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Adult congenital heart disease

Olga Trojnarska, Ludwina Szczepaniak-Chicheł, Marcin Gabriel, Agnieszka Bartczak-Rutkowska, Joanna Rupa-Matysek, Andrzej Tykarski, Stefan Grajek
BACKGROUND: Mortality in cyanotic patients with congenital heart diseases (CHD) is high, mainly due to cardiovascular complications. It is known that endothelial dysfunction, increased arterial stiffness, and impaired vascular function have negative influence on cardiovascular prognosis. The aim of the study was to assess parameters of arterial stiffness and vascular dysfunction in cyanotic patients with CHD as well as their potential relation to impaired blood oxygen saturation and polycythemia parameters typical for cyanosis...
October 15, 2016: Journal of Cardiology
Jagdish C Mohan, Vishwas Mohan, Madhu Shukla, Arvind Sethi
Hypoplastic right heart syndrome is a rare cyanotic congenital heart disease with under-development of the right ventricle, tricuspid, and pulmonary valves leading to right-to-left shunting of the blood through inter-atrial septal defect. Perinatal mortality is high with very few patients surviving to adulthood without corrective surgery. This report describes a 26-year-old young woman, who had recurrent abortions and stillbirths and detected to have marked cyanosis with hypoplastic right heart, sub-arterial ventricular septal defect, absent pulmonary valve, non-compaction of the left ventricle, and bicuspid aortic valve with aortic regurgitation...
September 2016: Indian Heart Journal
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
Alessandro Giamberti, Francesca R Pluchinotta, Massimo Chessa, Alessandro Varrica, Raffaele Vitale, Alessandro Frigiola, Carlo Pappone, Marco Ranucci
AIMS: Supraventricular arrhythmias are a major cause of morbidity and mortality in adult patients with congenital heart disease (CHD). Intraoperative ablation offers an alternative for patients who failed ablation procedures or are requiring concomitant surgical intervention. We present our long-term results with the surgical treatment of arrhythmias in adults with CHD (ACHD) undergoing elective cardiac surgery and the clinical predictors for arrhythmia recurrence. METHODS AND RESULTS: Between 2002 and 2013, 80 consecutive patients with CHD, mean age of 39 years, underwent intraoperative ablation with monopolar irrigated radiofrequency during cardiac surgery procedures...
October 12, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
Mostafa Behjati-Ardakani, Mohammad Golshan, Sedigheh Akhavan-Karbasi, Seyed-Masood Hosseini, Mohammad-Amin Behjati-Ardakani, Mohammadtaghi Sarebanhassanabadi
BACKGROUND: Atrial septal defect (ASD) is a common congenital heart disease. OBJECTIVES: The aim of this study was to assess the clinical course of ASD, as well as its frequency of spontaneous closure and regression of diameter. METHODS: In a study conducted from 2000 to 2011, 192 consecutive patients with an isolated ASD were followed up regularly with periodic echocardiographic evaluations according to a standardized protocol. The study was conducted in two series of patients: infants and children and adults...
August 2016: Iranian Journal of Pediatrics
Dipesh K Shah, Salil V Deo, Andrew D Althouse, Jeffery J Teuteberg, Soon J Park, Robert L Kormos, Harold M Burkhart, Victor O Morell
BACKGROUND: Adults with congenital heart disease may present with end-stage heart failure necessitating orthotopic heart transplant (OHT). We sought to review the United Network for Organ Sharing (UNOS) experience with this unique cohort focusing on surgical outcomes and survival. METHODS: From the UNOS registry, 737 adult congenital heart disease recipients (ACHDR) out of 26,993 OHT patients (2.7%) who underwent OHT were studied to analyze early and late outcomes and compared to non-congenital recipients (NCR) over a 15-year period (2000-2014)...
October 5, 2016: Journal of Cardiac Surgery
Zhanguo Sun, Wenjian Xu, Shuran Huang, Yueqin Chen, Xiang Guo, Zhitao Shi
BACKGROUND: Despite dual-source computed tomography (DSCT) technology has been performed well on adults or infants with heart disease, specific knowledge about children with congenital pulmonary valve stenosis (PS) remained to be established. OBJECTIVES: This original research aimed to establish a professional approach of DSCT performing technology on children and to assess the image quality performed by DSCT to establish a diagnostic evaluation for children with PS...
April 2016: Iranian Journal of Radiology: a Quarterly Journal Published By the Iranian Radiological Society
Christophe P Teuwen, Tim I M Korevaar, Rosa L Coolen, Twan van der Wel, Charlotte A Houck, Reinder Evertz, Ameeta Yaksh, Jolien W Roos-Hesselink, Ad J J C Bogers, Natasja M S de Groot
AIMS: Atrial fibrillation (AF) is increasingly observed in patients with congenital heart defects (CHDs) who survive nowadays into adulthood. Yet, predictors of AF are scarce in this high-risk population. This study therefore examined the predictive ability of atrial extrasystole (AES) for development of AF in CHD patients. METHODS AND RESULTS: Adult CHD patients who had a 24 h Holter registration were followed to determine who developed AF. A total of 573 patients (49% male, mean age 35 ± 12 years) were included; they had a simple/complete repaired CHD (n = 279), complex repaired CHD (n = 251), or univentricular heart (UVH, n = 43)...
October 4, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
Michelle Gurvitz, Raluca Ionescu-Ittu, Liming Guo, Mark J Eisenberg, Michal Abrahamowicz, Louise Pilote, Ariane J Marelli
The prevalence rate of cancer among adult patients with congenital heart disease (CHD) in North America has not been previously described. The Quebec adult CHD database was used to determine the prevalence rate of cancer among adult patients with CHD measured as the number of adults with CHD and cancer alive in 2005 per 1,000 adults with CHD. This prevalence rate was compared with the prevalence rate of cancer in the general population of adults in Canada. Types of cancer among the CHD group were described by gender and age...
August 31, 2016: American Journal of Cardiology
Ri-Tai Huang, Song Xue, Juan Wang, Jian-Yun Gu, Jia-Hong Xu, Yan-Jie Li, Ning Li, Xiao-Xiao Yang, Hua Liu, Xiao-Dong Zhang, Xin-Kai Qu, Ying-Jia Xu, Xing-Biao Qiu, Ruo-Gu Li, Yi-Qing Yang
As the most common form of birth defect in humans, congenital heart disease (CHD) is associated with substantial morbidity and mortality in both children and adults. Increasing evidence demonstrates that genetic defects play a pivotal role in the pathogenesis of CHD. However, CHD is of great heterogeneity, and in an overwhelming majority of cases, the genetic determinants underpinning CHD remain elusive. In the present investigation, the coding exons and flanking introns of the CASZ1 gene, which codes for a zinc finger transcription factor essential for the cardiovascular morphogenesis, were sequenced in 172 unrelated patients with CHD...
September 28, 2016: Gene
Jamie L Jackson, Gina M Gerardo, Curt J Daniels, Kathryn Vannatta
BACKGROUND: Disease-related stressors for survivors of congenital heart disease (CHD) have been qualitatively described but not quantified nor examined in relationship to important patient-reported outcomes (PROs). OBJECTIVE: The aims of this study are to (1) identify the types and degree of disease-related stress experienced by CHD survivors based on age, functional status, and sex, (2) examine differences in stress and PROs by age, functional status, and sex, and (3) determine the unique contribution of perceived stress to variability in PROs...
September 28, 2016: Journal of Cardiovascular Nursing
A E While, E Heery, A M Sheehan, I Coyne
BACKGROUND: The numbers of children with long-term illnesses surviving into adulthood and transferring from child to adult services has increased dramatically in the last 30 years. This study aimed to examine health-related quality of life pre- and post-transfer from child to adult healthcare for young people with three long-term illnesses. METHODS: A total of 217 young people with cystic fibrosis, congenital heart defects or diabetes attending child and adult hospital services in Dublin, Ireland completed a questionnaire survey...
September 27, 2016: Child: Care, Health and Development
A M M Strijbosch, R Zwart, N A Blom, B J Bouma, M Groenink, S M Boekholdt, R de Winter, B J M Mulder, A P Backx
INTRODUCTION: Adolescents with congenital heart disease transition from a paediatric to an adult setting. This is associated with loss-to-follow-up and suboptimal care. Increasing numbers of patients justify a special program. In this study we evaluated the cooperative program between paediatric and adult cardiology departments in a tertiary referral centre. METHODS: In this retrospective study, patients with congenital heart disease with at least one appointment scheduled at the transition program between January 2010 and January 2015 were included...
November 2016: Netherlands Heart Journal
Ana Ubeda Tikkanen, Jonathan Rhodes, Micheal Landzberg, Ami Bhatt, David M Systrom, Aaron Waxman, Lilamarie Moko, Robin Bradley, Scott Crouter, Alexander Opotowsky
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Seema Mital, Kiran Musunuru, Vidu Garg, Mark W Russell, David E Lanfear, Rajat M Gupta, Kathleen T Hickey, Michael J Ackerman, Marco V Perez, Dan M Roden, Daniel Woo, Caroline S Fox, Stephanie Ware
Advances in genomics are enhancing our understanding of the genetic basis of cardiovascular diseases, both congenital and acquired, and stroke. These advances include finding genes that cause or increase the risk for childhood and adult-onset diseases, finding genes that influence how patients respond to medications, and the development of genetics-guided therapies for diseases. However, the ability of cardiovascular and stroke clinicians to fully understand and apply this knowledge to the care of their patients has lagged...
September 26, 2016: Circulation. Cardiovascular Genetics
Giovanni Biglino, Claudio Capelli, Despina Koniordou, Di Robertshaw, Lindsay-Kay Leaver, Silvia Schievano, Andrew M Taylor, Jo Wray
BACKGROUND: Nurse education and training are key to providing congenital heart disease (CHD) patients with consistent high standards of care as well as enabling career progression. One approach for improving educational experience is the use of 3D patient-specific models. OBJECTIVES: To gather pilot data to assess the feasibility of using 3D models of CHD during a training course for cardiac nurses; to evaluate the potential of 3D models in this context, from the nurses' perspective; and to identify possible improvements to optimise their use for teaching...
September 26, 2016: Congenital Heart Disease
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
Charlien Gabriels, Julie De Backer, Agnes Pasquet, Bernard P Paelinck, Marielle Morissens, Frederik Helsen, Alexander Van De Bruaene, Werner Budts
OBJECTIVES: Studies evaluating the long-term outcome of adults with ventricular septal defect (VSD) are important to inform patients about prognosis. This study investigated the long-term outcome of patients with perimembranous VSD (pmVSD) followed in the Belgian Registry on Adult Congenital Heart Disease. METHODS: All pmVSD patients in the registry were analyzed. RESULTS: Two hundred and sixty-six patients were studied. Fifteen patients had Eisenmenger syndrome...
September 21, 2016: Cardiology
M J Schuuring, A P Backx, R Zwart, A H Veelenturf, D Robbers-Visser, M Groenink, A Abu-Hanna, N Bruining, M P Schijven, B J Mulder, B J Bouma
OBJECTIVE: Many adults with congenital heart disease (CHD) are affected lifelong by cardiac events, particularly arrhythmias and heart failure. Despite the care provided, the cardiac event rate remains high. Mobile health (mHealth) brings opportunities to enhance daily monitoring and hence timely response in an attempt to improve outcome. However, it is not known if adults with CHD are currently using mHealth and what type of mHealth they may need in the near future. METHODS: Consecutive adult patients with CHD who visited the outpatient clinic at the Academic Medical Center in Amsterdam were asked to fill out questionnaires...
November 2016: Netherlands Heart Journal
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