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https://www.readbyqxmd.com/read/28641300/prenatal-diagnosis-of-dextrocardia-with-complex-congenital-heart-disease-using-fetal-intelligent-navigation-echocardiography-fine-and-a-literature-review
#1
Lami Yeo, Suchaya Luewan, Dor Markush, Navleen Gill, Roberto Romero
Fetal dextrocardia is a type of cardiac malposition where the major axis from base to apex points to the right side. This condition is usually associated with a wide spectrum of complex cardiac defects. As a result, dextrocardia is conceptually difficult to understand and diagnose on prenatal ultrasound. The advantage of four-dimensional sonography with spatiotemporal image correlation (STIC) is that this modality can facilitate fetal cardiac examination. A novel method known as fetal intelligent navigation echocardiography (FINE) allows automatic generation of nine standard fetal echocardiography views in normal hearts by applying intelligent navigation technology to STIC volume datasets...
June 23, 2017: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/28636109/xq26-1-26-3-duplication-including-mospd1-and-gpc3-identified-in-boy-with-short-stature-and-double-outlet-right-ventricle
#2
Yukiko Hirota, Takaomi Minami, Tomoyuki Sato, Akiko Yokomizo, Auimi Matsumoto, Masahide Goto, Eriko Jinbo, Takanori Yamamgata
Xq25q26 duplication syndrome has been reported in individuals with clinical features such as short stature, intellectual disability, syndromic facial appearance, small hands and feet, and genital abnormalities. The symptoms are related to critical chromosome regions including Xq26.1-26.3. In this particular syndrome, no patient with congenital heart disease was previously reported. Here, we report a 6-year-old boy with typical symptoms of Xq25q26 duplication syndrome and double outlet right ventricle (DORV) with pulmonary atresia (PA)...
June 21, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28632654/an-overview-of-cardiac-computed-tomography-in-adults-with-congenital-heart-disease
#3
Pal Suranyi, Akos Varga-Szemes, Anthony M Hlavacek
Familiarity with congenital heart disease (CHD) and its manifestations in adults is becoming increasingly important for the practicing cardiothoracic imager. The use of computed tomographic angiography is becoming commonplace not only in adults with a history and subsequent interventions for CHD as a child but also in de novo detection of-typically-milder, survivable forms of CHD, which are clinically suspected because of declining cardiac performance, cardiac events, or murmurs. Occasionally, adult CHD (ACHD) is found incidentally on scans performed for other indications (eg, trauma or neoplasm staging) because of improvements in computed tomographic technology and advanced visualization...
July 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/28632653/imaging-adults-with-congenital-heart-disease-part-ii-advanced-cmr-techniques
#4
Anurag Sahu, Timothy C Slesnick
Because of great strides in medical care, survival into adulthood has become a common expectation in patients suffering from nearly all forms of congenital heart disease. As this aging population expands, the utilization of cardiac magnetic resonance imaging in their care continues to grow. Magnetic resonance technology has developed exponentially over the last 2 decades, and several advanced techniques for imaging adults with congenital heart disease have moved from the purely research arena into routine clinical care...
July 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/28632652/utility-of-cardiac-magnetic-resonance-imaging-in-the-management-of-adult-congenital-heart-disease
#5
Giuseppe Muscogiuri, Aurelio Secinaro, Paolo Ciliberti, Megan Fuqua, Arni Nutting
The increasing number of patients with adult congenital heart disease (ACHD) calls for the development of noninvasive imaging techniques that allow a long-term evaluation of native and postsurgical anatomy and function. Echocardiography remains the imaging modality of choice for congenital heart disease, but it is affected by limited acoustic windows and poor tissue characterization. Cardiac computed tomography and cardiac catheter angiography are 2 valid alternatives for the anatomic and functional assessment of ACHD; however, both use ionizing radiation, and cardiac catheter angiography requires an invasive approach...
July 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/28632650/advanced-cardiac-imaging-in-adults-with-congenital-heart-disease-the-great-wave
#6
Anthony M Hlavacek, U Joseph Schoepf
No abstract text is available yet for this article.
July 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/28630369/alpk3-gene-mutation-in-a-patient-with-congenital-cardiomyopathy-and-dysmorphic-features
#7
Ahmet Okay Cağlayan, Rabia Gonul Sezer, Hande Kaymakcalan, Ege Ulgen, Taner Yavuz, Jacob F Baranoski, Abdulkadir Bozaykut, Akdes Serin Harmanci, Yalim Yalcin, Mark W Youngblood, Katsuhito Yasuno, Kaya Bilguvar, Murat Gunel
Primary cardiomyopathy is one of the most common inherited cardiac diseases and harbors significant phenotypic and genetic heterogeneity. Because of this, genetic testing has become standard in treatment of this disease group. Indeed, in recent years, next-generation DNA sequencing has found broad applications in medicine, both as a routine diagnostic tool for genetic disorders and also as a high-throughput discovery tool for identifying novel disease causing genes. We describe a male infant with primary dilated cardiomyopathy that was diagnosed using intrauterine echocardiography, and found to progress to hypertrophic cardiomyopathy after birth...
June 19, 2017: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/28628748/does-young-age-really-put-the-heart-at-risk
#8
Michael P Belanger, Luke Yang Tan, Carin Wittnich
Despite significant advances in the management and treatment of heart disease in children, there continue to be patients who have worse outcomes than might be expected. A number of risk factors that could be responsible have been identified. Evidence based findings will be reviewed including whether young age and/or reduced body weight exacerbate these responses. For example, newborn children undergoing congenital cardiac surgery are known to have worse outcomes than older children. Evidence exists that newborn hearts do not tolerate ischemia as well as adults; developing irreversible injury sooner and exhibiting at risk metabolic profiles...
June 19, 2017: Canadian Journal of Physiology and Pharmacology
https://www.readbyqxmd.com/read/28628610/outcome-of-cardiac-surgery-in-patients-with-congenital-heart-disease-in-england-between-1997-and-2015
#9
Aleksander Kempny, Konstantinos Dimopoulos, Anselm Uebing, Gerhard-Paul Diller, Ulrich Rosendahl, George Belitsis, Michael A Gatzoulis, Stephen J Wort
BACKGROUND: The number of patients with congenital heart disease (CHD) is increasing worldwide and most of them will require cardiac surgery, once or more, during their lifetime. The total volume of cardiac surgery in CHD patients at a national level and the associated mortality and predictors of death associated with surgery are not known. We aimed to investigate the surgical volume and associated mortality in CHD patients in England. METHODS: Using a national hospital episode statistics database, we identified all CHD patients undergoing cardiac surgery in England between 1997 and 2015...
2017: PloS One
https://www.readbyqxmd.com/read/28626087/effect-of-obesity-and-underweight-status-on-perioperative-outcomes-of-congenital-heart-operations-in-children-adolescents-and-young-adults-an-analysis-of-data-from-the-society-of-thoracic-surgeons-database
#10
Michael L O'Byrne, Sunghee Kim, Christoph P Hornik, Babatunde A Yerokun, Roland A Matsouaka, Jeffrey P Jacobs, Marshall L Jacobs, Richard A Jonas
Background -Extreme BMI (either very high or very low) has been associated with increased risk of adverse perioperative outcome in adults undergoing cardiac surgery. The effect of body-mass index (BMI) on perioperative outcomes in congenital heart disease patients has not been evaluated. Methods -A multicenter retrospective cohort study was performed studying patients 10-35 years undergoing a congenital heart disease operation in the Society of Thoracic Surgeons Congenital Heart Surgery Database between 1/1/2010-12/31/2015...
June 16, 2017: Circulation
https://www.readbyqxmd.com/read/28626076/cardiac-fibroblast-transcriptome-analyses-support-a-role-for-interferogenic-profibrotic-and-inflammatory-genes-in-anti-ssa-ro-associated-congenital-heart-block
#11
Robert M Clancy, Androo J Markham, Tanisha Jackson, Sara E Rasmussen, Miroslav Blumenberg, Jill P Buyon
The signature lesion of SSA/Ro autoantibody-associated congenital heart block (CHB) is fibrosis and a macrophage infiltrate, supporting an experimental focus on cues influencing the fibroblast component. The transcriptomes of human fetal cardiac fibroblasts were analyzed using two complementary approaches. Cardiac injury conditions were simulated in vitro by incubating human fetal cardiac fibroblasts with supernatants from macrophages transfected with the SSA/Ro-associated hY3. The top ten upregulated transcripts in the stimulated fibroblasts reflected a type I interferon (IFN) response (e...
June 16, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28625174/quantification-of-cardiomyocyte-alignment-from-three-dimensional-3d-confocal-microscopy-of-engineered-tissue
#12
William J Kowalski, Fangping Yuan, Takeichiro Nakane, Hidetoshi Masumoto, Marc Dwenger, Fei Ye, Joseph P Tinney, Bradley B Keller
Biological tissues have complex, three-dimensional (3D) organizations of cells and matrix factors that provide the architecture necessary to meet morphogenic and functional demands. Disordered cell alignment is associated with congenital heart disease, cardiomyopathy, and neurodegenerative diseases and repairing or replacing these tissues using engineered constructs may improve regenerative capacity. However, optimizing cell alignment within engineered tissues requires quantitative 3D data on cell orientations and both efficient and validated processing algorithms...
June 19, 2017: Microscopy and Microanalysis
https://www.readbyqxmd.com/read/28624249/the-role-of-3-d-heart-models-in-planning-and-executing-interventional-procedures
#13
REVIEW
Elena K Grant, Laura J Olivieri
Percutaneous interventions aimed at addressing congenital and structural heart disease are simultaneously becoming more common and more complex as time progresses. An increasing number of heart defects that had previously required open heart surgery can now be successfully addressed in the cardiac catheterization laboratory. Adequate preprocedural preparation for these novel, complex procedures is critical to ensure their success. Diagnostic data can be collected before the intervention and displayed in multiple formats during the procedure...
February 24, 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28622145/clinical-analyses-of-383-cases-with-maternal-cardiac-diseases
#14
Emine Aydin, Ozgur Ozyuncu, Dila Kasapoglu, Gokcen Orgul, Necla Ozer, Murat Yurdakok, M Sinan Beksac
AIM: To evaluate the pregnancy outcomes of women with heart disease. MATERIALS AND METHODS: In this retrospective study, 383 pregnant women with cardiac diseases were examined. The cases were classified according to the World Health Organization (WHO) classification. The distribution of the cases according to class, congenital heart diseases, mean birthweight, mean gestational week at delivery, type of delivery [cesarean section (CS) or vaginal delivery], and cardivascular events (during pregnancy and puerperium) were evaluated...
June 5, 2017: Journal of Perinatal Medicine
https://www.readbyqxmd.com/read/28620681/cardiac-t1-mapping-in-congenital-heart-disease-bolus-vs-infusion-protocols-for-measurements-of-myocardial-extracellular-volume-fraction
#15
Nadya Al-Wakeel-Marquard, Sanaz Rastin, Frédéric Muench, Darach O H-Ici, Sevim Yilmaz, Felix Berger, Titus Kuehne, Daniel R Messroghli
Myocardial extracellular volume fraction (ECV) reflecting diffuse myocardial fibrosis can be measured with T1 mapping cardiovascular magnetic resonance (CMR) before and after the application of a gadolinium-based extracellular contrast agent. The equilibrium between blood and myocardium contrast concentration required for ECV measurements can be obtained with a primed contrast infusion (equilibrium contrast-CMR). We hypothesized that equilibrium can also be achieved with a single contrast bolus to accurately measure diffuse myocardial fibrosis in patients with congenital heart disease (CHD)...
June 15, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28617685/adult-congenital-heart-disease-magnitude-of-the-problem
#16
Akanksha N Thakkar, Ponraj Chinnadurai, C Huie Lin
PURPOSE OF REVIEW: To define the magnitude of problems faced by patients with adult congenital heart disease (ACHD) and to identify unmet needs for this population. RECENT FINDINGS: The ACHD population is estimated to include more than 1 million people in the United States and continues to grow at a steady rate. Owing to the decline in early mortality in this group, modern medicine is now faced by the long-term complications associated with congenital heart disease such as chronic heart failure, increased endocarditis risk, elevated burden of arrhythmias, pulmonary hypertension, valvular dysfunction, and pregnancy...
June 15, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/28616089/severe-pulmonary-arterial-hypertension-associated-with-congenital-cardiac-shunts-evolution-under-specific-treatment
#17
R I Negoi, I Ghiorghiu, F Filipoiu, M Hostiuc, I Negoi, C Ginghina
Objective: The aim of this study was to compare the effects of Sildenafil, Bosentan and combined therapy in patients with congenital cardiac shunts associated pulmonary artery hypertension (CCS-PAH). Design: Prospective observational study (February 2011 - January 2014) with a historical control group (January 2009 - January 2011). Setting: "CC Iliescu" Institute for Emergency Cardiovascular Diseases of Bucharest, a tertiary university-affiliated center. Patients: All cases with CCS-PAH. Interventions: Specific vasodilatory therapy: Sildenafil, Bosentan or combined therapy...
April 2017: Journal of Medicine and Life
https://www.readbyqxmd.com/read/28615160/nkx2-5-is-essential-to-establish-normal-heart-rate-variability-in-the-zebrafish-embryo
#18
Jamie K Harrington, Robert Sorabella, Abigail Tercek, Joseph R Isler, Kimara L Targoff
Heart rate variability (HRV) has become an important clinical marker of cardiovascular health and a research measure for the study of the cardiac conduction system and its autonomic controls. While zebrafish (Danio rerio) is an ideal vertebrate model for understanding heart development, HRV has only recently been investigated in this system. We have previously demonstrated that nkx2.5 and nkx2.7, two homologues of Nkx2-5 expressed in zebrafish cardiomyocytes, play vital roles in maintaining cardiac chamber-specific characteristics...
June 14, 2017: American Journal of Physiology. Regulatory, Integrative and Comparative Physiology
https://www.readbyqxmd.com/read/28614966/persistently-elevated-nuchal-translucency-and-the-fetal-heart
#19
Trisha V Vigneswaran, Tessa Homfray, Lindsey D Allan, John M Simpson, Vita Zidere
We describe the outcome of fifteen cases with an elevated nuchal translucency (NT) which persisted into the second trimester as nuchal edema (NE) > 6 mm whom underwent fetal echocardiography. Cases were identified following retrospective review of cardiac and genetic findings with NE. Minor congenital heart disease was identified in 3/15 by the second trimester. Agenesis of the ductus venosus was evident in four. Pulmonary valve stenosis was diagnosed in one fetus at the 20-week scan and hypertrophic cardiomyopathy in one...
June 14, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28611116/recent-advances-in-cardiovascular-magnetic-resonance-techniques-and-applications
#20
Michael Salerno, Behzad Sharif, Håkan Arheden, Andreas Kumar, Leon Axel, Debiao Li, Stefan Neubauer
Cardiovascular magnetic resonance imaging has become the gold standard for evaluating myocardial function, volumes, and scarring. Additionally, cardiovascular magnetic resonance imaging is unique in its comprehensive tissue characterization, including assessment of myocardial edema, myocardial siderosis, myocardial perfusion, and diffuse myocardial fibrosis. Cardiovascular magnetic resonance imaging has become an indispensable tool in the evaluation of congenital heart disease, heart failure, cardiac masses, pericardial disease, and coronary artery disease...
June 2017: Circulation. Cardiovascular Imaging
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