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Heterotaxy

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https://www.readbyqxmd.com/read/28521020/mid-term-outcomes-in-unbalanced-complete-atrioventricular-septal-defect-role-of-biventricular-conversion-from-single-ventricle-palliation%C3%A2
#1
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...
May 18, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28458851/ivemark-syndrome-bronchial-compression-from-anomalous-pulmonary-venous-anatomy
#2
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
#3
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
#4
Pei-Lan Shao, Mei-Hwan Wu, Jou-Kou Wang, Hui-Wen Hsu, Li-Min Huang, Shuenn-Nan Chiu
BACKGROUND: Pneumococcal vaccines, including pneumococcal polysaccharide vaccine (PPV) and pneumococcal conjugated vaccine (PCV), are important in preventing invasive pneumococcal disease. We analyzed the pneumococcal vaccination rate, efficacy and durability in patients with heterotaxy. METHODS: All patients with heterotaxy and CCHD who were followed 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...
April 18, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28381282/coronary-sinus-aneurysm-associated-with-multiple-venous-anomalies
#5
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
#6
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
https://www.readbyqxmd.com/read/28329229/isolated-severe-leftward-displacement-of-the-septum-primum-anatomic-and-3d-echocardiographic-findings-and-surgical-repair
#7
Fabio Cuttone, Khaled Hadeed, François Lacour-Gayet, Hugues Lucron, Sebastien Hascoet, Philippe Acar, Bertrand Leobon, Richard Van Praagh
OBJECTIVES: Leftward displacement of the septum primum is usually described as associated with hypoplastic left heart syndrome or visceral heterotaxy. This rare malformation results in partially or totally anomalous pulmonary venous drainage with a normal connection of the pulmonary veins to the left atrium, depending on the degree of septal shift. We report the 3D echocardiographic and anatomic findings as well as the surgical repair in a series of isolated severe leftward displacement of the septum primum, responsible for totally anomalous pulmonary venous drainage...
February 16, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28294539/angiographically-detectable-thebesian-veins-are-a-dynamic-and-reversible-finding-in-the-setting-of-congenital-heart-disease
#8
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
#9
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
#10
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
#11
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
#12
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 preprocedural 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
#13
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
#14
COMPARATIVE STUDY
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...
February 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/27864025/heterotaxy-syndrome-with-and-without-spleen-different-infection-risk-and-management
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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