Pulmonary atresia with ventricular septal defect

Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare cyanotic congenital heart malformation characterized by underdevelopment of the right ventricular outflow tract and atresia of the pulmonary valve, ventricular septal defect (VSD) and pulmonary collateral vessels. Clinical features depend on the anatomic variability of the lesion and patients may be minimally symptomatic, severely cyanotic or may develop congestive heart failure. PA-VSD may represent a severe form of Tetralogy of Fallot (see this term).

Aortopulmonary collateral arteries

Small ectopic arteries or arterial branches that connect the aorta, aortic branches and/or subclavian artery regions directly to the lung parenchyma, usually seen in conjunction with pulmonary atresia, ventricular septal defect (VSD) and/or closed ductus arteriosus.


Total: 11

                       


(per page)
PMID (PMCID)
30764888
 FEMALE Infant, Newborn
Utilisation of a three-dimensional printed model for the management of coronary-pulmonary artery fistula from left main coronary artery.
Misra A, Walters HL, Kobayashi D.
Cardiol Young. 2019;29(3):431-434.
Coronary-pulmonary artery fistula is a rare anomaly in which an aortopulmonary collateral artery arises from a coronary artery, often seen in patients with pulmonary atresia with ventricular septal defect.
28116756
 FEMALE
Branch pulmonary artery peel operation in a patient without a native intrapericardial pulmonary artery.
Kim H, Sung SC, Choi KH, Lee HD, Ban GH, Kim G, Kim HY.
J Card Surg. 2017;32(2):145-147.
A patient with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries without an intrapericardial pulmonary artery (PA) underwent a one-stage total correction, including both branch PA reconstructions, with a bovine pericardial roll at the age of 42 months.
25952246
 MALE Infant
Artery fistula causing aortic regurgitation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.
Furutake T, Koizumi J, Iwase T, Kamada T, Takahashi S, Oyama K, Okabayashi H, Ikai A.
Ann Thorac Surg. 2015;99(5):e121-3.
We report a case of aortic regurgitation (AR), coronary artery-to-pulmonary artery (CAPA) fistula, pulmonary atresia with ventricular septal defect (PA/VSD), and major aortopulmonary collateral arteries (MAPCAS).
26011192
 FEMALE Infant
Implantation of an Absorb bioresorbable vascular scaffold in the stenotic aortopulmonary collateral artery of a young child with Alagille syndrome.
Castro Rodriguez J, Dessy H, Demanet H.
Catheter Cardiovasc Interv. 2015;86(2):E76-80.
We report our experience with implantation of an Absorb bioresorbable stent in the stenotic major aortopulmonary collateral artery (MAPCA) of a 1-year-old girl born with pulmonary atresia with ventricular septal defect.
24958054
 MALE Adult
Combined Rastelli and Bentall Procedures for Pulmonary Atresia With Ventricular Septal Defect and a Large Aortopulmonary Collateral Artery and Ascending Aorta Aneurysm.
Chiostri B, Piccinini F, Vrancic M, Klinger D, Navia D, Lalor N, Longo F, Kreutzer C.
World J Pediatr Congenit Heart Surg. 2014;5(3):467-9.
Combined Rastelli and Bentall Procedures for Pulmonary Atresia With Ventricular Septal Defect and a Large Aortopulmonary Collateral Artery and Ascending Aorta Aneurysm.
17052781
 FEMALE
Successful use of a new Amplatzer Vascular plug for percutaneous closure of a large aortopulmonary collateral artery in a pulmonary atresia with ventricular septal defect prior to complete repair.
Tissot C, da Cruz E, Beghetti M, Aggoun Y.
Int J Cardiol. 2007;116(2):e39-41.
Successful use of a new Amplatzer Vascular plug for percutaneous closure of a large aortopulmonary collateral artery in a pulmonary atresia with ventricular septal defect prior to complete repair.
10339934
 FEMALE Infant
[Anesthetic management of an infant with pulmonary atresia and ventricular septal defect accompanied by excess pulmonary blood flow for systemic-pulmonary shunt operation].
Ataka T, Fukuda S, Kinoshita H, Kurokawa S, Kitahara Y, Shimoji K, Watanabe H, Sato S.
Masui. 1999;48(4):372-6.
A 6-month-old infant had pulmonary atresia with ventricular septal defect (PA + VSD) accompanied by excess pulmonary artery flow via major aortopulmonary collateral artery, underwent aortopulmonary shunt with artificial graft under midazolam-fentanyl anesthesia.
9068403
 MIXED_SAMPLE Infant, Newborn
Pulmonary atresia, "intact ventricular septum", and aortopulmonary collateral arteries.
Mildner RJ, Kiraly L, Sreeram N.
Heart. 1997;77(2):173-5.
In muscular pulmonary atresia, major aortopulmonary collateral arteries are characteristic of pulmonary atresia with ventricular septal defect and are rarely seen in pulmonary atresia with intact ventricular septum.
8811410
 FEMALE
One-stage repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries by a handmade trifurcated valved conduit.
Yang YJ, Wu JM, Tsai LM, Luo CY.
J Card Surg. 1996;11(2):151-4.
One-stage repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries by a handmade trifurcated valved conduit.
8811410
 FEMALE
One-stage repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries by a handmade trifurcated valved conduit.
Yang YJ, Wu JM, Tsai LM, Luo CY.
J Card Surg. 1996;11(2):151-4.
An 18-year-old girl with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (MAPCAs) was reported.
8283091
 FEMALE Child
[Surgical treatment of the patients with pulmonary atresia, ventricular septal defect associated with major aorto-pulmonary collateral arteries--a report of two cases].
Sangawa K, Oba O, Nakanishi K, Okada M, Yoshida H, Shiote A.
Nihon Kyobu Geka Gakkai Zasshi. 1993;41(11):2196-201.
Two patients undergoing corrective operation for pulmonary atresia with ventricular septal defect associated with major aortopulmonary collateral arteries (MAPCAs) were reported.