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Heterotaxy

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https://www.readbyqxmd.com/read/29094357/systematic-review-and-meta-analysis-of-the-performance-of-second-trimester-screening-for-prenatal-detection-of-congenital-heart-defects
#1
Christine L van Velzen, Johannes C F Ket, Peter M van de Ven, Nico A Blom, Monique C Haak
BACKGROUND: The prenatal detection rate of congenital heart defects (CHDs) is increasing, but reported rates vary. OBJECTIVE: To determine the performance of the second-trimester anomaly scan to detect CHD. SEARCH STRATEGY: PubMed and Embase were searched for relevant studies in any language from inception to February 3, 2017. The search terms included "prenatal diagnosis" or "pregnancy," "cardiovascular diseases" or "cardiac defects," "congenital," and "specificity," or "sensitivity," or "cohort study...
November 2, 2017: International Journal of Gynaecology and Obstetrics
https://www.readbyqxmd.com/read/29056267/airway-ciliary-dysfunction-association-with-adverse-postoperative-outcomes-in-nonheterotaxy-congenital-heart-disease-patients
#2
Eileen Stewart, Phillip S Adams, Xin Tian, Omar Khalifa, Peter Wearden, Maliha Zahid, Cecilia W Lo
OBJECTIVE: Heterotaxy (HTX) congenital heart disease (CHD) patients with ciliary dysfunction (CD) have been shown to have increased postoperative respiratory morbidity. We hypothesized that non-HTX CHD infants with CD also will have increased postoperative morbidity, particularly respiratory complications. METHODS: Sixty-three infants with non-HTX CHD undergoing cardiac surgery were enrolled. Tests commonly used to assess for CD, nasal nitric oxide (nNO) measurements and nasal epithelial ciliary motion (CM) assessment, were obtained...
September 20, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29040450/dilated-cardiomyopathy-complicated-with-visceral-heterotaxy
#3
Masaki Tsuji, Eisuke Amiya, Masaru Hatano, Issei Komuro
No abstract text is available yet for this article.
October 13, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28962659/polysplenia-syndrome-with-duodenal-and-pancreatic-dysplasia-in-a-holstein-calf-a-case-report
#4
Daisuke Kondoh, Tomomi Kawano, Tomoaki Kikuchi, Kaoru Hatate, Kenichi Watanabe, Motoki Sasaki, Norio Yamagishi, Hisashi Inokuma, Nobuo Kitamura
BACKGROUND: Laterality disorders of the abdominal organs include situs inversus totalis that mirrors the arrangements of all internal organs and heterotaxy syndrome (situs ambiguus) in which the thoracic or abdominal organs are abnormally arranged. Heterotaxy is often accompanied by multiple congenital malformations, and it generally comprises asplenia and polysplenia syndromes. To our knowledge, polysplenia syndrome has been reported in only three cattle, and computerized tomographic (CT) images of these animals were not obtained...
September 29, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/28875963/an-infant-with-abernethy-malformation-associated-with-heterotaxy-and-pulmonary-hypertension
#5
Xiao-Lin Zhang, Xiao-Min Duan, Fang-Yun Wang, Xin Zhang, Yan Sun, Ning Ma, Zhong-Dong Du
No abstract text is available yet for this article.
September 20, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28858039/fulminant-haemophilus-influenzae-type-a-infection-in-a-4-year-old-with-previously-undiagnosed-asplenic-heterotaxy
#6
Andrew Headrick, Erinn O Schmit, David W Kimberlin
The post Hib-vaccine era has resulted in increased cases of invasive, non-Hib, Haemophilus influenzae infections. One of the encapsulated species, type a, displays the most similarity in virulence to type b. Our patient, a fully-vaccinated four-year-old female, with newly diagnosed asplenia in the setting of heterotaxy, had a prolonged hospital course with disseminated Haemophilus influenzae type a infection.
August 26, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28741863/development-of-quality-metrics-for-ambulatory-pediatric-cardiology-infection-prevention
#7
Jonathan N Johnson, Cindy S Barrett, Wayne H Franklin, Eric M Graham, Nancy J Halnon, Brandy A Hattendorf, Catherine D Krawczeski, James J McGovern, Matthew J O'Connor, Amy H Schultz, Jeffrey M Vinocur, Devyani Chowdhury, Jeffrey B Anderson
INTRODUCTION: In 2012, the American College of Cardiology's (ACC) Adult Congenital and Pediatric Cardiology Council established a program to develop quality metrics to guide ambulatory practices for pediatric cardiology. The council chose five areas on which to focus their efforts; chest pain, Kawasaki Disease, tetralogy of Fallot, transposition of the great arteries after arterial switch, and infection prevention. Here, we sought to describe the process, evaluation, and results of the Infection Prevention Committee's metric design process...
July 25, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28696873/modified-aortic-uncrossing-procedure-a-novel-approach-for-norwood-palliation-of-complex-univentricular-congenital-heart-disease-with-a-circumflex-aorta
#8
Roosevelt Bryant, William Wallen, Raheel Rizwan, David L Morales
The circumflex aorta is a rare type of true vascular ring anomaly. It consists of a retroesophageal right aortic arch, a left-sided descending thoracic aorta, and a left-sided ligamentum arteriosum. The "aortic uncrossing procedure" described by Planché and Lacour-Gayet is the procedure of choice for managing this aortic anomaly in patients with a biventricular heart. The presence of a circumflex aorta in a patient with heterotaxy syndrome and univentricular congenital heart disease requiring Norwood palliation is highly unusual...
July 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28690992/diabetes-mellitus-due-to-agenesis-of-the-dorsal-pancreas-in-a-patient-with-heterotaxy-syndrome
#9
Jo Eun Jung, Jin Ho Hur, Mo Kyung Jung, Ahreum Kwon, Hyun Wook Chae, Duk Hee Kim, Ho-Seong Kim
Heterotaxy syndrome (HS) is a congenital disorder resulting from an abnormal arrangement of visceral organs across the normal left-right axis in the embryonic period. HS is usually associated with multiple anomalies, including defects of the major cardiovascular system and the extracardiovascular system such as intestinal malrotation, abnormal lung lobulation, bronchus anomalies, and pancreatic dysplasia. Although pancreatic dysplasia is occasionally accompanied with HS, the occurrence of diabetes mellitus (DM) due to pancreatic dysplasia in HS is rarely reported...
June 2017: Annals of Pediatric Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28621423/roles-of-the-cilium-associated-gene-ccdc11-in-left-right-patterning-and-in-laterality-disorders-in-humans
#10
Michal Gur, Enbal Ben-Tal Cohen, Olga Genin, Abraham Fainsod, Zeev Perles, Yuval Cinnamon
Axial determination occurs during early stages of embryogenesis. Flaws in laterality patterning result in abnormal positioning of visceral organs, as manifested in heterotaxy syndrome, or complete left-right inversion as in situs inversus totalis. These malformations are often associated with ciliopathies, as seen in primary ciliary dyskinesia. We have recently described a novel mutation in the Coiled-Coil Domain-Containing 11 (CCDC11) gene associated with laterality disorders in a consanguineous family of Arab-Muslim origin with two affected siblings presenting with diverse phenotypes, one with heterotaxy syndrome and the other with non-primary ciliary dyskinesia situs inversus totalis...
2017: International Journal of Developmental Biology
https://www.readbyqxmd.com/read/28603940/outcome-of-prenatally-diagnosed-fetal-heterotaxy-a-systematic-review-and-meta-analysis
#11
REVIEW
Danilo Italo Pio Buca, Asma Khalil, Giuseppe Rizzo, Alessandra Familiari, Silvia Di Giovanni, Marco Liberati, Daniela Murgano, Alessandra Ricciardulli, Francesco Fanfani, Giovanni Scambia, Francesco D'Antonio
OBJECTIVES: To assess the perinatal outcomes of fetuses affected by heterotaxy. METHODS: Medline, Embase and Cinhal were searched. Only studies reporting a prenatal diagnosis of isomerism were included. The outcomes observed were: associated cardiac and extra-cardiac anomalies, fetal arrhythmias, abnormal karyotype, type of surgical repair and perinatal mortality. The analysis was stratified according the type of heterotaxy syndrome (left, LAI, and right, RAI, atrial isomerism)...
June 12, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28594083/fetal-echocardiographic-evaluation-in-cases-of-heterotaxy-syndrome
#12
LETTER
Behnaz Moradi, Najme-Sadat Moosavi, Mohamad Ali Kazemi
No abstract text is available yet for this article.
September 2017: Journal of Clinical Ultrasound: JCU
https://www.readbyqxmd.com/read/28566826/isomerism-in-the-setting-of-the-so-called-heterotaxy-the-usefulness-of-computed-tomographic-analysis
#13
Shumpei Mori, Robert H Anderson, Tatsuya Nishii, Kensuke Matsumoto, Rohit S Loomba
The most complex combinations of congenital cardiac malformations are found in the setting of bodily isomerism. The question remains, however, as to whether evidence of cardiac isomerism is always to be found in the setting of bodily isomerism, also known as "heterotaxy." We have previously shown that, when assessed on the basis of the extent of the pectinate muscles relative to the atrioventricular junctions, there is always isomerism of the atrial appendages in this setting. Doubt has been remained, however, as to whether these cardiac features can accurately be recognized during life...
May 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28559696/the-determination-factors-of-left-right-asymmetry-disorders-a-short-review
#14
REVIEW
Andreea Catana, Adina Patricia Apostu
Laterality defects in humans, situs inversus and heterotaxy, are rare disorders, with an incidence of 1:8000 to 1:10 000 in the general population, and a multifactorial etiology. It has been proved that 1.44/10 000 of all cardiac problems are associated with malformations of left-right asymmetry and heterotaxy accounts for 3% of all congenital heart defects. It is considered that defects of situs appear due to genetic and environmental factors. Also, there is evidence that the ciliopathies (defects of structure or function) are involved in development abnormalities...
2017: Clujul Medical (1957)
https://www.readbyqxmd.com/read/28521020/mid-term-outcomes-in-unbalanced-complete-atrioventricular-septal-defect-role-of-biventricular-conversion-from-single-ventricle-palliation
#15
Meena Nathan, Sitaram Emani, Rinske IJsselhof, Hua Liu, Kimberlee Gauvreau, Pedro Del Nido
OBJECTIVES: Management strategy for unbalanced complete atrioventricular septal defects (CASVSDs) includes single-ventricle (SV) palliation and primary or staged biventricular (BiV) repair. More recently, BiV conversion (BiVC) from SV palliation and staged BiV recruitment (BiVR) have also been advocated. This study assesses mid-term outcomes in patients with unbalanced CASVSDs according to management strategy. METHODS: Consecutive patients with unbalanced CASVSDs who underwent surgery at a tertiary care centre from January 2000 to February 2016 with institutional review board approval...
September 1, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28458851/ivemark-syndrome-bronchial-compression-from-anomalous-pulmonary-venous-anatomy
#16
Pooja H Patel, Joel Hayden, Randy Richardson
Ivemark syndrome is a heterotaxy syndrome which affects multiple organs and affects roughly 1 in every 6000 deliveries. Specifically, it can cause total anomalous pulmonary venous return and cardiac defects, which ultimately lead to decreased life expectancy. In order to better understand the nature of cardiac structures, CT angiogram has been heavily relied upon as it also allows for 3D reconstruction and optimal visualization of those features. This specific case presents with an anomalous venous return accompanied by multi-organ right isomerism that was reconstructed with 3D CT angiogram to better visualize and understand the cardiopulmonary system, as well as contribute to a fund of knowledge in hopes of discovering a solution to this condition...
March 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28453807/attrition-in-patients-with-single-ventricle-and-trisomy-21-outcomes-after-a-total-cavopulmonary-connection
#17
Anastasios C Polimenakos, Sujata Subramanian, Chawki ElZein, Michel N Ilbawi
OBJECTIVES: Data are limited regarding the management of children with trisomy 21 (T21) syndrome and a functional single ventricle (FSV). We evaluated patients with T21 and a FSV who had a total cavopulmonary connection (TCPC). METHODS: From September 1999 to August 2012, 139 patients with a FSV underwent a TCPC. Sixty-five had unbalanced atrioventricular septal defect. Thirteen had T21. Three (of 13) had heterotaxy syndrome. The mean age at the Fontan operation was 27...
May 1, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28419083/pneumococcal-vaccination-and-efficacy-in-patients-with-heterotaxy-syndrome
#18
Pei-Lan Shao, Mei-Hwan Wu, Jou-Kou Wang, Hui-Wen Hsu, Li-Min Huang, Shuenn-Nan Chiu
BackgroundPneumococcal vaccines, including pneumococcal polysaccharide vaccine (PPV) and pneumococcal conjugated vaccine (PCV), are crucial in preventing invasive pneumococcal diseases. We analyzed the pneumococcal vaccination rate, efficacy, and durability in patients with heterotaxy.MethodsAll patients with heterotaxy and CCHD who were followed up at our institution between 2010 and 2015 were included. Pneumococcal vaccine status and geometric mean concentration (GMC) of serotypes 6B, 14, 19F, and 23F were analyzed...
July 2017: Pediatric Research
https://www.readbyqxmd.com/read/28381282/coronary-sinus-aneurysm-associated-with-multiple-venous-anomalies
#19
Guang Song, Ming Du, Weidong Ren, Ke Zhou, Lu Sun
BACKGROUND: Congenital anomalies of the venous system are rare, involve the inferior vena cava (IVC), a persistent left superior vena cava (PLSVC), and the left hepatic vein (LHV), and can make cardiac diagnostic and therapeutic procedures difficult. CASE PRESENTATION: We present a 67-year-old woman without heterotaxy syndrome associated with interruption of the left IVC that continued with the hemiazygos vein system, a PLSVC, and an anomalous LHV draining the into coronary sinus (CS)...
April 5, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28367761/segregating-bodily-isomerism-or-heterotaxy-potential-echocardiographic-correlations-of-morphological-findings
#20
Cornelia Tremblay, Rohit S Loomba, Peter C Frommelt, Donald Perrin, Diane E Spicer, Carl Backer, Robert H Anderson
BACKGROUND: Bodily isomerism, also referred to as heterotaxy, involves predominantly the thoracic organs, although other organs are usually abnormally positioned. Previously assessed on the basis of splenic anatomy, it is now understood that isomerism is better segregated on the basis of atrial appendage morphology. This allows for anticipation of associated findings. We aimed to assess the accuracy of segregation based on the morphology of the atrial appendages and other structures more easily identified by echocardiography...
April 3, 2017: Cardiology in the Young
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