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tetralogy of fallot adult not repair

Muhammad Yasir Qureshi, Robert J Sommer, Allison K Cabalka
Tricuspid valve abnormalities in congenital heart disease comprise a wide spectrum, with the most common being Ebstein anomaly and tricuspid valve dysplasia. Tricuspid valve dysfunction may also be secondary to other types of congenital heart disease, including functional tricuspid regurgitation seen in right heart volume overload conditions, such as atrial septal defect and repaired tetralogy of Fallot with severe pulmonary valve regurgitation. Congenitally corrected transposition and Mustard and Senning procedures maintain the right ventricle as the systemic ventricle, and the tricuspid valve is subject to unique hemodynamic stress not typically seen in normal circulation...
April 2019: JACC. Cardiovascular Imaging
Vinesh Appadurai, Jennifer Thoreau, Theresa Malpas, Mugur Nicolae
BACKGROUND: An improvement in life expectancy in patients suffering from adult congenital heart disease (ACHD) has corresponded with a rise in heart failure incidence within this group. An area that has not been addressed in ACHD heart failure guidelines has been the use of combined inhibition of angiotensin receptor-neprolysin pathways. This case series sought to demonstrate tolerability and 6-month outcome measures of sacubitril/valsartan use in ACHD patients with a severely impaired systemic ventricle...
December 17, 2018: Heart, Lung & Circulation
Alexander C Egbe, William R Miranda, Nandini Mehra, Naser M Ammash, Venkata R Missula, Malini Madhavan, Abhishek J Deshmukh, Mohamed Farouk Abdelsamid, Srikanth Kothapalli, Heidi M Connolly
Background Patients with tetralogy of Fallot ( TOF ) remain at risk for cardiovascular events despite successful repair. Some of the current risk stratification tools require advanced imaging and invasive studies, and hence are difficult to apply to routine patient care. A recent study showed that QRS fragmentation ( QRS -f) is predictive of mortality in patients with TOF. The current study aims to validate this result by assessing whether severity of QRS -f could predict all-cause mortality in a different TOF population...
December 18, 2018: Journal of the American Heart Association
Kenichiro Yamamura, Darren Yuen, Edward J Hickey, Xiaolin He, Rajiv R Chaturvedi, Mark K Friedberg, Lars Grosse-Wortmann, Kate Hanneman, Filio Billia, Michael E Farkouh, Rachel M Wald
OBJECTIVE: The relationship between right ventricular (RV) fibrosis and right heart reverse remodelling following pulmonary valve replacement (PVR) has not been well studied in adults with repaired tetralogy of Fallot (rTOF). Our aims were to histologically quantify RV fibrosis and to explore the relationship between fibrosis severity and cardiac remodelling post-PVR. METHODS: Adults with rTOF and pre-PVR cardiovascular (CMR) imaging were consented to procurement of RV muscle during PVR...
December 4, 2018: Heart
Alexander C Egbe, William R Miranda, Naser M Ammash, Nandan S Anavekar, Venkata R Missula, Srikanth Kothapalli, Arooj R Khan, Sameh M Said, Heidi M Connolly
BACKGROUND: Aortic dilation and aortic valve disease are known long-term complication of tetralogy of Fallot (TOF), but the risk of aortic dissection and the indications for prophylactic aortic surgery are unknown in this population. The purpose of this study was to: (1) determine the prevalence of significant aortic valve disease and/or significant aortic aneurysm (AVD-AA); (2) determine the incidence of progressive aortic dilation and aortic dissection in patients with TOF. METHODS: Retrospective review of adults with repaired TOF, and no prior aortic valve/aorta surgery, who had ≥2 measurements of the thoracic aorta >12 months apart, 1990-2017...
December 4, 2018: Heart
Natalia Dłużniewska, Piotr Podolec, Maciej Skubera, Monika Smaś-Suska, Jacek Pająk, Małgorzata Urbańczyk-Zawadzka, Wojciech Płazak, Maria Olszowska, Lidia Tomkiewicz-Pająk
BACKGROUND: Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease and the population of ToF repair survivors is growing rapidly. Adults with repaired ToF develop late complications. The aim of this study was to describe and analyze long-term follow-up of patients with repaired ToF. METHODS: This is a retrospective cohort study. Consecutive 83 patients with repaired ToF who did not undergo pulmonary valve replacement were included. Mean age of all patients was 30...
October 29, 2018: Cardiovascular Ultrasound
Cristina Cruz, Teresa Pinho, António José Madureira, Cláudia Camila Dias, Isabel Ramos, José Silva Cardoso, Maria Júlia Maciel
INTRODUCTION AND OBJECTIVES: Aortic dilatation can develop late after tetralogy of Fallot repair. Its extension beyond the aortic root is not clearly understood. We aimed to assess the prevalence and predictors of ascending aorta dilatation to set up an imaging protocol. METHODS: In this prospective study including adult patients after tetralogy of Fallot repair followed at a referral center, we assessed the aorta by cardiovascular magnetic resonance and defined ascending aorta dilatation as an observed-to-expected ratio >1...
September 2018: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
Cristina Cruz, Teresa Pinho, Vânia Ribeiro, Cláudia Camila Dias, José Silva Cardoso, Maria Júlia Maciel
INTRODUCTION AND AIMS: Intrinsic aortopathy can lead to dilatation late after tetralogy of Fallot (TOF) repair. Its extent and prevalence are not known. We aimed to assess aortic dimensions and elasticity and to find predictors of aortic dilatation. METHODS: A total of 126 adults were prospectively included after TOF repair and compared to 63 gender- and age-matched controls. Transthoracic echocardiography was used to assess aortic diameters at the level of the sinuses of Valsalva and ascending aorta and aortic dilatation was defined as z-score >+2...
July 2018: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
Julia Probst, Gerhard-Paul Diller, Holger Reinecke, Patrick Leitz, Gerrit Frommeyer, Stefan Orwat, Julia Vormbrock, Robert Radke, Fernando de Torres Alba, Gerrit Kaleschke, Helmut Baumgartner, Lars Eckardt, Kristina Wasmer
BACKGROUND: In patients with repaired Tetralogy of Fallot (ToF), implantable cardioverter defibrillators (ICD) are considered reasonable in selected adults with multiple risk factors for sudden cardiac death. PATIENTS AND METHODS: We performed a retrospective cohort study of all 174 patients with repaired ToF who are followed at the University Hospital of Muenster. We analyzed data according to the risk score previously proposed by Khairy and coworkers and patient outcome...
October 15, 2018: International Journal of Cardiology
Julia Hock, Anna-Luisa Häcker, Barbara Reiner, Renate Oberhoffer, Alfred Hager, Peter Ewert, Jan Müller
OBJECTIVE: Functional outcome measures are of growing importance in the aftercare of patients with congenital heart disease. This study addresses the functional status with regard to exercise capacity, health-related physical fitness (HRPF) and arterial stiffness in a recent cohort of children, adolescents and young adults with tetralogy of Fallot (ToF) after repair. DESIGN: Single-centre, uncontrolled and prospective cohort study. SETTING: Outpatient department of the German Heart Centre Munich; July 2014-January 2018...
February 2019: Archives of Disease in Childhood
Sabrina Lueck, Eike Bormann, Kathrin Rellensmann, Sven Martens, Andreas Rukosujew
BACKGROUND: Many patients with tetralogy of Fallot (TOF) who underwent surgical correction of their congenital cardiac malformation during infancy develop right ventricular dysfunction and exercise intolerance in the long term. The right ventricle (RV) dilates due to the development of severe pulmonary regurgitation (and secondary tricuspid insufficiency). To reduce RV dilation and improve exercise tolerance pulmonary valve replacement (PVR) is the common therapeutic strategy. Whether concomitant tricuspid valve repair (TVR) is beneficial in these pure volume-overload conditions is still unknown...
May 22, 2018: Journal of Cardiovascular Surgery
Ruchika Karnik, Santosh C Uppu, Meghan Tozzi, John Doucette, Irene D Lytrivi, Miwa Geiger, Berthold Klas, Ira A Parness, Rajesh Shenoy, Hari Rajagopal, Shubhika Srivastava
Left ventricular (LV) dysfunction is a risk factor for adverse outcomes in older children and adults with repaired Tetralogy of Fallot (rToF). Pulmonary regurgitation (PR), right ventricular (RV) dilation, and dysfunction have been shown to result in abnormal LV myocardial mechanics and dysfunction. The aim of our study was to evaluate LV rotational mechanics, especially apical rotation in young children with rToF with and without RV dilation. This is a retrospective, single center study in 28 asymptomatic young children with rToF (16 with RV dilation; 12 without RV dilation); 29 age-matched normal controls...
August 2018: Pediatric Cardiology
Wail Alkashkari, Amani Alsubei, Ziyad M Hijazi
PURPOSE OF REVIEW: The past couple of decades have brought tremendous advances to the field of pediatric and adult congenital heart disease (CHD). Percutaneous valve interventions are now a cornerstone of not just the congenital cardiologist treating patients with congenital heart disease, but also-and numerically more importantly-for adult interventional cardiologists treating patients with acquired heart valve disease. Transcatheter pulmonary valve replacement (tPVR) is one of the most exciting recent developments in the treatment of CHD and has evolved to become an attractive alternative to surgery in patients with right ventricular outflow tract (RVOT) dysfunction...
March 15, 2018: Current Cardiology Reports
Adam M Lubert, Jimmy C Lu, Albert P Rocchini, Mark D Norris, Sunkyung Yu, Prachi P Agarwal, Maryam Ghadimi Mahani, Adam L Dorfman
Epicardial fat produces multiple proinflammatory cytokines and is associated with adverse cardiovascular events. Inflammation and resultant endothelial dysfunction may play a role in progressive myocardial dysfunction among adults with single ventricle physiology after Fontan palliation, but the potential impact of increased epicardial fat volume (EFV) has not been studied. This study sought to determine if there is greater EFV in Fontan patients compared with a group of repaired tetralogy of Fallot (rTOF) patients...
April 1, 2018: American Journal of Cardiology
Ahmed Kheiwa, Punag Divanji, Vaikom S Mahadevan
Right ventricular outflow tract (RVOT) dysfunction is a common hemodynamic challenge for adults with congenital heart disease (ACHD), including patients with repaired tetralogy of Fallot (TOF), truncus arteriosus (TA), and those who have undergone the Ross procedure for congenital aortic stenosis and the Rastelli repair for transposition of great vessels. Pulmonary valve replacement (PVR) has become one of the most common procedures performed for ACHD patients. Areas covered: Given the advances in transcatheter technology, we conducted a detailed review of the available studies addressing the indications for PVR, historical background, evolving technology, procedural aspects, and the future direction, with an emphasis on ACHD patients...
March 2018: Expert Review of Cardiovascular Therapy
Neha Bansal, Daisuke Kobayashi, Thomas J Forbes, Wei Du, Jeffrey M Zerin, Aparna Joshi, Daniel R Turner
BACKGROUND: Transcatheter, bilateral branch pulmonary artery (PA) valve implantation is a novel treatment for patients with severe pulmonary insufficiency and oversized right ventricle (RV) outflow tract. There is scarce data on efficacy and safety of this approach. METHODS: This was a retrospective study of 8 patients with repaired tetralogy of fallot (TOF) who underwent bilateral branch PA valve implantation. Demographics, echocardiography, cardiac catheterization, and axial imaging data were reviewed...
April 1, 2018: Catheterization and Cardiovascular Interventions
Marko Novaković, Katja Prokšelj, Uroš Rajkovič, Tjaša Vižintin Cuderman, Katja Janša Trontelj, Zlatko Fras, Borut Jug
INTRODUCTION: Adults with repaired tetralogy of Fallot (ToF) have impaired exercise capacity, vascular and cardiac autonomic function, and quality of life (QoL). Specific effects of high-intensity interval or moderate continuous exercise training on these parameters in adults with repaired ToF remain unknown. METHODS AND RESULTS: Thirty adults with repaired ToF were randomized to either high-intensity interval, moderate intensity continuous training (36 sessions, 2-3 times a week) or usual care (no supervised exercise)...
March 15, 2018: International Journal of Cardiology
Luca Oechslin, Roberto Corti, Matthias Greutmann, Oliver Kretschmar, Oliver Gaemperli
OBJECTIVES: The aim of the study was to assess indications, procedural success, complications, echocardiographic, and clinical outcomes of percutaneous pulmonary valve implantation (PPVI) in adult patients with congenital heart disease (CHD). BACKGROUND: PPVI offers a non-surgical treatment option for failing prosthetic conduits in pulmonary position. However, efficacy and clinical outcomes after PPVI are still underreported. METHODS: From January 2008 to March 2016, 25 adult CHD patients with right ventricular outflow tract (RVOT) stenosis and/or pulmonary regurgitation underwent PPVI in our institution...
April 2018: Journal of Interventional Cardiology
Jouke P Bokma, Michiel M Winter, Arie P van Dijk, Hubert W Vliegen, Joost P van Melle, Folkert J Meijboom, Martijn C Post, Jacqueline K Berbee, S Matthijs Boekholdt, Maarten Groenink, Aeilko H Zwinderman, Barbara J M Mulder, Berto J Bouma
BACKGROUND: The effect of angiotensin II receptor blockers on right ventricular (RV) function is still unknown. Angiotensin II receptor blockers are beneficial in patients with acquired left ventricular dysfunction, and recent findings have suggested a favorable effect in symptomatic patients with systemic RV dysfunction. The current study aimed to determine the effect of losartan, an angiotensin II receptor blocker, on subpulmonary RV dysfunction in adults after repaired tetralogy of Fallot...
April 3, 2018: Circulation
Peter L M Kerkhof, B W Yoo, Peter M van de Ven, Neal Handly
Ejection fraction (EF) is applied as a clinically relevant metric to assess both left (LV) and right ventricular (RV) function. EF depends on the interplay between end-systolic volume (ESV) and end-diastolic volume (EDV). The role of the two constitutive components is of particular interest for the follow-up study of Fallot patients at risk for RV volume overload. The volume regulation graph (VRG) relates ESV to EDV and has been advanced as a central tool to describe LV and RV function. The method permits additional analysis of the impact of clinically relevant determinants such as sex and age...
July 2017: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
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