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Tetralogy fallot

Wail Alkashkari, Amani Alsubei, Ziyad M Hijazi
PURPOSE OF REVIEW: The past couple of decades have brought tremendous advances to the field of pediatric and adult congenital heart disease (CHD). Percutaneous valve interventions are now a cornerstone of not just the congenital cardiologist treating patients with congenital heart disease, but also-and numerically more importantly-for adult interventional cardiologists treating patients with acquired heart valve disease. Transcatheter pulmonary valve replacement (tPVR) is one of the most exciting recent developments in the treatment of CHD and has evolved to become an attractive alternative to surgery in patients with right ventricular outflow tract (RVOT) dysfunction...
March 15, 2018: Current Cardiology Reports
Ahmad Ali Amirghofran, Jamshid Badr, Mansour Jannati
BACKGROUND: Tetralogy of Fallot (TOF) is one of the congenital cardiac abnormality which occurs during embryonic time. Although surgical correction, especially early operation, is the best way to treat patients, still contributing factors in morbidity and mortality is controversial. The objective of this study is to investigate potential factors which might be correlated with post-operative outcomes of TOF. METHODS: In this retrospective study, 349 monitored patients with TOF correction were selected...
March 15, 2018: BMC Surgery
Michael L O'Byrne, Michael G McBride, Stephen Paridon, Elizabeth Goldmuntz
BACKGROUND: Obesity is associated with increased lifelong morbidity and reduced life span and is increasingly prevalent in the congenital heart disease population. Habitual exercise is an important aspect of a healthy lifestyle and primary prevention of obesity in the general population. The association between habitual activity and body mass index (BMI) has not been studied in children with congenital heart disease. METHODS: A cross-sectional analysis of two previously collected cohorts was performed, including participants 8 to 18 years old with tetralogy of Fallot, transposition of the great arteries, and single ventricle heart disease after a Fontan operation...
March 2018: World Journal for Pediatric & Congenital Heart Surgery
Zoel A Quinonez, Laura Downey, Rania K Abbasi, Calvin Kuan, Ritu Asija, Doff B McElhinney, Frank L Hanley, Richard D Mainwaring, Lisa Wise-Faberowski
Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals (TOF/PA/MAPCAs) is a heterogeneous disease with varying degrees of severity, requiring complex anesthetic management. Our institution has adopted the approach of early complete repair with incorporation of all lung segments, extensive lobar and branch segmental pulmonary artery reconstruction, and ventricular septal defect closure. While the surgical management of TOF/PA/MAPCAs has been extensively described and varies depending on the institution, there is a paucity of literature on the anesthetic management for such procedures...
March 2018: World Journal for Pediatric & Congenital Heart Surgery
Manar D Samad, Gregory J Wehner, Mohammad R Arbabshirani, Linyuan Jing, Andrew J Powell, Tal Geva, Christopher M Haggerty, Brandon K Fornwalt
Aims: Previous studies using regression analyses have failed to identify which patients with repaired tetralogy of Fallot (rTOF) are at risk for deterioration in ventricular size and function despite using common clinical and cardiac function parameters as well as cardiac mechanics (strain and dyssynchrony). This study used a machine learning pipeline to comprehensively investigate the predictive value of the baseline variables derived from cardiac magnetic resonance (CMR) imaging and provide models for identifying patients at risk for deterioration...
March 12, 2018: European Heart Journal Cardiovascular Imaging
Juanjuan Zhao, Mathilda T M Mommersteeg
The Slit ligands and their Robo receptors are well-known for their roles during axon guidance in the central nervous system, but are still relatively unknown in the cardiac field. However, data from different animal models suggest a broad involvement of the pathway in many aspects of heart development, from cardiac cell migration and alignment, lumen formation, chamber formation, to the formation of the ventricular septum, semilunar and atrioventricular valves, caval veins and pericardium. Absence of one or more of the genes in the pathway results in defects ranging from bicuspid aortic valves to ventricular septal defects and abnormal venous connections to the heart...
March 10, 2018: Cardiovascular Research
Priscila H A Oliveira, Beatriz S Souza, Eimi N Pacheco, Michele S Menegazzo, Ivan S Corrêa, Paulo R G Zen, Rafael F M Rosa, Claudia C Cesa, Lucia C Pellanda, Manuel A P Vilela
BACKGROUND: Numerous genetic syndromes associated with heart disease and ocular manifestations have been described. However, a compilation and a summarization of these syndromes for better consultation and comparison have not been performed yet. OBJECTIVE: The objective of this work is to systematize available evidence in the literature on different syndromes that may cause congenital heart diseases associated with ocular changes, focusing on the types of anatomical and functional changes...
January 2018: Arquivos Brasileiros de Cardiologia
Michał Orczykowski, Karolina Borowiec, Elzbieta Biernacka, Robert Bodalski, Piotr Urbanek, Pawel Derejko, Katarzyna Kodziszewska, Olgierd Wozniak, Aneta Fronczak, Kamil Marcinkiewicz, Krystyna Guzek, Agnieszka Fil, Grzegorz Warminski, Piotr Hoffman, Maria Bilinska, Lukasz Szumowski
BACKGROUND: After the surgical correction of Tetralogy of Fallot (TOF), surgical scars and natural obstacles form pathways capable of supporting an atrial tachyarrhythmia (AT). Radiofrequency ablation is effective, although the few studies to date on this topic had relatively short follow-up periods. AIM: The aims of the study were to evaluate the acute and long-term effects of RF ablation of AT and examine the characteristics of arrhythmia recurrence. METHODS: Tetralogy of Fallot patients (N 16, age 44...
March 14, 2018: Kardiologia Polska
Ping Hu, Fengchang Qiao, Yan Wang, Lulu Meng, Xiuqing Ji, Chunyu Luo, Tianhui Xu, Ran Zhou, Jingjing Zhang, Bin Yu, Leilei Wang, Ting Wang, Qiong Pan, Dingyuan Ma, Dong Liang, Zhengfeng Xu
OBJECTIVES: This study aimed to determine the diagnostic yield of targeted next-generation sequencing (NGS) in prenatal diagnosis of congenital heart defects (CHDs) and for investigating the possible genetic etiology of prenatal CHD cases. METHODS: Forty-four fetuses with CHDs and normal molecular karyotypes underwent targeted NGS in this study. Fetal genomic DNA was directly extracted from amniotic fluid cells in each prenatal case. A customized targeted NGS panel containing 77 CHD-associated genes was designed to detect variants in the coding regions and the splicing sites of these genes...
March 13, 2018: Ultrasound in Obstetrics & Gynecology
Laura Mercer-Rosa, Okan U Elci, Nelangi M Pinto, Ronn E Tanel, Elizabeth Goldmuntz
Deletion of 22q11.2 (del22q11) is associated with adverse outcomes in patients with tetralogy of Fallot (TOF). We sought to investigate its contribution to perioperative outcome in patients with a severe form of TOF characterized by pulmonary atresia (PA) or severe pulmonary stenosis (PS) and major aortopulmonary collateral arteries (MAPCAS). We conducted a retrospective review of patients with TOF/MAPCAS who underwent staged surgical reconstruction between 1995 and 2006. Groups were compared according to 22q11...
March 8, 2018: Pediatric Cardiology
Hiroo Kinami, Kiyozo Morita, Yoshihiro Ko, Gen Shinohara, Kazuhiro Hashimoto
Neonatal primary repair of tetralogy of Fallot (TOF) with absent pulmonary valve (APV) syndrome is associated with high mortality rates. Our plan involves a staged repair that avoids one-stage intracardiac repair (ICR), with a first palliation that closes the main pulmonary orifice using an expanded polytetrafluoroethylene (ePTFE) patch, pulmonary arterioplication, and an adjustable Blalock-Taussig (BT) shunt. This strategy was used for a neonatal case with TOF/APV syndrome with hypoplastic left ventricle (LV)...
March 7, 2018: Annals of Thoracic and Cardiovascular Surgery
Tarun Raina Ramman, Nilanjan Dutta, Kuntal Roy Chowdhuri, Sunny Agrawal, Sumir Girotra, Sushil Azad, Sitaraman Radhakrishnan, Parvathi Unninayar Iyer, Krishna Subramony Iyer
Persistent left superior vena cava is a common congenital anomaly of the thoracic venous system. Left superior vena cava draining into left atrium is a malformation of sinus venosus and caval system. The anomaly may be a cause of unexplained hypoxia even in adults. It may give rise to various diagnostic and technical challenges during cardiac catheterization and open-heart surgery. It is often detected serendipitously during diagnostic workup. Isolated left superior vena cava opening into left atrium is very commonly associated with other congenital heart defects...
January 1, 2018: World Journal for Pediatric & Congenital Heart Surgery
Saima Bibi, Syed Yasir Hussain Gilani, Shawana Bibi
BACKGROUND: Congenital heart disease is a significant problem world over especially in neonates. Early diagnosis and prompt interventions in neonatal period precludes the mortality associated with this disorder. The objective of this study was to highlight the diversity of congenital cardiac defects in our region so that appropriate interventions are devised to minimize significant morbidity and mortality associated with this disorder. METHODS: This descriptive cross-sectional study was conducted at the Neonatology Unit of Department of Paediatrics, Ayub Teaching Hospital from January 2015 to December 2016...
January 2018: Journal of Ayub Medical College, Abbottabad: JAMC
Isabel Kazour, Suraj D Serai, Stavra A Xanthakos, Robert J Fleck
The goal of this study was to assess the ability of quantitative T1 cardiovascular magnetic resonance (CMR) imaging to calculate liver extracellular volume (ECV) in patients with varying degrees of congestive hepatopathy (CH). T1 measurements and ECV calculations were performed retrospectively in three cohorts of patients: normal cardiac function, tetralogy of fallot (TOF) repair and Fontan palliation. All CMR studies included modified look-locker inversion recovery (MOLLI) T1 mapping scans performed pre- and post-injection of a gadolinium-based contrast agent (GBCA)...
March 3, 2018: Abdominal Radiology
Arkadiusz Krzyżanowski, Dariusz Swatowski, Tomasz Gęca, Maciej Kwiatek, Aleksandra Stupak, Sławomir Woźniak, Anna Kwaśniewska
BACKGROUND: Persistent right umbilical vein (PRUV) is usually an isolated finding but it may be accompanied by other fetal malformations. AIMS: We aimed to determine the incidence of prenatally diagnosed PRUV in a referral population, assess the neonatal outcome and discuss the findings together with those from previous publications. MATERIALS AND METHODS: A total of 2360 women with low-risk singleton pregnancies were examined in the second and third trimesters...
March 2, 2018: Australian & New Zealand Journal of Obstetrics & Gynaecology
Andrew M Kim, Timothy A Hunter, Brian F McQuillan, Derek F Franco, Timothy P Griffith, Brett W Carter, John P Lichtenberger
Early identification of congenital heart diseases, specifically those affecting the structural integrity and function of the interventricular septum, in childhood is important toward decreasing the morbidity and mortality of those affected. We review the pertinent clinical and imaging manifestations for those with ventricular septal defects, ventricular septal aneurysms, tetralogy of Fallot, and hypertrophic (obstructive) cardiomyopathy, in addition to discussing first-line imaging studies, including echocardiography, and indications for advanced imaging...
February 27, 2018: Journal of Thoracic Imaging
Maeve K Hopkins, Sarah A Goldstein, Cary C Ward, Jeffrey A Kuller
Objective: Congenital heart defects represent the most common major congenital anomalies. The objective of this review was to define the most common forms of congenital heart disease (CHD) in pregnancy, outline preconception counseling, discuss the associated morbidity and mortality of each lesion, and review current recommendations for management of CHD in pregnancy. Evidence Acquisition: A MEDLINE search of "congenital heart disease in pregnancy" and specific conditions in pregnancy including "ventricular septal defect," "atrial septal defect," "left outflow obstruction," "right outflow obstruction," "tetralogy of Fallot," and "transposition of the great vessels" was performed...
February 2018: Obstetrical & Gynecological Survey
Timothy Lee, Aaron J Weiss, Elbert E Williams, Fuad Kiblawi, Joanna Dong, Khanh H Nguyen
OBJECTIVES: Although the median sternotomy has been the traditional approach for congenital heart surgery, young patients and their families often find the midline scar to be cosmetically unappealing. At our center, a right transverse axillary incision has become the standard approach for many congenital cardiac lesions due to its safety, versatility, and unsurpassed aesthetic result. We present our experience with the axillary approach for a diverse array of congenital defects. METHODS: A retrospective review of patients receiving a right transverse axillary incision for congenital cardiac surgery between 2005-2016 was conducted...
February 21, 2018: Seminars in Thoracic and Cardiovascular Surgery
Wadi Mawad, Luc L Mertens
Cardiac imaging is central to today's pediatric cardiology practice not only to diagnose structural congenital defects and delineate cardiac and extracardiac anatomy but also for determining the hemodynamic impact of the structural defects and acquired pediatric diseases. Not so long ago, clinicians had to heavily rely on angiography as the main cardiac imaging modality to visualize the heart. Particularly, the development of echocardiography in the 1970s and 1980s together with the development of magnetic resonance imaging (MRI) and computed tomography (CT) resulted in a non-invasive diagnostic revolution with diagnostic catheterization becoming obsolete apart for very specific indications...
February 21, 2018: Current Treatment Options in Cardiovascular Medicine
Bojian Li, Sun Chen, Kun Sun, Rang Xu, Yurong Wu
Holt-Oram syndrome (HOS) is an autosomal dominant disorder, which is characterized by deformities of upper limbs and congenital heart defects. Alterations of TBX5 gene have been identified to be the leading cause of HOS, while some cases could not be explained by TBX5 mutations. In our study, we preliminarily diagnosed a newborn baby, who had Tetralogy of Fallot, thumb agenesis, facial dysplasia, and right ear canal malformation, as HOS. Chromosome microarray analyses showed no pathological deletions or replications of chromosome segments; whole exome sequencing screened out six candidate genes that were involved in cardiac diseases or syndromes among which SALL4 has been reported as HOS related gene...
February 20, 2018: DNA and Cell Biology
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