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Aortopulmonary Septal Defect

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Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand.

Kanthalas Lertsakulpiriya, Chodchanok Vijarnsorn, Prakul Chanthong, Paweena Chungsomprasong, Supaluck Kanjanauthai, Kritvikrom Durongpisitkul, Jarupim Soongswang, Thaworn Subtaweesin, Somchai Sriyoschati,

Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic congenital heart disease with a wide-range of presentations and treatment strategies, depending on the source of pulmonary circulation, anatomy of pulmonary arteries (PAs), and major aortopulmonary collateral arteries (MAPCAs). Data about the outcomes in developing countries is scarce. We ... Read more >>

Sci Rep (Scientific Reports)
[2020, 10(1):5165]

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Transcatheter closure of aortopulmonary window with Amplatzer duct occluder II: additional size.

Tayfun Uçar, Selen Karagözlü, Mehmet G Ramoğlu, Ercan Tutar,

Aortopulmonary window is a rare cardiac defect, and early management with surgery or transcatheter closure is lifesaving. Here, a 9-month-old patient, who underwent a successful device closure with additional size-Amplatzer duct occlude, is presented to make emphasis that it may be considered as the device of choice for defects in ... Read more >>

Cardiol Young (Cardiology in the young)
[2020, 30(3):424-426]

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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.

Katia Bravo-Jaimes, Brian Walton, Poyee Tung, Richard W Smalling,

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 ... Read more >>

Case Rep Cardiol (Case reports in cardiology)
[2020, 2020:4726529]

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Modified Yasui Operation Using Cryopreserved Femoral Vein Homograft.

Manan H Desai, Pranava Sinha, Richard A Jonas, Karthik Ramakrishnan,

The Yasui operation is indicated in interrupted aortic arch and a posterior mal-aligned ventricular septal defect with a narrow subaortic region. We present a modification of the Yasui procedure in which the aortic reconstruction was simplified using a non-valved cryopreserved femoral vein homograft to connect the pulmonary artery to the ... Read more >>

Ann. Thorac. Surg. (The Annals of thoracic surgery)
[2020, :]

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Aortopulmonary window with unreported interesting pulmonary artery intersection.

Mumun Sinha, Sheragaru Hanumanthappa Chandrashekhara, Arun Sharma,

Asian Cardiovasc Thorac Ann (Asian cardiovascular & thoracic annals)
[2020, 28(1):71-72]

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Systemic-to-pulmonary shunt vs right ventricle to pulmonary artery connection in the treatment of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries.

Fan Fan, Bo Peng, Zhimin Liu, Yinglong Liu, Qiang Wang,

OBJECTIVE:The systemic-to-pulmonary shunt (SPS) and right ventricle to pulmonary artery (RV-PA) connection were evaluated to pursue the goal of rehabilitating dysplastic native PAs via establishment of antegrade blood flow. However, the application of these two palliative operations was still confusing. We compared the two operations to determine their different effects ... Read more >>

J Card Surg (Journal of cardiac surgery)
[2020, 35(2):345-351]

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Effectiveness of Bidirectional Glenn Shunt Placement for Palliation in Complex Congenitally Corrected Transposed Great Arteries.

Kai Ma, Lei Qi, Zhongdong Hua, Keming Yang, Hao Zhang, Shoujun Li, Sen Zhang, Fengpu He, Guanxi Wang,

Surgery for complex congenitally corrected transposed great arteries is one of the greatest challenges in cardiovascular surgery. We report our experience with bidirectional Glenn shunt placement as a palliative procedure for complex congenitally corrected transposition. We retrospectively identified 50 consecutive patients who had been diagnosed with congenitally corrected transposition accompanied ... Read more >>

Tex Heart Inst J (Texas Heart Institute journal)
[2020, 47(1):15-22]

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Isolated persistence of the fifth aortic arch in an infant presenting with congestive heart failure.

Giovanni Meliota, Maristella Lombardi, Pierluigi Zaza, Maria Rosaria Tagliente, Paolo Versacci, Gabriele Scalzo, Ugo Vairo,

The persistence of the fifth aortic arch (PFAA) in postnatal life is an extremely rare and controversial cardiovascular malformation. PFAA is defined as an extra-pericardial vessel arising from the ascending aorta proximal to the origin of the brachiocephalic arteries, terminating either in the dorsal aorta or in the pulmonary arteries ... Read more >>

Ann Pediatr Cardiol (Annals of pediatric cardiology)
[2020, 13(1):91-94]

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Unifocalization cannot rely exclusively on native pulmonary arteries: the importance of recruitment of major aortopulmonary collaterals in 249 cases†.

David J Barron, Ramesh S Kutty, John Stickley, Oliver Stümper, Phil Botha, Natasha E Khan, Timothy J Jones, Nigel E Drury, William J Brawn,

OBJECTIVES:We sought to define the early and late outcomes of unifocalization based on a classification of the native pulmonary artery (nPA) system and major aortopulmonary collateral arteries (MAPCAs) with a policy of combined recruitment and rehabilitation and to analyse the role of unifocalization by leaving the ventricular septal defect (VSD) ... Read more >>

Eur J Cardiothorac Surg (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery)
[2019, 56(4):679-687]

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Asymptomatic phaeochromocytoma in a patient with Holt-Oram syndrome: a case report.

Perryn Ng, Doddabele Deepak, Lynette Teo, Ting Ting Low,

Background:Holt-Oram syndrome (HOS) is a rare congenital disease that affects the heart and upper limbs. Phaeochromocytoma, a catecholamine-secreting tumour, is a rare neuroendocrine disorder. We present an interesting case presentation of these two rare disorders in a patient who was asymptomatic for phaeochromocytoma. Case summary:A 28-year-old woman who was diagnosed ... Read more >>

Eur Heart J Case Rep (European heart journal. Case reports)
[2019, 3(4):1-5]

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Aortic Reconstruction Using a Main Pulmonary Artery Flap in an Isolated Aortopulmonary Window.

Hong Ju Shin, Younggi Jung, Jae Seung Shin,

Aortopulmonary window (APW) is a rare cardiac anomaly that was reported to occur in only 43 cases over 33 years at a large-volume cardiac center. It can present as an isolated anomaly or in combination with another cardiac anomaly. The surgical technique for APW has evolved from simple ligation to ... Read more >>

Korean J Thorac Cardiovasc Surg (The Korean journal of thoracic and cardiovascular surgery)
[2019, 52(4):236-238]

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Anomalous aortic origin of the pulmonary arteries: Case series and literature review.

Salvatore Agati, Carlos Guerra Sousa, Felice Davide Calvaruso, Rosanna Zanai, Ivana Campanella, Daniela Poli, Alfredo Di Pino, Luca Borro, Fiore Salvatore Iorio, Massimiliano Raponi, Robert H Anderson, Simone Reali, Andrea De Zorzi, Aurelio Secinaro,

Anomalous origin of the pulmonary arteries from the ascending aorta is a rare, but severe clinical entity necessitating a scrupulous evaluation. Either the right or the left pulmonary arteries can arise directly from the ascending aorta while the other pulmonary artery retains its origin from the right ventricular outflow tract. ... Read more >>

Ann Pediatr Cardiol (Annals of pediatric cardiology)
[2019, 12(3):248-253]

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Long-Term Assessment of Left Ventricular Ejection Fraction and Mitral Regurgitation Following Takeuchi Repair.

Gökmen Akkaya, Çağatay Bilen, Osman Nuri Tuncer, Mehmet Fatih Ayık, Yüksel Atay,

OBJECTIVE:This study aimed to evaluate the early operative outcomes and to compare the left ventricle and mitral valve functions after initial Takeuchi repair in patients with anomalous left coronary arising from pulmonary artery (ALCAPA). METHODS:Fourteen patients (5 males, 9 females; mean age 4.3 years, ranging from 25 days to 34 ... Read more >>

Braz J Cardiovasc Surg (Brazilian journal of cardiovascular surgery)
[2020, 34(6):687-693]

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Imaging of Pulmonary Atresia With Ventricular Septal Defect.

Ahmed Abdel Khalek Abdel Razek, Hala Al-Marsafawy, Maha Elmansy,

The aim of this article was to review computed tomography angiography and magnetic resonance angiography of pulmonary atresia with ventricular septal defect. This disorder is a rare complex congenital heart disease. Preoperative imaging of pulmonary atresia with ventricular septal defect with computed tomography angiography and magnetic resonance angiography is important ... Read more >>

J Comput Assist Tomogr (Journal of computer assisted tomography)
[2019, 43(6):906-911]

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Midline one-stage complete unifocalization early outcomes from a single center.

Oktay Korun, Okan Yurdakök, Mehmet Dedemoğlu, İlker Kemal Yücel, Ahmet Çelebi, Şefika Türkan Kudsioğlu, Ahmet Sasmazel, Numan Ali Aydemir,

OBJECTIVE:This study aims to present our experience with single-stage complete unifocalization and intraoperative flow study for the repair of ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. METHODS:This study was conducted through retrospective chart review of all the patients who underwent complete single-stage midline unifocalization in a single ... Read more >>

Anatol J Cardiol (Anatolian journal of cardiology)
[2019, 22(3):125-131]

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Stationary tissue background correction increases the precision of clinical evaluation of intra-cardiac shunts by cardiovascular magnetic resonance.

Jannike Nickander, Magnus Lundin, Goran Abdula, Jonas Jenner, Eva Maret, Peder Sörensson, Einar Heiberg, Andreas Sigfridsson, Martin Ugander,

We aimed to evaluate the clinical utility of stationary tissue background phase correction for affecting precision in the measurement of Qp/Qs by cardiovascular magnetic resonance (CMR). We enrolled consecutive patients (n = 91) referred for CMR at 1.5T without suspicion of cardiac shunt, and patients (n = 10) with verified cardiac shunts in this ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):5053]

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Novel JAG1 Deletion Variant in Patient with Atypical Alagille Syndrome.

Emanuele Micaglio, Andreea Alina Andronache, Paola Carrera, Michelle M Monasky, Emanuela T Locati, Barbara Pirola, Silvia Presi, Mario Carminati, Maurizio Ferrari, Alessandro Giamberti, Carlo Pappone,

Alagille syndrome (AGS) is an autosomal-dominant disorder characterized by various degrees of abnormalities in the liver, heart, eyes, vertebrae, kidneys, face, vasculature, skeleton, and pancreas. This case report describes a newborn child exhibiting a congenital neural tube defect and peculiar craniofacial appearance characterized by a prominent forehead, deep-set eyes, bulbous ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2019, 20(24):]

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Successful staged repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries in an extremely low birth weight infant.

Yujiro Ide, Masaya Murata, Maiko Tachi, Hiroki Ito, Mayumi Nagasawa, Mizuhiko Ishigaki, Sung-Hae Kim, Masaki Nii, Yasuhiko Tanaka, Kisaburo Sakamoto,

An extremely low birth weight infant (810 g) was born with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries accompanied by pulmonary overcirculation, which eventually resulted in intestinal hypoperfusion and focal intestinal perforation from the very early stage of life. Based on the echographic findings, we performed banding operations ... Read more >>

Gen Thorac Cardiovasc Surg (General thoracic and cardiovascular surgery)
[2019, :]

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Fatal thromboembolism of major aortopulmonary collateral arteries.

Anna Migdał, Małgorzata Żuk, Maria Zubrzycka, Bożena Rewers, Adam Koleśnik, Grażyna Brzezińska-Rajszys,

Kardiol Pol (Kardiologia polska)
[2019, 77(5):574-575]

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Assessment of intracardiac and extracardiac anomalies associated with coarctation of aorta and interrupted aortic arch using dual-source computed tomography.

Qin Zhao, Jin Wang, Zhi-Gang Yang, Ke Shi, Kai-Yue Diao, Shan Huang, Meng-Ting Shen, Ying-Kun Guo,

To evaluate the value of dual-source computed tomography (DSCT) compared with transthoracic echocardiography (TTE) in assessing intracardiac and extracardiac anomalies in patients with coarctation of aorta (CoA) and interrupted aortic arch (IAA). Seventy-five patients (63 with CoA and 12 with IAA) who received preoperative DSCT and TTE were retrospectively studied. ... Read more >>

Sci Rep (Scientific reports)
[2019, 9(1):11656]

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

Julia Illner, Holger Reinecke, Helmut Baumgartner, Gerrit Kaleschke,

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 ... Read more >>

Eur Heart J Case Rep (European heart journal. Case reports)
[2019, 3(4):1-4]

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Aortopulmonary window in adults: A rare entity leading to Eisenmenger syndrome.

Joud El Dick, Issam El-Rassi, Christelle Tayeh, Fadi Bitar, Mariam Arabi,

An aortopulmonary window (APW) is a rare congenital heart defect involving an abnormal communication between the ascending aorta and the pulmonary trunk with separate aortic and pulmonary valves. This defect accounts for 0.2% of all congenital cardiac anomalies and if left untreated can lead to Eisenmenger syndrome, severe pulmonary hypertension, ... Read more >>

Echocardiography (Echocardiography (Mount Kisco, N.Y.))
[2019, 36(6):1173-1178]

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Perfusion Methods and Modifications to the Cardiopulmonary Bypass Circuit for Midline Unifocalization Procedures.

Tristan D Margetson, Justin Sleasman, Sami Kollmann, Patrick J McCarthy, Ozzie Jahadi, Don Sheff, Paul Shuttleworth, Richard D Mainwaring, Frank L Hanley,

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a rare form of congenital heart disease. The midline unifocalization procedure has been developed for the treatment of PA/VSD/MAPCAs. These are complex and very lengthy procedures that require an entirely different method of perfusion. The purpose of ... Read more >>

J Extra Corpor Technol (The journal of extra-corporeal technology)
[2019, 51(3):147-152]

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Counseling for Prenatal Congenital Heart Disease-Recommendations Based on Empirical Assessment of Counseling Success.

Alexander Kovacevic, Andreas Simmelbauer, Sebastian Starystach, Michael Elsässer, Andreas Müller, Stefan Bär, Matthias Gorenflo,

Objectives: Empirical assessment of parental needs and affecting factors for counseling success after prenatal diagnosis of congenital heart disease (CHD). Methods:Counseling success after fetal diagnosis of CHD was assessed by a validated standardized questionnaire. The dependent variable "Effective Counseling" was measured in five created analytical dimensions (1. "Transfer of Medical ... Read more >>

Front Pediatr (Frontiers in Pediatrics)
[2020, 8:26]

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Outcomes of different rehabilitative procedures in patients with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries.

Dong Zhao, Keming Yang, Shoujun Li, Jun Yan, Zhongdong Hua, Nengxin Fang, Wenjun Su, Xiaodong Lv, Bing Yu,

OBJECTIVES:The objective of this study was to compare our clinical outcomes of the central shunt and the right ventricle-pulmonary artery (RV-PA) connection in patients with pulmonary atresia, ventricular septal defect and the major aortopulmonary collateral arteries. METHODS:From November 2009 to October 2017, a total of 157 consecutive patients with pulmonary ... Read more >>

Eur J Cardiothorac Surg (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery)
[2019, 55(5):837-844]

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