Comparison of staged repair versus single-stage complete repair for pulmonary atresia with ventricular septal defect: A systematic review and meta-analysis

2021 ◽  
Author(s):  
Huzeifa Elhedai ◽  
Mustafa Mohamed ◽  
Salma Saeed S. Mohammed ◽  
Khalid H. H. Mustafa ◽  
Mohamed Hassan A. Seedahmed ◽  
...  
Keyword(s):  
Systematic Review ◽  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Meta Analysis ◽  
Pulmonary Atresia ◽  
Single Stage ◽  
Complete Repair

Velo-Cardio-Facial Syndrome Associated With Ventricular Septal Defect, Pulmonary Atresia, and Hypoplastic Pulmonary Arteries

PEDIATRICS ◽  
1992 ◽  
Vol 89 (5) ◽  
pp. 915-919
Author(s):  
Kerry B. Jedele ◽  
Virginia V. Michels ◽  
Francisco J. Puga ◽  
Robert H. Feldt
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Severe Form ◽  
Arterial Tree ◽  
Complete Repair ◽  
Pulmonary Arterial ◽  
Ear Anomalies ◽  
Velo Cardio Facial Syndrome

We report on 15 patients with velo-cardio-facial syndrome who had a severe form of tetralogy of Fallot (pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary arteries). Noncardiac anomalies in these patients included typical facial and ear anomalies in 15, nasal speech in 13, palate anomalies in 10, and developmental delay in 10. Seven patients had significant bronchospasm, which has not been reported in association with the velo-cardio-facial syndrome. All 15 patients had severe abnormalities of the arborization of the pulmonary arterial tree, which also has not been reported in velo-cardio-facial syndrome. All patients underwent staging operations to prepare the true pulmonary vascular tree for complete repair of the defect (five underwent complete repair and three survived). Of the remaining 10 patients, 6 are awaiting further operation, 3 are not candidates for complete repair, and 1 has died.

Promoting Pulmonary Arterial Growth via Right Ventricle-to-Pulmonary Artery Connection in Children With Pulmonary Atresia, Ventricular Septal Defect, and Hypoplastic Pulmonary Arteries

2017 ◽  
Vol 8 (5) ◽  
pp. 564-569 ◽  
Author(s):  
Edon J. Rabinowitz ◽  
Shilpi Epstein ◽  
Nina Kohn ◽  
David B. Meyer
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Arterial Tree ◽  
Collateral Arteries ◽  
Complete Repair ◽  
Pulmonary Arterial

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.

scholarly journals Early extubation is associated with improved outcomes after complete surgical repair of pulmonary atresia with ventricular septal defect and hypoplastic pulmonary arteries in pediatric patients

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Yinan Li ◽  
Yuan Jia ◽  
Hongbai Wang ◽  
Xie Wu ◽  
Shoujun Li ◽  
...  
Keyword(s):  
Postoperative Complications ◽  
Ventricular Septal Defect ◽  
Medical Cost ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Length Of Hospital Stay ◽  
Early Extubation ◽  
Hospital Medical ◽  
Complete Repair ◽  
Postoperative Length

Abstract Background The aim of this study was to investigate the impact of an early extubation strategy on outcomes following complete repair of pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary artery. Methods One hundred thirteen patients undergoing complete repair surgery of pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary artery between 2016 and 2018 were included in our retrospective propensity-score matched study. Propensity score matching was conducted in 1 to 2 ratio to balance the covariables impacting on clinical outcomes between groups. The primary outcomes were defined as length of intensive care unit stay, postoperative length of hospital stay and in-hospital medical cost. The secondary outcomes included postoperative complications such as re-intubation, re-exploration, in-hospital mortality, arrhythmia and etc.. In addition, blood product consumption were also abstracted. Results Compared with matched controls, patients in the early extubation group were demonstrated with a significant reduced length of intensive care unit stay (Median: 1.9 d νs. 4.1 d, p = 0.039), postoperative length of hospital stay (Median: 9.0 d νs. 17.0 d, p = 0.007) and in-hospital medical cost (Median: 69.5 × 1000CNY νs. 113.6× 1000CNY, p = 0.041). As for the postoperative complications, the occurrence of re-intubation, re-exploration, in-hospital mortality, arrhythmia and renal replacement therapy was similar between groups. However, pulmonary complications (p = 0.049) were with a significantly lower rate in the early extubation group. In addition, fresh frozen plasma (p = 0.041) transfusion volume were significantly reduced in the early extubation group rather than packed red blood cells and platelets. Conclusions Early extubation following complete repair of pulmonary atresia improved clinical outcomes and reduced in-hospital medical cost without increasing any postoperative complications.

scholarly journals Analysis of achieving an “ideal” outcome following midline unifocalization

2018 ◽  
Vol 27 (1) ◽  
pp. 11-17 ◽  
Author(s):  
Richard D Mainwaring ◽  
William L Patrick ◽  
Tatiana R Rosenblatt ◽  
Teimour Nasirov ◽  
Komal Kamra ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Right Ventricle ◽  
Outcome Measures ◽  
Septal Defect ◽  
Pressure Ratio ◽  
Pulmonary Atresia ◽  
Major Aortopulmonary Collateral Arteries ◽  
Collateral Arteries ◽  
Complete Repair ◽  
Aortopulmonary Collateral

Objective Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is a complex form of congenital heart disease. Midline unifocalization has been developed for the surgical treatment of this condition. There are 3 outcome measures that determine long-term success: patients are alive, patients have achieved complete repair (i.e. ventricular septal defect closure), and patients have a relatively low right ventricle-to-aortic pressure ratio (<0.45). However, to date, no studies have combined these 3 outcome measures to analyze the likelihood of achieving an ideal outcome. Methods This was a retrospective review of 255 patients who underwent midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. The median age at unifocalization was 4.5 months. Results Two hundred thirty-five (92%) patients were alive at a mean follow-up of 5.3 years. Two hundred and seventeen (85%) patients underwent single-stage complete repair, and 38 (15%) had an initial unifocalization and shunt. Twenty-four of the 38 palliated patients have subsequently undergone repair. Thus 241 (94%) patients ultimately achieved complete repair. Of the 241 patients who were repaired, 219 (86%) had a right ventricle-to-aortic peak systolic pressure ratio <0.45. Combining these outcome measures, 77% of patients achieved an ideal outcome at one month, 73% at 6 months, 76% at one year, and 80% at 4 years. Conclusions Most (80%) patients can achieve all 3 measures of favorable outcome at 4 years following midline unifocalization. We speculate that this will be a favorable portend for the future of these patients.

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