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    1 OF 218

    Clinical implications of hypothermic ventricular fibrillation versus beating-heart technique during cardiopulmonary bypass for pulmonary valve replacement in patients with repaired tetralogy of Fallot.
    Interact Cardiovasc Thorac Surg 2017 May 23. Epub 2017 May 23.
    Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, Jongnogu, Seoul, Republic of Korea.
    Objectives: This study aimed to compare the effects of hypothermic ventricular fibrillation and beating-heart techniques during cardiopulmonary bypass (CPB) on postoperative outcomes after simple pulmonary valve replacement in patients with repaired tetralogy of Fallot (TOF).

    Methods: We retrospectively reviewed the data of 47 patients with repaired tetralogy of Fallot at a single institution, who received pulmonary valve replacement under the ventricular fibrillation or beating-heart technique without cardioplegic cardiac arrest during CPB between January 2005 and April 2015.

    Results: The patients were divided into fibrillation ( n  = 32) and beating-heart ( n  = 15) groups. Read More

    Psychiatric Disorders and Function in Adolescents with Tetralogy of Fallot.
    J Pediatr 2017 May 19. Epub 2017 May 19.
    Department of Psychiatry, Boston Children's Hospital, Boston, MA; Department of Psychiatry, Harvard Medical School, Boston, MA; Department of Cardiology, Boston Children's Hospital, Boston, MA. Electronic address:
    Objectives: To assess psychiatric disorders and function in adolescents with repaired tetralogy of Fallot (TOF) without and with a genetic diagnosis and to evaluate associations of functioning with medical factors, IQ, and demographics.

    Study Design: Adolescents with TOF (n = 91) and 87 healthy referents completed a clinician-rated structured psychiatric interview, parent-/self-report measures of psychopathology, and brain magnetic resonance imaging. Twenty-three of the adolescents with TOF had a known genetic diagnosis. Read More

    Gene expression in pediatric heart disease with emphasis on conotruncal defects.
    Prog Pediatr Cardiol 2005 Jul 9;20(2):127-141. Epub 2005 Jun 9.
    Section of Medical Genetics and Molecular Medicine, Children's Mercy Hospitals and Clinics and University of Missouri-Kansas City School of Medicine, 2401 Gillham Rd., Kansas City, MO 64108, United States.
    Developmental abnormalities of the heart are the underlying cause of many congenital heart malformations. The embryological development of the integrated cardiovascular tissue is the result of multiple tissue and cell-to-cell interactions involving temporal and spatial events under genetic control. Recent technological advances, like microarray analysis of gene expression, are providing new tools to aid in deciphering the complex networks of gene expression that regulate cardiac development. Read More

    Long-term respiratory outcomes after primary total correction for tetralogy of Fallot and absent pulmonary valve in patient with respiratory symptoms.
    Congenit Heart Dis 2017 May 19. Epub 2017 May 19.
    Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
    Objective: To review long-term respiratory outcomes for tetralogy of Fallot and absent pulmonary valve (TOF/APV) in respiratory symptomatic populations.

    Methods: Of 25 consecutive patients undergoing primary total correction for TOF/APV between 1987 and 2016, Sixteen patients (64%) with a preoperative respiratory disturbance were enrolled. The median age at operation was 1. Read More

    Virtual Surgery for Conduit Reconstruction of the Right Ventricular Outflow Tract.
    World J Pediatr Congenit Heart Surg 2017 May;8(3):391-393
    1 Division of Cardiac Surgery, Johns Hopkins Hospital, Baltimore, MD, USA.
    Purpose: Virtual surgery involves the planning and simulation of surgical reconstruction using three-dimensional (3D) modeling based upon individual patient data, augmented by simulation of planned surgical alterations including implantation of devices or grafts. Here we describe a case in which virtual cardiac surgery aided us in determining the optimal conduit size to use for the reconstruction of the right ventricular outflow tract.

    Description: The patient is a young adolescent male with a history of tetralogy of Fallot with pulmonary atresia, requiring right ventricle-to-pulmonary artery (RV-PA) conduit replacement. Read More

    Spectrum of pulmonary valve morphology and its relationship to pulmonary trunk in tetralogy of Fallot.
    Indian J Radiol Imaging 2017 Jan-Mar;27(1):65-69
    Department of Biostatistics, Christian Medical College, Vellore, Tamil Nadu, India.
    Background: Tetralogy of Fallot (TOF) is a complex congenital heart disease with anatomic variations. Although the pulmonary valve in TOF is abnormal, it has not been studied well, especially on newer imaging modalities such as multidetector computed tomography (CT), which gives excellent anatomic detail.

    Aims: The aim of this study was to assess the morphology of pulmonary valve in TOF on CT and evaluate its association with the degree of hypoplasia of infundibulum and pulmonary trunk. Read More

    Istaroxime, a potential anticancer drug in prostate cancer, exerts beneficial functional effects in healthy and diseased human myocardium.
    Oncotarget 2017 Apr 30. Epub 2017 Apr 30.
    Division of Cardiology, Department of Internal Medicine, Medical University of Graz, 8036 Graz, Austria.
    The current gold standard for prostate cancer treatment is androgen deprivation therapy and antiandrogenic agents. However, adverse cardiovascular events including heart failure can limit therapeutic use. Istaroxime, which combines Na+-K+-ATPase (NKA) inhibition with sarco/endoplasmic reticulum Ca2+-ATPase 2a (SERCA2a) stimulation, has recently shown promising anti-neoplastic effects in prostate cancer (PC) models and may also improve cardiac function. Read More

    Total Correction of Tetralogy of Fallot at Early Age: A Study of 183 Cases.
    Heart Lung Circ 2017 Apr 27. Epub 2017 Apr 27.
    Cardiothoracic Surgery Department, Zagazig University, Egypt.
    Background: Opinions regarding the optimal time for the repair of tetralogy of Fallot vary. A debate also exists about the timing of repair for the asymptomatic infant.

    Methods: This study included 183 patients with tetralogy of Fallot. Read More

    Conventional and right-sided screening for subcutaneous ICD in a population with congenital heart disease at high risk of sudden cardiac death.
    Ann Noninvasive Electrocardiol 2017 May 15. Epub 2017 May 15.
    Department of Cardiology, La Fe University Hospital, Valencia, Spain.
    Background: Information regarding suitability for subcutaneous defibrillator (sICD) implantation in tetralogy of Fallot (ToF) and systemic right ventricle is scarce and needs to be further explored. The main objective of our study was to determine the proportion of patients with ToF and systemic right ventricle eligible for sICD with both, standard and right-sided screening methods. Secondary objectives were: (i) to study sICD eligibility specifically in patients at high risk of sudden cardiac death, (ii) to identify independent predictors for sICD eligibility, and (iii) to compare the proportion of eligible patients in a nonselected ICD population. Read More

    Bronchoscopy in children with tetralogy of fallot, pulmonary atresia, and major aortopulmonary collaterals.
    Pediatr Pulmonol 2017 May 15. Epub 2017 May 15.
    Department of Pediatrics, Stanford University, Palo Alto, California.
    Objective: Children with Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals (TOF/PA/MAPCAs) undergoing unifocalization surgery are at risk for developing more postoperative respiratory complications than children undergoing other types of congenital heart surgery. Bronchoscopy is used in the perioperative period for diagnostic and therapeutic purposes. In this study, we describe bronchoscopic findings and identify factors associated with selection for bronchoscopy. Read More

    Klinefelter Syndrome in Association with Tetralogy of Fallot and Congenital Diaphragmatic Hernia.
    J Pediatr Genet 2017 Jun 3;6(2):115-117. Epub 2017 Jan 3.
    Department of Human Genetics, University of Miami, Miller School of Medicine, Dr. John T. Macdonald Foundation, Miami, Florida, United States.
    Klinefelter syndrome (KS) is the most common sex chromosomal aneuploidy in males. Major cardiovascular and diaphragmatic anomalies are uncommon in this syndrome. Here we report an infant with KS who had tetralogy of Fallot and congenital diaphragmatic hernia, all of which were identified prenatally and managed successfully after birth. Read More

    Evaluation of Congenital Heart Defects Treatment Options-Establishment of Pediatric Cardiology/Cardiosurgery in Bosnia and Herzegovina.
    Mater Sociomed 2017 Mar;29(1):73-75
    Pediatric Clinic, University Clinical Center Sarajevo, Sarajevo, Bosnia and Herzegovina.
    Introduction: Modern pediatric cardiology mainly deals with congenital heart defects (CHD), as the most common congenital anomalies. In most cases CHD requires surgical or interventional treatment.

    Goal: The goal of the research was to evaluate CHD treatment at Pediatric Clinic, University Clinical Center (UCC) Sarajevo, Bosnia and Herzegovina (B&H). Read More

    Impact of Structural Cerebral Damage in Adults With Tetralogy of Fallot.
    Circulation 2017 May;135(19):1873-1875
    From Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands (M.A.S., B.J.B., M.G., B.J.M.M.); Interuniversity Cardiology Institute of the Netherlands, Utrecht (M.A.S., B.J.M.M.); Department of Neurology, Academic Medical Center, Amsterdam, The Netherlands (E.R., J.W.v.D., L.L.v.W.); Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands (E.R.); Department of Medical Psychology, (L.L.v.W., B.A.S.), Department of Radiology (M.G., M.W.A.C., A.J.N., H.-J.M.M.M., C.B.L.M.M.), Academic Medical Center, Amsterdam, The Netherlands; Hurvitz Brain Sciences Program, Sunnybrook Research Institute, University of Toronto, Canada (H.-J.M.M.M.); and Department of Psychology, University of Amsterdam, The Netherlands (B.A.S.).

    Left aberrant subclavian artery. Systematic study in adult patients.
    Int J Cardiol 2017 Apr 25. Epub 2017 Apr 25.
    Department of Interventional Cardiology and Angiology, Institute of Cardiology, Warsaw, Poland.
    Background: Left aberrant subclavian artery (LASA), is a type of right aortic arch (RAA) branching, which takes-off distally to the right subclavian artery and usually crosses behind the esophagus to the left upper limb. Taking into account the rarity of RAA, LASA is much more rarely seen than the right aberrant subclavian artery (RASA) originating from the left aortic arch. However, RAA may be associated with much more frequent presence of LASA, than left aortic arch with RASA. Read More

    An antenatal marker of neurodevelopmental outcomes in infants with congenital heart disease.
    J Perinatol 2017 May 4. Epub 2017 May 4.
    Department of Pediatrics, Columbia University Medical Center, New York, NY, USA.
    Objective: Prenatal exposures are known to alter fetal neurodevelopment and autonomic control. We aimed to explore the correlation between fetal autonomic activity, measured by fetal heart rate variability, and 18-month developmental outcome in subjects with congenital heart disease.

    Study Design: From 2010 to 2013, 5 fetuses with hypoplastic left heart syndrome, 9 with transposition of the great arteries and 9 with tetralogy of Fallot were included in this prospective cohort study. Read More

    Twenty years of experience with intraoperative pulmonary artery stenting.
    Catheter Cardiovasc Interv 2017 May 4. Epub 2017 May 4.
    Department of Cardiac Surgery, University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, Michigan.
    Objectives: To describe our 20-year experience with intraoperative pulmonary artery (PA) stent placement and evaluate long-term patient outcomes, specifically the need and risk factors for reintervention.

    Background: Intraoperative PA stent placement is an alternative to surgical patch arterioplasty and percutaneous angioplasty or stent placement to treat branch PA stenosis.

    Methods: We performed a retrospective review of all intraoperative PA stents placed at our institution from 1994-2013. Read More

    Prevalence of Congenital Heart Disease among Infants from 2012 to 2014 in Langfang, China.
    Chin Med J (Engl) 2017 May;130(9):1069-1073
    Department of Ultrasound, PLA Army General Hospital, Beijing 100700, China.
    Background: Congenital heart disease (CHD) is the most common congenital malformations with high mortality and morbidity. The prevalence of CHD reported previously ranged from 4 per 1000 live births to 50 per 1000 live births. In this cross-sectional study, we aimed to document the prevalence of CHD in Langfang district of Hebei Province, China by analyzing data collected by hospitals located in 11 the counties of the district, as supported by a public health campaign. Read More

    Neonatal Cardiac Surgery in the New Era: Lessons learned from 1000 Consecutive Cases.
    Isr Med Assoc J 2016 Nov;18(11):645-648
    Division of Pediatric Heart Institute, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.
    Background: neonatal cardiac surgery has evolved over the last 50 years with a large percentage of the patients achieving complete physiological repair in the neonatal period. The remaining patients achieve staged palliation with an increasing amount of success.

    Objectives: To report our experience with 1000 neonatal cardiac surgical procedures performed in the last 10 years. Read More

    Forced vital capacity predicts morbidity and mortality in adults with repaired tetralogy of Fallot.
    Congenit Heart Dis 2017 May 2. Epub 2017 May 2.
    Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin.
    Objective: Abnormal lung function characterized by a reduced forced vital capacity (FVC) is common in adults with repaired tetralogy of Fallot (TOF) and is associated with previous thoracotomies and sternotomies. The impact of abnormal lung function on clinical outcomes in adult patients with repaired TOF is unclear. The aim of this study was to determine the impact of abnormal lung function on the outcome of hospitalization and death in adults with repaired TOF when analyzed with other traditional cardiac risk factors. Read More

    Revisiting the utility of technical performance scores following tetralogy of Fallot repair.
    J Thorac Cardiovasc Surg 2017 Apr 5. Epub 2017 Apr 5.
    Division of Pediatric Cardiac Surgery, Phoenix Children's Hospital, Phoenix, Ariz. Electronic address:
    Objective: Although an important quality metric, current technical performance scores may not be generalizable and may omit operative factors that influence outcomes. We examined factors not included in current technical performance scores that may contribute to increased postoperative length of stay, major complications, and cost after primary repair of tetralogy of Fallot.

    Methods: This is a retrospective single site study of patients younger than age 2 years with tetralogy of Fallot undergoing complete repair between 2007 and 2015. Read More

    The development of pacing induced ventricular dysfunction is influenced by the underlying structural heart defect in children with congenital heart disease.
    Indian Heart J 2017 Mar - Apr;69(2):240-243. Epub 2016 Dec 2.
    University Hospital, Cologne, Germany.
    Background: Right ventricular pacing can cause pacing-induced ventricular dysfunction (PIVD) correctable with biventricular pacing (BiVP). Factors associated with PIVD are poorly understood.

    Methods: We reviewed children receiving epicardial dual-chamber pacemakers for complete heart block (CHB) after congenital heart disease (CHD) surgery. Read More

    Is the presence of end-diastolic forward flow specific for restrictive right ventricular physiology in repaired tetralogy of Fallot?
    Int J Cardiol 2017 Apr 11. Epub 2017 Apr 11.
    Seirei Hamamatsu General Hospital, Department of Cardiovascular Surgery, Japan.
    Background: End-diastolic forward flow (EDFF) is recognized as restrictive right ventricular physiology (r-RVP), but conflicting results have been reported about effects on the clinical outcome in repaired tetralogy of Fallot (r-TOF).

    Objectives: We hypothesized that the EDFF by Doppler was not specific for diagnosing r-RVP.

    Methods: Sixty-two consecutive patients aged 15. Read More

    Clinical Aspects and Ablation of Ventricular Arrhythmias in Tetralogy of Fallot.
    Card Electrophysiol Clin 2017 Jun 13;9(2):285-294. Epub 2017 Mar 13.
    Department of Cardiology, Leiden University Medical Center, Postal Zone: C-05-P, PO Box 9600, Leiden 2300 RC, The Netherlands.
    Life expectancy of patients with rToF has considerably improved due to refined surgical interventions. Monomorphic fast VTs are frequently encountered in adult patients with rToF. The dominant substrate of VT is anatomical isthmuses bordered by surgical incisions, patch material and valve annuli. Read More

    Sudden Cardiac Death in Adult Congenital Heart Disease.
    Card Electrophysiol Clin 2017 Jun 18;9(2):225-234. Epub 2017 Mar 18.
    Adult Congenital Heart Center, Montreal Heart Institute, Université de Montréal, 5000 Belanger Street East, Montreal H1T 1C8, Canada. Electronic address:
    Sudden death of presumed arrhythmic etiology is a leading cause of mortality in adults with congenital heart disease. Anticipated benefits of the implantable cardioverter-defibrillator (ICD) must be weighed against high complication rates. Without robust evidence from randomized trials, caregivers face difficult decisions in selecting appropriate candidates. Read More

    Left Ventricular Stiffness in Adolescents and Young Adults with Repaired Tetralogy of Fallot.
    Sci Rep 2017 Apr 28;7(1):1252. Epub 2017 Apr 28.
    Department of Paediatrics and Adolescent Medicine, LKS Faculty of Medicine, The University of Hong Kong, Hong Kong, China.
    Left ventricular (LV) remodeling after tetralogy of Fallot (TOF) repair may influence LV stiffness. We hypothesized that LV stiffness is altered after TOF repair and related to myocardial calibrated integrated backscatter (cIB) and LV diastolic myocardial deformation. Seventy-seven TOF patients and 80 controls were studied. Read More

    Right vEntricular Dysfunction in tEtralogy of Fallot: INhibition of the rEnin-angiotensin-aldosterone system (REDEFINE) trial: Rationale and design of a randomized, double-blind, placebo-controlled clinical trial.
    Am Heart J 2017 Apr 2;186:83-90. Epub 2017 Jan 2.
    Department of Cardiology, Academic Medical Center Amsterdam, Amsterdam, the Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utretch, the Netherlands. Electronic address:
    Renin-angiotensin-aldosterone system (RAAS) inhibition with angiotensin II receptor blockers or angiotensin-converting enzyme inhibitors is beneficial in patients with acquired left ventricular dysfunction. Adult patients with tetralogy of Fallot (TOF) with right ventricular (RV) dysfunction are at high risk for heart failure, arrhythmias, and sudden cardiac death. However, the efficacy of RAAS inhibition has not been established in these patients. Read More

    MicroRNAs: pleiotropic players in congenital heart disease and regeneration.
    J Thorac Dis 2017 Mar;9(Suppl 1):S64-S81
    Division of Experimental Surgery, Department of Cardiovascular Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany.
    Congenital heart disease (CHD) is the leading cause of infant death, affecting approximately 4-14 live births per 1,000. Although surgical techniques and interventions have improved significantly, a large number of infants still face poor clinical outcomes. MicroRNAs (miRs) are known to coordinately regulate cardiac development and stimulate pathological processes in the heart, including fibrosis or hypertrophy and impair angiogenesis. Read More

    Surgical Strategy Toward Biventricular Repair for Severe Ebstein Anomaly in Neonates and Infancy.
    Ann Thorac Surg 2017 Apr 20. Epub 2017 Apr 20.
    Department of Surgery, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan.
    Background: Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair. Read More

    The effects of corrective surgery on endothelial biomarkers and anthropometric data in children with congenital heart disease.
    J Int Med Res 2017 Apr 1;45(2):493-503. Epub 2017 Jan 1.
    1 Department of Paediatrics, Linkou, Taiwan.
    Objective To investigate the influence of surgical correction on biomarkers of endothelial dysfunction in children with congenital heart disease and to evaluate anthropometric data. Methods Children with pulmonary hypertension (PH) or Tetralogy of Fallot (TOF) who were scheduled for corrective surgery were enrolled in this prospective study. Age-matched healthy children were included as controls. Read More

    Renal dysplasia characterized by prominent cartilaginous metaplasia lesions in VACTERL association: A case report.
    Medicine (Baltimore) 2017 Apr;96(15):e6499
    aDepartment of Pathology, Jichi Medical University bDepartment of Pediatric Urology, Children's Medical Center Tochigi and Jichi Medical University, Shimotsuke, Tochigi cDepartment of Pathology, Kanagawa Children's Medical Center, Yokohama, Kanagawa, Japan.
    Background: Renal dysplasia is the most important cause of end-stage renal disease in children. The histopathological characteristic of dysplasia is primitive tubules with fibromuscular disorganization. Renal dysplasia often includes metaplastic cartilage. Read More

    Sudden cardiac death in adults with congenital heart disease: does QRS-complex fragmentation discriminate in structurally abnormal hearts?
    Europace 2017 Apr 10. Epub 2017 Apr 10.
    Department of Cardiology, Heart Center, Academic Medical Center-University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.
    Aims: Sudden cardiac death (SCD) causes a large portion of all mortality in adult congenital heart disease (ACHD) patients. However, identification of high-risk patients remains challenging. Fragmented QRS-complexes (fQRS) are a marker for SCD in patients with acquired heart disease but data in ACHD patients are lacking. Read More

    Multiple variations of the cerebral arteries associated with tetralogy of Fallot: a case report.
    Surg Radiol Anat 2017 Apr 10. Epub 2017 Apr 10.
    Department of Pediatrics and Pediatric Cardiology, Saitama Medical University Saitama Medical Center, 1397-1 Yamane, Hidaka, Saitama, 350-1298, Japan.
    Tetralogy of Fallot (TOF) can be associated with vascular malformations or variations, especially of the aortic arch or supracardiac major venous systems. We report its association with an extremely rare combination of three such variations of the cerebral arteries-a right persistent hypoglossal artery, an extremely rare left carotid-right anterior cerebral artery (ACA) anastomosis, and a left accessory middle cerebral artery arising from the A1-A2 junction of the left ACA-which were diagnosed by magnetic resonance angiography in a 9-year-old girl during evaluation of multiple acute cerebral infarctions. Read More

    Noninvasive predictors of perioperative atrial arrhythmias in patients with tetralogy of Fallot undergoing pulmonary valve replacement.
    Clin Cardiol 2017 Apr 10. Epub 2017 Apr 10.
    Department of Cardiology, University of Colorado, Aurora.
    Background: Patients with tetralogy of Fallot (TOF) have increased risk of atrial arrhythmias.

    Hypothesis: A measure of atrial dispersion, the P-wave vector magnitude (Pvm), can identify patients at risk for perioperative atrial flutter (AFL) or intra-atrial re-entrant tachycardia (IART) in a large TOF cohort.

    Methods: We performed a blinded, retrospective analysis of 158 TOF patients undergoing pulmonary valve replacement between 1997 and 2015. Read More

    The utility of preoperative level of erythrocytosis in the prediction of postoperative blood loss and 30-day mortality in patients with tetralogy of fallot.
    Ann Card Anaesth 2017 Apr-Jun;20(2):188-192
    Department of Anesthesiology, Centro Medico Imbanaco, Cali, Colombia.
    Background: Postoperative major bleeding is a relatively common complication of patients undergoing corrective surgery of tetralogy of Fallot (TOF). Life-threatening blood losses can lead to aggressive transfusions or reoperation. Little is known about the risk factors associated with a bleeding tendency in TOF patients. Read More

    Association of airway abnormalities with 22q11.2 deletion syndrome.
    Int J Pediatr Otorhinolaryngol 2017 May 21;96:11-14. Epub 2017 Feb 21.
    22q and You and Clinical Genetics Centers, The Children's Hospital of Philadelphia, Philadelphia, PA, USA; Department of Pediatrics, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA. Electronic address:
    Introduction: 22q11.2 deletion syndrome (22q11.2DS) presents with complex but variable symptoms, including cardiac, immune, palatal, endocrine, cognitive, and psychiatric issues. Read More

    Coincidental Significant Tricuspid Regurgitation at the Time of Right Ventricle-to-Pulmonary Artery Conduit Intervention: Should We Address it, Ignore it, or Take a More Nuanced Approach?
    JACC Cardiovasc Interv 2017 Apr;10(7):709-711
    Division of Cardiology, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia; Division of Pediatric Cardiology, Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia.

    Tetralogy of Fallot Surgical Repair: Shunt Configurations, Ductus Arteriosus and the Circle of Willis.
    Cardiovasc Eng Technol 2017 Apr 5. Epub 2017 Apr 5.
    Department of Mechanical Engineering, Koç University, Rumeli Feneri Kampüsü, Sarıyer, Istanbul, Turkey.
    In this study, hemodynamic performance of three novel shunt configurations that are considered for the surgical repair of tetralogy of Fallot (TOF) disease are investigated in detail. Clinical experience suggests that the shunt location, connecting angle, and its diameter can influence the post-operative physiology and the neurodevelopment of the neonatal patient. An experimentally validated second order computational fluid dynamics (CFD) solver and a parametric neonatal diseased great artery model that incorporates the ductus arteriosus (DA) and the full patient-specific circle of Willis (CoW) are employed. Read More

    Use of Cardiac Computed Tomography for Ventricular Volumetry in Late Postoperative Patients with Tetralogy of Fallot.
    Korean J Thorac Cardiovasc Surg 2017 Apr 5;50(2):71-77. Epub 2017 Apr 5.
    Division of Pediatric Cardiac Surgery, Asan Medical Center, University of Ulsan College of Medicine.
    Background: Cardiac computed tomography (CT) has emerged as an alternative to magnetic resonance imaging (MRI) for ventricular volumetry. However, the clinical use of cardiac CT requires external validation.

    Methods: Both cardiac CT and MRI were performed prior to pulmonary valve implantation (PVI) in 11 patients (median age, 19 years) who had undergone total correction of tetralogy of Fallot during infancy. Read More

    Short-Term Change of Exercise Capacity in Patients with Pulmonary Valve Replacement after Tetralogy of Fallot Repair.
    Korean Circ J 2017 Mar 13;47(2):254-262. Epub 2017 Mar 13.
    Department of Pediatrics, Sejong General Hospital, Bucheon, Korea.
    Background And Objectives: The aim of this study was to investigate the effect of pulmonary valve replacement (PVR) on exercise capacity and determine cardiopulmonary exercise (CPEX) parameters associated with improvement in right ventricle (RV) function.

    Subjects And Methods: We retrospectively analyzed CPEX and magnetic resonance imaging parameters in a total of 245 patients who underwent PVR from January 1998 to October 2015. In addition, we analyzed the characteristics of the patients who showed improved exercise capacity after PVR. Read More

    Multiple gene variations contributed to congenital heart disease via GATA family transcriptional regulation.
    J Transl Med 2017 Apr 3;15(1):69. Epub 2017 Apr 3.
    Shanghai Key Lab of Birth Defect, Children's Hospital of Fudan University, Shanghai, 201102, China.
    Background: Congenital heart disease (CHD) is a common birth defect, and most cases occur sporadically. Mutations in key genes that are responsible for cardiac development could contribute to CHD. To date, the genetic causes of CHD remain largely unknown. Read More

    Acute right ventricular resynchronization improves haemodynamics in children after surgical repair of tetralogy of Fallot.
    Europace 2017 Mar 23. Epub 2017 Mar 23.
    Children's Heart Centre, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, V Úvalu 84, 150 06 Prague, Czech Republic.
    Aims: Right bundle branch block is associated with right ventricular (RV) electromechanical dyssynchrony, which may contribute to acute haemodynamic impairment after repair of tetralogy of Fallot (ToF). We sought to evaluate the effects of RV resynchronization on haemodynamics and tissue oxygenation during the first 24 h after surgery.

    Methods And Results: Arterial pressures, cardiac output, and tissue oxygenation were measured in 28 consecutive patients (median age 10. Read More

    Twenty Years of Anesthetic and Perioperative Management of Patients With Tetralogy of Fallot With Absent Pulmonary Valve.
    J Cardiothorac Vasc Anesth 2017 Feb 4. Epub 2017 Feb 4.
    Department of Anesthesiology, Perioperative and Pain Medicine, Division of Cardiac Anesthesia, Boston Children's Hospital, Boston, MA. Electronic address:
    Objective: Review the authors' institutional experience of the induction and perioperative airway management of children with tetralogy of Fallot with an absent pulmonary valve.

    Design: Retrospective chart review.

    Setting: Large academic children's hospital. Read More

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