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Toro Rocio R aDepartment of Medicine, Cadiz University School of Medicine, Cadiz bAdult Congenital Heart Disease Unit, Clinical Management Area of the Heart, University Hospital 'Virgen del Rocio', Seville cCardiology Department, Hospital Carlos III, Madrid, - - 2014
To assess left ventricle mechanics in Eisenmenger physiology patients with congenital shunts, and their relationship with the right ventricle, and to consider the clinical usefulness of this information. The study involved 28 patients with pulmonary artery hypertension (PAH) and congenital shunt, matched with 28 healthy participants. Standard echocardiography and pulsed ...
Rychik Jack J The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA - - 2014
Hypoplastic left heart syndrome (HLHS) is one of the most challenging forms of congenital heart disease to treat. Management of this condition consumes a substantial amount of energy, time, and bed capacity in many congenital heart centers in the western world today. Simply defined, HLHS is a congenital malformation in ...
Buber Jonathan J Department of Cardiology, Boston Children's Hospital, Boston, MA, United States. Electronic address: - - 2014
Subpulmonary ventricular outflow conduits are utilized routinely to repair complex congenital cardiac abnormalities, but are limited by the inevitable degeneration and need for reintervention. Data on conduit durability and propensity to dysfunction in the adult population are limited. The study included 288 consecutive patients ≥18years of age who were evaluated ...
Goot Benjamin H BH Division of Cardiology, Department of Pediatrics, Children's Hospital Colorado, University of Colorado, Aurora, CO, USA - - 2014
We report the clinical course of a female child with a normal karyotype and chromosomal microarray who presented as an infant with clinical findings consistent with congenital polyvalvular disease (CPVD). This clinical entity describes patients with multiple congenitally dysplastic valves, often showing nodular or cystic malformation in at least two ...
Yıldız Bekir Serhat BS Department of Cardiology, Pamukkale University Faculty of Medicine, Denizli, Turkey. - - 2014
Myocardial bridging (MB) is defined as the intramural course of a major epicardial coronary artery, and is mostly confined to the left ventricle and the left anterior descending coronary artery (LAD). MB is a common congenital abnormality of a coronary artery, and is usually thought to be a benign anatomical ...
Aboulhosn Jamil J Ahmanson/UCLA Adult Congenital Heart Center, Division of Cardiology, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, - - 2014
Left ventricular outflow obstructions (LVOTO) encompass a series of stenotic lesions beginning within the anatomic left ventricular outflow tract and ending in the descending thoracic aorta, occurring alone or in series. The degree of stenosis and hemodynamic consequences may vary from mild and well tolerated to severe and functionally limiting. ...
Bailliard Frédérique F 1Bailliard Henry Pediatric Cardiology,Raleigh,North Carolina,United States of - - 2014
Although steps are being taken to produce a universally acceptable coding system for categorisation of the congenitally malformed hearts, obstacles remain in the search for consensus. One of the groups of lesions continuing to produce the greatest problems is those that permit interventricular shunting. The difficulties relate partly to the ...
McGinley Andrea L AL Department of Biological Sciences, University of Illinois at Chicago, 900 S Ashland Ave.,, Chicago, IL, - - 2014
Congenital heart disease (CHD) is the most common birth defect. However, the majority of CHD cases have unknown etiology. Here we report the identification of ASXL2 and ASXL1, two homologous chromatin factors, as novel regulators of heart development. Asxl2(-/-) fetuses have reduced body weight and display congenital heart malformations including ...
Zanjani Keyhan S KS 1Department of Pediatrics,Children's Medical Center,Tehran University of Medical - - 2014
Left ventricular to right atrial communication is a rare congenital or acquired heart defect. There are many reports of successful transcatheter closure of this defect. We describe the device closure of one such communication in a 5-year-old girl using the Occlutech duct occluder. We believe that this device may have ...
Abreu Glória G Serviço de Cardiologia, Hospital de Braga, Braga, Portugal. Electronic address: - - 2014
Intercoronary communication or 'coronary arcade' is a rare congenital coronary anomaly. We present the case of a 65-year-old man with atypical chest pain for four months. The 12-lead ECG and echocardiogram were normal. Treadmill exercise testing was interrupted at peak exercise due to consecutive salvos of ventricular premature beats, without ...
Yin Senlin S Department of Cardiovascular Surgery, Pediatric Heart Center of West China Hospital, Sichuan University, Chengdu, Sichuan, - - 2014
Ventricular septal defect (VSD) is a common congenital heart disease. Open surgical repair with cardiopulmonary bypass (CPB) and sternotomy is the classic treatment. Percutaneous device closure is a minimally invasive alternative in treating selected VSDs, but it is limited by vascular access as well as radiation exposure especially in pediatric ...
Smith Paul J PJ East Anglia Cardiology Ltd, 5 Hall Lane, Great Hormead, Buntingford, Hertfordshire, UK. Electronic address: - - 2014
Double-chambered left ventricle is a rare congenital disorder in which the left ventricular cavity is subdivided into two cavities by an anomalous septum or muscle band. We describe a case of double-chambered left ventricle, most likely caused by the presence of excessive left ventricular bands, in an asymptomatic cat.
Ferreira António Miguel AM Cardiology and Cardiovascular Imaging Departments, Hospital da Luz, Avenida Lusíada, 100, 1500-650, Lisbon, Portugal, - - 2014
We report the incidental finding of a muscular congenital diverticulum of the left ventricular apex in a young adult with AV node reentry tachycardia. The role of cardiovascular magnetic resonance in the differential diagnosis of this rare cardiac malformation is briefly discussed.
Vanderlaan Rachel D RD Department of Surgery, Division of Cardiac Surgery, University of Toronto, Toronto, - - 2014
Heart failure can be a consequence of insufficient palliation of structural malformations in patients with congenital heart disease (CHD) or genetic perturbations resulting in cardiomyopathies. Although CHD is traditionally considered a pediatric clinical problem, there is a rapidly increasing population of patients surviving into adulthood with CHD and a corresponding ...
Subirana M Teresa MT Unidad de Cardiopatías Congénitas del Adolescente y Adulto Vall d'Hebron-Sant Pau, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. Electronic address: - - 2014
This article presents the most relevant developments in 2013 in 3 key areas of cardiology: congenital heart disease, clinical cardiology, and heart failure and transplant. Within the area of congenital heart disease, we reviewed contributions related to sudden death in adult congenital heart disease, the importance of specific echocardiographic parameters ...
Subirana M Teresa MT Unidad de Cardiopatías Congénitas del Adolescente y Adulto Vall d'Hebron-Sant Pau, Hospital de la Santa Creu i Sant Pau, Barcelona, España. Electronic address: - - 2014
This article presents the most relevant developments in 2013 in 3 key areas of cardiology: congenital heart disease, clinical cardiology, and heart failure and transplant. Within the area of congenital heart disease, we reviewed contributions related to sudden death in adult congenital heart disease, the importance of specific echocardiographic parameters ...
Suthar Renu R 1Department of Paediatrics, Division of Pediatric Neurology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, - - 2014
Stroke is an extremely rare complication of congenital heart block in children. We report a 2-year-old girl with congenital complete heart block who presented with acute-onset right middle cerebral artery territory stroke. The congenital heart block was secondary to maternal lupus.
Mylotte Darren - - 2014
In the context of congenital heart disease (CHD), the complex biochemical and physiologic response to the pressure- or volume-loaded ventricle can be induced by stenotic and shunt/regurgitant lesions, respectively. A range of transcatheter therapies have recently emerged to expand the therapeutic potential of the more traditional surgical and medical interventions ...
Patra Soumya S Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore, Karnataka, - - 2014
Afibrinogenemia is a rare autosomal recessive bleeding disorder with an estimated prevalence of 1:1,000,000. Usual presentation of this disorder is spontaneous bleeding, bleeding after minor trauma and excessive bleeding during interventional procedures. Paradoxically, few patients with afibrinogenemia may also suffer from severe thromboembolic complications. The management of these patients is ...
Cools E - - 2014
In this literature review, we try to give anesthesiologists a better understanding about Junctional Ectopic Tachycardia (JET), a narrow complex tachycardia that frequently occurs during and after surgery for congenital heart disease. Information was found in the databases of Pubmed, Science Direct, Medline and the Cochrane Library, by using the ...
Chung Jae Hoon JH Division of Internal Medicine, Sejong General Hospital, Bucheon, - - 2013
Congenital pericardial defects are rare and asymptomatic for both partial and complete defects. However, some patients can experience syncope, arrhythmia, and chest pain. When a patient experiences a symptom, it may be caused by herniation and dynamic compression or torsion of a heart structure including the coronary arteries. Diagnosis of ...
Gehin Connie - - 2013
Radiologic technologists perform imaging studies that are useful in the diagnosis of congenital heart defects in infants and adults. These studies also help to monitor congenital heart defect repairs in adults. This article describes the development and functional anatomy of the heart, along with the epidemiology and anatomy of congenital ...
K Shankarappa Ravindranath - - 2013
The double-chambered right ventricle (DCRV) is a rare congenital heart disease caused by hypertrophic anomalous muscle bands that divide the ventricular cavity, resulting in the formation of a high-pressure proximal chamber and other low-pressure distal one. Because of its evolving nature, its diagnosis is usually made during childhood/adolescence and very ...
Peters Bjoern - - 2013
Objective: Temporary pacing wires play a crucial role in the diagnosis and therapy of post-operative arrhythmia after surgery for congenital heart disease. At present, bipolar pacing wires are used in most institutions. In case of functional failure of these wires, a unipolar mode of stimulation and sensing should be theoretically ...
Dal Bianco Lucia - - 2013
This study describes the un-natural history of a 16-year-old patient, presenting with a critical bicuspid aortic valve stenosis, who was initially treated in the neonatal period and later with a valvuloplasty. This focused the attention on palliative interventions that, in young adulthood or middle age, are usually related to several ...
de Denus Simon - - 2013
Congenital heart disease (CHD) constitutes a lifelong challenge in heart failure management. Current therapy is based mainly on physiologic principles extrapolated from the management of left ventricular failure in adult populations with either ischemic or nonischemic cardiomyopathy. However, there is good evidence of genomic variability in the origin and progression ...
Zhang Zhi-Jun - - 2013
Congenital absence of the left atrial appendage (LAA) is extremely rare.We describe a case of congenial absence of the LAA in a patient scheduled for atrial fibrillation (AF) ablation. Absence of the LAA was found pre-procedurally by transoesophageal echocardiography (TEE) and cardiac computer tomography (CT). During the ablation procedure, the ...
Falsoleiman Homa H Mashhad Cardiac Research Center, School of Medicine, Mashhad University of Medical Sciences, Mashhad, - - 2013
Congenital afibrinogenemia is a rare autosomic recessive blood disorder. A 30-year-old lady, known to have congenital afibrinogenemia, presented with acute anterior myocardial infarction. We managed her with dual antiplatelet therapy and atorvastatin, but her chest pain did not subside and she was transferred to the catheterization laboratory. A proximal left ...
Preuss Christoph - - 2013
Heart failure is a major problem in the patient with congenital heart disease. Normally interpreted as a sequela of surgical interventions or abnormal preoperative loading conditions, there is increasing evidence that congenital heart malformations and abnormal ventricular function can have the same underlying genetic cause. With the changing demographic characteristics ...
Ellesøe Sabrina G - - 2013
Development of dilated cardiomyopathy in patients with congenital complete atrioventricular block with or without pacemaker is well described. We report a case of dilated cardiomyopathy in a child with congenital complete atrioventricular block, long-QT syndrome, and VVI pacemaker. Temporary pacing in the right ventricular outflow tract demonstrated a 63% increase ...
Kazmouz Suhaib - - 2013
Atrial septal defect (ASD) is one of the most common congenital heart defects, accounting for 7%-10% of all congenital heart disease (CHD) in children and 30%-33% of defects diagnosed in adults with CHD. This review highlights the evolution of transcatheter ASD closure, indications, follow-up, outcomes, and complications with a focus ...
Lowry Adam W - - 2013
Expanded surgical options and improved outcomes for children born with structural heart defects have ushered a greater clinical interest in the normal and abnormal development of the coronary circulation. Anatomic variations of the coronary system may impact surgical candidacy or operative technique during neonatal life, while others may impact long-term ...
Schallert Erica K - - 2013
The segmental classification of congenital heart disease, first conceptualized nearly 50 years ago, is now well established. The Van Praagh classification system, in particular, is commonly used throughout North America to facilitate communication between physicians from various specialties who are involved in diagnosing and managing congenital cardiovascular abnormalities. In the ...
Manieri S - - 2013
Congenital left ventricular diverticulum is a rare condition. When found, it is usually accompanied by other intracardiac malformations, so that again further examination is indicated. Furthermore, it is usually associated with thoracoabdominal wall defect, as seen in the spectrum of Cantrell's pentalogy, a congenital anomaly consisting of a lower sternal ...
Park Ji Young - - 2013
Cardiac surgery in neonates with congenital heart disease has progressed dramatically in the past three decades. However, low-birth-weight neonates with congenital heart disease continue to challenge the intellectual and technical skills of healthcare professionals. We present a case of a low-birth-weight neonate with pulmonary atresia and a ventricular septal defect, ...
Papangelou Alexander A Department of Anesthesiology and Critical Care Medicine, The Johns Hopkins University School of Medicine, Baltimore, MD 21287-7840, USA. - - 2013
Hypertonic saline (HS) can treat cerebral edema arising from a number of pathologic conditions. However, physicians are reluctant to use it during the first 24 h after stroke because of experimental evidence that it increases infarct volume when administered early after reperfusion. Here, we determined the effect of HS on ...
Almenar Luis L Unidad de Insuficiencia Cardiaca y Trasplante, Servicio de Cardiología, Hospital Universitario y Politécnico La Fe, Valencia, Spain. Electronic address: - - 2013
In the year 2012, 3 scientific sections-heart failure and transplant, congenital heart disease, and clinical cardiology-are presented together in the same article. The most relevant development in the area of heart failure and transplantation is the 2012 publication of the European guidelines for heart failure. These describe new possibilities for ...
Almenar Luis - - 2013
In the year 2012, 3 scientific sections-heart failure and transplant, congenital heart disease, and clinical cardiology-are presented together in the same article. The most relevant development in the area of heart failure and transplantation is the 2012 publication of the European guidelines for heart failure. These describe new possibilities for ...
Amuchástegui Marcos - - 2013
Isolated left ventricular noncompaction (ILVNC) is a rare cardiomyopathy with a genetic background characterized by numerous prominent trabeculations and deep intertrabecular recesses. It occurs in the absence of any coexisting congenital lesion or hemodynamic abnormality and is rare in the adult population. Heart failure, ventricular arrhythmias, and embolic events are ...
Yuan Shi-Min - - 2013
Kabuki syndrome (KS) is an entity of multiple congenital malformations with mental retardation with undetermined etiology. Congenital heart defects are one of the clinical manifestations of KS with insuffi cient elucidations. Literature of congenital heart defects associated with KS was comprehensively retrieved, collected and reviewed. The clinical features of the ...
Wang Bin - - 2013
Noninvasive injection of pro-angiogenic compounds such as vascular endothelial growth factor (VEGF) has shown promising results in regenerating cardiac microvasculature. However, these results have failed to translate into successful clinical trials in part due to the short half-life of VEGF in circulation. Increasing the dose of VEGF may increase its ...
Mavroudis Constantine - - 2013
Arrhythmia surgery in patients with congenital disease is challenged by the range of anatomic variants, arrhythmia types, and intramyocardial scar location. Experimental and clinical studies have elucidated the mechanisms of arrhythmias for accessory connections, atrial fibrillation, atrial reentry tachycardia, nodal reentry tachycardia, focal or automatic atrial tachycardia, and ventricular tachycardia. ...
Delmo Walter E M - - 2013
It is anticipated that as many as 10-20% of patients alive with anatomical congenital heart lesions may eventually develop heart failure. Most of these patients have undergone previous palliative or corrective surgeries. The Fontan procedure, although it has helped many patients with single-ventricle physiology to become hemodynamically functional, it is associated ...
Köhler Doreen D Department of Pediatric Cardiology, University of Heidelberg , Heidelberg , - - 2013
Right ventricular dysfunction represents a common problem in patients with congenital heart defects, such as Tetralogy of Fallot or pulmonary arterial hypertension. Patients with congenital heart defects may present with a pressure or volume overloaded right ventricle (RV) in a bi-ventricular heart or in a single ventricular circulation in which ...
Bordachar Pierre - - 2012
The incidence of congenital atrioventricular (AV) block is between 1 in 15000 and 1 in 20000 births. It may occur in isolation or as a consequence of anomalous development of the conduction tissue in the context of a cardiac malformation. In this review, we use the term "congenital AV bock" ...
Dinardo James A - - 2012
The population of adults with congenital heart disease (ACHD) now exceeds that of children with congenital heart disease. Within this relatively young population of adults in their third decade of life exist a population of patients with heart failure (HF) who, in functional status, closely resemble patients in their fifth ...
Spicer Diane E - - 2012
The ventricular mass extends from the atrioventricular to the ventriculo-arterial junctions. The junctions are obvious anatomic entities and provide discrete boundaries for the ventricles, which can then be subdivided into inlet, apical, and outlet components. The apical trabecular components are most constantly present when hearts are congenitally malformed. Abnormal ventricles, ...
Kolo P M - - 2012
This study aimed at determining the frequencies and pattern of congenital heart lesions among patients who had echocardiographic scan for various cardiovascular-related complaints at the University of Ilorin Teaching Hospital, Nigeria. All patients referred for echocardiography between May 2004 and April 2007 for various cardiovascular complaints were reviewed. Patients diagnosed ...
Krieger Eric V - - 2012
Ebstein anomaly (EA) is a rare congenital heart defect that may not be detected until late in adolescence or adulthood. Since the original description in a 19-year-old laborer with severe tricuspid valve (TV) regurgitation in 1866, our understanding of this rare condition has increased to the recognition that it is ...
Thomas Eapen - - 2012
Aortico-left ventricular tunnel is a rare congenital heart defect. Reports on successful device closure of these defects are scarce. We are reporting the first case of device closure of aortico-left ventricular tunnel using an Amplatzer vascular plug III.
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