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Pulmonary atresia

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https://www.readbyqxmd.com/read/29111286/role-of-computed-tomography-angiography-in-the-differentiation-of-feline-truncus-arteriosus-communis-from-pulmonary-atresia-with-ventricular-septal-defect
#1
Lauren E Markovic, Brian A Scansen, Brianna M Potter
Two domestic shorthair cats, a 6-month-old castrated male and a 7-month-old intact female, were diagnosed with complex congenital heart disease. Transthoracic echocardiography in both cats revealed a dilated arterial trunk overriding the interventricular septum with a large ventricular septal defect. The pulmonary trunk and branch pulmonary arteries were not visible using standard echocardiographic views in either cat. The differential diagnosis for both cats included truncus arteriosus communis vs. pulmonary atresia with ventricular septal defect...
October 27, 2017: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
https://www.readbyqxmd.com/read/29095737/long-term-right-ventricular-assist-device-therapy-in-an-adult-with-pulmonary-atresia-intact-ventricular-septum
#2
Joshua L Hermsen, Karen K Stout, April Stempien-Otero, Edward D Verrier, Nahush A Mokadam
Durable ventricular assist device (VAD) support is uncommonly employed in adult congenital heart disease and often involves supporting a systemic right ventricle (RV). Ventricular assist device support of a subpulmonic RV is even more unusual.
October 31, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/29050815/right-ventricular-outflow-tract-reintervention-after-primary-tetralogy-of-fallot-repair-in-neonates-and-young-infants
#3
Shyamasundar Balasubramanya, David Zurakowski, Michele Borisuk, Aditya K Kaza, Sitaram M Emani, Pedro J Del Nido, Christopher W Baird
OBJECTIVE: To assess the outcomes following primary tetralogy of Fallot (TOF) repair in neonates and young infants with pulmonary stenosis (PS) and pulmonary atresia and compare differences in reintervention on the right ventricular outflow tract (RVOT) among those undergoing valve sparing repair (VSR), transannular RVOT patch (TAP), and right ventricle-to-pulmonary artery (RV-PA) conduit surgeries. METHODS: Data were collected retrospectively in 101 patients who underwent TOF repair over a 10-year period between January 2005 and September 2015...
September 19, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29049818/dor-procedure-for-pulmonary-atresia-with-intact-ventricular-septum-in-an-infant
#4
Yoonjin Kang, Jae Gun Kwak, Eung Rae Kim, Woong-Han Kim
Pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation confers a high risk for myocardial infarction and complications of ischaemia, including left ventricle aneurysms. We describe an infant with pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation and a left ventricle apical aneurysm who successfully underwent the Dor procedure and a subsequent Fontan operation. Surgery for postinfarction left ventricle aneurysm can be considered in infants...
September 2, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29049701/effects-of-blood-flow-dynamics-on-autologous-pericardial-degeneration-in-reconstructed-pulmonary-arteries
#5
Nobuyasu Kato, Masaaki Yamagishi, Keiichi Itatani, Takako Miyazaki, Yoshinobu Maeda, Satoshi Asada, Yoshiro Matsui, Hitoshi Yaku
OBJECTIVES: To clarify the risk factors for abnormal degeneration of autologous pericardium, the mechanical stress on the endothelial tissue caused by turbulent blood flow in the pulmonary artery (PA) reconstructed with autologous pericardium and major aortopulmonary collateral arteries was assumed in pulmonary atresia and ventricular septal defect. METHODS: Patient-specific PA models were created for 6 patients based on their past computed tomography images taken after unifocalization of major aortopulmonary collateral arteries...
October 12, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29049668/one-stage-definitive-repair-of-complete-atrioventricular-septal-defect-and-pulmonary-atresia-with-major-aortopulmonary-collateral-arteries
#6
Yujiro Ide, Masaya Murata, Hiroki Ito, Kisaburo Sakamoto
Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is considered the most severe end of the tetralogy of Fallot spectrum, whereas complete atrioventricular septal defect associated with tetralogy of Fallot is a rare congenital defect. However, the combination of pulmonary atresia, major aortopulmonary collateral artery and complete atrioventricular septal defect is extremely rare, and surgical repair has not been reported. We describe a successful, 1-stage correction in a 7-month-old boy with this condition, who had an additional complication of a single aortic outlet from the right ventricle and significant atrioventricular valve regurgitation...
October 11, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29047321/perioperative-and-anesthetic-considerations-in-pulmonary-atresia-with-intact-ventricular-septum
#7
Stephen Gleich, Gregory J Latham, Denise Joffe, Faith J Ross
Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare right-heart obstructive lesion with a wide anatomic and physiologic spectrum of disease, ranging from simple membranous pulmonary valve atresia with a fully developed right ventricle (RV) to a severely hypoplastic RV and ventriculocoronary (RV-coronary) fistulas. Affected neonates are dependent on prostaglandin for adequate pulmonary blood flow. Depending on the severity of disease, treatment options range from transcatheter pulmonary valve perforation and ultimate biventricular repair to staged single-ventricle palliation...
October 1, 2017: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29021485/spontaneous-regression-of-epithelioid-angiosarcoma-in-a-young-woman
#8
Suya Hori, Motoko Tachihara, Daisuke Tamura, Kazuyuki Kobayashi, Kyosuke Nakata, Hiroshi Kamiryo, Yasuhiro Sakai, Tomoo Itoh, Takanori Hirose, Yoshihiro Nishimura
A 20-year-old Japanese woman with a history of pulmonary atresia was referred to our hospital after the detection of an abnormal mass in the right lung and mediastinal lymphadenopathy. A cytological specimen obtained by transbronchial brushing indicated that the pathological diagnosis was non-small cell lung cancer. During the follow-up period, the tumor spontaneously regressed. At four months after the diagnosis, she experienced sudden bleeding from the small intestine. The histological characteristics of the small intestine tumor were compatible with the cytological characteristics of the lung tumor...
October 11, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29019966/transthoracic-balloon-pulmonary-valvuloplasty-for-treatment-of-congenial-pulmonary-atresia-patients-with-intact-ventricular-septum
#9
Zhi-Qin Lin, Qiang Chen, Hua Cao, Liang-Wan Chen, Gui-Can Zhang, Dao-Zhong Chen, Qin-Min Wang, Han-Fan Qiu, Dong-Shan Liao, Feng Lin
BACKGROUND To summarize our clinical experience in performing transthoracic balloon pulmonary valvuloplasty for the treatment of patients suffering from congenial pulmonary atresia with intact ventricular septum (PA/IVS). MATERIAL AND METHODS Between April 2009 and April 2016, 38 patients with PA/IVS underwent transthoracic balloon pulmonary valvuloplasty in our hospital. All of them were combined with patent ductus arteriosus, tricuspid insufficiency, and atrial septal defect or patent foramen ovale. The valvuloplasty was performed from the right ventricular outflow tract through a median sternotomy incision under TEE guidance for all cases...
October 11, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28987686/can-the-pine-needle-sign-on-computed-tomography-be-used-to-differentiate-pulmonary-arteriovenous-malformation-from-its-mimics-analysis-based-on-dynamic-contrast-enhanced-chest-computed-tomography-in-adults
#10
Koji Tokunaga, Takeshi Kubo, Toshihide Yamaoka, Hiroyoshi Isoda, Kaori Togashi
OBJECTIVES/PURPOSE: To determine the diagnostic value of morphological features on computed tomography (CT) in the differentiation of pulmonary arteriovenous malformation (pAVM) and its mimics. MATERIALS AND METHODS: We retrospectively examined 59 consecutive patients (109 lesions) with initially suspected or occult pAVM who underwent contrast-enhanced chest CT from January 2006 to June 2016. All lesions were divided into two groups based on their diagnosis: AVM (n=93) and non-AVM (n=16)...
October 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/28983709/late-complications-of-biliary-atresia-hepatopulmonary-syndrome-and-portopulmonary-hypertension
#11
Frederick M Karrer, Bradley J Wallace, Arturo E Estrada
Children with biliary atresia (BA) following Kasai portoenterostomy have a high risk for portal hypertension, however, while variceal and hemorrhagic complications have been more commonly studied, less frequent but no less possibly devastating complications of hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPH) remain less well understood. HPS and PPH both occur in a setting of portal hypertension, however, paradoxically patients with HPS develop pulmonic vasculature dilation leading to shunting and hypoxia, while those with PPH develop an opposite progression of pulmonary vasoconstriction eventually leading to cor pulmonale and decompensation...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28953251/respiratory-morbidity-in-children-with-repaired-congenital-esophageal-atresia-with-or-without-tracheoesophageal-fistula
#12
REVIEW
Maria Francesca Patria, Stefano Ghislanzoni, Francesco Macchini, Mara Lelii, Alessandro Mori, Ernesto Leva, Nicola Principi, Susanna Esposito
Congenital esophageal atresia with or without tracheoesophageal fistula (CEA ± TEF) is a relatively common malformation that occurs in 1 of 2500-4500 live births. Despite the refinement of surgical techniques, a considerable proportion of children experience short- and long-term respiratory complications, which can significantly affect their health through adulthood. This review focuses on the underlying mechanisms and clinical presentation of respiratory morbidity in children with repaired CEA ± TEF. The reasons for the short-term pulmonary impairments are multifactorial and related to the surgical complications, such as anastomotic leaks, stenosis, and recurrence of fistula...
September 27, 2017: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/28928622/central-perforation-of-atretic-pulmonary-valve-using-coronary-microcatheter
#13
Saurabh Kumar Gupta, Rajnish Juneja, Anita Saxena
Percutaneous perforation of pulmonary valve, using 0.014" guidewires meant for coronary artery chronic total occlusion (CTO), is increasingly being performed for select cases of pulmonary atresia with intact ventricular septum (PA-IVS). Despite growing experience, procedural failures and complications are not uncommon. Even in infants treated successfully, the orifice created in the atretic pulmonary valve is eccentric. In this report, we present usefulness of coronary microcatheter in alignment of perforating coronary guidewire to the center of atretic pulmonary valve resulting in central perforation...
September 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28928619/percutaneous-management-of-complex-acquired-aortic-coarctation-in-an-adult-with-tetralogy-of-fallot-and-pulmonary-atresia
#14
Michael D Seckeler, Emily Lawson, Brent J Barber, Scott E Klewer
We present the case of a female adult with complex cyanotic congenital heart disease who had long-standing thoracic aortic obstruction due to scarring from earlier surgical procedures. She was symptomatic but felt to be too high risk for surgical intervention. With careful planning, she was able to undergo successful stenting of her aorta with subsequent clinical improvement. This case highlights some of the complexities of caring for adults with congenital heart disease and the importance of a thorough understanding of their anatomy and physiology and prior interventions before undertaking interventions...
September 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28915526/-exit-a-possible-intervention-for-new-and-earlyborn-babies-with-severe-hydrops-fetalis-and-hydrothoraces-on-both-sides
#15
Sandra Koch, Jochen Essers, Ortraud Beringer, Frank Reister, Helmut Hummler, Anja Moewes
The EXIT (ex utero intrapartum treatment) procedure is an established method of respiratory protection, originally used in the delivery of fetuses with congenital obstructive airway diseases (tumors in the throat area, hygromas, so-called congenital high airway obstruction syndrome (CHAOS)). Meanwhile, the procedure is also carried out in large perinatal centers for pronounced diaphragmatic hernia or other special indications (EXIT to ECMO, congenital lung airway malformations (CCAM), pulmonary atresia). We present our experience with adapted EXIT procedures in 5 preterm infants with secondary generalized hydrops fetalis and pronounced bilateral hydrothoraces...
September 15, 2017: Zeitschrift Für Geburtshilfe und Neonatologie
https://www.readbyqxmd.com/read/28914345/comparison-of-clinical-outcomes-between-open-and-thoracoscopic-repair-for-esophageal-atresia-with-tracheoesophageal-fistula-a-systematic-review-and-meta-analysis
#16
Yuhao Wu, Hongyu Kuang, Tiewei Lv, Chun Wu
OBJECTIVE: A meta-analysis was performed for a comparison of outcomes between open repair (OR) and thoracoscopic repair (TR) for esophageal atresia with tracheoesophageal fistula (EA with TEF). METHODS: Electronic databases, including PubMed, Cochrane Library, and Medline, were searched systematically for the literatures aimed mainly at comparing the therapeutic effects for EA with TEF administrated by OR and TR. Corresponding data sets were extracted and two reviewers independently assessed the methodological quality...
November 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28912834/molecular-and-clinical-characterization-of-new-patient-with-1-08%C3%A2-mb-deletion-in-10p15-3-region
#17
Anna Poluha, Joanna Bernaciak, Ilona Jaszczuk, Marta Kędzior, Beata Anna Nowakowska
BACKGROUND: Three distinct contiguous gene deletion syndromes are located at 10p chromosomal region. The deletion, involving 10p15.3 region, has been characterized by (DeScipio et al., Am J Med Genet A 158A:2152-61, 2012). However, because of the variation in size of the described deletions and lack of knowledge about the involved genes, the correlation between genotypes and patients' phenotypes remains unknown. CASE PRESENTATION: We describe female patient with de novo 1,08 Mb deletion in 10p15...
2017: Molecular Cytogenetics
https://www.readbyqxmd.com/read/28901225/promoting-pulmonary-arterial-growth-via-right-ventricle-to-pulmonary-artery-connection-in-children-with-pulmonary-atresia-ventricular-septal-defect-and-hypoplastic-pulmonary-arteries
#18
Edon J Rabinowitz, Shilpi Epstein, Nina Kohn, David B Meyer
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...
September 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28898455/coronary-pulmonary-arterial-fistula-in-a-neonate-with-pulmonary-atresia-ventricular-septal-defect-and-single-coronary-artery
#19
Naif Alkhushi, Osman O Al-Radi, Amr Ajlan, Gaser Abdelmohsen, Wael Attia
In cases of pulmonary atresia with ventricular septal defect (PA-VSD), coronary-pulmonary arterial fistula (CPAF) as the main source of pulmonary blood supply is extremely rare. These fistulae may arise from the left coronary artery, right coronary artery, or a single coronary artery. Fistulae from a single coronary artery are unusual. We are reporting a case of PA-VSD with single coronary artery and CPAF as the main source of pulmonary supply in addition to two major aortopulmonary collateral arteries (MAPCAS)...
October 2017: Echocardiography
https://www.readbyqxmd.com/read/28878607/genetic-testing-in-a-cohort-of-complex-esophageal-atresia
#20
Eliane Beauregard-Lacroix, Jessica Tardif, Emmanuelle Lemyre, Zoha Kibar, Christophe Faure, Philippe M Campeau
The objective of the present study is to describe a cohort of complex esophageal atresia and the yield of genetic tests performed for such patients. We selected 45 patients with complex esophageal atresia (EA), namely those having at least one associated anomaly. We reviewed their medical records to assess clinical features, other diagnoses, and genetic investigations. Most of the patients had a diagnosis of VACTERL association (56%) with no genetic variant identified. Interestingly, 5 patients in the cohort (11%) had a right pulmonary hypoplasia or agenesis...
August 2017: Molecular Syndromology
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