Outcomes of management of major aortopulmonary collaterals for pulmonary atresia and ventricular septal defect

2020 ◽  
pp. 1-9
Author(s):  
Dong Zhao ◽  
Keming Yang ◽  
Wei Feng ◽  
Shoujun Li ◽  
Jun Yan ◽  
...  

Abstract Objective: This study aimed to investigate the association between long-term survival and different management of major aortopulmonary collateral arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Methods: From November, 2009 to October, 2018, a total of 98 consecutive patients with pulmonary atresia, ventricular septal defect, major aortopulmonary collateral arteries, and hypoplastic pulmonary arteries treated with modified Blalock–Taussig shunt or right ventricle–pulmonary artery connection were included. Fifty-five patients who received occlusion or ligation of major aortopulmonary collateral arteries during or after palliative procedure were occlusion group, and the other 43 patients were no occlusion group. The early and late outcomes were compared. Results: The mean duration of follow-up was 30.9 months in no occlusion group and 49.8 months in the occlusion group (p < 0.001). Multivariate analysis showed that only no occlusion of major aortopulmonary collateral arteries was predictive of total mortality (Hazard Ratio: 4.42, 95% CI: 1.27 to 15.42, p = 0.02). The Kaplan–Meier survival curves confirmed that patients without occlusion of major aortopulmonary collateral arteries demonstrated worse survival as compared with the occlusion group (p = 0.013). The Kaplan–Meier survival curves of patients who underwent different palliative procedures showed no differences. Conclusions: For patients with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries when a primary repair is not feasible, those without occlusion of major aortopulmonary collateral arteries have a higher risk of death following an initial palliative procedure compared with patients who underwent occlusion of major aortopulmonary collateral arteries. The occlusion of major aortopulmonary collateral arteries is not associated with a higher rate of complete repair or better improvement of pulmonary artery growth.

Author(s):  
V.V. Ezhova

The case of prenatal diagnosis of pulmonary artery atresia with a ventricular septal defect and major aortopulmonary collateral arteries at 34 weeks is presented. Atresia of the pulmonary artery with a ventricular septal defect and major aortopulmonary collateral arteries (type B) was diagnosed. At 39 weeks birth occurred. After the examination, the prenatal diagnosis was confirmed. The condition of the newborn was rated as satisfactory. On the 7th day the child was discharged under the supervision of a cardiologist. Surgical treatment is planned. The article discusses the issues of differential prenatal diagnosis, modern possibilities of operative treatment and prognosis.


2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Zirou Huang ◽  
Fan Cao ◽  
Rongjun Zou ◽  
Minghui Zou ◽  
Weidan Chen ◽  
...  

Objective. We retrospectively analyzed the surgical results of pulmonary blood flow studies to guide ventricular septal defect (VSD) closure in the correction of pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries (PA/VSD/MAPCAs). Methods. A total of 57 children who were diagnosed with PA/VSD/MAPCAs and who underwent intraoperative pulmonary blood flow studies at our hospital between August 2016 and June 2019 were included. Surgery and cardiopulmonary bypass records were collected. The receiver operating characteristic (ROC) curve was used to verify the accuracy of pulmonary blood flow studies to predict VSD closure. Results. Complete VSD closure was achieved in 39 of 57 children (68.42%), with a median age of 2 years and 5 months (range: 7 months to 15 years and 9 months) and a median weight of 11.0 kg (5.7–36.5 kg). Partial VSD repair was recorded for 21 children (36.84%), including 4 children (19.05%) who underwent VSD closure in the later stages and 13 children (61.90%) who were under follow-up and waiting to undergo complete VSD closure. There was only one child (1.75%) with VSD left. After eliminating the data of four unqualified cases, the ROC curve for predicting VSD closure based on 53 pulmonary blood flow studies was obtained at a p value of <0.001, with an area under the curve of 0.922. The maximum Youden’s index was 0.713, which corresponded to an optimal mean pulmonary artery pressure cutoff value of 24.5 mmHg. Conclusion. The functional evaluation provided by pulmonary blood flow studies is highly accurate to predict intraoperative VSD repair. We recommend using pulmonary blood flow studies with a mean pulmonary artery pressure of ≤25 mmHg during blood perfusion at 3.0 L/min/m2 as the standard to repair VSD.


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