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tetralogy of fallot adult not repair

Richard J Dobson, Ify Mordi, Mark H Danton, Niki L Walker, Hamish A Walker, Nikolaos Tzemos
OBJECTIVE: Myocardial fibrosis has been associated with poorer outcomes in tetralogy of Fallot, however only a handful of studies have assessed its significance in the current era. Our aim was to quantify the amount of late gadolinium enhancement in both the LV and RV in a contemporary cohort of adults with surgically repaired tetralogy of Fallot, and assess the relationship with adverse clinical outcomes. DESIGN: Single centre cohort study SETTING: National tertiary referral center Patients: One hundred fourteen patients with surgically repaired tetralogy of Fallot with median age 29...
August 24, 2016: Congenital Heart Disease
Jimmy C Lu, Sunkyung Yu, Ray Lowery, Janaki Sagi, Amanda C Delong, Prachi P Agarwal, Maryam Ghadimi Mahani, Adam L Dorfman
Left ventricular (LV) ejection fraction (EF) and right ventricular (RV) ejection fraction by cardiovascular magnetic resonance (CMR) are associated with functional health status in patients with repaired tetralogy of Fallot (TOF) in cross-sectional studies, but few longitudinal data are available. This study aimed to determine predictors of subsequent decrease in functional health status in midterm follow-up. Patients with repaired TOF who had previously completed CMR and assessment with the Short Form 36 version 2 (SF-36) were recruited for repeat CMR, SF-36, and exercise test, if they had not had interval pulmonary valve replacement (PVR)...
October 2016: Pediatric Cardiology
Luís Almeida-Morais, Tiago Pereira-da-Silva, Luísa Branco, Ana T Timóteo, Ana Agapito, Lídia de Sousa, José A Oliveira, Boban Thomas, Nuno Jalles-Tavares, Rui Soares, Ana Galrinho, Rui Cruz-Ferreira
OBJECTIVE: The role of right ventricular longitudinal strain for assessing patients with repaired tetralogy of Fallot is not fully understood. In this study, we aimed to evaluate its relation with other structural and functional parameters in these patients. METHODS: Patients followed-up in a grown-up CHD unit, assessed by transthoracic echocardiography, cardiac MRI, and treadmill exercise testing, were retrospectively evaluated. Right ventricular size and function and pulmonary regurgitation severity were assessed by echocardiography and MRI...
May 26, 2016: Cardiology in the Young
Vishva A Wijesekera, Rekha Raju, Bruce Precious, Adam J Berger, Marla C Kiess, Jonathon A Leipsic, Jasmine Grewal
BACKGROUND: The natural history of right ventricular (RV) and left ventricular (LV) size and function among adults with tetralogy of Fallot (TOF) repair and hemodynamically significant pulmonary regurgitation (PR) is not known. The main aim of this study was to determine changes in RV and LV size and function over time in an adult population with TOF repair and hemodynamically significant pulmonary regurgitation. METHODS: Forty patients with repaired TOF and hemodynamically significant PR were included...
May 26, 2016: Congenital Heart Disease
Munetaka Masuda
Postoperative residua and sequelae after repair of tetralogy of Fallot in adults became one of the major concerns in the field of adult congenital heart disease. Right heart failure and arrhythmia can result fatal, and are considered to be related dilated right ventricle due to pulmonary valve regurgitation. Although precise indication of pulmonary valve replacement (PVR) is still not established, right ventricular end-diastolic volume index less than 150-170 ml/m(2) and right ventricular end systolic volume index less than 82-90 ml/m(2) may be border line for RV remodeling after PVR...
July 2016: General Thoracic and Cardiovascular Surgery
Nicola Pradegan, Vladimiro L Vida, Tal Geva, Giovanni Stellin, Matthew T White, Stephen P Sanders, Robert F Padera
Survival of patients after repair of tetralogy of Fallot (TOF) is worse than for the general population. We aimed to assess the time-related effects of surgical repair on right (RV) and left ventricle (LV) myocardium by quantifying hypertrophy and fibrosis. Cardiomyocyte transverse diameter and percent of fibrosis were measured in 8 adult heart specimens with late-repaired TOF, 6 with unrepaired TOF, and 11 normal hearts (controls). The RV and LV mean and median cardiomyocyte diameter and percent of fibrosis were significantly greater than controls in both repaired and unrepaired hearts...
May 2016: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
Mechthild Westhoff-Bleck, Finn Kornau, Arash Haghikia, Alexander Horke, Harald Bertram, Jens Treptau, Julian Widder, Johann Bauersachs, Michael-Ulrich Brehm
BACKGROUND: The goal of this study was to interrelate N-terminal B-type natriuretic peptide (NT-proBNP) levels and cardiac magnetic resonance imaging-derived ventricular function, mass, and volumes in adults with pulmonary regurgitation after Fallot repair and to evaluate the prognostic relevance of these parameters regarding adverse clinical outcome. METHODS: Eighty-one patients (aged 26.3 ± 7.4 years; male sex, 45.7%; New York Heart Association class I, 72.8%; pulmonary valve velocity, < 3 m/s) were included...
December 1, 2015: Canadian Journal of Cardiology
Michael Zdradzinski, Rachel Elkin, Scott Flamm, Richard Krasuski
BACKGROUND AND AIM OF THE STUDY: Cardiac magnetic resonance (CMR) is the 'gold standard' for quantifying pulmonic regurgitation (PR) in adults with congenital heart disease, but remains costly and is less readily available than echocardiography. Qualitative echocardiographic assessment of PR is challenging, and guiding criteria are limited. It is unknown if echocardiography is sufficient to screen for significant PR. The study aim was to determine whether cardiac MRI provides additional benefit in the assessment of PR in adults with congenital heart disease...
July 2015: Journal of Heart Valve Disease
Mustafa Kurkluoglu, Anitha S John, Russell Cross, David Chung, Can Yerebakan, David Zurakowski, Richard A Jonas, Pranava Sinha
Indications for prophylactic tricuspid annuloplasty in patients with pulmonary regurgitation (PR) after tetralogy of Fallot (TOF) repair are unclear and often extrapolated from acquired functional tricuspid regurgitation (TR) data in adults, where despite correction of primary left heart pathology, progressive tricuspid annular dilation is noted beyond a threshold diameter >4 cm (21 mm/m(2)). We hypothesized that unlike in adult functional TR, in pure volume-overload conditions such as patients with PR after TOF, the tricuspid valve size is likely to regress after pulmonary valve replacement (PVR)...
2015: Seminars in Thoracic and Cardiovascular Surgery
Michael R Joynt, Sunkyung Yu, Adam L Dorfman, Maryam Ghadimi Mahani, Prachi P Agarwal, Jimmy C Lu
Patients with repaired pulmonary stenosis (PS) or tetralogy of Fallot (TOF) both develop pulmonary regurgitation (PR) leading to right ventricular (RV) dilatation and dysfunction. We aimed to characterize differential effects of chronic PR in these populations. Patients with surgically repaired PS were matched 1:2 by age and PR fraction with patients with TOF. Patients with previous pulmonary valve replacement were excluded. Cardiovascular magnetic resonance data were compared; peak longitudinal and circumferential systolic strain by feature tracking were compared to evaluate differential contribution of the RV sinus and outflow tract, respectively...
January 15, 2016: American Journal of Cardiology
Jae Gun Kwak, Cheul Lee, Mina Lee, Chang-Ha Lee, So-Ick Jang, Sang Yun Lee, Su-Jin Park, Mi Kyoung Song, Seong-Ho Kim
OBJECTIVES: In a previous study, we identified factors affecting the durability of bioprosthetic valves in the pulmonary position following total repair of Tetralogy of Fallot (TOF). In this study, we aimed to identify factors affecting the durability of the bioprosthetic valve with regard to patient age and implanted valve size in order to guide valve choice in adolescent patients. METHODS: We enrolled and analysed 108 cases of pulmonary valve replacement (PVR) with stented bioprosthetic valves in TOF patients between January 1998 and February 2014...
April 2016: European Journal of Cardio-thoracic Surgery
Rachel S Beaty, Brady S Moffett, Stuart Hall, Jeffrey Kim
Cardiac arrhythmias occurring during the intraoperative period for cardiac surgery have been associated with excess morbidity and mortality. Several antiarrhythmics have been utilized for the management of intraoperative arrhythmias. These antiarrhythmic medications can cause undesirable adverse outcomes in the intensive care setting. The incidence and treatment of adult intraoperative arrhythmias have been studied. In addition, the prevalence, risk factors, and optimal treatment of pediatric postoperative arrhythmias have also been studied...
October 2015: Pediatric Cardiology
Seiji Ito, Doff B McElhinney, Robert Adams, Puneet Bhatla, Sohae Chung, Leon Axel
The aim is to compare tricuspid valve (TV) atrioventricular junction (AVJ) annular motion parameters in unrepaired atrial septal defect (ASD) and repaired Tetralogy of Fallot (TOF) by cardiac magnetic resonance (CMR) imaging. We retrospectively reviewed CMR studies performed between November 2007 and November 2013 in patients 16-45 years of age with unrepaired ASD (with or without partial anomalous pulmonary venous return) and with repaired TOF, who had previous infundibulotomy, but have not undergone pulmonary valve replacement...
August 2015: Pediatric Cardiology
Jouke P Bokma, Michiel M Winter, Thomas Oosterhof, Hubert W Vliegen, Arie P van Dijk, Mark G Hazekamp, Dave R Koolbergen, Maarten Groenink, Barbara J M Mulder, Berto J Bouma
OBJECTIVE: Patients with surgically repaired tetralogy of Fallot (rTOF) may develop functional tricuspid regurgitation (TR) due to annulus dilation. Guidelines suggest pulmonary valve replacement (PVR) in patients with rTOF with progressive TR, but data on clinical outcomes are lacking. Our objective was to determine whether TR was predictive for adverse events after PVR. METHODS: In this retrospective, multicenter cohort study, patients with rTOF who had undergone PVR after preoperative echocardiographic assessment of TR grade were included...
May 15, 2015: Heart: Official Journal of the British Cardiac Society
Petra Opić, Elisabeth M W J Utens, Judith A A E Cuypers, Maarten Witsenburg, Annemien van den Bosch, Ron van Domburg, Ad J J C Bogers, Eric Boersma, Antonio Pelliccia, Jolien W Roos-Hesselink
BACKGROUND: It is unclear whether sports participation in adults with repaired congenital heart disease is safe and has benefits. METHODS: Congenital heart disease (ConHD) patients who underwent corrective surgery for Atrial Septal Defect, Ventricular Septal Defect, Pulmonary Stenosis, Tetralogy of Fallot or Transposition of the Great Arteries in our center between 1968 and 1980 were included, and participated in our longitudinal follow-up study with serial evaluations in 2001 and 2011...
2015: International Journal of Cardiology
Makoto Mori, Ragavan Siddharthan, Brian Kogon
We describe the case of a 22-year-old with tetralogy of Fallot, who underwent complete repair with ventricular septal defect closure and right ventricle to pulmonary artery conduit placement. She has undergone numerous subsequent conduit changes, each complicated by early stenosis and failure. Unfortunately, serial conduit changes can become increasingly challenging. While extra-anatomic conduits have been described for complex left ventricular outflow tract obstruction, they have not been described for right-sided obstruction...
March 2015: Annals of Thoracic Surgery
Thomas J Moon, Nadine Choueiter, Tal Geva, Anne Marie Valente, Kimberlee Gauvreau, David M Harrild
Ventricular strain and dyssynchrony can be measured in patients with repaired tetralogy of Fallot (TOF), but their effect on clinical outcomes is poorly understood. The purpose of this study was to investigate if ventricular strain and dyssynchrony measured by cardiac magnetic resonance feature tracking are associated with death and sustained ventricular tachycardia. Patients with TOF who died or had ventricular tachycardia (TOF case, n = 16) were compared with age-matched patients with TOF with no adverse outcome (TOF control, n = 32)...
March 1, 2015: American Journal of Cardiology
Daniel Jeong, Petros V Anagnostopoulos, Alejandro Roldan-Alzate, Shardha Srinivasan, Mark L Schiebler, Oliver Wieben, Christopher J François
OBJECTIVE: Ventricular kinetic energy measurements may provide a novel imaging biomarker of declining ventricular efficiency in patients with repaired tetralogy of Fallot. Our purpose was to assess differences in ventricular kinetic energy with 4-dimensional flow magnetic resonance imaging between patients with repaired tetralogy of Fallot and healthy volunteers. METHODS: Cardiac magnetic resonance, including 4-dimensional flow magnetic resonance imaging, was performed at rest in 10 subjects with repaired tetralogy of Fallot and 9 healthy volunteers using clinical 1...
May 2015: Journal of Thoracic and Cardiovascular Surgery
Jonathan W Cramer, Salil Ginde, Garick D Hill, Scott B Cohen, Peter J Bartz, James S Tweddell, Michael G Earing
BACKGROUND: Chronic pulmonary regurgitation after tetralogy of Fallot repair often leads to progressive right ventricle dilation, dysfunction, and frequently, pulmonary valve replacement. For those with significant tricuspid regurgitation at the time of pulmonary valve replacement, it is unknown whether concomitant tricuspid valve repair improves postoperative outcomes. METHODS: This is a retrospective review of patients after tetralogy of Fallot repair who underwent pulmonary valve replacement between 1999 and 2012...
March 2015: Annals of Thoracic Surgery
Virginie Lambert, Elodie Gouadon, André Capderou, Emmanuel Le Bret, Mohamed Ly, Sylvie Dinanian, Jean-Francois Renaud, Michel Pucéat, Catherine Rücker-Martin
OBJECTIVE: Despite the increasing incidence of right ventricular (RV) failure in adult patients with congenital heart disease, current therapeutic options are still limited. By contrast to left-heart diseases, cell-based myocardial regeneration applied to the right ventricle is poorly studied, even though it may be a therapeutic solution. As human embryonic stem cell-derived cardiac progenitors seem to be good candidates owing to their proliferation capacity, our aim was to assess, in a large animal model of overloaded RV dysfunction, the feasibility and effects of such a cell therapy...
March 2015: Journal of Thoracic and Cardiovascular Surgery
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