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Transposition great arteries

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https://www.readbyqxmd.com/read/28645799/genotype-phenotype-evaluation-of-med13l-defects-in-the-light-of-a-novel-truncating-and-a-recurrent-missense-mutation
#1
Reza Asadollahi, Markus Zweier, Laura Gogoll, Raphael Schiffmann, Heinrich Sticht, Katharina Steindl, Anita Rauch
A decade after the designation of MED13L as a gene and its link to intellectual disability (ID) and dextro-looped transposition of great arteries in 2003, we previously described a recognizable syndrome due to MED13L haploinsufficiency. Subsequent reports of 22 further patients diagnosed by genome-wide testing further delineated the syndrome with expansion of the phenotypic spectrum and showed reduced penetrance for congenital heart defects. We now report two novel patients identified by whole exome sequencing, one with a de novo MED13L truncating mutation and the other with a de novo missense mutation...
June 20, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28645293/prenatal-screening-of-fetal-ventriculoarterial-connections-benefits-of-4d-technique-in-fetal-heart-imaging
#2
Yu Wang, Miao Fan, Faiza Amber Siddiqui, Meilian Wang, Wei Sun, Xue Sun, Wenjia Lei, Ying Zhang
BACKGROUND: Identification of prenatal ventriculoarterial connections in fetuses with conotruncal anomalies (CTA) remains one of the greatest challenges for sonographers performing screening examinations. Herein, we propose a novel protocol of 4D volume analysis that identifies ventriculoarterial connections and evaluate its clinical utility in routine screenings. METHODS: Twenty-nine cases of transposition of the great arteries (TGA), 22 cases of double-outlet right ventricle (DORV), 36 cases of tetralogy of Fallot (TOF), 14 cases of truncus arteriosus (TCA), and randomly selected 70 normal fetuses were reviewed in this study...
June 23, 2017: Cardiovascular Ultrasound
https://www.readbyqxmd.com/read/28632651/imaging-in-adult-congenital-heart-disease
#3
Stephanie S Gaydos, Akos Varga-Szemes, Rochelle N Judd, Pal Suranyi, David Gregg
There has been tremendous growth in the population of adults with congenital heart disease (CHD) over the last few decades because of advances in medical care. Whereas some cases are cured during childhood, most patients instead undergo palliation, which leaves them at risk for late complications. Lifelong clinical follow-up involving serial multimodality imaging is helpful to monitor and guide the treatment of late complications. Imaging of these individuals is challenging because of their unique anatomy and therefore requires careful consideration on a case-by-case basis...
July 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/28629628/left-ventricular-outflow-tract-obstruction-and-its-impact-on-systolic-ventricular-function-and-exercise-capacity-in-adults-with-a-subaortic-right-ventricle
#4
Annina Stauber, Céline Wey, Matthias Greutmann, Daniel Tobler, Kerstin Wustmann, Andreas Wahl, Emanuela R Valsangiacomo Buechel, Matthias Wilhelm, Markus Schwerzmann
BACKGROUND: In biventricular hearts the filling and contractility of one ventricle affects the performance of the other. In this study, we compared right ventricular systolic function and exercise capacity in patients with a subaortic right ventricle (RV) in relation to the presence of a left ventricular outflow tract obstruction (LVOTO). METHODS: Retrospective chart review of adults with congenitally corrected transposition of the great arteries (ccTGA) or with a previous atrial switch procedure for complete TGA (D-TGA)...
June 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28629487/learning-and-memory-in-adolescents-with-critical-biventricular-congenital-heart-disease
#5
Adam R Cassidy, Jane W Newburger, David C Bellinger
OBJECTIVES: Although evidence exists of broadly defined memory impairment among adolescents with critical congenital heart disease (CHD), nuanced investigations of declarative memory in this at-risk population have not been conducted. This study had two primary aims: (1) to conduct a fine-grained analysis of a range of relevant learning and memory processes in adolescents with critical biventricular CHD, and (2) to identify risk, odds, and predictors of memory impairment. METHODS: Data were combined from two single-center studies of neurodevelopmental outcomes in critical CHD...
June 20, 2017: Journal of the International Neuropsychological Society: JINS
https://www.readbyqxmd.com/read/28610439/congenitally-corrected-transposition-complex-anatomic-repair-or-fontan-pathway
#6
Supreet P Marathe, Matthew I Jones, Julian Ayer, Jessica Sun, Yishay Orr, Charlotte Verrall, Ian A Nicholson, Richard B Chard, Gary F Sholler, David S Winlaw
Background Successful anatomic repair of congenitally corrected transposition of the great arteries achieves excellent outcomes. Several centers report excellent long-term survival with the Fontan pathway as well. We have selectively applied both approaches depending on individual patient morphology, with anatomic repair preferred but utilizing the Fontan pathway when high technical complexity or operative risk is anticipated. Methods Hospital records over an 18-year period (1998-2016) were reviewed to identify patients with congenitally corrected transposition of the great arteries who underwent surgical management...
January 1, 2017: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/28608149/exercise-capacity-and-self-efficacy-are-associated-with-moderate-to-vigorous-intensity-physical-activity-in-children-with-congenital-heart-disease
#7
Laura Banks, Shelly Rosenthal, Cedric Manlhiot, Chun-Po Steve Fan, Adam McKillop, Patricia E Longmuir, Brian W McCrindle
This study sought to determine whether exercise capacity, self-efficacy, and gross motor skills are associated with moderate-to-vigorous physical activity (MVPA) levels in children, and if these associations differ by congenital heart disease (CHD) type. Medical history was abstracted from chart review. We assessed MVPA levels (via accelerometry), percent-predicted peak oxygen consumption ([Formula: see text] cardiopulmonary exercise test), gross motor skill percentiles (test of gross motor development version-2), and self-efficacy [children's self-perceptions of adequacy and predilection for physical activity scale (CSAPPA scale)]...
June 12, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28598551/coronary-artery-bypass-grafting-in-infants-children-and-young-adults-for-acquired-and-congenital-lesions
#8
Constantine Mavroudis
Coronary artery disease ranges from congenital in origin such as anomalous aortic origin of a coronary artery (AAOCA) to acquired diseases such as Kawasaki disease, and previously repaired conditions such as transposition of the great arteries. It is not uncommon, in the long run, for proximal coronary stenosis to develop following arterial switch, AAOCA repair, Ross procedure and Kawasaki disease leading to coronary artery bypass grafing (CABG). The objective of this report is to discuss the indications, challenges, and outcomes of CABG in infants, children, and young adults with acquired and congenital lesions...
June 9, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28597760/late-onset-pulmonary-arterial-hypertension-in-repaired-d-transposition-of-great-arteries-an-uncommon-complication
#9
Janani Baskaran, Kimara S March, Thenappan Thenappan
Late-onset pulmonary arterial hypertension (PAH) is a rare but fatal complication in patients with childhood surgical repair of dextro-transposition of great arteries (D-TGA), especially with the Mustard and Senning procedures. The pathogenic mechanisms of PAH in patients with repaired D-TGA are not well understood and treatment is not standardized. In this manuscript, we present a case of late-onset PAH in an adult D-TGA patient after Mustard repair and discuss the pathogenic mechanisms, diagnosis, and treatment of pulmonary hypertension in repaired D-TGA...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28591192/perinatal-outcomes-and-congenital-heart-defect-prognosis-in-53313-non-selected-perinatal-infants
#10
Donghua Xie, Hua Wang, Zhiyu Liu, Junqun Fang, Tubao Yang, Shujin Zhou, Aihua Wang, Jiabi Qin, Lili Xiong
OBJECTIVE: To evaluate perinatal outcomes and congenital heart defect (CHD) prognosis in a non-selected population. METHODS: The population-based surveillance data used in this assessment of CHDs were based on birth defect surveillance data collected from 2010-2012 in Liuyang City, China. Infants living with CHDs were followed up for 5 years to determine their prognosis. Prevalence, prenatal diagnosis, perinatal outcomes, and total and type-specific prognosis data were assessed using SPSS 18...
2017: PloS One
https://www.readbyqxmd.com/read/28580763/knowledge-based-reconstruction-for-measurement-of-right-ventricular-volumes-on-cardiovascular-magnetic-resonance-images-in-a-mixed-population
#11
Elise D Pieterman, Ricardo P J Budde, Daniëlle Robbers-Visser, Ron T van Domburg, Willem A Helbing
OBJECTIVE: Follow-up of right ventricular performance is important for patients with congenital heart disease. Cardiac magnetic resonance imaging is optimal for this purpose. However, observer-dependency of manual analysis of right ventricular volumes limit its use. Knowledge-based reconstruction is a new semiautomatic analysis tool that uses a database including knowledge of right ventricular shape in various congenital heart diseases. We evaluated whether knowledge-based reconstruction is a good alternative for conventional analysis...
June 5, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28579248/echocardiography-derived-left-ventricular-outflow-tract-gradient-and-left-ventricular-posterior-wall-thickening-are-associated-with-outcomes-for-anatomic-repair-in-congenitally-corrected-transposition-of-the-great-arteries
#12
Shreya Moodley, Sowmya Balasubramanian, Theresa A Tacy, Frandics Chan, Frank L Hanley, Rajesh Punn
BACKGROUND: Congenitally corrected transposition of the great arteries is a rare form of congenital heart disease. Management is controversial; options include observation, physiologic repair, and anatomic repair. Assessment of morphologic left ventricle preparedness is key in timing anatomic repair. This study's purpose was to review the modalities used to assess the morphologic left ventricle preoperatively and to determine if any echocardiographic variables are associated with outcomes...
June 1, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/28579227/d-tga-combined-with-left-arch-atresia-of-a-double-aortic-arch
#13
Dan Zhou, Laichun Song, Liang Tao, Hong Zhou
A three-month-old female underwent an arterial switch operation for transposition of the great arteries (TGA) with concomitant ventricular septal defect (VSD). After the operation, the patient suffered from stubborn pulmonary infection with increased airway resistance, and could not be weaned from a ventilator. Multispiral computed tomography (CT) scanning suggested a double aortic arch with left arch atresia. The patient underwent a second procedure to resection the left aortic arch. The patient gradually recovered and was successfully weaned from the ventilator...
May 5, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28566836/apparent-normal-arrangement-pattern-of-three-vessel-view-in-a-fetus-with-transposition-of-great-arteries-and-l-malposed-aorta
#14
Sudeep Verma, Shanthi Chidambaratanu, Raja Vijaylakshmi, Latha Srinivasan, Indrani Suresh
Transposition of great arteries (TGA) is more commonly associated with D-malposition of great arteries where anterior aorta produces characteristic "I" sign in the three-vessel view (3VV) in fetal heart imaging. We describe two cases with TGA and L-malposition of aorta where 3VV imaging showed an apparently normal arrangement of vessels while outflow tract imaging proved vital in diagnosing transposition anatomy. Apparently, normal 3VV in the presence of disproportionate vessel caliber and inability to produce normal outflow images should raise the suspicion...
May 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28566818/three-dimensional-printed-cardiac-prototypes-aid-surgical-decision-making-and-preoperative-planning-in-selected-cases-of-complex-congenital-heart-diseases-early-experience-and-proof-of-concept-in-a-resource-limited-environment
#15
Mahesh Kappanayil, Nageshwara Rao Koneti, Rajesh R Kannan, Brijesh P Kottayil, Krishna Kumar
INTRODUCTION: Three-dimensional. (3D) printing is an innovative manufacturing process that allows computer-assisted conversion of 3D imaging data into physical "printouts" Healthcare applications are currently in evolution. OBJECTIVE: The objective of this study was to explore the feasibility and impact of using patient-specific 3D-printed cardiac prototypes derived from high-resolution medical imaging data (cardiac magnetic resonance imaging/computed tomography [MRI/CT]) on surgical decision-making and preoperative planning in selected cases of complex congenital heart diseases (CHDs)...
May 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28566262/effect-of-phosphodiesterase-5-inhibition-with-tadalafil-on-systemic-right-ventricular-size-and-function-a-multi-center-double-blind-randomized-placebo-controlled-clinical-trial-serve-trial-rational-and-design
#16
Daniel Tobler, Judith Bouchardy, Engel Reto, Dik Heg, Christian Müller, André Frenk, Harald Gabriel, Jürg Schwitter, Tobias Rutz, Ronny R Buechel, Matthias Willhelm, Lukas Trachsel, Michael Freese, Matthias Greutmann, Markus Schwerzmann
BACKGROUND: Patients with a systemic right ventricle (RV) have a compromised late outcome caused by ventricular dysfunction. Standard medical heart failure therapy has not been shown to improve RV function and survival in these patients. Phosphodiesterase (PDE)-5 inhibition increases contractility in experimental models of RV hypertrophy, but not in the normal RV. In clinical practice, the effects of PDE-5 inhibition on systemic RV function and exercise capacity in adults with a systemic RV have not been tested...
May 23, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28549654/congenital-heart-defects-and-measures-of-prenatal-brain-growth-a-systematic-review
#17
REVIEW
Thommy Hansen, Tine Brink Henriksen, Cathrine Carlsen Bach, Niels Bjerregård Matthiesen
BACKGROUND: We summarize the evidence for an association between congenital heart defects and prenatal brain growth through a systematic literature review. Congenital heart defects are among the most common malformations, affecting approximately six per 1000 live births. The association between congenital heart defects and long-term neurodevelopmental disorders is well established. Increasing evidence suggests an association between impaired prenatal brain growth and neurodevelopmental disorders in children with congenital heart defects...
July 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28543795/permanent-nonselective-his-bundle-pacing-in-an-adult-with-l-transposition-of-the-great-arteries-and-complete-av-block
#18
Adam C Kean, W Aaron Kay, Jyoti K Patel, John M Miller, Gopi Dandamudi
We report the placement of a permanent transvenous nonselective His bundle pacing lead in conjunction with a transvenous pacemaker/ICD in an adult with Levo-Transposition of the Great Arteries (L-TGA) and a stenotic coronary sinus (CS) ostium (os), which would not accommodate a transvenous LV pacing lead. Nonselective His bundle pacing provided a nearly identical ventricular activation pattern in this previously unpaced patient. Many L-TGA patients will have an eventual need for permanent pacing and, given the challenges of CS cannulation, His bundle pacing may represent a preferred modality rather than pure morphologic LV pacing or surgical systemic ventricular lead placement to achieve optimal electrical synchrony...
May 24, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28542031/septic-coronary-embolization-in-a-patient-with-congenitally-corrected-transposition-of-the-great-arteries
#19
Mehdi Rambod, Abraham D Abernethy, Prospero B Gogo, William E Hopkins
No abstract text is available yet for this article.
May 24, 2017: Coronary Artery Disease
https://www.readbyqxmd.com/read/28537999/imaging-the-adult-with-transposition-of-the-great-arteries
#20
Jason H Anderson, Frank Cetta
PURPOSE OF REVIEW: Patients with complete and congenitally corrected transposition of the great arteries commonly survive into adulthood and present with a vast array of clinical residua. RECENT FINDINGS: Echocardiography remains the primary imaging modality in the routine assessment of the adult with transposition of the great arteries. It provides a comprehensive anatomic and hemodynamic evaluation. Limitations to echocardiography include evaluation of the following: the systemic right ventricle, baffle patency following atrial switch procedure, coronary arteries following arterial switch procedure or Nikadoh, and multilevel right ventricular outflow tract obstruction...
May 19, 2017: Current Opinion in Cardiology
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