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Pulmonary atresia

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https://www.readbyqxmd.com/read/28812458/congenital-cystic-lung-lesions-evolution-from-in-utero-detection-to-pathology-diagnosis-a-multidisciplinary-approach
#1
Steven Hardee, Lea Tuzovic, Cicero T Silva, Robert A Cowles, Joshua Copel, Raffaella A Morotti
Congenital cystic lung lesions are a group of rare pathologies that are usually diagnosed in the prenatal period. The majority of these lesions are diagnosed at pathology examination as congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestration (BPS). These lesions are typically managed by surgical intervention within the first year of life and have an excellent prognosis. We examined the evolution of imaging appearances from prenatal diagnosis to postnatal work-up of these lesions and correlate imaging and pathological findings...
September 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28803685/quantification-of-pulmonary-regurgitation-by-vector-flow-mapping-in-congenital-heart-patients-after-repair-of-right-ventricular-outflow-obstruction-a-preliminary-study
#2
Ashley Hoi-Man To, Vivian Wing-Yi Li, Ming-Yen Ng, Yiu-Fai Cheung
BACKGROUND: Vector flow mapping (VFM) enables direct visualization of flow pattern and estimation of flow volume. The aim of this study was to determine its accuracy in the quantification of pulmonary regurgitation (PR) in congenital heart patients after repair of right ventricular (RV) outflow obstruction. METHODS: This study comprised two parts: (1) validation of VFM in the quantification of PR in patients with repaired tetralogy of Fallot by cardiac magnetic resonance and (2) clinical application of VFM to determine PR in patients after biventricular repair of pulmonary atresia and stenosis with intact ventricular septum...
August 10, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/28770306/prenatal-echocardiographic-predictors-of-postnatal-management-strategy-in-the-fetus-with-right-ventricle-hypoplasia-and-pulmonary-atresia-or-stenosis
#3
Li Cao, Zhiyun Tian, Jack Rychik
Fetuses with pulmonary atresia or pulmonary stenosis with intact ventricular septum manifest variable degrees of right ventricle hypoplasia and inadequacy. We studied the relationship between prenatal echocardiographic parameters and their progression through gestation as potential predictors of postnatal single-ventricle or two-ventricle care strategy. Serial fetal echocardiograms of pulmonary atresia (n = 28) or severe pulmonary stenosis (n = 8) and intact ventricular septum were reviewed. Measurements included tricuspid valve and mitral valve diameter and Z scores, degree of tricuspid regurgitation, presence of subaortic stenosis, presence of coronary artery fistulae, and Doppler pulsatility indices in middle cerebral and umbilical artery...
August 2, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28765742/clinical-characteristics-and-independent-factors-related-to-long-term-outcomes-in-patients-with-left-isomerism
#4
Sun Hyang Lee, Bo Sang Kwon, Gi Beom Kim, Eun Jung Bae, Chung Il Noh, Hong Gook Lim, Woong Han Kim, Jeong Ryul Lee, Yong Jin Kim
BACKGROUND AND OBJECTIVES: We studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis. SUBJECTS AND METHODS: We reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014. RESULTS: Of the total study population, 29 patients (38.1%) had functional univentricular heart disease, 43 patients (56.5%) had cardiac anomalies suitable for biventricular hearts, and four patients (5...
July 2017: Korean Circulation Journal
https://www.readbyqxmd.com/read/28761211/holt-oram-syndrome-a-rare-variant
#5
Binoy Shankar, Euden Bhutia, Dinesh Kumar, Sunil Kishore, Shakti Pad Das
Holt-Oram syndrome is an autosomal dominant disorder, characterised by skeletal abnormalities of the upper limb associated with congenital heart defect, mainly atrial and ventricular septal defects. Skeletal defects exclusively affect the upper limbs in the preaxial radial ray distribution and are bilateral and asymmetrical. They range from clinodactyly, absent or digitalised thumb, hypoplastic or absent radii, and first metacarpal to hypoplastic ulna and carpal bone anomalies. Cardiac involvement ranges from asymptomatic conduction disturbances to multiple structural defects...
July 2017: Iranian Journal of Medical Sciences
https://www.readbyqxmd.com/read/28752252/prenatal-diagnosis-of-pulmonary-atresia-with-ventricular-septal-defect
#6
Shui-Hua Yang, Peng-Hui Luo, Xiao-Xian Tian, Xin-Yan Li, Xue-Qin Li, Zuo-Jian Yang, Sheng-Li Li
Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare complex congenital heart defect. Major artery-pulmonary collateral arteries (MAPCAs) are characteristic of PA-VSD. Prenatal diagnosis can be achieved in most cases of PA-VSD with recent advances in echocardiography. However, it is extremely rare that all MAPCAs can be observed on the echocardiograph. Here, we report a case of prenatally diagnosed type C PA-VSD in which all the MAPCAs could be seen on the echocardiograph, with the diagnosis supported by autopsy evidence...
July 27, 2017: Journal of Medical Ultrasonics
https://www.readbyqxmd.com/read/28738792/functional-study-of-dand5-variant-in-patients-with-congenital-heart-disease-and-laterality-defects
#7
Fernando Cristo, José M Inácio, Salomé de Almeida, Patrícia Mendes, Duarte Saraiva Martins, José Maio, Rui Anjos, José A Belo
BACKGROUND: Perturbations on the Left-Right axis establishment lead to laterality defects, with frequently associated Congenital Heart Diseases (CHDs). Indeed, in the last decade, it has been reported that the etiology of isolated cases of CHDs or cases of laterality defects with associated CHDs is linked with variants of genes involved in the Nodal signaling pathway. METHODS: With this in mind, we analyzed a cohort of 38 unrelated patients with Congenital Heart Defects that can arise from initial perturbations in the formation of the Left-Right axis and 40 unrelated ethnically matched healthy individuals as a control population...
July 24, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28736712/successful-ablation-for-non-pulmonary-multi-foci-atrial-fibrillation-tachycardia-in-a-patient-with-coronary-sinus-ostial-atresia-by-transseptal-puncture-and-epicardial-approach
#8
Yukihiro Inamura, Junichi Nitta, Akira Sato, Masahiko Goya, Mitsuaki Isobe, Kenzo Hirao
No abstract text is available yet for this article.
May 2017: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/28734439/coronary-sinus-orifice-atresia-after-the-fontan-completion-unique-repair-technique
#9
Shinya Yokoyama, Takeo Yonekura, Noboru Nishiwaki, Shigeki Taniguchi
We report a successful unroofing of the coronary sinus via a unique technique. Our patient was diagnosed with single ventricle morphology. Pulmonary artery banding and bidirectional cavopulmonary shunt were performed at 1 and 6 months old, respectively. Ordinary univentricular repair strategy was performed; extracardiac total cavopulmonary connection was achieved at 18 months old. Catheterization performed 1 year after the Fontan procedure revealed the coronary sinus orifice atresia, diffuse coronary artery stenosis, and sinusoid formation of the coronary system...
August 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28719049/pulmonary-vein-stenosis-in-patients-with-smith-lemli-opitz-syndrome
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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)...
September 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
#18
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
#19
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
#20
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
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