Right Ventricle-to-Pulmonary Artery Shunt in Pulmonary Atresia with a Ventricular Septal Defect: A Word of Caution

Background: Complete repair of pulmonary atresia (PA) ventricular septal defect (VSD) with hypoplastic or absent native pulmonary arteries, often with major aortopulmonary collateral arteries (MAPCAs), involves construction of an adequate sized pulmonary arterial tree. We report our results with a previously described staged strategy using initial right ventricle (RV)-to-reconstructed pulmonary arterial tree (RV-PA) connection to promote pulmonary arterial growth and facilitate later ventricular septation. Methods: We retrospectively reviewed data for all patients (N = 10) with initial echocardiographic diagnosis of PA-VSD and hypoplastic pulmonary arteries operated in our center from October 2008 to August 2016. Pulmonary arterial vessel size measured on preoperative and postoperative angiography was used to calculate Nakata index. Results: Seven patients had PA-VSD, three had virtual PA-VSD, and seven had MAPCAs. All underwent creation of RV-PA connection at a median age of 7.5 days and weight 3.6 kg. Eight patients had RV-PA conduits, two had a transannular patches, and seven had major pulmonary artery reconstruction simultaneously. There were no deaths or serious morbidity; one conduit required revision prior to complete repair. Complete repair with ventricular septation and RV pressure less than half systemic was achieved in all patients at a median age of 239 days. Nakata index in neonatal period was 54 mm2/m2 (range 15-144 mm2/m2) and at time of septation 184 mm2/m2 (range 56-510 mm2/m2; P = .004). Growth rates of right and left branch pulmonary arteries were similar. The 10 patients underwent 28 catheterizations with 13 interventions in 8 patients prior to full repair. Conclusion: Early palliative RV-PA connection promotes pulmonary arterial growth and facilitates eventual full repair with VSD closure with low RV pressure and operative risk.

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Pulmonary atresia with ventricular septal defect and tetralogy of Fallot: transannular path augmentation versus systemic to pulmonary artery shunt for first-stage palliation

Cardiology in the Young ◽

10.1017/s1047951120002553 ◽

2020 ◽

Vol 30 (11) ◽

pp. 1679-1687

Author(s):

Marien Lenoir ◽

Virginie Fouilloux ◽

Beatrice Desnous ◽

Bilal Rahmani ◽

Nabila El Gueddari ◽

...

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Hospital Mortality ◽

Right Ventricle ◽

Tetralogy Of Fallot ◽

Septal Defect ◽

Pulmonary Atresia ◽

Biventricular Repair ◽

Palliative Procedure ◽

Blalock Taussig Shunt

AbstractBackground:Pulmonary atresia with ventricular septal defect and severe tetralogy of Fallot require a palliative procedure for pulmonary artery rehabilitation. For first-stage palliation, two main surgical options are still debated: right ventricle to pulmonary artery connection and modified Blalock–Taussig shunt. We compared the clinical outcomes of the two procedures.Methods:From 1995 to 2018, 88 patients needed palliation (pulmonary atresia with ventricular septal defect n = 47; tetralogy of Fallot n = 41). Among these patients, 70 modified Blalock–Taussig shunt and 18 transannular path augmentation were performed before 6 months of age. Using a 1:1 propensity score match analysis, 20 patients were included in the analysis. The primary outcome was in-hospital mortality and pulmonary artery growth.Results:After matching, the pre-operative Nakata was smaller in transannular path augmentation 54 ± 24 mm2/m2 than modified Blalock–Taussig shunt 109 ± 31 mm2/m2 (p < 0.001). The age and weight were similar (p = 0.31 and p = 0.9, respectively). There was no difference in in-hospital mortality (p = 0.3). The Nakata index before biventricular repair and delta Nakata were smaller in modified Blalock–Taussig shunt group (206 ± 80 mm2/m2, 75 ± 103 mm2/m2) than transannular path augmentation (365 ± 170 mm2/m2, 214 ± 165 mm2/m2; p = 0.03; p < 0.001). Median time to biventricular repair was similar (p = 0.46). The rate of interstage reintervention was similar (p = 0.63).Conclusions:The transannular path augmentation is better for the rehabilitation of the native pulmonary artery. Despite a smaller pulmonary artery, right ventricle to pulmonary artery connection is equivalent to modified Blalock–Taussig shunt for rate of biventricular repair and time to biventricular repair.

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Mid-Term Outcome of Right Ventricle to Pulmonary Artery Shunt for Older Children and Young Adults With Ventricular Septal Defect, Pulmonary Atresia, and Hypoplastic Pulmonary Arteries

Seminars in Thoracic and Cardiovascular Surgery ◽

10.1053/j.semtcvs.2019.05.014 ◽

2019 ◽

Vol 31 (4) ◽

pp. 837-844 ◽

Cited By ~ 2

Author(s):

Kartik Patel ◽

Suresh Kumar Rajan ◽

Pankaj Garg ◽

Trushar Gajjar ◽

Amit Mishra ◽

...

Keyword(s):

Young Adults ◽

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Right Ventricle ◽

Septal Defect ◽

Pulmonary Arteries ◽

Pulmonary Atresia ◽

Older Children ◽

Term Outcome ◽

Children And Young Adults

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Outcomes of palliative right ventricle to pulmonary artery connection for pulmonary atresia with ventricular septal defect†

European Journal of Cardio-Thoracic Surgery ◽

10.1093/ejcts/ezx194 ◽

2017 ◽

Vol 52 (3) ◽

pp. 590-598 ◽

Cited By ~ 8

Author(s):

Marien Lenoir ◽

Margaux Pontailler ◽

Régis Gaudin ◽

Sébastien Gerelli ◽

Daniel Tamisier ◽

...

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Right Ventricle ◽

Septal Defect ◽

Pulmonary Atresia

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Establishment of right ventricle-pulmonary artery continuity as the first-stage palliation in older infants with pulmonary atresia with ventricular septal defect may be preferable to use of an arterial shunt

Interactive Cardiovascular and Thoracic Surgery ◽

10.1093/icvts/ivu052 ◽

2014 ◽

Vol 19 (1) ◽

pp. 88-94 ◽

Cited By ~ 6

Author(s):

Shuai Zheng ◽

Keming Yang ◽

Kun Li ◽

Shoujun Li

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Right Ventricle ◽

Septal Defect ◽

Pulmonary Atresia

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Pulmonary Atresia with Ventricular Septal Defect and Right Ventricle–to–Pulmonary Artery Conduits

Sabiston and Spencer's Surgery of the Chest ◽

10.1016/b978-1-4160-5225-8.00120-3 ◽

2010 ◽

pp. 1897-1910

Author(s):

Sitaram M. Emani ◽

John E. Mayer

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Right Ventricle ◽

Septal Defect ◽

Pulmonary Atresia

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

Cardiology in the Young ◽

10.1017/s1047951120004047 ◽

2020 ◽

pp. 1-9

Author(s):

Dong Zhao ◽

Keming Yang ◽

Wei Feng ◽

Shoujun Li ◽

Jun Yan ◽

...

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Septal Defect ◽

Pulmonary Atresia ◽

Survival Curves ◽

Major Aortopulmonary Collateral Arteries ◽

Palliative Procedure ◽

Collateral Arteries ◽

Kaplan Meier ◽

Aortopulmonary Collateral

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.

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