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Congenital Heart Disease

Madalena Coutinho Cruz, André Viveiros Monteiro, Guilherme Portugal, Sérgio Laranjo, Ana Lousinha, Bruno Valente, Paulo Osório, Pedro Silva Cunha, Lídia de Sousa, José Alberto Oliveira, Ana Agapito, Mário Martins Oliveira, Fátima Pinto, Rui Cruz Ferreira
OBJECTIVE: Sudden cardiac death is common in the adult congenital heart disease (ACHD) population. Knowledge and experience about the use of implantable cardioverter defibrillators (ICD) in ACHD patients is very limited. We aimed to characterize a cohort of patients with ACHD and ICDs. DESIGN: Thirty consecutive ACHD patients submitted to an ICD implantation in a single tertiary center were evaluated. Data on baseline clinical features, heart defect, indication for ICD, type of device, appropriate therapies, ICD-related complication, and mortality during follow-up were collected...
March 19, 2019: Congenital Heart Disease
Jenny Alenius Dahlqvist, Jan Sunnegårdh, Katarina Hanséus, Eva Strömvall Larsson, Anders Nygren, Magnus Dalén, Håkan Berggren, Jens Johansson Ramgren, Urban Wiklund, Annika Rydberg
OBJECTIVE: Fontan surgery is performed in children with univentricular heart defects. Previous data regarding permanent pacemaker implantation frequency and indications in Fontan patients are limited and conflicting. We examined the prevalence of and risk factors for pacemaker treatment in a consecutive national cohort of patients after Fontan surgery in Sweden. METHODS: We retrospectively reviewed all Swedish patients who underwent Fontan surgery from 1982 to 2017 (n = 599)...
March 18, 2019: Congenital Heart Disease
James A Kuo, Tyler Hamby, Maham N Munawar, Eldad Erez, Vincent K H Tam
OBJECTIVE: Various options exist for right ventricular outflow tract (RVOT) reconstruction in congenital heart disease. The Freestyle porcine aortic root may be used but its longevity is not well defined. DESIGN: We performed a retrospective review of all non-Ross RVOT reconstructions using the Freestyle root in our institution. Survival and reintervention, either by surgery, transcatheter valve implantation, balloon valvuloplasty, or bare metal stent placement, were recorded...
March 12, 2019: Congenital Heart Disease
An Van Berendoncks, Roderick Van Grootel, Jackie McGhie, Matthijs van Kranenburg, Myrthe Menting, Judith A A E Cuypers, Ad J J C Bogers, Maarten Witsenburg, Jolien W Roos-Hesselink, Annemien E van den Bosch
AIMS: Reliable evaluation of the severity and consequences of pulmonary regurgitation (PR) in patients with repaired tetralogy of Fallot (TOF) is crucial to timely identify the need for pulmonary valve intervention. We aimed to identify the accuracy of echocardiographic parameters to differentiate between moderate and severe PR, using phase contrast cardiac magnetic resonance imaging (CMR) as gold standard. METHODS AND RESULTS: In this cross-sectional study, 45 TOF patients with both echocardiographic and CMR measurements of PR were enrolled...
March 7, 2019: Congenital Heart Disease
Stephen J Dolgner, Eric V Krieger, Jacob Wilkes, Susan L Bratton, Ravi R Thiagarajan, Cindy S Barrett, Titus Chan
OBJECTIVE: Adult congenital heart disease (ACHD) patients who undergo cardiac surgery are at risk for poor outcomes, including extracorporeal membrane oxygenation support (ECMO) and death. Prior studies have demonstrated risk factors for mortality, but have not fully examined risk factors for ECMO or death without ECMO (DWE). We sought to identify risk factors for ECMO and DWE in adults undergoing congenital heart surgery in tertiary care children's hospitals. DESIGN: All adults (≥18 years) undergoing congenital heart surgery in the Pediatric Health Information System (PHIS) database between 2003 and 2014 were included...
March 5, 2019: Congenital Heart Disease
Darcy N Marckini, Bennett P Samuel, Jessica L Parker, Stephen C Cook
BACKGROUND: Physician burnout has many undesirable consequences, including negative impact on patient care delivery and physician career satisfaction. Electronic health records (EHRs) may exacerbate burnout by increasing physician workload. OBJECTIVE: To determine burnout in adult congenital heart disease (ACHD) specialists by assessing stress associated with EHRs. DESIGN: Electronic survey study of ACHD providers. SETTING: Canada and United States...
March 1, 2019: Congenital Heart Disease
Xing Chen, Wu Zhou, Qinghua Hu, Lingjin Huang
OBJECTIVE: This study explores the role of the Notch3-HES5 signal pathway in monocrotaline-induced pulmonary hypertension (PH) using rat models. METHOD: Sprague Dawley rats (n = 45) were randomly grouped into normal group, control group, and model group. Rats in the model group were used to establish the PH rat model. Four weeks after model establishment, right catheterization was used to measure the mean pulmonary arterial pressure (mPAP) and right ventricular systolic pressure (RVSP) to analyze hemodynamic changes...
February 27, 2019: Congenital Heart Disease
Donald J Hagler, William R Miranda, Brielle J Haggerty, Jason H Anderson, Jonathan N Johnson, Frank Cetta, Sameh M Said, Nathaniel W Taggart
BACKGROUND: Stenosis of the venous connections and conduits is a well-known late complication of the Fontan procedure. Currently, data on the outcomes of percutaneous intervention for the treatment of extra- or intracardiac conduits and lateral tunnel baffles obstruction are limited. In an attempt to better define the nature and severity of the stenosis and the results of catheter interventional management, we reviewed Fontan patients with obstructed extra- or intracardiac conduits and lateral tunnel baffles...
February 25, 2019: Congenital Heart Disease
Varisara Pornprasertchai, Chodchanok Vijarnsorn, Supaluck Kanjanauthai, Paweena Chungsomprasong, Prakul Chanthong, Kritvikrom Durongpisitkul, Jarupim Soongswang
BACKGROUND: The increasing number of patients with Ebstein anomaly (EA) surviving into adulthood implies improvements in the treatments for the complex lesion. We revisited the clinical outcomes of patients with EA to demonstrate their "real world" survival. OBJECTIVES: To identify the survival and predictors of mortality in patients with EA who underwent medical or surgical management in the present era. METHODS: All patients who had EA with atrioventricular concordance between 1994 and 2016 were retrospectively reviewed...
February 21, 2019: Congenital Heart Disease
Yuli Y Kim, Leah A Goldberg, Katherine Awh, Tanmay Bhamare, David Drajpuch, Adi Hirshberg, Sara L Partington, Rachel Rogers, Emily Ruckdeschel, Lynda Tobin, Morgan Venuti, Lisa D Levine
OBJECTIVE: To assess performance of risk stratification schemes in predicting adverse cardiac outcomes in pregnant women with congenital heart disease (CHD) and to compare these schemes to clinical factors alone. DESIGN: Single-center retrospective study. SETTING: Tertiary care academic hospital. PATIENTS: Women ≥18 years with International Classification of Diseases, Ninth Revision, Clinical Modification codes indicating CHD who delivered between 1998 and 2014...
February 6, 2019: Congenital Heart Disease
Fei Zhang, Yifeng Yang, Qin Wu, Wancun Jin, Haisong Bu, Sijie Wu, Tianli Zhao, Shijun Hu
OBJECTIVE: To evaluate safety and effectiveness of intraoperative device closure for secundum atrial septal defect (ASD) not referred to percutaneous closure. DESIGN AND PATIENTS: From April 2010 to December 2018, 231 secundum ASD children (≤14 years) directly recommended to surgical repair were enrolled in this study. These patients were divided into two groups according to the parents' choice based on surgeons' recommendation. Follow-up evaluations were adopted at 2 weeks, 3 months, 6 months, and 12 months after the procedure and yearly thereafter...
February 4, 2019: Congenital Heart Disease
Maayke A Sluman, Silke Apers, Judith K Sluiter, Karen Nieuwenhuijsen, Philip Moons, Koen Luyckx, Adrienne H Kovacs, Corina Thomet, Werner Budts, Junko Enomoto, Hsiao-Ling Yang, Jamie L Jackson, Paul Khairy, Stephen C Cook, Raghavan Subramanyan, Luis Alday, Katrine Eriksen, Mikael Dellborg, Malin Berghammer, Eva Mattsson, Andrew S Mackie, Samuel Menahem, Maryanne Caruana, Kathy Gosney, Alexandra Soufi, Susan M Fernandes, Kamila S White, Edward Callus, Shelby Kutty, Berto J Bouma, Barbara J M Mulder
BACKGROUND: Conflicting results have been reported regarding employment status and work ability in adults with congenital heart disease (CHD). Since this is an important determinant for quality of life, we assessed this in a large international adult CHD cohort. METHODS: Data from 4028 adults with CHD (53% women) from 15 different countries were collected by a uniform survey in the cross-sectional APPROACH International Study. Predictors for employment and work limitations were studied using general linear mixed models...
February 4, 2019: Congenital Heart Disease
Amy Sanyahumbi, Andrea Beaton, Danielle Guffey, Mina C Hosseinipour, Melissa Karlsten, Charles G Minard, Daniel J Penny, Craig A Sable, Peter N Kazembe
BACKGROUND: In asymptomatic children, screening echocardiography has been used to attempt to diagnose rheumatic heart disease (RHD) at an early stage (latent RHD). World Heart Federation guidelines have standardized categorization of "definite," "borderline," or no RHD by echo findings. The progression of RHD diagnosed through echo screening is not known. In 2014, we screened 1450 schoolchildren in Malawi. OBJECTIVE: Our objective was to evaluate 2-year RHD evolution among those diagnosed through screening...
January 31, 2019: Congenital Heart Disease
Jeremy M Steele, Tamar J Preminger, Francine G Erenberg, Lu Wang, Katherine Dell, Tarek Alsaied, Kenneth G Zahka
OBJECTIVES: To determine the prevalence, age of onset, and risk factors for overweight and obesity in children with congenital heart disease (CHD). STUDY DESIGN: Children with CHD who were seen at our institution from 1996 to 2017 were studied. Patients were full-time residents of the United States and were receiving all cardiac care at our institution. Patients were categorized by age and CHD diagnosis. The date of last normal weight for age and the date of first recorded weight in the range of overweight and obese were documented...
January 30, 2019: Congenital Heart Disease
Clifford L Cua, Melissa Moore-Clingenpeel, Nazia Husain, Ralf Holzer, John P Cheatham, Janaki Gokhale
BACKGROUND: Increased ventricular end-diastolic pressure (VEDP) is a known risk factor for morbidity and mortality in patients with single right ventricle (RV) physiology. Previous studies have shown mixed results correlating echocardiographic measurements with catheter-derived VEDP in this population. Goal of this study was to evaluate if echocardiographic systolic/diastolic ratio (S/D) correlated with VEDP. METHODS: Patients with single RV physiology who underwent simultaneous echocardiography and catheterization were evaluated...
January 30, 2019: Congenital Heart Disease
Christopher R Broda
Heart failure is an emerging issue with important implications in adult patients with congenital heart disease. Practitioners with expertise in both adult congenital heart disease and heart failure are needed to manage this growing and often complex population. In the United States, the optimal training pathway to enable practitioners to best care for these patients is ill-defined. This article explores possibilities and issues that interested trainees may encounter during their training experience.
January 25, 2019: Congenital Heart Disease
Ginnie Abarbanell, Naomi K Tepper, Sherry L Farr
OBJECTIVE: Women with congenital heart disease (CHD) are at increased risk of pregnancy complications and need information on safe, effective contraceptive methods to avoid unintended pregnancy. This systematic review examines evidence regarding safety of contraceptive use among women with CHD. METHODS: The PubMed database was searched for any peer-reviewed articles published through April 2018 that included safety outcomes associated with reversible contraceptive methods among women with CHD...
January 25, 2019: Congenital Heart Disease
Mahmoud Zaqout, Kristof Vandekerckhove, Nathalie Michels, Laurent Demulier, Thierry Bove, Katrien François, Julie De Backer, Stefaan De Henauw, Daniel De Wolf
OBJECTIVE: To investigate the status of body mass index (BMI) in adult people with congenital heart disease (ACHD). METHODS: Five hundred thirty-nine adults with CHD (53.8% men) were seen in the outpatient clinic from 2013 to 2015 and compared to a reference population (n = 1737). The severity of CHD was categorized as mild, moderate, and severe according to standard guidelines. Patients were categorized based on BMI as underweight (<18.5), overweight (25-30), or obese (>30)...
January 25, 2019: Congenital Heart Disease
Mehmet Salih Bilal, Arda Özyüksel, Mustafa Kemal Avşar, Şener Demiroluk, Osman Küçükosmanoğlu, Yalım Yalçın
OBJECTIVE: Management of the patients with transposition of the great arteries and intact ventricular septum may be challenging beyond the newborn period. Herein, we would like to present our alternative strategy for training the left ventricle in these patients. METHODS: Six patients with transposition of the great arteries and intact ventricular septum were evaluated in our clinic. Two of them were palliated with Glenn procedure and pulmonary banding as a definitive treatment strategy at other centers...
January 25, 2019: Congenital Heart Disease
Thomas G Wilson, Yves d'Udekem, David S Winlaw, Rachael L Cordina, Julian Ayer, Thomas L Gentles, Robert G Weintraub, Leeanne E Grigg, Michael Cheung, Timothy M Cain, Padma Rao, Charlotte Verrall, Karin Du Plessis, Kathryn Rice, Ajay J Iyengar
BACKGROUND: Patients with a Fontan circulation are at risk of renal dysfunction. We analyzed cross-sectional data in pediatric and adult Fontan patients in order to assess the accuracy of commonly used serum creatinine-based methods in estimating glomerular filtration rate (GFR). METHODS: A total of 124 Fontan patients (58 children, 66 adults) were enrolled across three study centers. Measurement of GFR (mGFR) using in vivo 99m Tc-DTPA clearance was performed. Various serum creatinine-based equations were used to calculate estimated GFR (eGFR)...
January 21, 2019: Congenital Heart Disease
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