Coronary to pulmonary artery collaterals in patients with pulmonary atresia and ventricular septal defect

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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Double aortic arch: an elusive diagnosis in a child with CHD

Cardiology in the Young ◽

10.1017/s1047951121004856 ◽

2021 ◽

pp. 1-3

Author(s):

Mariana Lemos ◽

Miguel Fogaça da Mata ◽

Ana Coutinho Santos

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Aortic Arch ◽

Ct Angiography ◽

Septal Defect ◽

Vascular Ring ◽

Pulmonary Atresia ◽

Double Aortic Arch ◽

Shunt Surgery

Abstract An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.

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Stenting of modified Blalock–Taussig shunt in adult with palliated pulmonary atresia and ventricular septal defect: a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytz201 ◽

2019 ◽

Vol 3 (4) ◽

pp. 1-4

Author(s):

Julia Illner ◽

Holger Reinecke ◽

Helmut Baumgartner ◽

Gerrit Kaleschke

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Congenital Heart ◽

Septal Defect ◽

Pulmonary Atresia ◽

Complex Congenital Heart Disease ◽

Bare Metal

Abstract Background Adults with complex congenital heart disease palliated with systemic-to-pulmonary artery shunts have become rare and represent a particularly challenging patient group for the cardiologist. One of the complications and causes of severe clinical deterioration during long-term follow-up are progressive obstruction or total occlusion of the shunt. The risk for surgical intervention is frequently high and catheter intervention may be complicated by complex anatomy and shunt calcification. Case summary We report the case of a 47-year-old man with uncorrected (palliated) pulmonary atresia and ventricular septal defect who presented with progressive cyanosis (oxygen saturation 69%) and decreasing exercise capacity. Computed tomography revealed a totally occluded modified left Blalock–Taussig (BT) shunt and a severely stenosed central shunt (Waterston–Cooley) in a patient with confluent but hypoplastic pulmonary arteries and multiple major aortic pulmonary collaterals. Due to a high operative risk, an interventional, percutaneous approach was preferred to re-do surgery. From a radial access the calcified BT shunt could be crossed with a hydrophilic guidewire. Then, a rotational thrombectomy, balloon dilatation, and bare-metal stenting at the proximal and distal anastomoses were performed. Post-interventionally, peripheral oxygen saturation increased from 69% to 82%. Clopidogrel was administered for 1 month after bare-metal stenting. At 1-year follow-up, the BT shunt was still patent on echocardiography and exercise tolerance markedly improved. Discussion This case highlights the benefit of percutaneous rotational thrombectomy followed by stenting of chronically occluded systemic-to-pulmonary artery shunts for further palliation in adult patients with complex congenital heart disease not suitable for surgical repair.

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An Unusual Cause of Hypoxia: Ventricular Septal Defect, Pulmonary Artery Atresia, and Major Aortopulmonary Collaterals Diagnosed in the Adult Cardiac Catheterization Lab

Case Reports in Cardiology ◽

10.1155/2020/4726529 ◽

2020 ◽

Vol 2020 ◽

pp. 1-3

Author(s):

Katia Bravo-Jaimes ◽

Brian Walton ◽

Poyee Tung ◽

Richard W. Smalling

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Cardiac Catheterization ◽

Mortality Risk ◽

Septal Defect ◽

Pulmonary Atresia ◽

High Mortality Risk ◽

Catheterization Laboratory ◽

Aortopulmonary Collaterals ◽

Major Aortopulmonary Collaterals

The association of pulmonary atresia, ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCA) is an extreme form of tetralogy of Fallot (TOF). It carries a high mortality risk if not intervened on during infancy with only 20% of unoperated patients surviving into adulthood. We present the case of a 40-year-old man who presented for evaluation prior to retinal surgery and was found to have hypoxia and a loud murmur. Cardiac catheterization was performed in the general catheterization laboratory, demonstrating a membranous VSD, pulmonary atresia, and MAPCA. We highlight the challenges and limitations that an adult interventional cardiologist may have when encountering these patients.

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Coronary artery to pulmonary artery communications in pulmonary atresia with ventricular septal defect

Indian Heart Journal ◽

10.1016/j.ihj.2013.08.017 ◽

2013 ◽

Vol 65 (5) ◽

pp. 636-638

Author(s):

Anuradha Sridhar ◽

Raghavan Subramanyan ◽

Kotturathu Mammen Cherian

Keyword(s):

Coronary Artery ◽

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Septal Defect ◽

Pulmonary Atresia

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Coronary artery to pulmonary artery collateral as the principal source of pulmonary blood supply in pulmonary atresia and ventricular septal defect with absent left pulmonary artery

Cardiology in the Young ◽

10.1017/s1047951120003315 ◽

2020 ◽

Vol 30 (11) ◽

pp. 1728-1729

Author(s):

Ibrahima S. Barry ◽

Stefano Di Bernardo ◽

Milan Prša

Keyword(s):

Coronary Artery ◽

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Blood Supply ◽

Septal Defect ◽

Pulmonary Atresia ◽

Left Pulmonary Artery ◽

Principal Source ◽

Absent Left Pulmonary Artery

AbstractPulmonary atresia and ventricular septal defect is associated with variable sources of pulmonary blood supply. We present a case of a coronary artery to pulmonary artery collateral as the principal source of pulmonary blood supply.

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