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tetralogy of fallot adult not repair

Mark Dennis, Ben Moore, Irina Kotchetkova, Lynne Pressley, Rachael Cordina, David S Celermajer
OBJECTIVE: Survival of patients with repaired tetralogy of Fallot (rToF) into young adulthood is very good. Concerns exist, however, over long-term morbidity and mortality as these subjects reach middle age. We aimed to assess survival and the prevalence of complications in patients with rToF seen in our Adult Congenital Heart Disease (ACHD) service. METHODS: One hundred and sixty-eight consecutive patients with 'simple rToF', aged over 16 years, followed up at our tertiary-level ACHD service in Sydney, Australia since 2000, were included...
2017: Open Heart
Clare T M Lai, Enders K O Ng, Pak-Cheong Chow, Ava Kwong, Yiu-Fai Cheung
BACKGROUND: Emerging data suggest that heart-related microRNAs (miRs) may serve as circulating biomarkers of myocardial injury. We aimed to determine the circulating profile of miRs in patients with volume-overloaded right ventricles after repair of tetralogy (TOF). MATERIALS AND METHODS: A total of 104 TOF patients and 70 controls were recruited. The study was conducted in two phases: (1) determination of circulating heart-related miRs described in left heart diseases (miR-1, miR-133a, miR-208a, miR-208b and miR423-5p) by quantitative real-time PCR in 49 patients and 30 controls and followed by validation in an independent cohort of 55 patients and 40 controls; (2) expression profiling of serum samples from eight patients and eight controls, followed by validation...
June 29, 2017: European Journal of Clinical Investigation
Maki Kobayashi, Ken Takahashi, Mariko Yamada, Kana Yazaki, Kotoko Matsui, Noboru Tanaka, Sachie Shigemitsu, Katsumi Akimoto, Masahiko Kishiro, Keisuke Nakanishi, Shiori Kawasaki, Masaki Nii, Keiichi Itatani, Toshiaki Shimizu
Assessment of left ventricular (LV) dysfunction is vital in patients with repaired tetralogy of Fallot (rTOF). The early diastolic intraventricular pressure gradient (IVPG) in the LV plays an important role in diastolic function. IVPG is calculated as the intraventricular pressure difference divided by the LV length, which allows to account for differences in LV size and therefore calculate IVPG in children. We aimed to investigate the mechanisms of LV diastolic dysfunction by measuring mid-to-apical IVPG as an indicator of the active suction force sucking blood from the left atrium into the LV...
June 20, 2017: Heart and Vessels
Aleksander Kempny, Konstantinos Dimopoulos, Anselm Uebing, Gerhard-Paul Diller, Ulrich Rosendahl, George Belitsis, Michael A Gatzoulis, Stephen J Wort
BACKGROUND: The number of patients with congenital heart disease (CHD) is increasing worldwide and most of them will require cardiac surgery, once or more, during their lifetime. The total volume of cardiac surgery in CHD patients at a national level and the associated mortality and predictors of death associated with surgery are not known. We aimed to investigate the surgical volume and associated mortality in CHD patients in England. METHODS: Using a national hospital episode statistics database, we identified all CHD patients undergoing cardiac surgery in England between 1997 and 2015...
2017: PloS One
Mikaël Laredo, Robert Frank, Xavier Waintraub, Estelle Gandjbakhch, Laurence Iserin, Sebastien Hascoët, Caroline Himbert, Yves Gallais, Françoise Hidden-Lucet, Guillaume Duthoit
BACKGROUND: Monomorphic ventricular tachycardia (MVT) is common in adults with repaired tetralogy of Fallot (TOF), and is associated with sudden cardiac death. Management of MVT is not defined, and results of catheter ablation (CA) are limited. AIMS: To evaluate long-term outcomes of MVT CA in repaired TOF. METHODS: Thirty-four patients (mean age 32±10.3 years; 59% male) with repaired TOF underwent CA for symptomatic MVT between 1990 and 2012 in our centre; direct-current ablation (DCA) was used in 6%, radiofrequency followed by DCA in 29% and radiofrequency alone in 65%...
March 24, 2017: Archives of Cardiovascular Diseases
Deane Yim, Eugenie Riesenkampff, Pablo Caro-Dominguez, Shi-Joon Yoo, Mike Seed, Lars Grosse-Wortmann
BACKGROUND: Myocardial fibrosis is linked with adverse clinical outcomes in adults after tetralogy of Fallot repair (rTOF). Native T1 times (T1) by cardiac magnetic resonance have been shown to be a surrogate marker of diffuse myocardial fibrosis. The objective was to quantify native T1 in children post-rTOF and to evaluate their relationship with surgical, imaging, and clinical factors. METHODS AND RESULTS: A retrospective cross-sectional study was performed. Midventricular native T1 were obtained in 100 children post-rTOF using a modified look-locker inversion recovery cardiac magnetic resonance sequence and compared with 35 pediatric controls...
March 2017: Circulation. Cardiovascular Imaging
François Roubertie, Pierre-Emmanuel Séguéla, Zakaria Jalal, Xavier Iriart, Xavier Roques, Bernard Kreitmann, Mohammed Al-Yamani, Xavier Pillois, Jean-Benoît Thambo
BACKGROUND: Pulmonary valve replacement (PVR) often is performed in adults with repaired tetralogy of Fallot (TOF). For patients who have tricuspid regurgitation (TR), tricuspid valve (TV) repair associated to PVR is still debated. OBJECTIVE: We sought to evaluate perioperative factors related to TV repair when performed at the time of PVR in patients with repaired TOF. METHODS: We retrospectively reviewed 104 patients with repaired TOF (or its equivalent) who underwent PVR (2002-2014)...
February 10, 2017: Journal of Thoracic and Cardiovascular Surgery
Marko Novaković, Katja Prokšelj, Vito Starc, Borut Jug
PURPOSE: Adults after surgical repair of tetralogy of Fallot (ToF) may have impaired vascular and cardiac autonomic function. Thus, we wanted to assess interrelations between heart rate variability (HRV) and heart rate recovery (HRR), as parameters of cardiac autonomic function, and arterial stiffness, as a parameter of vascular function, in adults with repaired ToF as compared to healthy controls. METHODS: In a case-control study of adults with repaired ToF and healthy age-matched controls we measured: 5-min HRV variability (with time and frequency domain data collected), carotid artery stiffness (through pulse-wave analysis using echo-tracking ultrasound) and post-exercise HRR (cycle ergometer exercise testing)...
March 8, 2017: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
Magdalena A Sajnach-Menke, Sarah C Walpole
Purpose The aim of this study was to determine whether the exercise tolerance test can provide diagnostic and prognostic information regarding children and young adults and help predict outcome. METHODS: A total of 87 patients, aged 7-29 years (median 13, mean 13.4) were selected retrospectively. They underwent exercise test at the Freeman Hospital from December, 2015 to May, 2016. There were two groups of patients - 46 had symptoms such as chest pain, palpitations, syncope, or dyspnoea on exertion and no cardiac diagnosis, and 40 patients had a cardiac diagnosis such as hypertrophic cardiomyopathy, transposition of the great arteries with post-arterial switch operation, aortic stenosis or regurgitation, tetralogy of Fallot, abnormal coronary arteries, Wolff-Parkinson-White syndrome, or supraventricular tachycardia...
September 2017: Cardiology in the Young
Yumi Shiina, Koichiro Niwa
It is challenging to evaluate aortopathy in congenital heart disease using conventional investigations such as brachial-ankle pulse wave velocity (baPWV). Therefore, we evaluated the cardio-ankle vascular index (CAVI), a dimension of the ascending aorta and plasma transforming growth factor-β1 (TGF-β1) level, in order to find novel noninvasive parameters of aortopathy in adults with repaired tetralogy of Fallot (TOF). Prospectively, we enrolled 42 consecutive adults with TOF (28 patients with repaired TOF were not on ARB, 8 patients with repaired TOF on ARB, 6 patients with no repair or only palliative repair) and 20 age-matched healthy controls...
February 2017: Pediatric Cardiology
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...
January 2017: 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...
April 2017: 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...
December 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...
October 2016: 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
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