Efficacy of bosentan therapy for segmental pulmonary artery hypertension due to major aortopulmonary collateral arteries in children

2012 ◽  
Vol 161 (1) ◽  
pp. e1-e3 ◽  
Author(s):  
Kenichiro Yamamura ◽  
Hazumu Nagata ◽  
Kazuyuki Ikeda ◽  
Kenji Ihara ◽  
Toshiro Hara
Keyword(s):  
Pulmonary Artery ◽  
Pulmonary Artery Hypertension ◽  
Major Aortopulmonary Collateral Arteries ◽  
Collateral Arteries ◽  
Aortopulmonary Collateral

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.

Stenting of the right ventricular outflow tract as an initial intervention in Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries

2020 ◽  
pp. 1-8
Author(s):  
Mohammad Abumehdi ◽  
Deepa Sasikumar ◽  
Milind Chaudhari ◽  
Vinay Bhole ◽  
Phil Botha ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Pulmonary Stenosis ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Major Aortopulmonary Collateral Arteries ◽  
Collateral Arteries ◽  
Aortopulmonary Collateral

Abstract Objectives: To assess the role of right ventricular outflow tract stenting as the primary intervention in Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries. Background: The management of a subset of infants with Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries requires a staged approach including rehabilitation of diminutive native pulmonary arteries, conventionally using an aortopulmonary shunt. We share our experience of pulmonary artery rehabilitation with right ventricular outflow tract stenting. Methods: Retrospective review of all patients with Tetralogy of Fallot with pulmonary stenosis who underwent right ventricular outflow tract stenting as primary intervention over an 8-year period. Results: Ten patients underwent right ventricular outflow tract stent insertion at a median age of 61 days (interquartile range (IQR) 8.3–155 days). Median weight at stent deployment was 4.2 kg (IQR 3.2–5.7 kg). Oxygen saturations improved from a median of 79% (IQR 76–80%) to 92% (IQR 90–95%), p = 0.012. The median right and left pulmonary artery z score increased from −3.51 (IQR −4.59 to −2.80) and −2.07 (IQR −3.72 to 0.15) to a median of −1.17 (IQR −2.26 to 0.16) p < 0.05, and 0.24 (IQR −1.09 to 1.84) p < 0.05, respectively, at subsequent angiogram. Nine patients underwent further catheterisation. Four patients underwent complete anatomical repair. Only one patient required unifocalisation, as most patients had a native supply to all-important lung segments. Conclusion: Right ventricular outflow tract stenting is a useful procedure in the subset of patients with Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries, where native pulmonary arterial growth is required to facilitate repair.

Prenatal ultrasound diagnosis of pulmonary atresia with a ventricular septal defect and major aortopulmonary collateral arteries (MARCA)

2020 ◽  
Author(s):  
V.V. Ezhova
Keyword(s):  
Prenatal Diagnosis ◽  
Surgical Treatment ◽  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Type B ◽  
Major Aortopulmonary Collateral Arteries ◽  
Collateral Arteries ◽  
Aortopulmonary Collateral

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.

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