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Adult congenital heart disease, structural heart disease

Manavi Tyagi, Theodora Fteropoulli, Catherine S Hurt, Shashivadan P Hirani, Lorna Rixon, Anna Davies, Nathalie Picaut, Fiona Kennedy, John Deanfield, Shay Cullen, Stanton P Newman
OBJECTIVE: We carried out a cross-sectional study to assess cognitive function in a sample of adult CHD patients, within the Functioning in Adult Congenital Heart Disease study London. The association between cognitive functioning and disease complexity was examined. METHODS: A total of 310 patients participated in this study. Patients were classified into four structural complexity groups - tetralogy of Fallot, transposition of the great arteries, single ventricle, and simple conditions...
October 18, 2016: Cardiology in the Young
Patricia E Thomas, Scott L Macicek
BACKGROUND: Catheter ablation has been used to manage supraventricular arrhythmia in children since 1990. This article reviews the history of catheter ablation used to treat arrhythmia in children and discusses new frontiers in the field. We also address ablation in adult patients with a history of congenital heart disease (CHD) that was diagnosed and initially treated in childhood. METHODS: We conducted an evidence-based literature review to gather available data on ablation for supraventricular tachycardia in children and adult patients with CHD...
2016: Ochsner Journal
Leda Klouda, Wayne J Franklin, Anita Saraf, Dhaval R Parekh, David D Schwartz
OBJECTIVE: Congenital heart disease (CHD) can affect the developing central nervous system, resulting in neurocognitive and behavioral deficits. Preoperative neurological abnormalities as well as sequelae of the open heart operations required to correct structural abnormalities of the heart contribute to these deficits. There are few studies examining the neurocognitive functioning of adults with CHD. This study sought to investigate multiple domains of neurocognitive functioning in adult survivors of CHD who had childhood cardiac surgery with either moderate or severe disease complexity...
September 21, 2016: Congenital Heart Disease
Reena Khantwal Joshi, Neeraj Aggarwal, Mridul Agarwal, Veronique Dinand, Raja Joshi
OBJECTIVE: To delineate risk factors for failure of extubation in the operating room among pediatric cardiac surgery patients. DESIGN: Prospective, observational study. SETTING: Single center, tertiary care, teaching hospital. PARTICIPANTS: The study comprised 448 congenital cardiac surgery patients who were enrolled for intended extubation in the pediatric cardiac operating room over 5 years. INTERVENTIONS: The airways of enrolled patients were extubated in the operating room if predetermined suitability criteria were met...
June 16, 2016: Journal of Cardiothoracic and Vascular Anesthesia
Mohammed Ebrahim, Sanjeet Hegde, Beth Printz, Mark Abcede, James A Proudfoot, Christopher Davis
Noninvasive measurement of cardiac output (CO) and particularly stroke volume (SV) remain difficult but potentially valuable. These variables can be particularly challenging to measure in children with congenital heart disease (CHD). Impedance cardiography (IC) is a technique shown to be accurate in measuring SV in adults and in children with structurally normal hearts. The ease of use and rapidity of SV measurement using IC makes it potentially attractive for young patients with CHD. Advances in IC technology have led to more sophisticated signal-morphology IC (SMIC) devices that may further improve accuracy...
August 25, 2016: Pediatric Cardiology
Steven Promislow, Joseph G Abunassar, Behnam Banihashemi, Benjamin J Chow, Girish Dwivedi, Kasra Maftoon, Ian G Burwash
BACKGROUND: Many free-form-text referral requisitions for transthoracic echocardiography (TTE) provide insufficient information to adequately evaluate their adherence to Appropriate Use Criteria (AUC). We developed a structured referral requisition algorithm based on requisition deficiencies identified retrospectively in a derivation cohort of 1303 TTE referrals and evaluated the performance of the algorithm in a consecutive series of cardiology outpatient referrals. METHODS: The validation cohort comprised 286 consecutive TTE outpatient cardiology referrals over a 2-week period...
2016: Cardiovascular Ultrasound
Yi-Meng Zhou, Xiao-Yong Dai, Ri-Tai Huang, Song Xue, Ying-Jia Xu, Xing-Biao Qiu, Yi-Qing Yang
Dilated cardiomyopathy (DCM) is the most prevalent form of primary cardiomyopathy in humans and is a leading cause of heart failure and sudden cardiac death. Genetic abnormalities have been demonstrated to be a major contributor to the development of DCM. However, DCM is a genetically heterogeneous disease, and the genetic basis underlying DCM in a significant proportion of patients remains unclear. In the current study, the coding exons and splicing junction sites of the T‑Box 20 (TBX20) gene, which encodes a T‑box transcription factor essential for cardiac morphogenesis and structural remodeling, were sequenced in 115 unrelated patients with idiopathic DCM, and a novel heterozygous mutation, p...
October 2016: Molecular Medicine Reports
Kristina N Mayer, Beatrice Latal, Walter Knirsch, Ianina Scheer, Michael von Rhein, Bettina Reich, Jürgen Bauer, Kerstin Gummel, Neil Roberts, Ruth O'Gorman Tuura
INTRODUCTION: The accurate and precise measurement of brain volumes in young children is important for early identification of children with reduced brain volumes and an increased risk for neurodevelopmental impairment. Brain volumes can be measured from cerebral MRI (cMRI), but most neuroimaging tools used for cerebral segmentation and volumetry were developed for use in adults and have not been validated in infants or young children. Here, we investigate the feasibility and accuracy of three automated software methods (i...
September 2016: Neuroradiology
Mechthild Westhoff-Bleck, Juliane Briest, Daniela Fraccarollo, Denise Hilfiker-Kleiner, Lotta Winter, Ulrike Maske, Markus A Busch, Stefan Bleich, Johann Bauersachs, Kai G Kahl
OBJECTIVE: In adult congenital heart disease (ACHD), mental health status and quality of life become important issues due to improved life expectancy. Current literature provides conflicting data regarding mental health status in ACHD. Furthermore, none of the studies so far compared prevalence rates with a matched control group. METHODS: The prevalence of mental disorders was assessed in 150 ACHD using a structured interview, and compared to 12-months estimates of the general German population...
November 1, 2016: Journal of Affective Disorders
Daniel R Brown, Leigh Ann Samsa, Li Qian, Jiandong Liu
Animal models of cardiovascular disease are key players in the translational medicine pipeline used to define the conserved genetic and molecular basis of disease. Congenital heart diseases (CHDs) are the most common type of human birth defect and feature structural abnormalities that arise during cardiac development and maturation. The zebrafish, Danio rerio, is a valuable vertebrate model organism, offering advantages over traditional mammalian models. These advantages include the rapid, stereotyped and external development of transparent embryos produced in large numbers from inexpensively housed adults, vast capacity for genetic manipulation, and amenability to high-throughput screening...
June 2016: Journal of Cardiovascular Development and Disease
Anne Lee Solevåg, Georg M Schmölzer, Po-Yin Cheung
Background Infant and neonatal myocardial infarction (MI) has been described in association with congenital heart disease, coronary artery abnormalities, myocarditis, and tumors. MI in the perinatal period in a structurally normal heart and with ventricular arrhythmia as a presenting feature has not been thoroughly described. Published case reports describe treatment methods extrapolated from adult MI. However, due to the rare occurrence, the most appropriate acute treatment for both MI and ventricular arrhythmia in newborn infants remains unknown...
April 2016: American Journal of Perinatology Reports
Andrew S Mackie, Gwen R Rempel, Adrienne H Kovacs, Miriam Kaufman, Kathryn N Rankin, Ahlexxi Jelen, Cedric Manlhiot, Samantha J Anthony, Joyce Magill-Evans, David Nicholas, Renee Sananes, Erwin Oechslin, Dimi Dragieva, Sonila Mustafa, Elina Williams, Michelle Schuh, Brian W McCrindle
BACKGROUND: The population of adolescents and young adults with congenital heart disease (CHD) is growing exponentially. These survivors are at risk of late cardiac complications and require lifelong cardiology care. However, there is a paucity of data on how to prepare adolescents to assume responsibility for their health and function within the adult health care system. Evidence-based transition strategies are required. METHODS: The Congenital Heart Adolescents Participating in Transition Evaluation Research (CHAPTER 2) Study is a two-site cluster randomized clinical trial designed to evaluate the efficacy of a nurse-led transition intervention for 16-17 year olds with moderate or complex CHD...
2016: BMC Cardiovascular Disorders
Josue Chery, Joshua Wong, Shan Huang, Shuyun Wang, Ming-Sing Si
Hypoplastic left heart syndrome (HLHS), the most severe and common form of single ventricle congenital heart lesions, is characterized by hypoplasia of the mitral valve, left ventricle (LV), and all LV outflow structures. While advances in surgical technique and medical management have allowed survival into adulthood, HLHS patients have severe morbidities, decreased quality of life, and a shortened lifespan. The single right ventricle (RV) is especially prone to early failure because of its vulnerability to chronic pressure overload, a mode of failure distinct from ischemic cardiomyopathy encountered in acquired heart disease...
June 28, 2016: Tissue Engineering. Part B, Reviews
Stephen A Gaeta, Cary Ward, Richard A Krasuski
Advancement in correction or palliation of congenital cardiac lesions has greatly improved the lifespan of congenital heart disease patients, resulting in a rapidly growing adult congenital heart disease (ACHD) population. As this group has increased in number and age, emerging science has highlighted the systemic nature of ACHD. Providers caring for these patients are tasked with long-term management of multiple neurologic, pulmonary, hepatic, renal, and endocrine manifestations that arise as syndromic associations with congenital heart defects or as sequelae of primary structural or hemodynamic abnormalities...
October 2016: Trends in Cardiovascular Medicine
Jan Janoušek, Peter Kubuš
Cardiac resynchronization therapy (CRT) is an established treatment option for adult patients suffering heart failure due to idiopathic or ischemic cardiomyopathy associated with electromechanical dyssynchrony. There is limited evidence suggesting similar efficacy of CRT in patients with congenital heart disease (CHD). Due to the heterogeneity of structural and functional substrates, CRT implantation techniques are different with a thoracotomy or hybrid approach prevailing. Efficacy of CRT in CHD seems to depend on the anatomy of the systemic ventricle with best results achieved in systemic left ventricular patients upgraded to CRT from conventional pacing...
June 2016: Herzschrittmachertherapie & Elektrophysiologie
Kristina Wasmer, Julia Köbe, Gerhard Diller, Lars Eckardt
Arrhythmia management is one of the main challenges in the treatment of adult patients with congenital heart disease (ACHD). Apart from heart failure, arrhythmias are mainly responsible for morbidity and mortality in these patients. Supraventricular tachycardia is more frequent than ventricular arrhythmias and is not only associated with debilitating symptoms, but is often as threatening as ventricular tachycardia. The incidence depends on the underlying defect, type, and time of repair. For the overall ACHD population the incidence of supraventricular tachycardia is up to 50 % and increases with age and time since surgery...
June 2016: Herzschrittmachertherapie & Elektrophysiologie
Ariane Marelli, Steven P Miller, Bradley Scott Marino, Angela L Jefferson, Jane W Newburger
The number of patients surviving with congenital heart disease (CHD) has soared over the last 3 decades. Adults constitute the fastest-growing segment of the CHD population, now outnumbering children. Research to date on the heart-brain intersection in this population has been focused largely on neurodevelopmental outcomes in childhood and adolescence. Mutations in genes that are highly expressed in heart and brain may cause cerebral dysgenesis. Together with altered cerebral perfusion in utero, these factors are associated with abnormalities of brain structure and brain immaturity in a significant portion of neonates with critical CHD even before they undergo cardiac surgery...
May 17, 2016: Circulation
Fiona Campbell, Katie Biggs, Susie K Aldiss, Philip M O'Neill, Mark Clowes, Janet McDonagh, Alison While, Faith Gibson
BACKGROUND: There is evidence that the process of transition from paediatric (child) to adult health services is often associated with deterioration in the health of adolescents with chronic conditions.Transitional care is the term used to describe services that seek to bridge this care gap. It has been defined as 'the purposeful, planned movement of adolescents and young adults with chronic physical and medical conditions from child-centred to adult-oriented health care systems'. In order to develop appropriate services for adolescents, evidence of what works and what factors act as barriers and facilitators of effective interventions is needed...
2016: Cochrane Database of Systematic Reviews
Kanwal M Farooqi, Omar Saeed, Ali Zaidi, Javier Sanz, James C Nielsen, Daphne T Hsu, Ulrich P Jorde
As the population of adults with congenital heart disease continues to grow, so does the number of these patients with heart failure. Ventricular assist devices are underutilized in adults with congenital heart disease due to their complex anatomic arrangements and physiology. Advanced imaging techniques that may increase the utilization of mechanical circulatory support in this population must be explored. Three-dimensional printing offers individualized structural models that would enable pre-surgical planning of cannula and device placement in adults with congenital cardiac disease and heart failure who are candidates for such therapies...
April 2016: JACC. Heart Failure
Michele D'Alto, Konstantinos Dimopoulos, Werner Budts, Gerhard-Paul Diller, Giovanni Di Salvo, Santo Dellegrottaglie, Pierluigi Festa, Giancarlo Scognamiglio, Gaetano Rea, Lamia Ait Ali, Wei Li, Michael A Gatzoulis
Pulmonary arterial hypertension (PAH) in adult patients with congenital heart disease (CHD) is associated with increased morbidity and mortality. The present review aims to discuss the clinical applications of invasive and non-invasive diagnostic modalities and to describe the strengths and weaknesses of each technique. Chest radiograph is an inexpensive investigation providing information on pulmonary arterial and hilar dilatation, pruning of peripheral pulmonary arteries and cardiomegaly. Transthoracic two-dimensional and Doppler echocardiography is the most widely used imaging tool...
June 15, 2016: Heart: Official Journal of the British Cardiac Society
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