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Pulmonary atresia

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https://www.readbyqxmd.com/read/28915526/-exit-a-possible-intervention-for-new-and-earlyborn-babies-with-severe-hydrops-fetalis-and-hydrothoraces-on-both-sides
#1
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
#2
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...
September 15, 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
#3
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
#4
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
#5
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)...
September 12, 2017: Echocardiography
https://www.readbyqxmd.com/read/28878607/genetic-testing-in-a-cohort-of-complex-esophageal-atresia
#6
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
https://www.readbyqxmd.com/read/28874283/mortality-in-adults-with-congenital-heart-disease
#7
Pavithra Naidu, Leeanne Grigg, Dominica Zentner
AIM: Retrospective ascertainment of the causes of mortality in the adult congenital heart disease (ACHD) cohort of the Royal Melbourne Hospital (RMH). METHODS: Deceased patients (n=73) of the 2519 ACHD patients in the Royal Melbourne Hospital registry (commenced in 1991) were identified. Retrospective analysis was undertaken. Age, gender of deceased individuals, and frequency and cause of death in different congenital diagnosis groups was explored. RESULTS: Between 1991 and 2015, death occurred in 3...
October 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28871366/predictors-of-mortality-in-children-with-pulmonary-atresia-with-intact-ventricular-septum
#8
Stephanie Grant, David Faraoni, James DiNardo, Kirsten Odegard
Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare cardiac congenital lesion characterized by imperforate pulmonary valve, intact ventricular septum, and atrial level shunt. Although different management strategies to establish a source of non-ductal dependent pulmonary blood flow have been described, studies have not assessed the relationship between the therapeutic approach, patient characteristics, and outcomes. The purpose of this study was to identify predictors of mortality for patients with PA/IVS...
September 4, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28871319/onset-ages-of-hepatopulmonary-syndrome-and-pulmonary-hypertension-in-patients-with-biliary-atresia
#9
Takehisa Ueno, Ryuta Saka, Yuichi Takama, Hiroaki Yamanaka, Yuko Tazuke, Kazuhiko Bessho, Hiroomi Okuyama
PURPOSE: Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are extrahepatic complications of biliary atresia (BA). Their detection is sometimes delayed, which may result in missed opportunities for liver transplantation. The aim of this study was to determine the onset ages of HPS and PoPH in BA patients. METHODS: BA patients followed at our institution were identified. Patients visited our clinic for routine blood work, as well as regular electrocardiography, chest X-rays, and arterial blood gas tests...
September 4, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28870391/determinants-of-adverse-outcomes-after-systemic-to-pulmonary-shunts-in-biventricular-circulation
#10
Ben Hobbes, Yves d'Udekem, Diana Zannino, Igor E Konstantinov, Christian Brizard, Johann Brink
BACKGROUND: Systemic-to-pulmonary shunts are useful palliative procedures, although many teams have been deterred by high mortality and morbidity. We aimed to identify predictors of adverse outcomes after shunts in biventricular lesions. METHODS: From 2004 to 2014, 173 children had shunt procedures. Morphologies included: tetralogy of Fallot, pulmonary atresia with ventricular septal defect (VSD) with and without major aortopulmonary collaterals (MAPCAs), transposition of great arteries with pulmonary stenosis, and double outlet right ventricle...
September 1, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28867155/the-boundaries-of-fetal-cardiac-intervention-expand-or-tighten
#11
REVIEW
Laura Gellis, Wayne Tworetzky
Fetal cardiac intervention (FCI) is a relatively new and continually evolving field, and, for select cardiac defects, offers the potential to alter the progression of the disease and improve outcomes. It is a procedure that requires a collaborative effort between maternal-fetal medicine, interventional cardiology and fetal echo/ultrasound specialists, as well as fetal and maternal anesthesiologists, nursing specialists, and social workers. This article reviews the most recently reported data and advances in FCI...
August 31, 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28853209/inferior-vena-cava-atresia-predisposing-to-acute-lower-extremity-deep-vein-thrombosis-in-children-a-descriptive-dual-center-study
#12
Cristina Tarango, Riten Kumar, Manish Patel, Anne Blackmore, Patrick Warren, Joseph S Palumbo
PURPOSE: Thrombosis in the healthy pediatric population is a rare occurrence. Little is known about the optimal treatment or outcomes of children with unprovoked acute lower extremity (LE) deep vein thrombosis (DVT) associated with atresia of the inferior vena cava (IVC). METHODS: We retrospectively analyzed the records of patients with acute LE DVT subsequently found to have IVC atresia who presented to two tertiary pediatric institutions between 2008 and 2016...
August 29, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28835981/a-perforation-procedure-for-pulmonary-atresia-with-intact-ventricular-septum-egyptian-experience-and-adaptations
#13
S A El Saiedi, W A Attia, O M Abd El-Aziz, W N Lotfy, A M Abd El-Rahim, H Hassanein, S Qureshi
BACKGROUND: Pulmonary atresia with intact ventricular septum (PA-IVS) is an uncommon disorder with significant morphological heterogeneity. The use of percutaneous radiofrequency (RF)-assisted perforation of the atretic valve and subsequent balloon dilation provides a relatively easy but expensive procedure that is expected to establish ante-grade flow through the pulmonary valve in most patients. OBJECTIVES: The aim of the study was to attempt a cost reduction by using catheters and wires readily available in our catheter laboratory...
August 23, 2017: Herz
https://www.readbyqxmd.com/read/28821333/impact-of-pulmonary-flow-study-pressure-on-outcomes-after-one-stage-unifocalization
#14
Matteo Trezzi, Sonia B Albanese, Antonio Albano, Gabriele Rinelli, Carolina D'Anna, Angelo Polito, Enrico Cetrano, Adriano Carotti
BACKGROUND: The purpose of this study was to evaluate the accuracy of the pulmonary flow study in (1) predicting the feasibility of concomitant intracardiac repair after one-stage unifocalization; and in (2) predicting long-term survival and the onset of right ventricular dysfunction after surgery. METHODS: Between October 1996 and July 2015, a flow study was obtained in 95 patients undergoing complete one-stage unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals...
August 16, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28812458/congenital-cystic-lung-lesions-evolution-from-in-utero-detection-to-pathology-diagnosis-a-multidisciplinary-approach
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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