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    11033 results match your criteria Tetralogy of Fallot

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    Right ventricular outflow tract reintervention after primary tetralogy of Fallot repair in neonates and young infants.
    J Thorac Cardiovasc Surg 2017 Sep 19. Epub 2017 Sep 19.
    Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Mass.
    Objective: To assess the outcomes following primary tetralogy of Fallot (TOF) repair in neonates and young infants with pulmonary stenosis (PS) and pulmonary atresia and compare differences in reintervention on the right ventricular outflow tract (RVOT) among those undergoing valve sparing repair (VSR), transannular RVOT patch (TAP), and right ventricle-to-pulmonary artery (RV-PA) conduit surgeries.

    Methods: Data were collected retrospectively in 101 patients who underwent TOF repair over a 10-year period between January 2005 and September 2015. The primary endpoint was reintervention on the RVOT, defined as a surgical procedure or cardiac catheterization-based RVOT reintervention. Read More

    Influence of staged repair and primary repair on outcomes in patients with complete atrioventricular septal defect and tetralogy of Fallot: a systematic review and meta-analysis.
    Interact Cardiovasc Thorac Surg 2017 Aug 25. Epub 2017 Aug 25.
    Department of Congenital Heart Surgery, Siberian Biomedical Research Center, Novosibirsk, Russian Federation.
    Objectives: Complete atrioventricular septal defect with tetralogy of Fallot is a rare congenital heart disease. The combination of these lesions occurs in about 1% of all patients with atrioventricular canal defects and in 5-6% of patients with tetralogy of Fallot. To assess the influence of surgical strategy on the survival and reintervention rate for the left atrioventricular valve and right ventricular outflow tract. Read More

    One-stage definitive repair of complete atrioventricular septal defect and pulmonary atresia with major aortopulmonary collateral arteries.
    Interact Cardiovasc Thorac Surg 2017 Oct 11. Epub 2017 Oct 11.
    Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan.
    Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is considered the most severe end of the tetralogy of Fallot spectrum, whereas complete atrioventricular septal defect associated with tetralogy of Fallot is a rare congenital defect. However, the combination of pulmonary atresia, major aortopulmonary collateral artery and complete atrioventricular septal defect is extremely rare, and surgical repair has not been reported. We describe a successful, 1-stage correction in a 7-month-old boy with this condition, who had an additional complication of a single aortic outlet from the right ventricle and significant atrioventricular valve regurgitation. Read More

    Pulmonary valve restitution following transannular patch repair of tetralogy of Fallot.
    Interact Cardiovasc Thorac Surg 2017 Jun 26. Epub 2017 Jun 26.
    Department of Pediatrics, Penn State Children's Hospital, Hershey, PA, USA.
    Chronic pulmonary insufficiency following transannular patch repair of tetralogy of Fallot may mandate restoration of a competent pulmonary valve. Pulmonary valve leaflets that are preserved at initial surgery may grow and develop normal morphology and subsequent valve repair may be possible. We reviewed our experience with native pulmonary valve restitution following transannular patch repair (2001-15). Read More

    The relationship between cardiovascular magnetic resonance imaging measurement of extracellular volume fraction and clinical outcomes in adults with repaired tetralogy of Fallot.
    Eur Heart J Cardiovasc Imaging 2017 Oct 17. Epub 2017 Oct 17.
    Department of Medical Imaging, Peter Munk Cardiac Center, University Health Network, University of Toronto, Toronto General Hospital, 585 University Avenue, 1PMB-298, Toronto, Ontario M5G 2N2, Canada.
    Aims: Our aims were to explore cardiac magnetic resonance quantification of myocardial extracellular volume (ECV) in adults with repaired tetralogy of Fallot (rTOF) when compared with healthy controls and to investigate the association between ECV and major adverse cardiovascular outcomes.

    Methods And Results: We prospectively recruited adults with rTOF (n = 44, 59% male, 32.9 ± 13. Read More

    Genetic Origins of Tetralogy of Fallot.
    Cardiol Rev 2017 Oct 17. Epub 2017 Oct 17.
    From the Departments of Medicine, *Dalhousie University, Halifax, Nova Scotia and **New York Medical College/Westchester Medical Center, Valhalla NY.
    Due to improved survival and clinical outcomes, congenital heart disease is an area of growing importance within the medical community. As these patients reach adulthood and have children, there has been a growing appreciation for the increased risk of CHD among their offspring, strongly implying a genetic element. Given the growing wealth of genetic data available and these clinical implications, this review serves to re-examine the role of genetics within CHD, using Tetralogy of Fallot as a model pathology. Read More

    Pediatrics Spanish Registry of Percutaneous Melody Pulmonary Valve Implantation in Patients Younger Than 18 Years.
    Rev Esp Cardiol (Engl Ed) 2017 Oct 14. Epub 2017 Oct 14.
    Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain; Unidad de Cardiología Infantil, Hospital Universitario Gregorio Marañón, Madrid, Spain.
    Introduction And Objectives: A decade has passed since the first Spanish percutaneous pulmonary Melody valve implant (PPVI) in March 2007. Our objective was to analyze its results in terms of valvular function and possible mid-term follow-up complications.

    Methods: Spanish retrospective descriptive multicenter analysis of Melody PPVI in patients < 18 years from the first implant in March 2007 until January 1, 2016. Read More

    Progression of late post-operative atrial fibrillation in patients with tetralogy of fallot.
    J Cardiovasc Electrophysiol 2017 Oct 13. Epub 2017 Oct 13.
    Departments of Cardiology.
    Introduction: ToF patients are at risk for ventricular deterioration at a relatively young age, which can be aggravated by AF development. Therefore, knowledge on AF development and its timespan of progression is essential to guide treatment strategies for AF.

    Objective: We examined late post-operative AF onset and progression in ToF patients during long-term follow-up after ToF correction. Read More

    Genome-Wide Association Study to Find Modifiers for Tetralogy of Fallot in the 22q11.2 Deletion Syndrome Identifies Variants in the GPR98 Locus on 5q14.3.
    Circ Cardiovasc Genet 2017 Oct;10(5)
    From the Department of Genetics (T.G., J.H.C., H.N., C.L.C., T.W., B.E.M.) and Department of Epidemiology and Population Health (T.W.), Albert Einstein College of Medicine, Bronx, NY; Center for Human Genetics, Facultad de Medicina Clinica Alemana Universidad del Desarrollo, Santiago, Chile (G.M.R.); Division of Human Genetics (D.M.M.M., E.E.M., E.Z., B.S.E.), Division of Cardiology (E.G.), and Department of Pediatrics (E.G.), Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia; Department of Genetics, Wroclaw Medical University, Poland (A.B.); Clinical Genetics Research Program, Center for Addiction and Mental Health and Department of Psychiatry, University of Toronto (A.S.B., E.W.C.C.); Dalglish Family 22q Clinic, Department of Psychiatry and Toronto General Research Institute, University Health Network, Canada (A.S.B.); Campbell Family Mental Health Research Institute, Centre for Addiction and Mental Health, Toronto, Canada (A.S.B.); Center for Human Genetics, University of Leuven (KU Leuven), Belgium (A.S., J.V., K.D.); The Child Psychiatry Division, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer, Ramat Gan, Israel (D.G.); Sackler Faculty of Medicine and Sagol School of Neuroscience, Tel Aviv University, Israel (D.G., M.C., E.M.); Felsenstein Medical Research Center, Sackler Faculty of Medicine, Tel Aviv University, Petah Tikva, Israel (M.C., E.M.); Developmental Imaging and Psychopathology Lab, University of Geneva School of Medicine, Switzerland (M.S., S.E.); Department of Genetic Medicine, UNIGE and iGE3 Institute of Genetics and Genomics of Geneva, University of Geneva Medical Center, Switzerland (S.E.A.); Marcus Autism Center, Children's Healthcare of Atlanta, GA (K.C.); Division of Pediatric Cardiovascular Surgery, Children's Hospital of Wisconsin, Milwaukee (A.T.-M., M.E.M.); Department of Surgery, Medical College of Wisconsin, Milwaukee (A.T.-M., M.E.M.); Department of Medical Genetics, Bambino Gesù Hospital, Rome, Italy (M.C.D., B.D.); Department of Pediatrics, La Sapienza University of Rome, Italy (B.M.); Department of Medical Genetics, Aix Marseille University, APHM, GMGF, Timone Hospital, France (N.P., T.B.); Department of Psychiatry and Biobehavioral Sciences, Semel Institute for Neuroscience and Human Behavior, University of California at Los Angeles (L.K.-W., C.E.B.); Department of Genetics, Polish Mother's Memorial Hospital, Research Institute, Łódź, Poland (M.P., W.H.); Department of Cardiology and Division of Genetics, Boston Children's Hospital, MA (A.E.R.); M.I.N.D. Institute and Department of Psychiatry and Behavioral Sciences (F.T.) and M.I.N.D. Institute and Department of Biochemistry and Molecular Medicine (T.J.S.), University of California, Davis; Department of Psychiatry and Psychology, University of Maastricht, The Netherlands (E.D.A.V.D., T.A.v.A.); Department of Psychiatry and Behavioral Sciences, and Program in Neuroscience, SUNY Upstate Medical University, Syracuse, NY (T.A.v.A., W.R.K.); Department of Human Genetics, Emory University School of Medicine, Atlanta, GA (H.R.J., D.J.C.); Department of Biostatistics and Bioinformatics, Emory University Rollins School of Public Health, Atlanta, GA (H.R.J.); and Human Genetics Center and Department of Epidemiology, Human Genetics and Environmental Sciences, UTHealth School of Public Health, Houston, TX (A.J.A., L.E.M.).
    Background: The 22q11.2 deletion syndrome (22q11.2DS; DiGeorge syndrome/velocardiofacial syndrome) occurs in 1 of 4000 live births, and 60% to 70% of affected individuals have congenital heart disease, ranging from mild to severe. Read More

    Additional mechanism for left ventricular dysfunction: chronic pulmonary regurgitation decreases left ventricular preload in patients with tetralogy of Fallot.
    Cardiol Young 2017 Oct 11:1-6. Epub 2017 Oct 11.
    1Children's Hospital,University of Helsinki,Helsinki University Central Hospital,Helsinki,Finland.
    Background: Right ventricular dysfunction in patients with tetralogy of Fallot and significant pulmonary regurgitation may lead to systolic dysfunction of the left ventricle due to altered ventricular interaction. We were interested in determining whether chronic pulmonary regurgitation affects the preload of the left ventricle. In addition, we wanted to study whether severe chronic pulmonary regurgitation would alter the preload of the left ventricle when compared with patients having preserved pulmonary valve annulus. Read More

    Early outcomes of percutaneous pulmonary valve implantation using the Edwards SAPIEN XT transcatheter heart valve system.
    Int J Cardiol 2017 Oct 6. Epub 2017 Oct 6.
    Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich, Technical University Munich, Germany. Electronic address:
    Background: Patients with congenital or acquired heart defects affecting the pulmonary valve and right ventricular outflow tract (RVOT) commonly require multiple surgical interventions, resulting in significant morbidity. A less invasive alternative is percutaneous pulmonary valve implantation (PPVI). Though studies have previously reported the safety and efficacy of the early generation transcatheter heart valves (THVs), data on more recent devices are severely lacking. Read More

    Risk comparison for prenatal use of analgesics and selected birth defects, National Birth Defects Prevention Study 1997-2011.
    Ann Epidemiol 2017 Sep 20. Epub 2017 Sep 20.
    Division of Congenital and Developmental Disorders, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA.
    Purpose: To compare the use of nonsteroidal anti-inflammatory drugs (NSAIDs) and/or opioids to the use of acetaminophen without NSAIDs or opioids with respect to associations with birth defects.

    Methods: We used data from the National Birth Defects Prevention Study (1997-2011). Exposure was self-reported maternal analgesic use from the month before through the third month of pregnancy (periconceptional). Read More

    Extreme Tetralogy of Fallot With Polycythemia in a Ferret (Mustela putorius furo).
    Top Companion Anim Med 2017 Jun 10;32(2):80-85. Epub 2017 Aug 10.
    Department of Medicina I Cirurgia Animals, Universitat Autònoma de Barcelona, Barcelona, Spain.
    A 5-month-old, intact male ferret (Mustela putorius furo) was presented with apathy, tachypnea and exercise intolerance. On initial physical examination, tachypnea and cyanosis were the two primary clinical signs detected. The complete blood count also revealed a severe polycythemia, with lymphopenia, neutrophilia and eosinophilia. Read More

    Contribution of rare inherited and de novo variants in 2,871 congenital heart disease probands.
    Nat Genet 2017 Oct 9. Epub 2017 Oct 9.
    Department of Genetics, Yale University School of Medicine, New Haven, Connecticut, USA.
    Congenital heart disease (CHD) is the leading cause of mortality from birth defects. Here, exome sequencing of a single cohort of 2,871 CHD probands, including 2,645 parent-offspring trios, implicated rare inherited mutations in 1.8%, including a recessive founder mutation in GDF1 accounting for ∼5% of severe CHD in Ashkenazim, recessive genotypes in MYH6 accounting for ∼11% of Shone complex, and dominant FLT4 mutations accounting for 2. Read More

    Improving the role of echocardiography in studying the right ventricle of repaired tetralogy of Fallot patients: comparison with cardiac magnetic resonance.
    Int J Cardiovasc Imaging 2017 Oct 7. Epub 2017 Oct 7.
    Echocardiography Unit, Division of Pediatric Cardiology and Arrhythmology, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children Hospital-IRCCS, Rome, Italy.
    Right ventricular (RV) evaluation represents one of the major clinical tasks in the follow-up of repaired tetralogy of Fallot patients (rToF) with pulmonary valve regurgitation, as both severe RV dilatation and dysfunction are key factors in defining the need of pulmonary valve replacement. The aim of our study was to report the diagnostic accuracy of echocardiography in the identification of rToF patients with severely dilated and/or depressed RV as compared to cardiac magnetic resonance (CMR). Among our patients with rToF, a subgroup of 95 (17. Read More

    Quantification of pulmonary regurgitation in patients with repaired Tetralogy of Fallot by 2D phase-contrast MRI: Differences between the standard method of velocity averaging and a pixel-wise analysis.
    JRSM Cardiovasc Dis 2017 Jan-Dec;6:2048004017731986. Epub 2017 Sep 21.
    Pontificia Universidad Católica de Chile, Santiago, Chile.
    Objectives: To compare the values of pulmonary regurgitation in patients with repaired Tetralogy of Fallot quantified from two-dimensional phase-contrast data, by using a new pixel-wise analysis and the standard velocity-averaging method.

    Design: Quantitative in silico and in vivo analysis.

    Setting: Hospital Sótero del Río. Read More

    Is mesocardia with left-sided caval vein draining to coronary sinus a contraindication for a percutaneous pulmonary valve implantation? A case description.
    Cardiol Young 2017 Oct 4:1-5. Epub 2017 Oct 4.
    1Centre for Congenital Heart Disease,Heart and Diabetes Centre,Bad Oeynhausen,Ruhr University of Bochum,North Rhine Westphalia,Germany.
    Introduction Although the right jugular vein approach for percutaneous pulmonary valve implantation is well described, there are no reports that describe a percutaneous pulmonary valve implantation through a left superior caval vein to coronary sinus pathway. Case A 14-year-old female with tetralogy of Fallot, mesocardia, left superior caval vein draining into the coronary sinus, and hemiazygos continuation of the inferior caval vein underwent ventricular septal defect closure, with homograft insertion from the right ventricle to the pulmonary artery, patch augmentation of the left pulmonary artery, and creation of an atrial communication. Thereafter followed numerous catheterisations and interventions with stent implantation for stenosis of the left pulmonary artery and the homograft, as did device closure of the atrial communication. Read More

    The Need for Extracorporeal Membrane Oxygenation in Adults Undergoing Congenital Heart Surgery: Impact and Trends of Utilization.
    Indian J Crit Care Med 2017 Sep;21(9):547-551
    Division of Cardiology, Chicago Medical School, North Chicago, IL, USA.
    Introduction: Adults with congenital heart disease (ACHD) represent a population with unique health-care needs. Many patients require cardiac surgery, with some requiring postoperative extracorporeal membrane oxygenation (ECMO). This study aimed to identify the risk factors for the need of postoperative ECMO and characterize the impact of ECMO on admission characteristics. Read More

    A novel method for evaluating regional RV function in the adult congenital heart with low-dose CT and SQUEEZ processing.
    Int J Cardiol 2017 Sep 29. Epub 2017 Sep 29.
    Department of Bioengineering, UC San Diego, La Jolla, CA, United States; Department of Radiology, UC San Diego, La Jolla, CA, United States; Division of Cardiology, Department of Medicine, UC San Diego, La Jolla, CA, United States. Electronic address:
    Background: Measuring local RV function in adult congenital heart disease (ACHD) with echocardiography or MRI is challenging because of the complex geometry and existing pacing devices. Visual assessment of ventricular function via low-dose cardiac CT has been recently performed. This pilot study assessed whether low-dose 4D cine CT combined with automatic measurement of regional shortening could quantify right-ventricular function in ACHD patients. Read More

    Physical Fitness and Metabolic Syndrome in Children with Repaired Congenital Heart Disease Compared with Healthy Children.
    J Pediatr 2017 Sep 28. Epub 2017 Sep 28.
    Department of Pediatric Cardiology, Ghent University Hospital, Ghent, Belgium.
    Objective: To determine whether children who underwent surgery for congenital heart disease (CHD) are as fit as their peers.

    Study Design: We studied 66 children (6-14 years) who underwent surgery for ventricular septal defect (n = 19), coarctation of aorta (n = 10), tetralogy of Fallot (n = 15), and transposition of great arteries (n = 22); and 520 healthy children (6-12 years). All children performed physical fitness tests: cardiorespiratory fitness, muscular strength, balance, flexibility, and speed. Read More

    Combining computer modelling and cardiac imaging to understand right ventricular pump function.
    Cardiovasc Res 2017 Oct;113(12):1486-1498
    CARIM School for Cardiovascular Diseases, Maastricht University Medical Center, Maastricht, The Netherlands.
    Right ventricular (RV) dysfunction is a strong predictor of outcome in heart failure and is a key determinant of exercise capacity. Despite these crucial findings, the RV remains understudied in the clinical, experimental, and computer modelling literature. This review outlines how recent advances in using computer modelling and cardiac imaging synergistically help to understand RV function in health and disease. Read More

    Percutaneous pulmonary valve implantation for reconstruction of a patch-repaired right ventricular outflow tract.
    J Interv Cardiol 2017 Sep 20. Epub 2017 Sep 20.
    Pediatric Heart Center of Hessen, Goethe University, Frankfurt/Main, Germany.
    Percutaneous pulmonary valve implantation (PPVI) is nowadays an accepted treatment option to repair post-surgical conduit dysfunction of the right ventricular outflow tract (RVOT). In addition, many patients need a pulmonary valve to reconstruct a hemodynamically incompetent native or conduit free outflow tract. Based on our experience with percutaneous stent-valve placement in a cohort of 125 patients, we report here transvenous reconstruction of a conduit-free, patch repaired outflow tract by utilizing balloon-expandable stent-valves in 23 patients with a median age of 22 years (5-60 years). Read More

    Ventricular Fibrillation and Myocardial Depression Following Pulmonary Valve Replacement in Tetralogy of Fallot With an Intramural Coronary Artery and Coronary Artery Compression by Chest Tube.
    World J Pediatr Congenit Heart Surg 2017 Jan 1:2150135117716421. Epub 2017 Jan 1.
    5 Department of Anesthesiology, Medical College of Wisconsin, Children's Hospital of Wisconsin, Milwaukee, WI, USA.
    We describe the case of a 10-year-old male with a history of repaired Tetralogy of Fallot and known intramural right coronary artery (RCA) who presented for bioprosthetic pulmonary valve replacement. The operation was complicated by postoperative ventricular fibrillation arrest. Selective coronary angiography revealed external compression of the mid-RCA by a mediastinal chest tube that improved immediately upon removal of the tube. Read More

    Estimating pressure gradients by auscultation: How technology (echocardiography) can help improve clinical skills.
    World J Cardiol 2017 Aug;9(8):693-701
    Division of Pediatric Cardiology, Hassenfeld Children's Hospital of New York at NYU Langone, Fink Children's Center, New York, NY 10016, United States.
    Aim: To extend our previously-published experience in estimating pressure gradients (PG) via physical examination in a large patient cohort.

    Methods: From January 1, 1997 through December 31, 2009, an attending pediatric cardiologist compared clinical examination (EXAM) with Doppler-echo (ECHO), in 1193 patients with pulmonic stenosis (PS, including tetralogy of Fallot), aortic stenosis (AS), and ventricular septal defect (VSD). EXAM PG estimates were based primarily on a murmur's pitch, grade, and length. Read More

    Percutaneous management of complex acquired aortic coarctation in an adult with tetralogy of Fallot and pulmonary atresia.
    Ann Pediatr Cardiol 2017 Sep-Dec;10(3):295-297
    Department of Pediatrics (Cardiology), University of Arizona, Tucson, AZ, USA.
    We present the case of a female adult with complex cyanotic congenital heart disease who had long-standing thoracic aortic obstruction due to scarring from earlier surgical procedures. She was symptomatic but felt to be too high risk for surgical intervention. With careful planning, she was able to undergo successful stenting of her aorta with subsequent clinical improvement. Read More

    Stenting of right ventricular outflow tract in Tetralogy of Fallot with subarterial ventricular septal defect: A word of caution.
    Ann Pediatr Cardiol 2017 Sep-Dec;10(3):281-283
    Department of Pediatric Cardiology, National Heart Institute, Kuala Lumpur, Malaysia.
    We report a case of Tetralogy of Fallot with severe cyanosis who underwent a successful right ventricular outflow tract stenting. Follow-up echocardiography revealed moderate aortic regurgitation due to the impingement of the stent on the aortic valve. The patient underwent successful surgical correction at which time the stent was removed completely with a resolution of the aortic regurgitation. Read More

    Intraocular pressure in children after congenital heart surgery: A single-center study.
    Ann Pediatr Cardiol 2017 Sep-Dec;10(3):234-239
    Department of Pediatrics, Division of Pediatric Cardiology, Arkansas Children's Hospital, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.
    Background: The impact of varied cardiac physiologies on intraocular pressure (IOP) among children undergoing heart operations is unknown.

    Aim: The aim of this study was to determine the IOP among children with varying cardiovascular physiologies and varying hemodynamics after their heart operation.

    Setting And Design: This was a prospective, observational study. Read More

    A case of schizophrenia comorbid for tetralogy of Fallot treated with clozapine: further considerations on a role for 22q.11.2 in the proneness for seizures.
    Neuropsychiatr Dis Treat 2017 29;13:2271-2273. Epub 2017 Aug 29.
    Department of Forensic Psychiatry.
    We present a case of schizophrenia comorbid for tetralogy of Fallot, without chromosome 22q.11.2 deletion or duplication, treated successfully with a combination of clozapine and antiepileptic drugs. Read More

    Pseudoaneurysm complicating right ventricle-to-pulmonary artery conduit surgery: Incidence and risk factors.
    J Thorac Cardiovasc Surg 2017 Aug 24. Epub 2017 Aug 24.
    Department of Cardiology, Boston Children's Hospital, Boston, Mass.
    Objectives: Although pseudoaneurysm is an uncommon complication after right ventricle-to-pulmonary artery conduit placement, it has the potential to cause significant morbidity and mortality.

    Methods: We performed a review of patients with pseudoaneurysms diagnosed at our institution in a 20-year period (from 1995 through 2015) and compared their clinical characteristics with a group of age- and sex-matched control patients.

    Results: We found that younger age, smaller size, the diagnosis of tetralogy of Fallot, the use of a pulmonary homograft conduit, the presence of an unrestrictive ventricular septal defect after conduit placement, and having at least systemic right ventricular pressure were all more common in patients who had pseudoaneurysms develop. Read More

    Complications Associated With Implantable Cardioverter Defibrillators in Adults With Congenital Heart Disease or Left Ventricular Noncompaction Cardiomyopathy (From the NCDR(®) Implantable Cardioverter-Defibrillator Registry).
    Am J Cardiol 2017 Aug 8. Epub 2017 Aug 8.
    University of Washington School of Medicine, Seattle, Washington.
    Patients with childhood heart disease are living longer and entering adulthood, and may undergo implantable cardioverter-defibrillator (ICD) implantation to reduce the risk of sudden death. We evaluated the characteristics of adult patients with congenital heart disease or left ventricular noncompaction cardiomyopathy (LVNC) in the National Cardiovascular Disease Registry ICD Registry and determined ICD-related in-hospital complications. Patients with LVNC or transposition of the great arteries, tetralogy of Fallot, Ebstein's anomaly, atrial septal defect, ventricular septal defect, or common ventricle were identified in the registry. Read More

    Molecular and clinical characterization of new patient with 1,08 Mb deletion in 10p15.3 region.
    Mol Cytogenet 2017 7;10:34. Epub 2017 Sep 7.
    Department of Medical Genetics, Cytogenetics, Institute of Mother and Child, Kasprzaka 17, A 01-211 Warsaw, Poland.
    Background: Three distinct contiguous gene deletion syndromes are located at 10p chromosomal region. The deletion, involving 10p15.3 region, has been characterized by (DeScipio et al. Read More

    Current Surgical Outcomes of Congenital Heart Surgery for Patients With Down Syndrome in Japan.
    Circ J 2017 Sep 12. Epub 2017 Sep 12.
    The Japan Cardiovascular Surgery Database Organization.
    Background: Current surgical outcomes of congenital heart surgery for patients with Down syndrome are unclear.Methods and Results:Of 29,087 operations between 2008 and 2012 registered in the Japan Congenital Cardiovascular Surgery Database (JCCVSD), 2,651 were carried out for patients with Down syndrome (9%). Of those, 5 major biventricular repair procedures [ventricular septal defect repair (n=752), atrioventricular septal defect repair (n=452), patent ductus arteriosus closure (n=184), atrial septal defect repair (n=167), tetralogy of Fallot (TOF) repair (n=108)], as well as 2 major single ventricular palliations [bidirectional Glenn (n=21) and Fontan operation (n=25)] were selected and their outcomes were compared. Read More

    Prevalence and characteristics of coronary artery anomalies in children with congenital heart disease diagnosed with coronary angiography.
    Turk Kardiyol Dern Ars 2017 Sep;45(6):527-532
    Department of Pediatrics, Gaziantep University Faculty of Medicine, Gaziantep, Turkey.
    Objective: Aim of the present study was to determine the prevalence of coronary artery anomalies in children with congenital heart disease.

    Methods: Data of 1138 consecutive patients who were referred for cardiac catheterization and angiography for assessment of coronary anomaly between January 2005 and December 2009 were retrospectively analyzed. Total of 515 patients whose coronary arteries could be examined through left ventricle and aortic root injection were included in the study. Read More

    Clinical Progress in the Management of Tetralogy of Fallot in the Dominican Republic: A Case Series.
    World J Pediatr Congenit Heart Surg 2017 Sep;8(5):584-589
    3 Department of Pediatric Cardiology, CEDIMAT Centro Cardiovascular, Santo Domingo, Republica Dominicana.
    Background: Definitive surgical interventions for Dominican children with congenital heart disease, like those of other low- and middle-income countries, have been historically limited.

    Methods: We undertook review of a case series focusing on the surgical correction of complex forms of tetralogy of Fallot at a single center, CEDIMAT Centro Cardiovascular, in the Dominican Republic, over a 30-month period.

    Results: According to our criteria, 43 cases were determined to be complex tetralogy of Fallot repairs from the two-year period. Read More

    Early leaflet thrombosis complicating transcatheter implantation of a Sapien 3 valve in a native right ventricular outflow tract.
    Catheter Cardiovasc Interv 2017 Sep 12. Epub 2017 Sep 12.
    Division of Cardiology, St. Paul's Hospital, Vancouver, British Columbia, Canada.
    A 59-year-old female with Tetralogy of Fallot had a previous complete repair with RVOT patch enlargement. She developed subsequent severe symptomatic (NYHA III) pulmonary regurgitation with severe RV dilatation. She had a concomitant interstitial lung disease secondary to hypersensitivity pneumonitis that precluded her from cardiac surgery. Read More

    Cyanotic congenital heart diseases among Nigerian children.
    Cardiovasc Diagn Ther 2017 Aug;7(4):389-396
    Deaprtment of Pediatrics, Lagos University Teaching Hospital, Idi-araba, Lagos, Nigeria.
    Background: There are only few reports on cyanotic congenital heart diseases (CCHD) among Nigerian and African Children. The current report aim to provide the most recent hospital based data on the distribution of CCHD in children less than 14 years of age, the demographic characteristics and risk factors identified.

    Methods: Prospective and cross-sectional involving consecutive cases of CCHD diagnosed with echocardiography at the Lagos State University Teaching Hospital between January 2007 and June 2016. Read More

    Stenting of the Right Ventricular Outflow Tract Promotes Better Pulmonary Arterial Growth Compared With Modified Blalock-Taussig Shunt Palliation in Tetralogy of Fallot-Type Lesions.
    JACC Cardiovasc Interv 2017 Sep;10(17):1774-1784
    The Heart Unit, Birmingham Children's Hospital, Birmingham, United Kingdom. Electronic address:
    Objectives: This study sought to compare pulmonary arterial (PA) growth during palliation after right ventricular outflow tract (RVOT) stenting versus modified Blalock-Taussig shunt (mBTS) in patients coming forward for complete repair of tetralogy of Fallot-type lesions.

    Background: RVOT stenting is a recent alternative to mBTS in the initial palliation of selected patients with Fallot-type lesions.

    Methods: This was a retrospective, single-center study of nonrandomized, consecutive palliated Fallot patients over a 10-year period. Read More

    Harmony Feasibility Trial: Acute and Short-Term Outcomes With a Self-Expanding Transcatheter Pulmonary Valve.
    JACC Cardiovasc Interv 2017 Sep;10(17):1763-1773
    Department of Cardiology, Nationwide Children's Hospital, Columbus, Ohio.
    Objectives: This study sought to obtain in vivo data to confirm assumptions on device loading conditions and assess procedural feasibility, safety, and valve performance.

    Background: The Harmony transcatheter pulmonary valve (Medtronic, Minneapolis, Minnesota) was designed for patients with severe pulmonary regurgitation who require pulmonary valve replacement.

    Methods: Three sites participated in this first Food and Drug Administration-approved early feasibility study using an innovative device design to accommodate the complex anatomy of the right ventricular outflow tract. Read More

    Development of quality metrics for ambulatory care in pediatric patients with tetralogy of Fallot.
    Congenit Heart Dis 2017 Sep 7. Epub 2017 Sep 7.
    Department of Cardiology, Children's National Heart Institute, Arlington, Virginia, USA.
    Objective: The objective of this study was to develop quality metrics (QMs) relating to the ambulatory care of children after complete repair of tetralogy of Fallot (TOF).

    Design: A workgroup team (WT) of pediatric cardiologists with expertise in all aspects of ambulatory cardiac management was formed at the request of the American College of Cardiology (ACC) and the Adult Congenital and Pediatric Cardiology Council (ACPC), to review published guidelines and consensus data relating to the ambulatory care of repaired TOF patients under the age of 18 years. A set of quality metrics (QMs) was proposed by the WT. Read More

    Echocardiographic Classification and Surgical Approaches to Double-Outlet Right Ventricle for Great Arteries Arising Almost Exclusively from the Right Ventricle.
    Tex Heart Inst J 2017 Aug 1;44(4):245-251. Epub 2017 Aug 1.
    Selecting an appropriate surgical approach for double-outlet right ventricle (DORV), a complex congenital cardiac malformation with many anatomic variations, is difficult. Therefore, we determined the feasibility of using an echocardiographic classification system, which describes the anatomic variations in more precise terms than the current system does, to determine whether it could help direct surgical plans. Our system includes 8 DORV subtypes, categorized according to 3 factors: the relative positions of the great arteries (normal or abnormal), the relationship between the great arteries and the ventricular septal defect (committed or noncommitted), and the presence or absence of right ventricular outflow tract obstruction (RVOTO). Read More

    Human hepatic organoids for the analysis of human genetic diseases.
    JCI Insight 2017 Sep 7;2(17). Epub 2017 Sep 7.
    Department of Anesthesia.
    We developed an in vitro model system where induced pluripotent stem cells (iPSCs) differentiate into 3-dimensional human hepatic organoids (HOs) through stages that resemble human liver during its embryonic development. The HOs consist of hepatocytes, and cholangiocytes, which are organized into epithelia that surround the lumina of bile duct-like structures. The organoids provide a potentially new model for liver regenerative processes, and were used to characterize the effect of different JAG1 mutations that cause: (a) Alagille syndrome (ALGS), a genetic disorder where NOTCH signaling pathway mutations impair bile duct formation, which has substantial variability in its associated clinical features; and (b) Tetralogy of Fallot (TOF), which is the most common form of a complex congenital heart disease, and is associated with several different heritable disorders. Read More

    Wnt11 regulates cardiac chamber development and disease during perinatal maturation.
    JCI Insight 2017 Sep 7;2(17). Epub 2017 Sep 7.
    Cardiovascular Research Laboratory.
    Ventricular chamber growth and development during perinatal circulatory transition is critical for functional adaptation of the heart. However, the chamber-specific programs of neonatal heart growth are poorly understood. We used integrated systems genomic and functional biology analyses of the perinatal chamber specific transcriptome and we identified Wnt11 as a prominent regulator of chamber-specific proliferation. Read More

    Pulmonary Right Ventricular Resynchronization in Congenital Heart Disease: Acute Improvement in Right Ventricular Mechanics and Contraction Efficiency.
    Circ Cardiovasc Imaging 2017 Sep;10(9)
    From the Children's Heart Center, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic (J.J., J.K., M.L., V.T., P.V., R.G., P.K., M.K.); and CARIM School for Cardiovascular Diseases, Maastricht University Medical Center, The Netherlands (J.L., T.D., F.P.).
    Background: Electromechanical discoordination may contribute to long-term pulmonary right ventricular (RV) dysfunction in patients after surgery for congenital heart disease. We sought to evaluate changes in RV function after temporary RV cardiac resynchronization therapy.

    Methods And Results: Twenty-five patients aged median 12. Read More

    Mortality in adults with congenital heart disease.
    Int J Cardiol 2017 Oct;245:125-130
    Department of Cardiology, 300 Grattan Street, Parkville, Melbourne, Victoria, Australia.
    Aim: Retrospective ascertainment of the causes of mortality in the adult congenital heart disease (ACHD) cohort of the Royal Melbourne Hospital (RMH).

    Methods: Deceased patients (n=73) of the 2519 ACHD patients in the Royal Melbourne Hospital registry (commenced in 1991) were identified. Retrospective analysis was undertaken. Read More

    Clinical, echocardiographic, and therapeutic aspects of congenital heart diseases of children at Douala General Hospital: A cross-sectional study in sub-Saharan Africa.
    Congenit Heart Dis 2017 Sep 4. Epub 2017 Sep 4.
    Department of Internal Medicine and Specialties, Faculty of Medicine and Biomedical Sciences, University of Yaounde 1, Yaounde, Cameroon.
    Introduction: Cardiovascular diseases in pediatric pathologies have emerged in the recent years in sub-Saharan Africa (SSA), with congenital heart diseases (CHDs) being the most frequent. Unfortunately, their diagnosis is usually delayed, thereby increasing childhood morbidity and mortality.

    Objectives: Describe the clinical, echocardiographic, and therapeutic aspects of CHDs of children at Douala General Hospital. Read More

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