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Pulmonary atresia

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https://www.readbyqxmd.com/read/28329111/hypoplastic-left-heart-syndrome-a-novel-surgical-strategy-for-small-volume-centres-%C3%A2
#1
Margaux Pontailler, Régis Gaudin, Marien Lenoir, Ayman Haydar, Diala Kraiche, Damien Bonnet, Pascal Vouhé, Olivier Raisky
OBJECTIVES: We describe in a prospective study, a novel surgical technique for the management of hypoplastic left heart syndrome inspired by the hybrid Norwood approach. METHODS: This new neonatal palliation comprises replacement of the patent ductus arteriosus (PDA) and aortic arch plasty with a pulmonary homograft associated with the banding of both pulmonary arteries and atrial septectomy, under cardiopulmonary bypass without aortic clamping and cardioplegia...
February 17, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28329049/surgical-treatment-of-pulmonary-atresia-with-major-aortopulmonary-collateral-arteries-in-83-consecutive-patients%C3%A2
#2
Oleksandr D Babliak, Yaroslav B Mykychak, Oleksandra O Motrechko, Illya M Yemets
OBJECTIVES: This article reports the safety and efficacy of a morphology-based algorithm for the surgical management of pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries. METHODS: A total of 83 patients were operated from 2007 to 2014 using 3 surgical approaches: one-stage total repair, one-stage unifocalization with central shunt and delayed total repair and multistage unifocalization with subsequent total repair. Patients were divided into 2 groups depending on the surgical strategy used to choose the surgical approach...
March 2, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28300528/functional-near-tricuspid-atresia-in-a-patient-with-absent-pulmonary-valve-and-an-intact-ventricular-septum
#3
Yasunobu Miki, Toshikatsu Tanaka, Yoshihiro Oshima
Absent pulmonary valve with an intact ventricular septum is a rare malformation. We report a case of absent pulmonary valve and intact ventricular septum with functional near-tricuspid atresia caused by pulmonary regurgitation. Initial palliation with main pulmonary artery ligation and bilateral pulmonary artery banding was performed at 1 day of age. More antegrade flow across the tricuspid valve was recognised postoperatively, resulting in a successful right ventricular outflow tract reconstruction by a hand-sewn bileaflet polytetrafluoroethylene valve and modified Blalock-Taussig shunt at 11 days of age...
August 30, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/28296740/diagnosis-and-treatment-of-communicating-bronchopulmonary-foregut-malformation-report-of-two-cases-and-review-of-the-literature
#4
Hongxia Ren, Liqiong Duan, Baohong Zhao, Xiaoxia Wu, Hongyi Zhang, Caixia Liu
RATIONALE: Communicating bronchopulmonary foregut malformation (CBPFM) is a rare congenital malformation involving both the digestive and respiratory systems. To our best knowledge, most cases of CBPFM reported in the literature were in infancy or adulthood and CBPFM in infantile is even rarer with a high case-fatality rate partly due to misdiagnosis. PATIENT CONCERNS: We presented 2 cases of neonatal CBPFM. A 11-hour male newborn was admitted because of moaning for 7 hours, and a 1-day male newborn was referred to us with profuse foams, choking on breast-milk feeding and facial cyanosis...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28293976/evolution-of-hybrid-interventions-for-congenital-heart-disease
#5
Hitesh Agrawal, Wail Alkashkari, Damien Kenny
Surgical and transcatheter interventions have seen a tremendous evolution in last three decades. Hybrid technology combines the achievements of both disciplines to bring substantial hemodynamic benefit to patients with congenital heart disease (CHD) in a minimally invasive manner. This collaboration between surgeons and interventionalists will continue to grow as the technology evolves to meet the demands of CHD patients, potentially avoiding cardiopulmonary bypass and vascular access complications as well as optimizing immediate technical outcomes with exit angiography...
March 15, 2017: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/28274523/intraoperative-stenting-of-pulmonary-artery-stenosis-in-children-with-congenital-heart-disease
#6
Mathilde Meot, Bruno Lefort, Jean Marc El Arid, Nathalie Soulé, Julie Lothion-Boulanger, François Lengellé, Alain Chantepie, Paul Neville
BACKGROUND: Branch pulmonary artery (BPA) stenosis is frequently associated with congenital heart disease. Management of BPA stenosis is challenging for surgeons due to a high rate of recurrence. The purpose of this study was to assess the results of intraoperative pulmonary artery stenting associated with or without surgical angioplasty. METHODS: We included 33 children from our center between January 2008 and July 2014. Patients had pulmonary atresia with ventricular septal defect (13), tetralogy of Fallot (10), troncus arteriosus (4), double outlet right ventricle (2), and single left or right ventricle (4)...
March 5, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28272662/fibromuscular-dysplasia-of-the-coronary-arteries-a-case-report-and-review-of-the-literature
#7
Julia Kuzyk, Oksana Boiko, Taras Stetsko
Fibromuscular dysplasia is a nonatherosclerotic and non-inflammatory vascular disease with primary lesion of renal and internal carotid arteries. We present a neonatal case of fibromuscular dysplasia who died on the second day of life. The newborn suffered from fibromuscular dysplasia of the coronary arteries and a congenital heart defect. The interesting feature of this case was the formation of aneurysms of the coronary arteries with pulmonary atresia. This case demonstrates a casuistically rare form of association between fibromuscular dysplasia of the coronary arteries and pulmonary artery atresia...
February 4, 2017: Türk Patoloji Dergisi
https://www.readbyqxmd.com/read/28260150/importance-of-multidisciplinary-management-for-pulmonary-atresia-ventricular-septal-defect-major-aorto-pulmonary-collateral-arteries-and-completely-absent-central-pulmonary-arteries
#8
Takaya Hoashi, Satoshi Yazaki, Koji Kagisaki, Masataka Kitano, Masatoshi Shimada, Isao Shiraishi, Hajime Ichikawa
BACKGROUND: To review long-term clinical outcomes for pulmonary atresia, ventricular septal defect (PA/VSD), major aorto-pulmonary collateral arteries (MAPCAs) and completely absent central pulmonary arteries (cPAs). METHODS: Of all 120 surgically treated patients with PA/VSD, MAPCAs between 1981 and 2011, 15 patients (12.5%) with completely absent cPAs were enrolled. The median age at initial surgery was 3.8 ± 4.9 years old (range 0.1-17.6) and 3.5 (2-6)...
March 4, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28258242/successful-cardiac-transplantation-outcomes-in-patients-with-adult-congenital-heart-disease
#9
Jonathan N Menachem, Jessica R Golbus, Maria Molina, Jeremy A Mazurek, Nicole Hornsby, Pavan Atluri, Stephanie Fuller, Edo Y Birati, Yuli Y Kim, Lee R Goldberg, Joyce W Wald
OBJECTIVES: The purpose of our study is (1) to characterise patients with congenital heart disease undergoing heart transplantation by adult cardiac surgeons in a large academic medical centre and (2) to describe successful outcomes associated with our multidisciplinary approach to the evaluation and treatment of adults with congenital heart disease (ACHD) undergoing orthotopic heart transplantation (OHT). BACKGROUND: Heart failure is the leading cause of death in patients with ACHD leading to increasing referrals for OHT...
March 3, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28248409/immediate-and-long-term-outcomes-of-percutaneous-transcatheter-pulmonary-valve-implantation
#10
Roland Fiszer, Paweł Dryżek, Małgorzata Szkutnik, Sebastian Góreczny, Alexandra Krawczuk, Jadwiga Moll, Tomasz Moszura, Szymon Pawlak, Jacek Białkowski
BACKGROUND: Transcutaneous pulmonary valve replacement (TPVR) has become an alternative to heart surgery for patients after previous right ventricular outflow tract (RVOT) or pulmonary artery (PA) surgical interventions. The objective was to present immediate and long-term outcomes of transcutaneous pulmonary valve replacement. METHODS: Between 06/2009 and 06/2016, 46 patients underwent TPVR. Initial diagnoses included tetralogy of Fallot, common arterial trunk, transposition of great arteries post Rastelli correction, left ventricle outflow obstruction after Ross operation, pulmonary atresia, and isolated dysplastic pulmonary valve stenosis...
March 1, 2017: Cardiology Journal
https://www.readbyqxmd.com/read/28242012/national-in-hospital-outcomes-of-pregnancy-in-women-with-single-ventricle-congenital-heart-disease
#11
R Thomas Collins, Di Chang, Adam Sandlin, Anthony Goudie, James M Robbins
Most patients with single ventricle (SV) congenital heart disease are expected to survive to adulthood. Women with SV are often counseled against pregnancy; however, data on pregnancies in these women are lacking. We sought to evaluate in-hospital outcomes of pregnancy in women with SV. We used nationally representative data from the 1998 to 2012 National Inpatient Sample to identify women ≥18 years of age admitted to the hospital with International Classification of Diseases-9th Revision codes for an intrauterine pregnancy and a diagnosis of hypoplastic left heart syndrome, tricuspid atresia, or common ventricle...
April 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28208970/isolated-hypoplasia-of-left-pulmonary-artery-with-agenesis-of-left-lobe-of-thyroid-a-case-report
#12
Mohammed Abdul Khadir, Ganesh Narayana, Ganavi Ramagopal, Pradeep G Nayar
Isolated Unilateral hypoplasia or agenesis of a branch of pulmonary artery is very rare. It is usually seen associated with congenital heart diseases such as tetralogy of Fallot, atrial septal defect, coarctation of the aorta, right aortic arch, truncus arteriosus, patent ductus arteriosus and pulmonary atresia. It occurs as a result of lack of embryological development of either the left or right sixth aortic arch and has been found to present itself with various clinical manifestations as during childhood it presents as contralateral pulmonary hypertension and in adults as haemoptysis...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28205731/p-19-impaired-pulmonary-function-and-ventilatory-limitation-in-children-with-successfully-repaired-oesophageal-atresia
#13
J McBride, P Field, C Clarkson, J Menzies, J Hughes, M Doumit, C Wu, S Adams, M Soma, U Krishnan, Y Belessis
No abstract text is available yet for this article.
April 1, 2016: Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus
https://www.readbyqxmd.com/read/28190605/the-importance-of-age-and-weight-on-cavopulmonary-shunt-stage-ii-outcomes-after-the-norwood-procedure-planned-versus-unplanned-surgery
#14
David J Barron, Intisar Ul Haq, Adrian Crucean, John Stickley, Phil Botha, Natasha Khan, Timothy J Jones, William J Brawn
OBJECTIVE: The study objective was to evaluate the outcomes of the cavopulmonary shunt after the Norwood procedure with a particular focus on age, weight, and whether surgery was planned or expedited by clinical findings. METHODS: We studied 297 consecutive patients with hypoplastic left heart syndrome undergoing the cavopulmonary shunt operation between 2002 and 2014. All patients underwent the Norwood procedure with a right ventricle to pulmonary artery conduit...
January 17, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28163422/transcatheter-pulmonary-valve-perforation-using-chronic-total-occlusion-wire-in-pulmonary-atresia-with-intact-ventricular-septum
#15
Shweta Bakhru, Shilpa Marathe, Manish Saxena, Sudeep Verma, Rajan Saileela, Tapan K Dash, Nageswara Rao Koneti
BACKGROUND: Perforation of pulmonary valve using radiofrequency ablation in pulmonary atresia with intact ventricular septum (PA IVS) is a treatment of choice. However, significant cost of the equipment limits its utility, especially in the developing economies. OBJECTIVE: To assess the feasibility, safety, and efficacy of perforation of pulmonary valve using chronic total occlusion (CTO) wires in patients with PA IVS as an alternative to radiofrequency ablation...
January 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28154913/right-ventricle-to-pulmonary-artery-shunt-in-pulmonary-atresia-with-a-ventricular-septal-defect-a-word-of-caution
#16
Kwang Ho Choi, Si Chan Sung, Hyungtae Kim, Hyoung Doo Lee, Gil Ho Ban, Geena Kim, Hoon Ko
The management of pulmonary atresia with a ventricular septal defect (PA/VSD) depends on the anatomy of the pulmonary artery or on the surgical strategy used at individual institutions. In our institution, we adopted a right ventricle-to-pulmonary artery (RV-PA) shunt in 2011 as a palliative procedure for PA/VSD to overcome the disadvantages of a Blalock-Taussig shunt. We evaluated the results of the RV-PA shunt as initial palliative surgery for PA/VSD. Thirteen patients with ductus-dependent PA/VSD from August 2011 to August 2015 were enrolled...
February 3, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28148318/utility-of-4d-flow-mapping-in-eisenmenger-syndrome-with-pulmonary-atresia
#17
Soha Romeih, Heba Aguib, Magdi Yacoub
Management of patients with Eisenmenger syndrome with pulmonary atresia is challenging because of the complexity of the structure-function relationship of the components of the syndrome. Multi-modality imaging including cardiac magnetic resonance (CMR) 4D Flow offers unprecedented opportunities to unravel, at least in part, some of these components, and thus help in the management of these patients. In this study, we describe the use of these integrated methods with particular reference to CMR 4D Flow in a patient with Eisenmenger syndrome and pulmonary atresia and outline both the utility and the limitations...
December 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/28132091/a-case-of-unusual-configuration-of-the-right-bronchial-arteries-combined-with-cryptogenic-severe-bilateral-hypertrophy
#18
Santiago Rojas, Eduard Quintana, Marisa Ortega, Alfonso Rodríguez-Baeza
Bronchial arteries commonly originate from thoracic aorta between T5 and T6. Ectopic origins from aortic arch, supraortic trunks and their branches, coronary arteries, and even abdominal aorta have been described in the literature. In some circumstances, such as pulmonary artery malformations, chronic embolism, or inflammatory diseases of the lung, the bronchial arteries become hypertrophied and eventually could be the only supply of pulmonary circulation. Here, we describe a case of an elderly man who presented an unusual pattern of bronchial arteries of the right lung combined with severe bilateral hypertrophy of bronchial vessels...
January 28, 2017: Surgical and Radiologic Anatomy: SRA
https://www.readbyqxmd.com/read/28122228/temporally-distinct-six2-positive-second-heart-field-progenitors-regulate-mammalian-heart-development-and-disease
#19
Zhengfang Zhou, Jingying Wang, Chaoshe Guo, Weiting Chang, Jian Zhuang, Ping Zhu, Xue Li
The embryonic process of forming a complex structure such as the heart remains poorly understood. Here, we show that Six2 marks a dynamic subset of second heart field progenitors. Six2-positive (Six2(+)) progenitors are rapidly recruited and assigned, and their descendants are allocated successively to regions of the heart from the right ventricle (RV) to the pulmonary trunk. Global ablation of Six2(+) progenitors resulted in RV hypoplasia and pulmonary atresia. An early stage-specific ablation of a small subset of Six2(+) progenitors did not cause any apparent structural defect at birth but rather resulted in adult-onset cardiac hypertrophy and dysfunction...
January 24, 2017: Cell Reports
https://www.readbyqxmd.com/read/28120278/long-term-management-challenges-in-esophageal-atresia
#20
REVIEW
Abby White, Raphael Bueno
Esophageal atresia is a rare congenital anomaly, but improved surgical and critical care has resulted in survival rates exceeding 90%. Long-term survival is associated with numerous management challenges including chronic motility disorders, dysphagia, strictures, reflux, esophagitis and attendant complications, tracheomalacia and chronic restrictive lung disease, and recurrent pulmonary infections. No guidelines for adolescents and younger or older adults exist for the treatment and monitoring of this specialized patient population...
March 2017: Current Treatment Options in Gastroenterology
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