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heterotaxy syndrome

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https://www.readbyqxmd.com/read/29779315/-improving-outcomes-of-patients-with-heterotaxy-and-functional-single-ventricle-a-10-year-follow-up-of-70-cases-in-a-single-institution
#1
F Cao, M H Zou, L Ma, Y S Xia, S C Yang, W D Chen, G D Huang, X X Chen
Objective: To review current-era palliation outcomes of patients with heterotaxy and functional single ventricle in a single institution. Methods: The clinical data of 70 consecutive patients with heterotaxy undergoing multistage single ventricle palliation in Guangzhou Women and Children's Medical Center from January 2008 to December 2017 were retrospectively reviewed, and the prognosis factors for mortality were analyzed. There were 53 male and 17 female patients.The median age was 13.3 months (range: 6 days to 150 months)...
May 1, 2018: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/29724030/some-isolated-cardiac-malformations-can-be-related-to-laterality-defects
#2
REVIEW
Paolo Versacci, Flaminia Pugnaloni, Maria Cristina Digilio, Carolina Putotto, Marta Unolt, Giulio Calcagni, Anwar Baban, Bruno Marino
Human beings are characterized by a left⁻right asymmetric arrangement of their internal organs, and the heart is the first organ to break symmetry in the developing embryo. Aberrations in normal left⁻right axis determination during embryogenesis lead to a wide spectrum of abnormal internal laterality phenotypes, including situs inversus and heterotaxy . In more than 90% of instances, the latter condition is accompanied by complex and severe cardiovascular malformations. Atrioventricular canal defect and transposition of the great arteries—which are particularly frequent in the setting of heterotaxy —are commonly found in situs solitus with or without genetic syndromes...
May 2, 2018: Journal of Cardiovascular Development and Disease
https://www.readbyqxmd.com/read/29722020/atrioventricular-canal-defect-and-genetic-syndromes-the-unifying-role-of-sonic-hedgehog
#3
REVIEW
M C Digilio, F Pugnaloni, A De Luca, G Calcagni, A Baban, M L Dentici, P Versacci, B Dallapiccola, M Tartaglia, B Marino
The atrioventricular canal defect (AVCD) is a congenital heart defect (CHD) frequently associated with extracardiac anomalies (75%). Previous observations from a personal series of patients with AVCD and "polydactyly syndromes" demonstrated that the distinct morphology and combination of AVCD features in some of these syndromes is reminiscent of the cardiac phenotype found in heterotaxy, a malformation complex previously associated with functional cilia abnormalities and aberrant Hedgehog (Hh) signaling...
May 3, 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29609227/-characteristics-and-prognosis-of-interrupted-inferior-vena-cava-with-azygous-continuation
#4
Q Xu, H X Sun, J S Xie, J L Wang, Q Y Pei, X H Zhang
Objective: To explore the clinical characteristics of interrupted of the inferior vena cava with azygous continuation and the prognosis. Methods: Retrospective analysis of 21 fetuses diagnosed with interrupted inferior vena cava with azygous continuation among 28 567 pregnant women who underwent routine ultrasound scan. The clinical data, ultrasonographic features, genetic information and prognosis were collected. Results: Interrupted of the inferior vena cava with azygous continuation occurred in 21(0.07%, 21/28 567) of 28 567 patients...
March 25, 2018: Zhonghua Fu Chan Ke za Zhi
https://www.readbyqxmd.com/read/29605269/is-isomerism-a-risk-factor-for-intestinal-volvulus
#5
Rachel M Landisch, Rohit S Loomba, Jose H Salazar, Matthew W Buelow, Michele Frommelt, Robert H Anderson, Amy J Wagner
INTRODUCTION: Isomerism, or heterotaxy syndrome, affects many organ systems anatomically and functionally. Intestinal malrotation is common in patients with isomerism. Despite a low reported risk of volvulus, some physicians perform routine screening and prophylactic Ladd procedures on asymptomatic patients with isomerism who are found to have intestinal malrotation. The primary aim of this study was to determine if isomerism is an independent risk factor for volvulus. METHODS: Kid's Inpatient Database data from 1997 to 2012 was utilized for this study...
March 6, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29559246/intrahepatic-cholangiocarcinoma-after-fontan-procedure-in-an-adult-with-visceral-heterotaxy
#6
Dehua Wang, Darren Marshall, Gruschen Veldtman, Anita Gupta, Andrew T Trout, Juan Villafane, Kevin Bove
Hepatic dysfunction, including development of hepatocellular carcinoma and other liver lesions has been increasingly reported following Fontan procedure for congenital heart disease. We report a unique case of intrahepatic cholangiocarcinoma 28 years after a Fontan procedure in a 31year old female with heterotaxy syndrome. The subcapsular mass-forming tumor was composed of poorly differentiated tumor cells arranged in small vague glandular or slit-lumen nests, and focally fused or anastomosing large trabecular patterns within the prominent fibrotic stroma...
March 15, 2018: Pathology, Research and Practice
https://www.readbyqxmd.com/read/29552537/primary-ciliary-dyskinesia-and-situs-ambiguus-a-rare-association
#7
Kumar Narahari Narahari, Anu Kapoor, Sarat Kumar Sanamandra, Surya Ramachandra Varma Gunturi
Primary ciliary dyskinesia (PCD) is a rare disorder with impaired ciliary function resulting in a spectrum of clinical manifestations of varying severity. PCD affects approximately one in every 20,000 individuals with a reported prevalence between 1:4000 and 1:50,000. Due to its nonspecific clinical features, the condition is usually diagnosed late in its course, unless situs inversus (SI) or organ laterality defects are discovered at imaging. A small subset of patients with PCD display associated organ laterality defects, different from the classical SI totalis...
January 2018: International Journal of Applied and Basic Medical Research
https://www.readbyqxmd.com/read/29544591/heterotaxy-syndrome
#8
Khalid Mahmood, Mukhtar A Memon, Shabbir A Naeem
No abstract text is available yet for this article.
March 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/29479560/atypical-perioperative-management-for-duodenal-obstruction-in-an-infant-with-heterotaxy-syndrome-a-case-report
#9
Hirofumi Obinata, Shinichi Nishibe, Yoko Ishihara
Background: Heterotaxy syndrome (HS) is characterized by a wide variety of cardiac and extra-cardiac malformations, including pulmonary valve stenosis, interruption of the inferior vena cava, total anomalous pulmonary venous connection (TAPVC), asplenia, polysplenia, intestinal malrotation, and preduodenal portal vein (PDPV). We report the case of a heterotaxic infant with an infracardiac TAPVC and preduodenal portal vein who experienced repetitive hemodynamic instability during urgent laparotomy for duodenal obstruction...
2018: JA Clinical Reports
https://www.readbyqxmd.com/read/29457852/successful-management-of-living-donor-liver-transplantation-for-biliary-atresia-with-single-ventricle-physiology-from-peri-transplant-through-total-cavopulmonary-connection-a-case-report
#10
Yohei Yamada, Ken Hoshino, Takayuki Oyanagi, Ryohei Gatayama, Jun Maeda, Nobuyuki Katori, Yasushi Fuchimoto, Taizo Hibi, Masahiro Shinoda, Kentaro Matsubara, Hideaki Obara, Ryo Aeba, Yuko Kitagawa, Hiroyuki Yamagishi, Tatsuo Kuroda
Children with single ventricle physiology have complete mixing of the pulmonary and systemic circulations, requiring staged procedures to achieve a separation of these circulations, or Fontan circulation. The single ventricle physiology significantly increases the risk of mortality in children undergoing non-cardiac surgery. As liver transplantation for patients with single ventricle physiology is particularly challenging, only a few reports have been published. We herein report a case of successful LDLTx for an 8-month-old pediatric patient with biliary atresia, heterotaxy, and complex heart disease of single ventricle physiology...
May 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29453132/malrotation-is-not-associated-with-adverse-outcomes-after-cardiac-surgery-in-patients-with-heterotaxy-syndrome
#11
Shelby C White, Peter N Dean, Eugene D McGahren, James J Gangemi, Jeffrey Vergales
BACKGROUND: Patients with heterotaxy syndrome (HS) often have asymptomatic malrotation. There is a lack of consensus regarding the management of these patients, particularly in patients with complex congenital heart disease (CHD). We sought to describe the prevalence of malrotation and incidence of volvulus in a population of patients with complex CHD and to identify the impact of malrotation on morbidity and mortality following cardiac surgery. METHODS: We performed a retrospective review of all patients with HS and complex CHD who required cardiac surgery in the first year of life at a single center between October 1995 and September 2015...
January 31, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29445263/noncompaction-cardiomyopathy-and-heterotaxy-syndrome
#12
Hugo R Martinez, Stephanie M Ware, Marcus S Schamberger, John J Parent
Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by compact and trabecular layers of the left ventricular myocardium. This cardiomyopathy may occur with congenital heart disease (CHD). Single cases document co-occurrence of LVNC and heterotaxy, but no data exist regarding the prevalence of this association. This study sought to determine whether a non-random association of LVNC and heterotaxy exists by evaluating the prevalence of LVNC in patients with heterotaxy. In a retrospective review of the Indiana Network for Patient Care, we identified 172 patients with heterotaxy (69 male, 103 female)...
September 2017: Progress in Pediatric Cardiology
https://www.readbyqxmd.com/read/29444812/classifying-heterotaxy-syndrome-time-for-a-new-approach
#13
EDITORIAL
Stephen P Sanders, Tal Geva
No abstract text is available yet for this article.
February 2018: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29442328/zic3-in-heterotaxy
#14
Helen M Bellchambers, Stephanie M Ware
Mutation of ZIC3 causes X-linked heterotaxy, a syndrome in which the laterality of internal organs is disrupted. Analysis of model organisms and gene expression during early development suggests ZIC3-related heterotaxy occurs due to defects at the earliest stage of left-right axis formation. Although there are data to support abnormalities of the node and cilia as underlying causes, it is unclear at the molecular level why loss of ZIC3 function causes such these defects. ZIC3 has putative roles in a number of developmental signalling pathways that have distinct roles in establishing the left-right axis...
2018: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29415491/is-an-appreciation-of-isomerism-the-key-to-unlocking-the-mysteries-of-the-cardiac-findings-in-heterotaxy
#15
REVIEW
Robert H Anderson, Diane E Spicer, Rohit Loomba
Pediatric cardiologists treating patients with severe congenital cardiac defects define "visceral heterotaxy" on the basis of isomerism of the atrial appendages. The isomeric features represent an obvious manifestation of disruption of left-right asymmetry during embryonic development. Thus, there are two subsets of individuals within the overall syndrome, with features of either right or left isomerism. Within the heart, it is only the atrial appendages that are truly isomeric. The remainder of the cardiac components shows variable morphology, as does the arrangement of the remaining body organs...
February 6, 2018: Journal of Cardiovascular Development and Disease
https://www.readbyqxmd.com/read/29383228/heterotaxy-syndrome-with-intestinal-malrotation-polysplenia-and-azygos-continuity
#16
Stéphanie Cupers, Christine Van Linthout, Brigitte Desprechins, Léon Rausin, Martine Demarche, Marie-Christine Seghaye
No abstract text is available yet for this article.
January 8, 2018: Clinics and Practice
https://www.readbyqxmd.com/read/29326834/ablation-of-atrial-fibrillation-in-patients-with-congenital-heart-disease
#17
Marwan M Refaat, Jad Ballout, Moussa Mansour
With improved surgical techniques and medical management for patients with congenital heart diseases, more patients are living longer and well into adulthood. This improved survival comes with a price of increased morbidity, mainly secondary to increased risk of tachyarrhythmias. One of the major arrhythmias commonly encountered in this subset of cardiac patients is AF. Similar to the general population, the risk of AF increases with advancing age, and is mainly secondary to the abnormal anatomy, abnormal pressure and volume parameters in the hearts of these patients and to the increased scarring and inflammation seen in the left atrium following multiple surgical procedures...
December 2017: Arrhythmia & Electrophysiology Review
https://www.readbyqxmd.com/read/29299107/the-missing-link-a-case-of-absent-pituitary-infundibulum-and-ectopic-neurohypophysis-in-a-pediatric-patient-with-heterotaxy-syndrome
#18
Adil Omer, Dana Haddad, Leszek Pisinski, Alan V Krauthamer
We report a case of absent pituitary infundibulum and ectopic neurohypophysis in a 4-year-old patient presenting clinically with hypopituitarism as well as heterotaxy syndrome complicated by global developmental delay and growth retardation. The clinical and laboratory workup of our patient suggested underlying hypopituitarism related to either congenital or acquired pathology, necessitating MRI to distinguish between them. We explain the various structural causes of hypopituitarism and detail how to predict the MRI findings and treatment, based on a fundamental understanding of the anatomy and pathophysiology of the hypothalamic pituitary axis and distinguishing anterior versus posterior pituitary hormone derangements...
September 2017: Journal of Radiology Case Reports
https://www.readbyqxmd.com/read/29245220/case-report-of-intestinal-non-rotation-heterotaxy-and-polysplenia-in-a-patient-with-pancreatic-cancer
#19
Spyridon Pagkratis, Sara Kryeziu, Miranda Lin, Samah Hoque, Juan Carlos Bucobo, Jonathan M Buscaglia, Georgios V Georgakis, Aaron R Sasson, Joseph Kim
RATIONALE: Heterotaxy with polysplenia is an extremely rare congenital condition resulting from abnormal arrangement of organs in the abdominal and thoracic cavities during embryologic development. When a malignancy such as pancreatic cancer develops under these conditions, surgical resection becomes particularly complex. This case report demonstrates successful pancreatic cancer resection despite the patient's complicated anatomy. PATIENT CONCERNS: An 82-year-old female presented to our institution with complaints of mild right upper quadrant pain radiating to the mid-epigastric region...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29169330/comparing-levocardia-and-dextrocardia-in-fetuses-with-heterotaxy-syndrome-prenatal-features-clinical-significance-and-outcomes
#20
Xiaofang Wang, Yifan Shi, Shi Zeng, Jiawei Zhou, Jia Zhou, Hongxia Yuan, Lin Wang, Weiyuan Shi, Qichang Zhou
BACKGROUND: To investigate the differences in cardiovascular disease, extracardiac anomalies and outcomes between fetuses with levocardia and dextrocardia. METHODS: Clinical demographics, prenatal features, postnatal characteristics and the outcomes of fetuses with levocardia or dextrocardia were recorded and analyzed. RESULTS: Sixty-five fetuses with dextrocardia and thirty-eight fetuses with levocardia were enrolled. Right ventricle outlet obstruction, atrioventricular septal defect and intestinal malrotation were common in both groups...
November 23, 2017: BMC Pregnancy and Childbirth
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