scholarly journals Complete repair of pulmonary atresia, ventricular septal defect, and severe peripheral arborization abnormalities of the central pulmonary arteries

1989 ◽  
Vol 98 (6) ◽  
pp. 1018-1029 ◽  
Author(s):  
Francisco J. Puga ◽  
Franco E. Leoni ◽  
Paul R. Julsrud ◽  
Douglas D. Mair
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
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 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

scholarly journals A central shunt to rehabilitate diminutive pulmonary arteries in patients with pulmonary atresia with ventricular septal defect

2015 ◽  
Vol 149 (2) ◽  
pp. 515-520.e1 ◽  
Author(s):  
Hyungtae Kim ◽  
Si Chan Sung ◽  
Kwang Ho Choi ◽  
Hyoung Doo Lee ◽  
Gil Ho Ban ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia

scholarly journals Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto–pulmonary collaterals

2001 ◽  
Vol 20 (3) ◽  
pp. 590-597 ◽  
Author(s):  
Dominique Metras ◽  
Philippe Chetaille ◽  
Bernard Kreitmann ◽  
Alain Fraisse ◽  
Olivier Ghez ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia

scholarly journals Staged Correction of Pulmonary Atresia, Ventricular Septal Defect and Collateral Arteries

2021 ◽  
Author(s):  
Pieter van de Woestijne ◽  
M. Mokhles ◽  
Ingrid van Beynum ◽  
Peter de Jong ◽  
Jeroen Wilschut ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Pulmonary Vasculature ◽  
Collateral Arteries ◽  
Staged Approach ◽  
The Right ◽  
Rv Function

Objectives Pulmonary atresia (PA) with ventricular septal defect (VSD) and systemic-pulmonary collateral arteries (SPCA’s) has a variable anatomy with regard to the pulmonary vasculature, asking for an individualized surgical treatment. A protocol was applied consisting of staged unifocalization and correction. Methods Since 1989 39 consecutive patients were included (median age at first operation 13 months). In selected cases a central aorto-pulmonary shunt was performed as first procedure. Unifocalization procedures were performed through a lateral thoracotomy. Correction consisted of shunt takedown, VSD closure and interposition of an allograft between the right ventricle and the reconstructed pulmonary artery. Postoperatively and at follow up echocardiographic data were obtained. Results In 39 patients 66 unifocalization procedures were performed. Early mortality was 5%. Seven patients were considered not suitable for correction, four of them died. One patient is awaiting further correction. Correction was done successfully in 28 patients. Operative mortality was 3% and late mortality 11%. Median follow-up after correction was 19 years. Eleven patients needed homograft replacement. Freedom from conduit replacement was 88%, 73% and 60% at 5, 10 and 15 years respectively. Right ventricular function was reasonable or good in 75 % of the patients. Conclusions After complete unifocalization 30/37 patients (81%) were considered correctable. The main reasons for palliative treatment without correction were pulmonary hypertension and/or inadequate outgrowth of pulmonary arteries. Staged approach of PA, VSD and SPCA’s results in adequate correction and good functional capacity. RV function after correction remains reasonable or good in the majority of patients.

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