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Repaired Tetralogy of Fallot

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
Zoel A Quinonez, Laura Downey, Rania K Abbasi, Calvin Kuan, Ritu Asija, Doff B McElhinney, Frank L Hanley, Richard D Mainwaring, Lisa Wise-Faberowski
Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals (TOF/PA/MAPCAs) is a heterogeneous disease with varying degrees of severity, requiring complex anesthetic management. Our institution has adopted the approach of early complete repair with incorporation of all lung segments, extensive lobar and branch segmental pulmonary artery reconstruction, and ventricular septal defect closure. While the surgical management of TOF/PA/MAPCAs has been extensively described and varies depending on the institution, there is a paucity of literature on the anesthetic management for such procedures...
March 2018: World Journal for Pediatric & Congenital Heart Surgery
Manar D Samad, Gregory J Wehner, Mohammad R Arbabshirani, Linyuan Jing, Andrew J Powell, Tal Geva, Christopher M Haggerty, Brandon K Fornwalt
Aims: Previous studies using regression analyses have failed to identify which patients with repaired tetralogy of Fallot (rTOF) are at risk for deterioration in ventricular size and function despite using common clinical and cardiac function parameters as well as cardiac mechanics (strain and dyssynchrony). This study used a machine learning pipeline to comprehensively investigate the predictive value of the baseline variables derived from cardiac magnetic resonance (CMR) imaging and provide models for identifying patients at risk for deterioration...
March 12, 2018: European Heart Journal Cardiovascular Imaging
Michał Orczykowski, Karolina Borowiec, Elzbieta Biernacka, Robert Bodalski, Piotr Urbanek, Pawel Derejko, Katarzyna Kodziszewska, Olgierd Wozniak, Aneta Fronczak, Kamil Marcinkiewicz, Krystyna Guzek, Agnieszka Fil, Grzegorz Warminski, Piotr Hoffman, Maria Bilinska, Lukasz Szumowski
BACKGROUND: After the surgical correction of Tetralogy of Fallot (TOF), surgical scars and natural obstacles form pathways capable of supporting an atrial tachyarrhythmia (AT). Radiofrequency ablation is effective, although the few studies to date on this topic had relatively short follow-up periods. AIM: The aims of the study were to evaluate the acute and long-term effects of RF ablation of AT and examine the characteristics of arrhythmia recurrence. METHODS: Tetralogy of Fallot patients (N 16, age 44...
March 14, 2018: Kardiologia Polska
Hiroo Kinami, Kiyozo Morita, Yoshihiro Ko, Gen Shinohara, Kazuhiro Hashimoto
Neonatal primary repair of tetralogy of Fallot (TOF) with absent pulmonary valve (APV) syndrome is associated with high mortality rates. Our plan involves a staged repair that avoids one-stage intracardiac repair (ICR), with a first palliation that closes the main pulmonary orifice using an expanded polytetrafluoroethylene (ePTFE) patch, pulmonary arterioplication, and an adjustable Blalock-Taussig (BT) shunt. This strategy was used for a neonatal case with TOF/APV syndrome with hypoplastic left ventricle (LV)...
March 7, 2018: Annals of Thoracic and Cardiovascular Surgery
Isabel Kazour, Suraj D Serai, Stavra A Xanthakos, Robert J Fleck
The goal of this study was to assess the ability of quantitative T1 cardiovascular magnetic resonance (CMR) imaging to calculate liver extracellular volume (ECV) in patients with varying degrees of congestive hepatopathy (CH). T1 measurements and ECV calculations were performed retrospectively in three cohorts of patients: normal cardiac function, tetralogy of fallot (TOF) repair and Fontan palliation. All CMR studies included modified look-locker inversion recovery (MOLLI) T1 mapping scans performed pre- and post-injection of a gadolinium-based contrast agent (GBCA)...
March 3, 2018: Abdominal Radiology
Timothy Lee, Aaron J Weiss, Elbert E Williams, Fuad Kiblawi, Joanna Dong, Khanh H Nguyen
OBJECTIVES: Although the median sternotomy has been the traditional approach for congenital heart surgery, young patients and their families often find the midline scar to be cosmetically unappealing. At our center, a right transverse axillary incision has become the standard approach for many congenital cardiac lesions due to its safety, versatility, and unsurpassed aesthetic result. We present our experience with the axillary approach for a diverse array of congenital defects. METHODS: A retrospective review of patients receiving a right transverse axillary incision for congenital cardiac surgery between 2005-2016 was conducted...
February 21, 2018: Seminars in Thoracic and Cardiovascular Surgery
Damien Kenny, Terence Prendiville, Kevin P Walsh, Jonathan McGuinness
Repair of tetralogy of Fallot with atrioventricular septal defect may be associated with hemodynamic challenges in the postoperative period particularly as left atrial hypertension secondary to left atrioventricular valve dysfunction may exacerbate pulmonary regurgitation and augment low cardiac output. We present a case describing hybrid strategies to treat severe left atrioventricular and pulmonary valve regurgitation with modified balloon expandable stent valves to counter low cardiac output secondary to valve dysfunction...
March 2018: Annals of Thoracic Surgery
Natalia Dłużniewska, Piotr Podolec, Tomasz Miszalski-Jamka, Maciej Krupiński, Paweł Banyś, Małgorzata Urbańczyk, Bogdan Suder, Grzegorz Kopeć, Maria Olszowska, Lidia Tomkiewicz-Pająk
OBJECTIVES: Investigate the effects of left and right ventricular function and severity of pulmonary valve regurgitation, quantified by cardiac magnetic resonance (CMR), on exercise tolerance in adult patients who underwent ToF repair at a young age. METHODS: This is a retrospective cohort study of 52 patients after ToF surgery and 33 age- and sex-matched healthy volunteers. CMR and cardiopulmonary exercise testing (CPET) were performed on all patients; CPET was performed on control subjects...
January 2018: Indian Heart Journal
Miyuki Shibata, Keiichi Itatani, Taiyu Hayashi, Takashi Honda, Atsushi Kitagawa, Kagami Miyaji, Minoru Ono
The optimal timing for pulmonary valve replacement after Tetralogy of Fallot (TOF) repair remains controversial. In this study, we estimated the feasibility of using flow energy loss (FEL) to predict right ventricular (RV) deterioration due to pulmonary regurgitation after TOF repair. We examined RV outflow tract (RVOT) flow in nine patients who underwent TOF or double-outlet right ventricle repair in the intervention group (Group I) and compared them with three healthy children in the control group (Group C)...
February 16, 2018: Pediatric Cardiology
Michael P DiLorenzo, Elizabeth Goldmuntz, Susan C Nicolson, Mark A Fogel, Laura Mercer-Rosa
Introduction The right ventricular adaptations early after surgery in infants with tetralogy of Fallot are important to understand the changes that occur later on in life; this physiology has not been fully delineated. We sought to assess early postoperative right ventricular remodelling in patients with tetralogy of Fallot by cardiac MRI. Materials and method Subjects with tetralogy of Fallot under 1 year of age were recruited following complete surgical repair for tetralogy of Fallot. Protocol-based cardiac MRI to assess anatomy, function, and flows was performed before hospital discharge using the feed and sleep technique, an unsedated imaging technique...
February 15, 2018: Cardiology in the Young
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...
January 9, 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
Michael Ma, Richard D Mainwaring, Frank L Hanley
The heterogenous anatomy of Tetralogy of Fallot with major aortopulmonary collateral arteries has engendered a similar degree of diversity in its management and, ultimately, outcome. We summarize our comprehensive treatment paradigm for this lesion evolved over 15 years of experience through 458 patients and the results obtained. An updated analysis of 307 patients treated primarily at our institution is included. A review of recent literature, comparison of management strategies, and discussion of ongoing controversies are provided...
March 2018: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
Anis Cerovac, Dzenita Ljuca, Bedreldin Khodary, Gordana Grgic
Aim: The aim of the paper is to present the risk of pregnancy for mother and her child in a young patient who had a surgery to repair Tetralogy of Fallot (ToF), who gave a birth to her firstborn by having a cesarean section. Case report: 23 years old patient, in 28 weeks of pregnancy was admitted to the clinic due to her medical record. She did not have any subjective complaints. She had two surgeries to repair ToF. After the surgery, she had residual ventricular septal defect (VSD)...
February 2018: Medical Archives
Hyungtae Kim, Si Chan Sung, Kwang Ho Choi, Hyoung Doo Lee, Geena Kim, Hoon Ko, Young Seok Lee
OBJECTIVES: We adopted an operative technique of pulmonary valve (PV) annular enlargement with valve repair in tetralogy of Fallot (TOF) correction to reduce postoperative pulmonary regurgitation (PR) 16 years ago. Here, we have evaluated the long-term results. METHODS: Between April 2000 and August 2005, 43 patients (26 men) with tetralogy of Fallot with pulmonary stenosis underwent PV annular enlargement with valve repair. The median age and body weight at the time of surgery were 14 months and 10...
January 22, 2018: European Journal of Cardio-thoracic Surgery
Amr A Arafat, Elatafy E Elatafy, Sahar Elshedoudy, Mahmoud Zalat, Neamet Abdallah, Ahmed Elmahrouk
BACKGROUND: Right ventricular (RV) volume overload increases morbidity and mortality after tetralogy of Fallot (TOF) repair. Surgical strategies like pulmonary leaflets sparing and tricuspid valve repair at time of primary repair may decrease RV overload. Our objective is to evaluate early and midterm results of pulmonary leaflets sparing with infundibular preservation and tricuspid valve repair in selected TOF patients with moderate pulmonary annular hypoplasia. METHODS: From 2011 to 2016; 46 patients with TOF and moderate pulmonary annular hypoplasia had surgical repair with sparing of the pulmonary valve leaflets...
January 22, 2018: Journal of Cardiothoracic Surgery
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...
January 22, 2018: Catheterization and Cardiovascular Interventions
Takashi Kido, Takayoshi Ueno, Masaki Taira, Hideto Ozawa, Koichi Toda, Toru Kuratani, Yoshiki Sawa
BACKGROUND: This study aimed to identify the clinical predictors of the degree of right ventricular (RV) myocardial fibrosis in patients with repaired tetralogy of Fallot (TOF) with special focus on the RV pressure load.Methods and Results:From April 2004 to March 2017, 30 patients with repaired TOF underwent pulmonary valve replacement and concomitant RV myocardial biopsy. The stroke volume ratio (RV stroke volume/left ventricular stroke volume), RV end-diastolic volume index, and right-to-left ventricular systolic pressure ratio were evaluated with respect to their prognostic value for the degree of RV myocardial fibrosis...
January 19, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
Holly Bauser-Heaton, Alejandro Borquez, Ritu Asija, Lisa Wise-Faberowski, Yulin Zhang, Laura Downey, Stanton B Perry, Andrew Koth, Lynn F Peng, Claudia A Algaze, Frank L Hanley, Doff B McElhinney
BACKGROUND: Our institutional approach to tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) emphasizes unifocalization and augmentation of the reconstructed pulmonary arterial (PA) circulation and complete intracardiac repair in infancy, usually in a single procedure. This approach yields a high rate of complete repair with excellent survival and low right ventricular (RV) pressure. However, little is known about remodeling of the unifocalized and reconstructed pulmonary circulation or about reinterventions on the reconstructed PAs or the RV outflow tract conduit...
December 13, 2017: Journal of Thoracic and Cardiovascular Surgery
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