%) patients had bicuspid aortic valve, and the remaining 54 (73%) had a tricuspid aortic valve. The median aortic valve annulus diameter was 6.0 mm (IQR: 2.0). Patients’ median age was 7 ± 29 days (range, 2–150); median weight was 3.3 ± 0.7 kg (range, 1.5–6.0), with 21 (28%) patients <3.0 kg. Selective brain perfusion through the innominate artery and selective coronary perfusion through the aortic root during aortic arch reconstruction were used in all patients. Statistical analysis was performed using SPSS version 20.0 software (SPSS Inc., Chicago, IL, USA).

The early mortality was 1.3%. One premature neonate died in the hospital with extracorporeal membrane oxygenation after aortic coarctation plus ventricular septal defect repair. There was no further mortality. Median follow-up was 5.7 years (IQR: 10.48). Reinterventions occurred in 36 (49%) patients: balloon angioplasty in 18 (24%) patients, reoperations in 4 (5%) patients, and both in 14 (19%) patients. A total of 86 follow-up procedures were required in these 36 (49%) patients: aortic valve valvulopasty (n = 6, 8%), stent implantation (n = 8, 11%), balloon dilatation (n = 39, 53%), and reoperation (n = 33, 45%). The median time to reinterventions was 9.094 years (SE 0.890). A potential risk factor for reintervention after interrupted aortic arch and aortic coarctation with ventricular septal defect repair was bicuspid aortic valve (p = 0.019, Chi2 (1) = 5.457). In addition, a multivariate Cox analysis with backward selection and significance level <0.015 was applied to all variables that showed significant effects in univariable analyzes. This regression confirmed that bicuspid aortic valve (HR = 0.381, p = .016), and interrupted aortic arch (HR = 0.412, p = 0.043) were predictors of late reintervention. All patients had no obvious neurologic impairment in routine examinations at last follow-up.

Bicuspid aortic valve was a significant risk factor for valve-related reintervention after one-stage repair for aortic arch obstruction with ventricular septal defect due to later development of stenosis associated with higher late morbidity and mortality. Particularly neonates with bicuspid aortic valve will possibly require reintervention in the future. Regular lifelong cardiac follow-up is recommended.