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heterotaxy syndrome

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https://www.readbyqxmd.com/read/27864025/heterotaxy-syndrome-with-and-without-spleen-different-infection-risk-and-management
#1
Eva Piano Mortari, Anwar Baban, Nicoletta Cantarutti, Chiara Bocci, Rachele Adorisio, Rita Carsetti
We compared the clinical disease and immunological conditions of Heterotaxy syndrome patients with or without spleen and found that asplenia is associated to significant increased frequency of infection and reduction of IgM memory B cells.
November 15, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27844411/fetal-situs-isomerism-heterotaxy-syndrome-diagnostic-evaluation-and-implication-for-postnatal-management
#2
REVIEW
Karl Degenhardt, Jack Rychik
A hallmark of vertebrate anatomy is asymmetry of structures, especially internal organs, on the left and right side of the body. Heterotaxy syndrome is the combination of correct-sided, and incorrect-sided organs. The establishment of the left-right axis is an early event in vertebrate embryogenesis. Failure to establish this axis has numerous consequences for later development and can result in a wide range of potential defects. Congenital heart disease is among the more frequent and serious problems. Heterotaxy syndrome is diagnosed prenatally with increasing frequency due to improved screening practices...
December 2016: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/27843561/predictors-of-poor-outcome-among-children-with-heterotaxy-syndrome-a-retrospective-review
#3
Eiméar McGovern, Eoin Kelleher, James E Potts, John O'Brien, Kevin Walsh, Lars Nolke, Colin J McMahon
OBJECTIVE: To determine predictors of poor outcome in patients with heterotaxy syndrome. METHODS: A retrospective review of children with heterotaxy syndrome, in a single tertiary paediatric cardiology centre, was conducted between 1 January 1997 and 1 January 2014 to determine predictors of poor outcome. Poor outcome was defined as death, cardiac transplantation or New York Heart Association (NYHA) functional class III or IV. RESULTS: There were 35 patients diagnosed with heterotaxy syndrome, 17 of whom were diagnosed antenatally...
2016: Open Heart
https://www.readbyqxmd.com/read/27821535/copy-number-variation-as-a-genetic-basis-for-heterotaxy-and-heterotaxy-spectrum-congenital-heart-defects
#4
Jason R Cowan, Muhammad Tariq, Chad Shaw, Mitchell Rao, John W Belmont, Seema R Lalani, Teresa A Smolarek, Stephanie M Ware
Genomic disorders and rare copy number abnormalities are identified in 15-25% of patients with syndromic conditions, but their prevalence in individuals with isolated birth defects is less clear. A spectrum of congenital heart defects (CHDs) is seen in heterotaxy, a highly heritable and genetically heterogeneous multiple congenital anomaly syndrome resulting from failure to properly establish left-right (L-R) organ asymmetry during early embryonic development. To identify novel genetic causes of heterotaxy, we analysed copy number variants (CNVs) in 225 patients with heterotaxy and heterotaxy-spectrum CHDs using array-based genotyping methods...
December 19, 2016: Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences
https://www.readbyqxmd.com/read/27817752/right-aortic-arch-with-isolation-of-the-left-subclavian-artery-a-rare-association-with-airway-obstruction
#5
Putri Yubbu, Haifa A Latiff, Abdel Moneim Adam Abbaker
We present two interesting cases of isolated left subclavian artery from the pulmonary artery with symptoms of upper airway obstruction. The first patient had tetralogy of Fallot, pulmonary artery sling, bilateral superior caval veins, and left bronchial isomerism, suggesting heterotaxy syndrome. The second patient had a right aortic arch, isolated left subclavian artery, and bilateral arterial ducts. These two cases are interesting because of their rarity and uncommon presentation.
November 7, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27735157/ivemark-syndrome
#6
Pushpa Masiwal, K S Chenthil, B Priyadarsini, J Gnanaprakasam, Iyengaran Srihari
Ivemark syndrome or right atrial isomerism is a rare syndrome of asplenia / hyposplenia with malformation of heart and abnormal arrangement of internal organs of chest and abdomen and is classified under heterotaxy disorder. We describe here the case of a 14 year old boy diagnosed with asplenia, dextrocardia with double outlet right ventricle and midline liver.
May 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27713611/fontan-operation-in-a-patient-with-severe-hypoplastic-right-pulmonary-artery-single-ventricle-and-heterotaxy-syndrome
#7
Jun-Yen Pan, Chu-Chuan Lin, Jen-Ping Chang
Assessment of the pulmonary circulation status including pressure, resistance, size, and absence of anatomical distortion, is crucial to the successful Fontan operation. Most patients are found to have acceptable pulmonary arteries after previous palliation, although some degree of distortion is not uncommon. However, in rare instances, some patients have only one functioning lung with another pulmonary artery seriously hypoplastic or atretic. For theses patients, completion of a Fontan operation will be challenging...
September 2016: Acta Cardiologica Sinica
https://www.readbyqxmd.com/read/27679871/factors-influencing-bacteraemia-in-patients-with-isomerism-and-chd-the-effects-of-functional-splenic-status-and-antibiotic-prophylaxis
#8
Rohit S Loomba, Andrew N Pelech, Robert H Anderson
BACKGROUND: "Heterotaxy syndrome", best segregated as isomerism, is characterised by laterality defects of the thoraco-abdominal organs, causing functional impairment. In particular, the spleen is frequently affected, increasing susceptibility to bacteraemia. This study explored factors that may increase the risk of bacteraemia in patients with isomerism. METHODS: We identified patients with CHD and isomerism. Review of outpatient, inpatient, and surgical records was conducted to collect data and determine trends in the cohort...
September 29, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27660582/pulmonary-hypertension-in-a-patient-with-congenital-heart-defects-and-heterotaxy-syndrome
#9
Tariq Yousuf, Jason Kramer, Brody Jones, Hesam Keshmiri, Muhyaldeen Dia
BACKGROUND: Heterotaxy syndrome, also called isomerism, is a condition in which abdominal and thoracic organs are located in abnormal body positions. Pulmonary hypertension (PHTN) is an uncommon clinical feature of heterotaxy syndrome. CASE REPORT: We describe the case of a 26-year-old male who developed PHTN as a rare manifestation of heterotaxy syndrome. To our knowledge, PHTN has never been reported as a prominent clinical feature in a patient with heterotaxy syndome and congenital cardiac abnormalities...
2016: Ochsner Journal
https://www.readbyqxmd.com/read/27641144/high-burden-of-genetic-conditions-diagnosed-in-a-cardiac-neurodevelopmental-clinic
#10
Paula C Goldenberg, Betsy J Adler, Ashley Parrott, Julia Anixt, Karen Mason, Jannel Phillips, David S Cooper, Stephanie M Ware, Bradley S Marino
BACKGROUND: There is a known high prevalence of genetic and clinical syndrome diagnoses in the paediatric cardiac population. These disorders often have multisystem effects, which may have an important impact on neurodevelopmental outcomes. Taken together, these facts suggest that patients and families may benefit from consultation by genetic specialists in a cardiac neurodevelopmental clinic. OBJECTIVE: This study assessed the burden of genetic disorders and utility of genetics evaluation in a cardiac neurodevelopmental clinic...
September 19, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27610349/single-institution-experience-with-the-ladd-s-procedure-in-patients-with-heterotaxy-and-stage-i-palliated-single-ventricle
#11
Kurt D Piggott, Grace George, Harun Fakioglu, Carlos Blanco, Sukumar Saguna Narasimhulu, Kamal Pourmoghadam, Hamish Munroe, William Decampli
AIM: To investigate and describe our current institutional management protocol for single-ventricle patients who must undergo a Ladd's procedure. METHODS: We retrospectively reviewed the charts of all patients from January 2005 to March 2014 who were diagnosed with heterotaxy syndrome and an associated intestinal rotation anomaly who carried a cardiac diagnosis of functional single ventricle and were status post stage I palliation. A total of 8 patients with a history of stage I single-ventricle palliation underwent Ladd's procedure during this time period...
August 8, 2016: World Journal of Clinical Pediatrics
https://www.readbyqxmd.com/read/27504813/-twin-biliary-trees-in-a-patient-with-heterotaxy-syndrome
#12
Daniel Kelly, Neil Johnson, Grushen Veldtman, Samuel Kocoshis, Rohit Kohli
No abstract text is available yet for this article.
August 6, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27405593/pediatric-echocardiography-by-work-relative-value-units-is-study-complexity-adequately-captured
#13
Sowmya Balasubramanian, Alaina K Kipps, Shea N Smith, Theresa A Tacy, Elif Seda Selamet Tierney
BACKGROUND: Present resource-based relative value unit (RVU) assignment for echocardiography is based on Current Procedural Terminology (CPT) codes, which do not incorporate complexity of diagnosis, time spent for image acquisition, or interpretation of echocardiograms. The objective of this study was to determine whether CPT-based RVU assignment accurately reflects physician effort in performing and interpreting pediatric echocardiographic examinations. METHODS: Cardiac complexity category (CCC) and physician time for study interpretation of 123 echocardiograms (June to September 2013) were prospectively assigned...
July 9, 2016: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/27403672/intestinal-rotation-anomalies
#14
Juan Carlos Pelayo, Andrea Lo
Intestinal rotation abnormality (IRA) predisposes to lethal midgut volvulus. An understanding of intestinal development illustrates the process of normal intestinal rotation and fixation. An appreciation of the clinical presentation and consequences of missed IRA will enhance clinical suspicion and timely evaluation. Selecting the appropriate imaging modality to diagnose IRA requires an understanding of the benefits and limitations of each. The Ladd's procedure continues to be the appropriate surgical treatment for IRA with or without volvulus...
July 1, 2016: Pediatric Annals
https://www.readbyqxmd.com/read/27292912/utility-of-fetal-magnetic-resonance-imaging-in-assessing-the-fetus-with-cardiac-malposition
#15
Lasya Gaur, Lauren Talemal, Dorothy Bulas, Mary T Donofrio
OBJECTIVE: Abnormal cardiac axis and/or malposition prompts evaluation of congenital heart disease; however, etiology may be difficult to clarify using obstetrical ultrasound or fetal echocardiography (echo) alone. We aimed to use fetal magnetic resonance imaging (MRI) as a complementary tool to identify causes of cardiac malposition. METHODS: Review of fetuses diagnosed with cardiac malposition by fetal ultrasound and echo was performed. Etiology was classified as either because of heterotaxy syndrome or extracardiac masses...
August 2016: Prenatal Diagnosis
https://www.readbyqxmd.com/read/27126401/anomalous-pulmonary-venous-return-insights-into-prenatal-detection
#16
Richelle Olsen, Zoe Doyle, Denis Levy, Tracy Anton, Delaram Molkara, Maryam Tarsa, Mark Sklansky, Dolores H Pretorius
OBJECTIVES: To review all cases of total anomalous pulmonary venous return (TAPVR) or partial anomalous pulmonary venous return (PAPVR) identified prenatally or postnatally at a single institution and to identify factors that may lead to a correct or missed diagnosis in both high- and low-risk fetuses on screening examinations. METHODS: Fetal images from 16 cases of prenatally or postnatally diagnosed T/PAPVR were retrospectively reviewed to analyze factors that influenced interpretations and diagnoses...
June 2016: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/27085619/the-concept-of-palliation-in-children-with-heterotaxy-syndrome
#17
EDITORIAL
Eva W Cheung, Emile A Bacha
No abstract text is available yet for this article.
May 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27085618/outcomes-of-multistage-palliation-of-infants-with-functional-single-ventricle-and-heterotaxy-syndrome
#18
COMPARATIVE STUDY
Bahaaldin Alsoufi, Courtney McCracken, Brian Schlosser, Ritu Sachdeva, Andrew Well, Brian Kogon, William Border, Kirk Kanter
BACKGROUND: Management of infants with heterotaxy syndrome and functional single ventricle is complicated due to associated cardiac and extracardiac anomalies. We report current era palliation results. METHODS: Between 2002 and 2012, 67 infants with heterotaxy syndrome underwent multistage palliation. Competing risks analyses modeled events after surgery (death vs Glenn procedure) and examined factors associated with survival. In addition, early and late outcomes following first-stage palliation surgery were compared with a matched contemporaneous control group of patients with nonheterotaxy single ventricle anomalies...
May 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27061183/prenatal-diagnosis-of-functionally-univentricular-heart-associations-and-perinatal-outcomes
#19
Aline Wolter, Sina Nosbüsch, Andreea Kawecki, Jan Degenhardt, Christian Enzensberger, Oliver Graupner, Carina Vorisek, Hakan Akintürk, Can Yerebakan, Markus Khalil, Dietmar Schranz, Jochen Ritgen, Rüdiger Stressig, Roland Axt-Fliedner
OBJECTIVE: Functionally univentricular hearts (UVHs) represent cardiac anomalies in which either the pulmonary or systemic circulation cannot be supported independently. The purpose of our study was to determine perinatal outcomes after prenatal diagnosis of functionally UVH. METHODS: We retrospectively evaluated patients who presented between 2008 and June 2015 in our centre and in prenatal practice praenatal.de in Cologne. We included double inlet left ventricle (DILV), tricuspid valve atresia (TA), pulmonary valve atresia and intact ventricular septum (PA:IVS), unbalanced atrioventricular septal defect (AVSD), heterotaxy, hypoplastic left heart syndrome (HLHS) and hypoplastic left heart complex (HLHC)...
June 2016: Prenatal Diagnosis
https://www.readbyqxmd.com/read/26991659/when-the-right-drug-should-be-left-prenatal-drug-exposure-and-heterotaxy-syndrome
#20
Nicole R van Veenendaal, Cynthia D J Kusters, Roelof-Jan Oostra, Jorieke E H Bergman, Jan-Maarten Cobben
BACKGROUND: Recent studies reported an association between prenatal propylthiouracil exposure and birth defects, including abnormal arrangement across the left-right body axis, suggesting an association with heterotaxy syndrome. METHODS: This case-control and case-finding study used data from 1981 to 2013 from the EUROCAT birth defect registry in the Northern Netherlands. First, we explored prenatal exposures in heterotaxy syndrome (cases) and Down syndrome (controls)...
July 2016: Birth Defects Research. Part A, Clinical and Molecular Teratology
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