Systemic to Pulmonary Artery Versus Right Ventricular to Pulmonary Artery Shunts for Patients With Pulmonary Atresia, Ventricular Septal Defect, and Hypoplastic Pulmonary Arteries

2015 ◽  
Vol 30 (11) ◽  
pp. 840-845 ◽  
Author(s):  
Xu Wang ◽  
Zhongyuan Lu ◽  
Shoujun Li ◽  
Jun Yan ◽  
Keming Yang ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Right Ventricular ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia

scholarly journals Management of pulmonary atresia with ventricular septal defect and hypoplastic pulmonary arteries by right ventricular outflow construction

1980 ◽  
Vol 80 (4) ◽  
pp. 552-567 ◽  
Author(s):  
Jeffrey M. Piehler ◽  
Gordon K. Danielson ◽  
Dwight C. McGoon ◽  
Robert B. Wallace ◽  
Richard E. Fulton ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Right Ventricular ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia

Abstract 12635: Nationwide Long-term Follow-up of Pulmonary Atresia With Ventricular Septal Defect

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Anu Kaskinen ◽  
Juha-Matti Happonen ◽  
Ilkka P Mattila ◽  
Olli M Pitkänen
Keyword(s):  
Surgical Treatment ◽  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Palliative Surgery ◽  
Total Study Population ◽  
Collateral Arteries

Introduction: The naturally poor survival of pulmonary atresia with ventricular septal defect (PA+VSD) has improved due to evolved perioperative and surgical treatment. Studies including PA+VSD patients, both with and without major aortopulmonary collateral arteries (MAPCAs), with extensive follow-up are scarce. This nationwide study aimed to investigate survival and surgical treatment in PA+VSD patients with and without MAPCAs. Methods: Study comprised 109 PA+VSD patients born in Finland between 1970 and 2007. We reviewed retrospectively medical records and operative reports through December 2011, as well as first available angiograms and preoperative angiograms prior to repair attempt. Results: The median follow-up time for the total study population, including also patients who died during the follow-up, was 11.4 years (IQR 0.8 - 21.1). The incidence of PA+VSD, which could be determined reliably from 1995 to 2007, was 6.1 per 100 000 live births. Although the patients with (n = 43) or without MAPCAs (n = 66) showed no difference in survival (p = 0.74), the patients without MAPCAs had better probability to achieve repair (64% vs. 28%, p < 0.0001). The bigger size of true central pulmonary arteries assessed by McGoon index at first angiogram [HR 0.66 (CI95% 0.49 - 0.88) per 0.5 McGoon index units, p = 0.006] and achievement of repair [HR 0.07 (CI95% 0.03 - 0.17), p < 0.0001] improved the overall survival. After successful repair survival was 93% at 1 year and 91% from 2 years on. Palliated patients, instead, had survival at 1, 5, 10, and 20 years of age of 55%, 42%, 34%, and 20% respectively. However, patients with right ventricle - pulmonary artery connection and septal fenestration had better survival than rest of the palliated patients (p = 0.001). Palliation with a systemic-pulmonary artery shunt increased McGoon index by 41% (p < 0.0001). Conclusions: The patients with MAPCAs had higher risk to remain palliated than patients without, although their survival was similar. Survival of PA+VSD was influenced by the initial size of true central pulmonary arteries and whether repair was achieved. Although palliative procedures may not improve the final outcome of PA+VSD, palliative surgery may have a role in its treatment.

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.

Pulmonary Atresia, VSD in Association with Coronary-Pulmonary Artery Fistula

2007 ◽  
Vol 15 (4) ◽  
pp. 335-338 ◽  
Author(s):  
Hani K Najm ◽  
Neerod K Jha ◽  
Michael Godman ◽  
Mansour Al Mutairi ◽  
Ahmed I Rezk ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Left Pulmonary Artery ◽  
Corrective Surgery ◽  
Aortopulmonary Collaterals ◽  
One Year ◽  
Pulmonary Artery Fistula

Congenital coronary-pulmonary artery fistula is rare in patients with pulmonary atresia and ventricular septal defect. The nomenclature, physiological, clinical, and surgical implications of these fistulas are yet to be defined. We report a one-year-old child with pulmonary atresia, ventricular septal defect, and a right coronary-pulmonary artery fistula who also had a diminutive, disconnected left pulmonary artery in addition to aortopulmonary collaterals. The patient underwent corrective surgery. However, the fate of diminutive pulmonary arteries is unknown. The literature was reviewed to explore the clinical or surgical implications of such fistulas for improved understanding and management in the future.

Palliative reconstruction of right ventricular outflow tract by using femoral vein allograft in a newborn with hypoplastic pulmonary artery and ventricular septal defect: case report

2018 ◽  
Vol 22 (2) ◽  
pp. 68
Author(s):  
Alexey V. Voytov ◽  
Alexander Yu. Omelchenko ◽  
Ilyya A. Soynov ◽  
Nataliya R. Nichay ◽  
Artem V. Gorbatyh ◽  
...  
Keyword(s):  
Informed Consent ◽  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Conflict Of Interest ◽  
Septal Defect ◽  
Femoral Vein ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Cardiac Surgeons ◽  
Vein Allograft

<p>Treating patients with pulmonary atresia and ventricular septal defect, who have hypoplastic native pulmonary arteries, is a challenge for cardiac surgeons. We have reported a case of successful pulmonary bed rehabilitation using a femoral vein allograft in a patient with pulmonary atresia and ventricular septal defect. No similar cases with a detailed description of surgery stages and pulmonary artery development supported by illustrative materials are described both in the literature. The strategy of rehabilitation of the native pulmonary artery bed facilitates antegrade pulmonary blood flow, increases native pulmonary arteries growth, contributes to successful implementation of staged endovascular interventions and allows for complete repair. The technique of “short” cardiopulmonary bypass with induced ventricular fibrillation is a safe and effective option for such patients. This clinical case might be useful in medical practice of pediatric cardiac surgeons involved in the problem of pulmonary artery rehabilitation.</p><p>Received 4 June 2018. Revised 4 July 2018. Accepted 5 July 2018.</p><p><strong>Informed consent:</strong> The legal representative (infant’s mother) has voluntarily given her written informed consent to use the infant’s medical data obtained in the course of treatment for scientific purposes and for their publication.</p><p><strong>Funding:</strong> The study had no sponsorship.</p><p><strong>Conflict of interest:</strong> Authors declare no conflict of interest.</p>

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 ◽  
...  
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.

Sign in / Sign up

Export Citation Format

Share Document