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Heterotaxy

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https://www.readbyqxmd.com/read/28294539/angiographically-detectable-thebesian-veins-are-a-dynamic-and-reversible-finding-in-the-setting-of-congenital-heart-disease
#1
Paul Tannous, Sunil J Ghelani, Audrey C Marshall, Diego Porras
OBJECTIVE: Angiographically detectable Thebesian veins (ThVs) are a rare finding sometimes associated with coronary steal and myocardial ischemia in adults, but there are limited data regarding prominent ThVs in the setting of complex congenital heart disease (CHD). This study represents the largest series to date describing the presence and temporal changes of angiographically detectable ThVs in children with CHD. METHODS: This is a single center case series describing the clinical characteristics and coronary anatomy in children with CHD and angiographicall detectable ThVs...
March 10, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28256002/a-case-of-prenatally-detected-left-isomerism-and-hemiazygos-continuation-of-inferior-vena-cava
#2
Behnaz Moradi, Najme-Sadat Moosavi, Mohamad Ali Kazemi, Ahmad-Reza Tahmasebpour, Farzaneh Fattahi Masrour
Heterotaxy syndromes are defined as the disorders that involve abnormal arrangement of viscera. We present a case of prenatally diagnosed left isomerism in a 30-year-old primigravida woman referred to our hospital for complex cardiac abnormality. Sonographic findings included heart block, unbalanced atrioventricular septal defect, interruption of the inferior vena cava with hemiazygos continuation, double superior vena cava, a right-sided stomach, and biliary atresia. The hemiazygos vein drained into the right atrium by the persistent left superior vena cava...
March 3, 2017: Journal of Clinical Ultrasound: JCU
https://www.readbyqxmd.com/read/28199048/practice-variability-in-management-of-infectious-issues-in-heterotaxy-a-survey-of-pediatric-cardiologists
#3
Rohit S Loomba, Gabrielle Geddes, Amanda J Shillingford, David A Hehir
BACKGROUND: Splenic dysfunction is common in heterotaxy syndrome, and increases the risk of bacteremia and bacteremia related mortality. Despite the risks associated with bacteremia in this setting, best practice guidelines for management of infectious concerns are lacking. We conducted a survey of pediatric cardiologists to characterize practice regarding the diagnosis of splenic dysfunction, approach to antibiotic prophylaxis, and management of possible bacterial infection. METHODS: A 22-item web-based survey was distributed via email to pediatric cardiologists in North America...
February 15, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28195132/analysis-of-craniocardiac-malformations-in-xenopus-using-optical-coherence-tomography
#4
Engin Deniz, Stephan Jonas, Michael Hooper, John N Griffin, Michael A Choma, Mustafa K Khokha
Birth defects affect 3% of children in the United States. Among the birth defects, congenital heart disease and craniofacial malformations are major causes of mortality and morbidity. Unfortunately, the genetic mechanisms underlying craniocardiac malformations remain largely uncharacterized. To address this, human genomic studies are identifying sequence variations in patients, resulting in numerous candidate genes. However, the molecular mechanisms of pathogenesis for most candidate genes are unknown. Therefore, there is a need for functional analyses in rapid and efficient animal models of human disease...
February 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28191656/utility-and-safety-of-combined-interventional-catheterization-and-electrophysiology-procedures-in-a-children-s-hospital
#5
S Yukiko Asaki, Jeffrey W Orcutt, Christina Y Miyake, Henri Justino, Caridad M DE LA Uz, Jeffrey J Kim, Santiago O Valdes, Athar M Qureshi
BACKGROUND: Interventional cardiac catheterization (cath) and electrophysiology (EP) procedures are not routinely performed together. There are several perceived barriers affecting this practice, though there are also advantages for both the patient and practitioner to a combined approach. METHODS: This was a single center retrospective study reviewing combined cath and EP procedures with a pre-procedural intention to intervene at Texas Children's Hospital from 2001 to 2014...
February 13, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28055329/percutaneous-transhepatic-fontan-kreutzer-completion-of-hepatic-vein-inclusion
#6
Ignacio Juaneda, Alejandro Peirone, Adolfo Ferrero Guadagnoli, Alejandro Contreras, Santiago Orozco, Juan Diaz, Christian Kreutzer
We report the case of an 11-year-old girl with heterotaxy syndrome, dextrocardia, and azygos continuation of an interrupted inferior vena cava who had developed pulmonary arteriovenous fistulas after a Kawashima procedure consisting of bilateral superior cavopulmonary anastomoses. She presented with profound cyanosis, fatigue, and failure to thrive. An operative procedure to direct hepatic vein effluent to the pulmonary circulation was performed with placement of an extracardiac conduit between the hepatic veins and the left pulmonary artery...
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/27878633/cardiopulmonary-exercise-testing-in-fontan-patients-with-and-without-isomerism-heterotaxy-as-compared-to-patients-with-primary-ciliary-dyskinesia-and-subjects-with-structurally-normal-hearts
#7
Rohit S Loomba, Michael Danduran, Kim G Nielsen, Astrid M Ring, Joshua Kovach, Robert H Anderson
Isomerism, also known as heterotaxy, is a clinical entity that impacts multiple organ systems both anatomically and functionally. The airways and lungs are involved in a great number of these patients, leading to increased sinopulmonary symptoms, increased need for oxygenation, and increased postoperative ventilatory support. Additionally, these patients often have congenital heart disease requiring Fontan palliation. What has not been previously described, and is the focus of this study, is the results of cardiopulmonary exercise testing in those who have undergone Fontan palliation with and without isomerism...
November 23, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27864025/heterotaxy-syndrome-with-and-without-spleen-different-infection-risk-and-management
#8
Eva Piano Mortari, Anwar Baban, Nicoletta Cantarutti, Chiara Bocci, Rachele Adorisio, Rita Carsetti
No abstract text is available yet for this article.
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
#9
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
#10
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/27832130/detecting-mechanisms-of-karyotype-evolution-in-heterotaxis-orchidaceae
#11
Ana Paula Moraes, André Olmos Simões, Dario Isidro Ojeda Alayon, Fábio de Barros, Eliana Regina Forni-Martins
The karyotype is shaped by different chromosome rearrangements during species evolution. However, determining which rearrangements are responsible for karyotype changes is a challenging task and the combination of a robust phylogeny with refined karyotype characterization, GS measurements and bioinformatic modelling is necessary. Here, this approach was applied in Heterotaxis to determine what chromosome rearrangements were responsible for the dysploidy variation. We used two datasets (nrDNA and cpDNA, both under MP and BI) to infer the phylogenetic relationships among Heterotaxis species and the closely related genera Nitidobulbon and Ornithidium...
2016: PloS One
https://www.readbyqxmd.com/read/27821535/copy-number-variation-as-a-genetic-basis-for-heterotaxy-and-heterotaxy-spectrum-congenital-heart-defects
#12
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/27821534/heterotaxy-in-caenorhabditis-widespread-natural-variation-in-left-right-arrangement-of-the-major-organs
#13
Melissa R Alcorn, Davon C Callander, Agustín López-Santos, Yamila N Torres Cleuren, Bilge Birsoy, Pradeep M Joshi, Anna W Santure, Joel H Rothman
Although the arrangement of internal organs in most metazoans is profoundly left-right (L/R) asymmetric with a predominant handedness, rare individuals show full (mirror-symmetric) or partial (heterotaxy) reversals. While the nematode Caenorhabditis elegans is known for its highly determinate development, including stereotyped L/R organ handedness, we found that L/R asymmetry of the major organs, the gut and gonad, varies among natural isolates of the species in both males and hermaphrodites. In hermaphrodites, heterotaxy can involve one or both bilaterally asymmetric gonad arms...
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
#14
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
#15
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
#16
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
#17
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/27676165/transhepatic-permanent-pacing-in-a-child-with-complex-cyanotic-heart-disease-after-total-cavo-pulmonary-shunt-kawashima-repair
#18
Anilkumar Singhi, Ejaz Ahmed Sheriff, Kothandam Sivakumar
Complex cyanotic congenital heart diseases with left isomerism are sometimes associated with atrioventricular nodal conduction disturbances that may need permanent pacing. Surgical palliation in such anatomy connecting the superior vena cava to the pulmonary artery precludes a transvenous access for an endocardial pacing lead to the ventricles. Epicardial leads in these patients fail if the pacing thresholds are very high. We report transhepatic permanent ventricular lead implantation for a young boy with heterotaxy complicated by complete heart block...
March 2016: Indian Pacing and Electrophysiology Journal
https://www.readbyqxmd.com/read/27660582/pulmonary-hypertension-in-a-patient-with-congenital-heart-defects-and-heterotaxy-syndrome
#19
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
#20
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
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