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Pulmonary atresia

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https://www.readbyqxmd.com/read/28719049/pulmonary-vein-stenosis-in-patients-with-smith-lemli-opitz-syndrome
#1
Aaron R Prosnitz, Jane Leopold, Mira Irons, Kathy Jenkins, Amy E Roberts
OBJECTIVE: To describe a group of children with co-incident pulmonary vein stenosis and Smith-Lemli-Opitz syndrome and to generate hypotheses as to the shared pathogenesis of these disorders. DESIGN: Retrospective case series. PATIENTS: Five subjects in a pulmonary vein stenosis cohort of 170 subjects were diagnosed with Smith-Lemli-Opitz syndrome soon after birth. RESULTS: All five cases were diagnosed with Smith-Lemli-Opitz syndrome within 6 weeks of life, with no family history of either disorder...
July 18, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28705054/radiofrequency-perforation-of-the-pulmonary-valve-an-efficient-low-cost-solution
#2
Stephen C Brown, Bjorn Cools, Derize Boshoff, Ruth Heying, Benedicte Eyskens, Marc Gewillig
Objective The aim of the study was to assess the feasibility of using commonly available catheterization laboratory equipment for radiofrequency perforation of the pulmonary valve in patients with pulmonary atresia and intact ventricular septum. Methods The system (off-label use for all items) is made up of a co-axial telescopic arrangement consisting of a 0.014" PT 2 ™ coronary guidewire, for insulation inside a 2.7-F microcatheter which has an inner lumen of 0.021". The microcatheter was passed via a standard 4-F right coronary catheter to just below the atretic pulmonary valve...
July 14, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28701598/the-blalock-and-taussig-shunt-revisited
#3
REVIEW
Usha Kiran, Shivani Aggarwal, Arin Choudhary, B Uma, Poonam Malhotra Kapoor
The systemic to pulmonary artery shunts are done as palliative procedures for cyanotic congenital heart diseases ranging from simple tetralogy of Fallots (TOFs)/pulmonary atresia (PA) to complex univentricular hearts. They allow growth of pulmonary arteries and maintain regulated blood flow to the lungs till a proper age and body weight suitable for definitive corrective repair is reached. We have reviewed the BT shunt with its anaesthtic considerations and management of associated complications.
July 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28685640/presentation-of-unrepaired-pulmonary-atresia-with-ventricular-septum-defect-and-major-aortopulmonary-collateral-arteries-arising-from-a-left-brachiocephalic-trunk-in-a-52-year-old-female
#4
Joerg Kellermair, Barbara Wichert-Schmitt, Kaveh Akbari, Clemens Steinwender
No abstract text is available yet for this article.
July 7, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28669502/the-impact-of-22q11-2-deletion-syndrome-on-surgical-repair-outcomes-of-conotruncal-cardiac-anomalies
#5
Bahaaldin Alsoufi, Courtney McCracken, Subhadra Shashidharan, Shriprasad Deshpande, Kirk Kanter, Brian Kogon
BACKGROUND: We aim to describe the impact of 22q11.2 deletion syndrome (22q11DS) on clinical characteristics, postoperative course, and early and late outcomes of neonates undergoing surgery for conotruncal anomalies. METHODS: A retrospective review was performed (2002 to 2012) of 224 neonates who underwent surgery for interrupted aortic arch (n = 67), truncus arteriosus (n = 85), or ductal-dependent pulmonary atresia and ventricular septal defect (n = 72). Patients were divided into three groups: group 1, n = 119, no genetic syndrome; group 2, n = 64, 22q11DS; and group 3, n = 41, other genetic syndrome...
June 29, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28653340/aortic-perfusion-score-for-pulmonary-atresia-with-intact-ventricular-septum-an-antegrade-coronary-perfusion-scoring-system-that-is-predictive-of-need-for-transplant-and-mortality
#6
Rohit S Loomba, Andrew N Pelech
BACKGROUND: Pulmonary atresia with intact ventricular septum is a unique congenital malformation of the heart in which patients may undergo single-, two- or 1½- ventricle repair. Size of the tricuspid valve annulus, morphology of the right ventricle and presence of ventriculocoronary connections can all impact the selection of appropriate palliative strategy. We developed the aortic perfusion score, a novel scoring system based on anterograde coronary perfusion with the aim of being able to identify patients at risk for death or transplant...
June 27, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28651676/anomalous-origin-of-the-left-coronary-artery-from-the-right-pulmonary-artery-in-a-univentricular-heart
#7
Ziyad M Binsalamah, Diego A Lara, Emmett D McKenzie
Anomalous origin of the left coronary artery from the right pulmonary artery in single ventricles is a very rare congenital anomaly. Failure to recognise it preoperatively may lead to adverse outcomes, including death. We report the case of a neonate with a univentricular heart in the form of a double-outlet right ventricle, mitral atresia with discrete coarctation of the aorta, and an incidental intraoperative finding of an anomalous origin of the left coronary artery from the right pulmonary artery. The patient underwent a successful repair with an uneventful postoperative course...
June 27, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28636109/xq26-1-26-3-duplication-including-mospd1-and-gpc3-identified-in-boy-with-short-stature-and-double-outlet-right-ventricle
#8
Yukiko Hirota, Takaomi Minami, Tomoyuki Sato, Akiko Yokomizo, Auimi Matsumoto, Masahide Goto, Eriko Jinbo, Takanori Yamamgata
Xq25q26 duplication syndrome has been reported in individuals with clinical features such as short stature, intellectual disability, syndromic facial appearance, small hands and feet, and genital abnormalities. The symptoms are related to critical chromosome regions including Xq26.1-26.3. In this particular syndrome, no patient with congenital heart disease was previously reported. Here, we report a 6-year-old boy with typical symptoms of Xq25q26 duplication syndrome and double outlet right ventricle (DORV) with pulmonary atresia (PA)...
June 21, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28633393/outcomes-of-palliative-right-ventricle-to-pulmonary-artery-connection-for-pulmonary-atresia-with-ventricular-septal-defect%C3%A2
#9
Marien Lenoir, Margaux Pontailler, Régis Gaudin, Sébastien Gerelli, Daniel Tamisier, Damien Bonnet, Bari Murtuza, Pascal R Vouhé, Olivier Raisky
OBJECTIVES: To determine the early, intermediate and long-term outcomes of pulmonary atresia with ventricular septal defect (PA/VSD) Types I, II and III initially palliated by a right ventricle to pulmonary artery (RVPA) connection. METHODS: We performed a retrospective study from 2000 to 2014 that included 109 patients with PA/VSD who had undergone an RVPA connection (tetralogy of Fallot and PA/VSD Type IV excluded). The end-points of this strategy were adequate pulmonary artery tree post-palliation, second palliation, biventricular repair, right ventricular pressure post-biventricular repair and late reoperation...
June 15, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28624838/dental-management-of-pediatric-patients-affected-by-pulmonary-atresia-with-ventricular-septal-defect-a-scoping-review
#10
A Garrocho-Rangel, A-C Echavarría-García, M-A Rosales-Bérber, J Flores-Velázquez, A Pozos-Guillén
BACKGROUND: Congenital Heart Diseases (CHD) involves a wide range of pathological conditions, such as Pulmonary Atresia with Ventricle Septal Defect (PA/VSD). This disorder leads to the systemic circulation of oxygen-poor blood (cyanosis), with associated features and consequences in the oral cavity. MATERIAL AND METHODS: Using scoping review methodology for screening and article selection, the primary objectives of this paper were as follows: first, to pose a research question; second, to identify relevant studies in order to answer the research question; third, to select and retrieve the studies; fourth, to chart the critical data, and finally, to collate, summarize, and report the results from the most important articles on the dental management of children affected with PA/VSD...
July 1, 2017: Medicina Oral, Patología Oral y Cirugía Bucal
https://www.readbyqxmd.com/read/28611969/anastomotic-strictures-after-esophageal-atresia-repair-incidence-investigations-and-management-including-treatment-of-refractory-and-recurrent-strictures
#11
REVIEW
Renato Tambucci, Giulia Angelino, Paola De Angelis, Filippo Torroni, Tamara Caldaro, Valerio Balassone, Anna Chiara Contini, Erminia Romeo, Francesca Rea, Simona Faraci, Giovanni Federici di Abriola, Luigi Dall'Oglio
Improved surgical techniques, as well as preoperative and postoperative care, have dramatically changed survival of children with esophageal atresia (EA) over the last decades. Nowadays, we are increasingly seeing EA patients experiencing significant short- and long-term gastrointestinal morbidities. Anastomotic stricture (AS) is the most common complication following operative repair. An esophageal stricture is defined as an intrinsic luminal narrowing in a clinically symptomatic patient, but no symptoms are sensitive or specific enough to diagnose an AS...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28583299/arresting-pulmonary-atresia-intact-ventricular-septum%C3%A2-only-if-you-have-to
#12
EDITORIAL
Harold M Burkhart, Jess L Thompson, Ron E Angona
No abstract text is available yet for this article.
May 19, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28579227/d-tga-combined-with-left-arch-atresia-of-a-double-aortic-arch
#13
Dan Zhou, Laichun Song, Liang Tao, Hong Zhou
A three-month-old female underwent an arterial switch operation for transposition of the great arteries (TGA) with concomitant ventricular septal defect (VSD). After the operation, the patient suffered from stubborn pulmonary infection with increased airway resistance, and could not be weaned from a ventilator. Multispiral computed tomography (CT) scanning suggested a double aortic arch with left arch atresia. The patient underwent a second procedure to resection the left aortic arch. The patient gradually recovered and was successfully weaned from the ventilator...
May 5, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28566832/recanalization-of-an-occluded-left-pulmonary-artery-a-case-report-and-review-of-the-literature
#14
Ali Ibrahim Elarabi, Ming Chern Leong, Mazeni Alwi
We report an 8-year-old male child with tetralogy of Fallot (TOF), who developed left pulmonary artery (LPA) atresia, following surgical repair of TOF and left pulmonary arterioplasty at the age of 6 years. He underwent successful radiofrequency recanalization and stenting of the LPA. The LPA exhibited satisfactory growth for 3 months, following recanalization and stenting.
May 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28566818/three-dimensional-printed-cardiac-prototypes-aid-surgical-decision-making-and-preoperative-planning-in-selected-cases-of-complex-congenital-heart-diseases-early-experience-and-proof-of-concept-in-a-resource-limited-environment
#15
Mahesh Kappanayil, Nageshwara Rao Koneti, Rajesh R Kannan, Brijesh P Kottayil, Krishna Kumar
INTRODUCTION: Three-dimensional. (3D) printing is an innovative manufacturing process that allows computer-assisted conversion of 3D imaging data into physical "printouts" Healthcare applications are currently in evolution. OBJECTIVE: The objective of this study was to explore the feasibility and impact of using patient-specific 3D-printed cardiac prototypes derived from high-resolution medical imaging data (cardiac magnetic resonance imaging/computed tomography [MRI/CT]) on surgical decision-making and preoperative planning in selected cases of complex congenital heart diseases (CHDs)...
May 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28532389/the-use-of-macitentan-in-fontan-circulation-a-case-report
#16
Polyvios Demetriades, Amir Aziz, Robin Condliffe, Sarah E Bowater, Paul F Clift
BACKGROUND: The Fontan circulation, a result of a palliative procedure in patients with single systemic ventricles, is defined by chronically elevated pulmonary vascular resistance. When traditional heart failure therapies fail, pharmacological agents that reduce pulmonary artery pressures may be used. These include endothelial-receptor antagonists, prostanoids and phosphodiesterase type 5 inhibitors. We report the first use of macitentan, an endothelin-receptor antagonist, in a patient with a Fontan circulation...
May 22, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28527961/major-aortopulmonary-collateral-arteries-with-anatomy-other-than-pulmonary-atresia-ventricular-septal-defect
#17
William L Patrick, Richard D Mainwaring, Olaf Reinhartz, Rajesh Punn, Theresa Tacy, Frank L Hanley
BACKGROUND: Major aortopulmonary collateral arteries (MAPCAs) are frequently found in association with pulmonary atresia with ventricular septal defect (PA/VSD). However, some patients with MAPCAs do not have PA/VSD but have a variety of other "atypical" anatomic diagnoses. METHODS: This was a retrospective review of patients with MAPCAs and atypical anatomy. The 50 patients with MAPCAs could be divided into two subgroups: (1) single ventricle anatomy (n = 33) and (2) two ventricle anatomy (n = 17)...
May 17, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28520540/virtual-surgery-for-conduit-reconstruction-of-the-right-ventricular-outflow-tract
#18
Chin Siang Ong, Yue-Hin Loke, Justin Opfermann, Laura Olivieri, Luca Vricella, Axel Krieger, Narutoshi Hibino
PURPOSE: Virtual surgery involves the planning and simulation of surgical reconstruction using three-dimensional (3D) modeling based upon individual patient data, augmented by simulation of planned surgical alterations including implantation of devices or grafts. Here we describe a case in which virtual cardiac surgery aided us in determining the optimal conduit size to use for the reconstruction of the right ventricular outflow tract. DESCRIPTION: The patient is a young adolescent male with a history of tetralogy of Fallot with pulmonary atresia, requiring right ventricle-to-pulmonary artery (RV-PA) conduit replacement...
May 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28520535/optimal-z-score-use-in-surgical-decision-making-in-pulmonary-atresia-with-intact-ventricular-septum
#19
Mark Nelson Awori, Nikita P Mehta, Frederick O Mitema, Naomi Kebba
OBJECTIVES: In the surgical treatment of pulmonary atresia with intact ventricular septum, the size of the tricuspid valve annulus (as measured by z-scores) has emerged as a significant factor in deciding which repair to perform. Various tricuspid valve annulus z-scores are reported as "cutoffs" for successful biventricular repair. We aimed to determine whether the use of different z-score data sets contributed to the gross variation in "cutoffs" for successful biventricular repair reported in the literature...
May 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28515589/spectrum-of-pulmonary-valve-morphology-and-its-relationship-to-pulmonary-trunk-in-tetralogy-of-fallot
#20
Binita R Chacko, George K Chiramel, Leena R Vimala, Devi A Manuel, Elizabeth Joseph, K Reka
BACKGROUND: Tetralogy of Fallot (TOF) is a complex congenital heart disease with anatomic variations. Although the pulmonary valve in TOF is abnormal, it has not been studied well, especially on newer imaging modalities such as multidetector computed tomography (CT), which gives excellent anatomic detail. AIMS: The aim of this study was to assess the morphology of pulmonary valve in TOF on CT and evaluate its association with the degree of hypoplasia of infundibulum and pulmonary trunk...
January 2017: Indian Journal of Radiology & Imaging
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