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Atresia pulmonary

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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
#1
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
#2
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
#3
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...
June 18, 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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
https://www.readbyqxmd.com/read/28504356/bronchoscopy-in-children-with-tetralogy-of-fallot-pulmonary-atresia-and-major-aortopulmonary-collaterals
#14
Douglas R Sidell, Andrew M Koth, Holly Bauser-Heaton, Doff B McElhinney, Lisa Wise-Faberowski, Michael C Tracy, Frank L Hanley, Ritu Asija
OBJECTIVE: Children with Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals (TOF/PA/MAPCAs) undergoing unifocalization surgery are at risk for developing more postoperative respiratory complications than children undergoing other types of congenital heart surgery. Bronchoscopy is used in the perioperative period for diagnostic and therapeutic purposes. In this study, we describe bronchoscopic findings and identify factors associated with selection for bronchoscopy...
May 15, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28500137/outcomes-after-decompression-of-the-right-ventricle-in-infants-with-pulmonary-atresia-with-intact-ventricular-septum-are-associated-with-degree-of-tricuspid-regurgitation-results-from-the-congenital-catheterization-research-collaborative
#15
Christopher J Petit, Andrew C Glatz, Athar M Qureshi, Ritu Sachdeva, Shiraz A Maskatia, Henri Justino, David J Goldberg, Namrita Mozumdar, Wendy Whiteside, Lindsay S Rogers, George T Nicholson, Courtney McCracken, Mike Kelleman, Bryan H Goldstein
BACKGROUND: Outcomes after right ventricle (RV) decompression in infants with pulmonary atresia with intact ventricular septum vary widely. Descriptions of outcomes are limited to small single-center studies. METHODS AND RESULTS: Neonates undergoing RV decompression for pulmonary atresia with intact ventricular septum were included from 4 pediatric centers. Primary end point was reintervention post-RV decompression; secondary end points included circulation type at latest follow-up...
May 2017: Circulation. Cardiovascular Interventions
https://www.readbyqxmd.com/read/28496023/bilateral-coronary-pulmonary-artery-fistulas-in-pulmonary-atresia-with-ventricular-septal-defect
#16
Yusaku Nagatomo, Jun Muneuchi, Mamie Watanabe, Kunihiko Joo, Yoshie Ochiai
We present a very rare case of bilateral coronary to pulmonary artery fistulas associated with pulmonary atresia with ventricular septal defect. The courses of coronary to pulmonary artery fistulas have to be clearly delineated by detailed angiography prior to corrective surgery.
May 12, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28489830/-clinical-and-laboratory-characteristics-of-patients-with-pulmonary-hypertension-and-pulmonary-vascular-complications-hospitalized-at-the-instituto-nacional-de-salud-del-ni%C3%A3-o
#17
Alexis Jose Ormeño Julca, Carlos Melchor Alvarez Murillo, Pedro Miguel Amoretti Alvino, Angel Aladino Florian Florian, Rosa Aurora Castro Johanson, Maria Danisa Celi Perez, Olga Rocío Huamán Prado
The hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHN) are distinct pulmonary vascular complications of portal hypertension (PHT) and are associated with increased morbidity and mortality. OBJECTIVES: To describe the clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño. MATERIALS AND METHODS: We included patients with HTP hospitalized from January 2012 to June 2013 and that during its evolution progressed with SHP or HTPP...
January 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
https://www.readbyqxmd.com/read/28472241/does-morphological-vessel-analysis-address-pulmonary-vascular-physiology-in-patients-with-pulmonary-atresia-ventricular-septal-defect-and-major-aortopulmonary-collateral-arteries
#18
Adriano Carotti
No abstract text is available yet for this article.
May 2, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28441832/-fetal-cardiac-interventions-for-pulmonary-atresia
#19
N C Hong, K Sun
No abstract text is available yet for this article.
April 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28441821/-assessment-of-undiagnosed-critical-congenital-heart-disease-before-discharge-from-the-maternity-hospital
#20
Q M Zhao, F Liu, L Wu, M Ye, B Jia, X J Ma, G Y Huang
Objective: Undiagnosed critical congenital heart disease (CCHD) was assessed before discharge from maternity hospital.Basic information was provided for screening CCHD in the early neonatal stage.Chi-squared test was used for comparison of categorical variables(detection rate of different types of CCHD). Method: A retrospective cohort study was conducted in neonates with CCHD who were admitted to Children's Hospital of Fudan University between 1 January 2012 and 31 December 2015. For comparing with the previously reported undiagnosed rate of CCHD at discharge, CCHD was defined as all duct dependent congenital heart disease (DDCHD) and any cyanotic CHD that required early surgery...
April 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
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