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    10766 results match your criteria Tetralogy of Fallot

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    Preprocedural Risk Assessment Prior to PPVI with CMR and Cardiac CT.
    Pediatr Cardiol 2017 Feb 16. Epub 2017 Feb 16.
    Department of Radiology, Children's Hospital Colorado, Aurora, Colorado, USA.
    Percutaneous pulmonary valve intervention (PPVI) is a less invasive and less costly approach to pulmonary valve replacement compared with the surgical alternative. Potential complications of PPVI include coronary compression and pulmonary arterial injury/rupture. The purpose of this study was to characterize the morphological risk factors for PPVI complication with cardiac MRI and cardiac CTA. Read More

    A Rare Association of Pentalogy of Fallot with Situs Inversus Totalis Complicated by Brain Abscess in an Adolescent-Case Report.
    J Clin Diagn Res 2016 Dec 1;10(12):SD06-SD07. Epub 2016 Dec 1.
    Associate Professor, Department of Paediatrics, MKCG Medical College , Brahmapur, Odisha, India .
    Tetralogy of Fallot (TOF) is the most frequently diagnosed congenital cyanotic heart disease. It is often associated with additional findings, such as atrial septal defect (i.e. Read More

    Isolated Hypoplasia of Left Pulmonary Artery with Agenesis of Left Lobe of Thyroid: A Case Report.
    J Clin Diagn Res 2016 Dec 1;10(12):SD04-SD05. Epub 2016 Dec 1.
    Professor and Head, Department of Cardiology, Chettinad Hospital and Research Institute , Chennai, Tamil Nadu, India .
    Isolated Unilateral hypoplasia or agenesis of a branch of pulmonary artery is very rare. It is usually seen associated with congenital heart diseases such as tetralogy of Fallot, atrial septal defect, coarctation of the aorta, right aortic arch, truncus arteriosus, patent ductus arteriosus and pulmonary atresia. It occurs as a result of lack of embryological development of either the left or right sixth aortic arch and has been found to present itself with various clinical manifestations as during childhood it presents as contralateral pulmonary hypertension and in adults as haemoptysis. Read More

    Left Atrial Deformation Analysis in Patients with Corrected Tetralogy of Fallot by 3D Speckle-Tracking Echocardiography (from the MAGYAR-Path Study).
    Arq Bras Cardiol 2017 Feb 13. Epub 2017 Feb 13.
    2nd Department of Medicine and Cardiology Center - Medical Faculty - Albert Szent-Györgyi Clinical Center - University of Szeged - Szeged - Hungary.
    Background:: Three-dimensional (3D) echocardiography coupled with speckle-tracking echocardiographic (STE) capability is a novel methodology which has been demontrated to be useful for the assessment of left atrial (LA) volumes and functional properties. There is increased scientific interest on myocardial deformation analysis in adult patients with corrected tetralogy of Fallot (cTOF).

    Objectives:: To compare LA volumes, volume-based functional properties and strain parameters between cTOF patients and age- and gender-matched healthy controls. Read More

    Complete Repair of Tetralogy of Fallot in the Neonatal Versus Non-neonatal Period: A Meta-analysis.
    Pediatr Cardiol 2017 Feb 11. Epub 2017 Feb 11.
    Division of Cardiology and Cardiothoracic Surgery, Children's Hospital of Wisconsin/ Medical College of Wisconsin, 9000 Wisconsin Avenue, Milwaukee, WI, 53226, USA.
    It is unclear if neonatal tetralogy of Fallot repair offers better outcomes compared to repair later in infancy. We therefore conducted a meta-analysis comparing outcomes of neonatal and non-neonatal repair. Manuscripts were identified and reviewed for quality and bias with favorably scored manuscripts being included in the final meta-analysis. Read More

    Junctional ectopic tachycardia in late period after early postoperative complete atrioventricular block: Messenger of return to normal sinus rhythm? : Explanation with four case series.
    J Electrocardiol 2017 Feb 1. Epub 2017 Feb 1.
    Department of Pediatric Cardiology, İstanbul Mehmet Akif Ersoy, Thoracic and Cardiovascular Surgery Center and Research Hospital, Istanbul, Turkey.
    Junctional ectopic tachycardia(JET) is a rare childhood arrhythmia originating from the area adjacent to the atrioventricular(AV) node. It often occurs after surgical procedures like repair of Tetralogy of Fallot, atrioventricular septal defect and ventricular septal defect, which are all performed in that area. While AV block (AVB) can occur after JET, it is very rare for late JET occurring after early postoperative AVB to be followed by normal sinus rhythm (NSR). Read More

    Outcomes Following Melody Transcatheter Pulmonary Valve Implantation for Right Ventricular Outflow Tract Dysfunction in Repaired Congenital Heart Disease: First Reported Australian Single Centre Experience.
    Heart Lung Circ 2017 Jan 24. Epub 2017 Jan 24.
    Heart & Lung Institute, The Prince Charles Hospital, Brisbane, Qld, Australia; University of Queensland, Brisbane, Qld, Australia.
    Background: Transcatheter pulmonary valve implantation (TPVI) with the Melody® transcatheter pulmonary valve (TPV) has demonstrated good haemodynamic and clinical outcomes in the treatment of right ventricular outflow tract (RVOT) conduit dysfunction in patients with repaired congenital heart disease CHD. We present the first Australian single centre experience of patients treated with Melody TPV.

    Method: A prospective, observational registry was developed to monitor clinical and haemodynamic outcomes in patients with RVOT dysfunction treated with the Melody TPV (Medtronic Inc, Minneapolis, United States). Read More

    Concomitant Right Ventricular Outflow Tract Cryoablation during Pulmonary Valve Replacement in a Patient with Tetralogy of Fallot.
    Korean J Thorac Cardiovasc Surg 2017 Feb 5;50(1):41-43. Epub 2017 Feb 5.
    Division of Cardiovascular Surgery, Department of Thoracic and Cardiovascular Surgery, Severance Cardiovascular Hospital, Yonsei University College of Medicine.
    A 38-year-old female patient with a history of tetralogy of Fallot repair at 10 years of age underwent pulmonary valve replacement with a mechanical prosthesis, tricuspid annuloplasty, and right ventricular outflow tract cryoablation due to pulmonary regurgitation, tricuspid regurgitation, and multiple premature ventricular contractions with sustained ventricular tachycardia. After surgery, she had an uneventful postoperative course with arrhythmia monitoring. She was discharged without incident, and a follow-up Holter examination showed a decrease in the number of ventricular ectopic beats from 702 to 41. Read More

    Fetal Right Aortic Arch: Associated Anomalies, Genetic Anomalies with Chromosomal Microarray Analysis and Postnatal Outcome.
    Prenat Diagn 2017 Feb 6. Epub 2017 Feb 6.
    Department of Ultrasonic Medicine, Fetal Medical Centre, Guangzhou, China.
    Objectives: To assess the associated prenatal findings, genetic anomalies with chromosomal microarray analysis (CMA) and postnatal outcome of fetal right aortic arch (RAA).

    Methods: This retrospective study reviewed 92 fetuses diagnosed with RAA and the findings of CMA using Affymetrix CytoScan HD array in our institution between 2013 and 2016.

    Results: Postnatal data were not available for six cases and genetic data were not available for 26 cases. Read More

    Trends in pediatric echocardiography and the yield for congenital heart disease in a major cardiac referral hospital in Cameroon.
    Transl Pediatr 2017 Jan;6(1):40-45
    Non-Communicable Diseases Research Unit, South African Medical Research Council of South Africa, Cape Town, South Africa.
    Background: Congenital heart disease (CHD) is a common condition in children in Sub-Saharan Africa (SSA), where it is associated with poor outcomes. Diagnosis of CHD in SSA depends essentially on echocardiography, which is available only in few urban referral centers. Our aim was to assess time changes in the pattern of referral for pediatric echocardiography and the subsequent diagnosis of structural CHD in a major SSA city. Read More

    Mechanism of valve failure and efficacy of reintervention through catheterization in patients with bioprosthetic valves in the pulmonary position.
    Ann Pediatr Cardiol 2017 Jan-Apr;10(1):11-17
    Department of Cardiology, Harvard Medical School, Boston Children's Hospital, Boston, MA, USA.
    Background: Surgical and transcatheter bioprosthetic valves (BPVs) in the pulmonary position in patients with congenital heart disease may ultimately fail and undergo transcatheter reintervention. Angiographic assessment of the mechanism of BPV failure has not been previously described.

    Aims: The aim of this study was to determine the mode of BPV failure (stenosis/regurgitation) requiring transcatheter reintervention and to describe the angiographic characteristics of the failed BPVs and report the types and efficacy of reinterventions. Read More

    Development of Quality Metrics in Ambulatory Pediatric Cardiology.
    J Am Coll Cardiol 2017 Feb;69(5):541-555
    Department of Pediatrics, Harvard Medical School and Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts.
    The American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Section had attempted to create quality metrics (QM) for ambulatory pediatric practice, but limited evidence made the process difficult. The ACPC sought to develop QMs for ambulatory pediatric cardiology practice. Five areas of interest were identified, and QMs were developed in a 2-step review process. Read More

    Inflammatory cytokine response and cardiac troponin I changes in cardiopulmonary bypass using two cardioplegia solutions; del Nido and modified St. Thomas': a randomized controlled trial.
    Perfusion 2017 Feb 1:267659117691119. Epub 2017 Feb 1.
    4 Cellular and Molecular Research Center, Iran University of Medical Sciences, Tehran, Iran.
    Background: Long intervals of del Nido (DN) solution administration, 90 minutes, may result in rewarming of the myocardial tissue and elevate metabolic demand and hypoxia. This will potentially increase inflammatory response due to ischemia-reperfusion injury. We conducted this study to compare the inflammatory response between patients receiving DN and multi-dose St Thomas' cardioplegia solution (MST) in cardiopulmonary bypass (CPB) surgery for the correction of tetralogy of Fallot (TF). Read More

    First in human experience of a new self-expandable percutaneous pulmonary valve implantation using knitted nitinol-wire and tri-leaflet porcine pericardial valve in the native right ventricular outflow tract.
    Catheter Cardiovasc Interv 2017 Feb 1. Epub 2017 Feb 1.
    Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Jongno-gu, Seoul, 03080, South Korea.
    Balloon-expandable percutaneous pulmonary valve systems using the Melody and Edwards SAPIEN transcatheter heart valves have been increasingly used instead of surgically implantable pulmonary valves. However, limited patients with right ventricular outflow tract (RVOT) lesions are suitable candidates for percutaneous pulmonary valve implantation (PPVI) using these systems after surgical correction of tetralogy of Fallot. Therefore, larger self-expandable valved-stents are being developed for native RVOT lesions. Read More

    The Impact of the Right Ventricular Outflow Tract Patch on Right Ventricular Strain in Tetralogy of Fallot: A Comparison with Valvar Pulmonary Stenosis Utilizing Cardiac Magnetic Resonance.
    Pediatr Cardiol 2017 Jan 31. Epub 2017 Jan 31.
    Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia PA. 3401 Civic Center Boulevard, Philadelphia, PA, 19104, USA.
    A non-contractile transannular patch (TAP) in the right ventricular outflow tract (RVOT) contributes to ventricular dysfunction after tetralogy of Fallot (TOF) repair. We compared regional right ventricular (RV) strain in repaired TOF with valvar pulmonary stenosis (VPS) after balloon valvuloplasty to investigate the effects of TAP. Retrospective review of 26 cardiac magnetic resonance studies of TOF (n = 13) and VPS (n = 13) subjects matched by degree and duration of pulmonary regurgitation (PR). Read More

    Aortopulmonary window: Morphology, diagnosis, and long-term results.
    J Card Surg 2017 Feb 30;32(2):138-144. Epub 2017 Jan 30.
    Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India.
    Objective: Aortopulmonary window (APW) is a rare congenital heart defect. We reviewed our experience with this condition over the last two decades.

    Methods: Between September 1993 and December 2013, 62 patients underwent surgery for APW. Read More

    Hemadsorption with Adult CytoSorb® in a Low Weight Pediatric Case.
    Case Rep Crit Care 2017 3;2017:6987167. Epub 2017 Jan 3.
    "Carol Davila" University of Medicine & Pharmacy Bucharest, 37 Dionisie Lupu Street, District 1, 020021 Bucharest, Romania.
    Cytokine adsorber (CytoSorb) has been used successfully as adjunctive treatment for adult patients with elevated cytokine levels in the setting with severe sepsis and septic shock and to reduce blood myoglobin, unconjugated bilirubin, and conjugated bilirubin. In this article we present the case of a nine-month-old male infant who was admitted to the NICU due to sepsis after cardiac surgery, Fallot tetralogy, and multisystem organ failure (MSOF) including liver failure and renal failure which was successfully treated by a combination of continuous hemodiafiltration (HDF) and hemadsorption with CytoSorb. HDF was safe and effective from the first day for urea removal, but the patient's bilirubin levels kept increasing gradually, culminating on the 9th day with a maximum value of 54 mg/dL of total bilirubin and 31. Read More

    Coexistence of abnormal systolic motion of mitral valve in a consecutive group of 324 adult Tetralogy of Fallot patients assessed with echocardiography.
    Open Heart 2016 30;3(2):e000518. Epub 2016 Dec 30.
    University of California San Francisco (UCSF) , San Francisco, California , USA.
    Background: The presence of mitral valve prolapse (MVP) in congenital heart disease (CHD) patients is not well described. Tetralogy of Fallot (TOF) is the most common cyanotic CHD associated with overall good long-term survival after palliation. Since MVP is more often identified in adults and TOF patients are now surviving longer, we thus sought to perform this cohort study with a case-control design to (1) determine the prevalence of MVP and systolic displacement of mitral leaflets (SDML) in adult TOF patients, and (2) describe their clinical and imaging characteristics. Read More

    Temporally Distinct Six2-Positive Second Heart Field Progenitors Regulate Mammalian Heart Development and Disease.
    Cell Rep 2017 Jan;18(4):1019-1032
    Departments of Urology and Surgery, Boston Children's Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA. Electronic address:
    The embryonic process of forming a complex structure such as the heart remains poorly understood. Here, we show that Six2 marks a dynamic subset of second heart field progenitors. Six2-positive (Six2(+)) progenitors are rapidly recruited and assigned, and their descendants are allocated successively to regions of the heart from the right ventricle (RV) to the pulmonary trunk. Read More

    Cardiac resynchronization therapy in adults with congenital heart disease.
    Europace 2017 Jan 20. Epub 2017 Jan 20.
    Division of Cardiology, Peter Munk Cardiac Center, Toronto Congenital Cardiac Center for Adults, and University of Toronto, Toronto, Canada.
    Aims: In adults with congenital heart disease (CHD) heart failure is one of the leading causes of morbidity and mortality but experience with and reported outcome of cardiac resynchronization therapy (CRT) is limited. We investigated the efficacy of CRT in adults with CHD.

    Methods And Results: This was a retrospective study including 48 adults with CHD who received CRT since 2003 in four tertiary referral centres. Read More

    Surgical repair of a complete atrioventricular septal defect with tetralogy of Fallot using a classical one-patch technique and pulmonary valve preservation.
    Multimed Man Cardiothorac Surg 2016 Sep 28;2017. Epub 2016 Sep 28.
    The association of complete atrial ventricular septal defect (cAVSD) with tetralogy of Fallot remains a complex malformation that involves both inlet and outlet components of the ventricles. The surgical treatment used to be, in the 1980s, a staged repair strategy due to the high mortality rate of first primary repairs (PRs). However, nowadays, PRs have better outcomes. Read More

    Isolated Left Subclavian Artery, Complete Atrioventricular Block, and Tricuspid Atresia in a Neonate.
    Tex Heart Inst J 2016 Dec 1;43(6):546-549. Epub 2016 Dec 1.
    Isolated left subclavian artery is one of the rarer aortic arch anomalies. It has been associated with other congenital heart diseases, typically tetralogy of Fallot, double-outlet right ventricle, and atrial and ventricular septal defects. Its significant clinical implications include a left-to-right shunt from the vertebrobasilar system, which causes pulmonary overcirculation and subclavian steal. Read More

    Unrepaired Tetralogy of Fallot with Absent Pulmonary Valve in a Mildly Symptomatic 16-Year-Old Boy.
    Tex Heart Inst J 2016 Dec 1;43(6):517-519. Epub 2016 Dec 1.
    Absent pulmonary valve is a rare and severe variant seen in only 3% to 6% of patients with tetralogy of Fallot. Fetuses with this combined condition who survive through birth typically need intervention in infancy or early childhood because of respiratory distress, heart failure, or failure to thrive. We describe the unusual case of a mildly symptomatic 16-year-old boy with these conditions who underwent successful primary repair. Read More

    Stress echo 2020: the international stress echo study in ischemic and non-ischemic heart disease.
    Cardiovasc Ultrasound 2017 Jan 18;15(1). Epub 2017 Jan 18.
    Cardiology Hospital, Policlinico of Bari, Bari, Italy.
    Background: Stress echocardiography (SE) has an established role in evidence-based guidelines, but recently its breadth and variety of applications have extended well beyond coronary artery disease (CAD). We lack a prospective research study of SE applications, in and beyond CAD, also considering a variety of signs in addition to regional wall motion abnormalities.

    Methods: In a prospective, multicenter, international, observational study design, > 100 certified high-volume SE labs (initially from Italy, Brazil, Hungary, and Serbia) will be networked with an organized system of clinical, laboratory and imaging data collection at the time of physical or pharmacological SE, with structured follow-up information. Read More

    How pacemakers work and simple programming: a primer for the non-electrophysiologist.
    Cardiol Young 2017 Jan;27(S1):S115-S120
    Department of Cardiology,The Hospital for Sick Children,Toronto,Ontario,Canada.
    Paediatric pacemaker management is challenging because of the small patient size, complex cardiac anatomy, and unique programming considerations. Hardware placement options include epicardial and transvenous systems. When pacemaker malfunction is suspected, a systematic approach is required to determine whether there is malfunction in pacing, sensing, or no output due to hardware problem. Read More

    Sudden cardiac arrest in patients following surgery for CHD.
    Cardiol Young 2017 Jan;27(S1):S68-S74
    Division of Cardiology,Nicklaus Children's Hospital,Miami,Florida,United States of America.
    The prevalence of sudden cardiac arrest after surgery for CHD is primarily related to the complexity of the congenital defect and the presence of residual defects, especially ventricular dysfunction. Among all causes of death in patients having CHD, about 19% lead to sudden mortality. The specific risk factors associated with the various congenital defects are poorly understood. Read More

    At what age should tetralogy of Fallot be corrected?
    Cardiol Young 2017 Jan 12:1-5. Epub 2017 Jan 12.
    Department of Pediatrics,University of California,San Francisco,California,United States of America.
    Tetralogy of Fallot can be corrected with very low mortality at any age, even in neonates, but this does not necessarily mean that it should be corrected in the neonatal period. Although there are many advantages to early correction, a high proportion of these neonates have residual stenosis or pulmonary regurgitation that impairs ventricular function and may require further surgery or implantation of a pulmonary valve. Before we had the ability to correct this anomaly with low mortality in small children, a variety of palliative procedures had to be performed. Read More

    Main pulmonary artery cross-section ratio is low in fetuses with tetralogy of Fallot and ductus arteriosus-dependent pulmonary circulation.
    Cardiol Young 2017 Jan 12:1-5. Epub 2017 Jan 12.
    Department of Pediatric Cardiology,National Cerebral and Cardiovascular Center,Osaka,Japan.
    Objectives: This study aimed to determine fetal echocardiographic features of tetralogy of Fallot in association with postnatal outcomes.

    Methods: The Z-scores of the main and bilateral pulmonary arteries and the aorta were measured, and the following variables were calculated in 13 fetuses with tetralogy of Fallot: pulmonary artery-to-aorta ratio and main pulmonary artery cross-section ratio - the main pulmonary artery diameter squared divided by the sum of the diameter squared of the left and right pulmonary arteries. Fetuses were classified as having ductus arteriosus-dependent or ductus arteriosus-independent pulmonary circulation. Read More

    Isolated persistent left superior vena cava: A case report and its clinical implications.
    Ann Card Anaesth 2017 Jan-Mar;20(1):104-107
    Department of Cardiothoracic and Vascular Surgery, Apollo Hospitals, Bhubaneswar, Odisha, India.
    The venous anomaly of a persistent left superior vena cava (PLSVC) affects 0.3%-0.5% of the general population. Read More

    Basic arterial blood gas biomarkers as a predictor of mortality in tetralogy of Fallot patients.
    Ann Card Anaesth 2017 Jan-Mar;20(1):67-71
    Department of CTVS, CTC, AIIMS, New Delhi, India.
    Background: Serum lactate and base deficit have been shown to be a predictor of morbidity and mortality in critically ill patients. Poor preoperative oxygenation appears to be one of the significant factors that affects early mortality in tetralogy of Fallot (TOF). There is little published literature evaluating the utility of serum lactate, base excess (BE), and oxygen partial pressure (PO 2 ) as simple, widely available, prognostic markers in patients undergoing surgical repair of TOF. Read More

    Acquired coronary artery disease in adult patients with congenital heart disease: a true or a false problem?
    J Cardiovasc Med (Hagerstown) 2017 Jan 7. Epub 2017 Jan 7.
    aCongenital Cardiac Surgery Unit bPediatric and Adult Congenital Heart Center cDepartment of Cardiac Surgery, IRCCS Policlinico San Donato, Milan, Italy.
    Background: The population of adults with congenital heart disease (ACHD) is increasing and aging, and a large percentage of this population is now over 65 years of age. For this reason, it is probable that acquired coronary artery disease (CAD) will become an important issue that needs to be addressed also in these patients. We retrospectively analyzed all ACHD patients who underwent surgery in our Institution with the aim to investigate the incidence of associated CAD and the results of surgical treatment. Read More

    The Role of Conventional and Right-Sided ECG Screening for Subcutaneous ICD in a Tetralogy of Fallot Population.
    Pacing Clin Electrophysiol 2017 Feb;40(2):145-153
    Electrophysiology Section, Cardiology Department, La Fe University Hospital, Valencia, Spain.
    Background: Information regarding suitability for subcutaneous implantable cardioverter-defibrillator (S-ICD) implant in tetralogy of Fallot (ToF) population is scarce and needs to be further explored.

    The Aims Of Our Study Were: (1) to determine the proportion of patients with ToF eligible for S-ICD, (2) to identify the optimal sensing vector in ToF patients, (3) to test specifically the eligibility for S-ICD with right-sided screening, and (4) to compare with the proportion of eligible patients in a nonselected ICD population.

    Methods: We recruited 60 consecutive patients with ToF and 40 consecutive nonselected patients. Read More

    Cardiac Magnetic Resonance Feature Tracking Biventricular Two-Dimensional and Three-Dimensional Strains to Evaluate Ventricular Function in Children After Repaired Tetralogy of Fallot as Compared with Healthy Children.
    Pediatr Cardiol 2017 Jan 5. Epub 2017 Jan 5.
    Division of Pediatric Cardiology, Memorial Hermann Children's Hospital, University of Texas Medical School at Houston, Houston, TX, USA.
    Cardiac magnetic resonance imaging is an important tool to evaluate cardiac anatomy and ventricular size and function after repaired tetralogy of Fallot. Magnetic resonance tissue tagging is the gold standard for evaluation of myocardial strain. However, myocardial tagging strain requires tagged images to be obtained prospectively, during the scan and with limited temporal resolution. Read More

    Persistent left superior vena cava accompanying congenital heart disease in children: Experience of a tertiary care center.
    Echocardiography 2017 Jan 3. Epub 2017 Jan 3.
    Department of Pediatric Cardiology, Dr. Sami Ulus Obstetrics and Gynecology, Children's Health and Diseases Training and Research Hospital, Ankara, Turkey.
    Background: This study focuses on determining concomitant persistent left superior vena cava (SVC) in patients with congenital heart disease (CHD).

    Methods: Between 2005 and 2012, a total of 2.663 patients with CHD, 88 (3. Read More

    Proarrhythmic remodelling of the right ventricle in a porcine model of repaired tetralogy of Fallot.
    Heart 2017 Mar 8;103(5):347-354. Epub 2016 Oct 8.
    IHU LIRYC, L'Institut de Rythmologie et Modélisation Cardiaque, Fondation Bordeaux Université, Bordeaux, France.
    Objective: The growing adult population with surgically corrected tetralogy of Fallot (TOF) is at risk of arrhythmias and sudden cardiac death. We sought to investigate the contribution of right ventricular (RV) structural and electrophysiological remodelling to arrhythmia generation in a preclinical animal model of repaired TOF (rTOF).

    Methods And Results: Pigs mimicking rTOF underwent cardiac MRI functional characterisation and presented with pulmonary regurgitation, RV hypertrophy, dilatation and dysfunction compared with Sham-operated animals (Sham). Read More

    Case report: maternal mosaicism resulting in inheritance of a novel GATA6 mutation causing pancreatic agenesis and neonatal diabetes mellitus.
    Diagn Pathol 2017 Jan 3;12(1). Epub 2017 Jan 3.
    Division of Pediatric Endocrinology Montreal Children's Hospital, McGill University Health Centre, 1001 Boulevard Decarie, Montreal, H4A 3J1, Quebec, Canada.
    Background: Haploinsufficiency of the GATA6 transcription factor gene was recently found to be the most common cause of pancreatic agenesis, a rare cause of neonatal diabetes mellitus. Although most cases are de novo, we describe three siblings with inherited GATA6 haploinsufficiency and the rare finding of parental mosaicism.

    Case Presentation: The proband was born at term with severe intrauterine growth restriction, the first child of non-consanguineous parents. Read More

    Postsystolic Shortening Is Associated with Altered Right Ventricular Function in Children after Tetralogy of Fallot Surgical Repair.
    PLoS One 2017 3;12(1):e0169178. Epub 2017 Jan 3.
    Department of Pediatric Cardiology and General Pediatrics. Medical University of Warsaw Zwirki i Wigury, Warsaw, Poland.
    The aim of the study was to determine whether segmental interactions, as expressed by postsystolic shortening (PSS), affects RV mechanics and are connected with impaired systolic and diastolic function in rTOF children.

    Patients And Methods: 55 rTOF adolescent (study group), and 34 healthy volunteers (control group) were examined using classical Doppler flow (Doppler), Tissue Doppler Imaging (TDI) and Speckle Tracking Echocardiography (STE). PSS was found to occur when time to peak (TTP) was longer than pulmonary valve closure time (PVCT). Read More

    Right Ventricular Outflow Tract Reconstruction With a Polytetrafluoroethylene Monocusp Valve: A 20-Year Experience.
    Semin Thorac Cardiovasc Surg 2016 Summer;28(2):463-470. Epub 2016 Jun 2.
    Section of Cardiothoracic Surgery, Department of Surgery, James Whitcomb Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, Indiana. Electronic address:
    In patients with tetralogy of Fallot (TOF), pulmonary atresia (PA), and other congenital right ventricular outflow tract (RVOT) malformations, polytetrafluoroethylene (PTFE) monocusp outflow tract patches (MOTP) relieve obstruction and provide pulmonary valve competence. The purpose of this study was to determine whether our PTFE-MOTP was an acceptable short- and mid-term remedy for patients with TOF or PA as assessed by freedom from severe pulmonary regurgitation and freedom from reoperation. From 1994-2014, 171 patients (mean age 1. Read More

    Porcine Intestinal Submucosa (CorMatrix) for Semilunar Valve Repair in Children: A Word of Caution After Midterm Results.
    Semin Thorac Cardiovasc Surg 2016 Summer;28(2):436-445. Epub 2016 Jun 1.
    Pediatric and Congenital Cardiac Surgery Unit, Department of Cardiac Thoracic and Vascular Sciences, School of Medicine, University of Padova, Padova, Italy.
    Surgery for congenital valve anomalies in children is a challenging topic. We aim to assess early and late functional outcomes of CorMatrix scaffold after repair of aortic and pulmonary valves (PV) in congenital heart disease in a prospective nonrandomized clinical study on children with congenital aortic (Group 1) or PV (Group 2) disease. Primary endpoints were reoperation or reintervention on semilunar valves and echocardiographic evidence of regurgitation or stenosis greater than mild. Read More

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