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Frank W DiPaola, Kurt R Schumacher, Caren S Goldberg, Joshua Friedland-Little, Aishwarya Parameswaran, Jonathan R Dillman
OBJECTIVES: Assess liver stiffness using ultrasound point shear wave elastography (US P-SWE) in children before and after the Fontan operation. METHODS: Eighteen children undergoing the Fontan operation were prospectively enrolled. Eight US P-SWE measurements were obtained from the right hepatic lobe before surgery, and at multiple postoperative time points. Temporally related inferior vena cava pressure (IVC) data was collected from medical records, when available...
October 17, 2016: European Radiology
Thomas A Carins, William Y Shi, Ajay J Iyengar, Ashley Nisbet, Victoria Forsdick, Diana Zannino, Thomas Gentles, Dorothy J Radford, Robert Justo, David S Celermajer, Andrew Bullock, David Winlaw, Gavin Wheaton, Leeanne Grigg, Yves d'Udekem
OBJECTIVES: Patients living with a Fontan circulation are prone to develop arrhythmias. However, their prognostic impact has been seldom studied. As such, we aimed to determine the incidence and predictors of arrhythmias after the Fontan procedure and the long-term outcomes after the first onset of arrhythmias. METHODS: Of the 1034 patients who have undergone a Fontan procedure as recorded in the Australian and New Zealand Fontan Registry, we identified those in whom a tachyarrhythmia or bradyarrhythmia developed...
November 2016: Journal of Thoracic and Cardiovascular Surgery
Stanimir Georgiev, Gunter Balling, Bettina Ruf, Kilian Ackermann, Jelena P von Ohain, Christian Schreiber, Peter Ewert
OBJECTIVES: We aimed to investigate whether early postoperative extubation following the Fontan operation is universally feasible and can be used as a management tool in unstable patients. METHODS: All patients undergoing the Fontan operation in our centre between 2004 and 2013 (n=253) were analysed. Until 2008, patients were extubated according to standard criteria and comprised group 1. Group 2 included all patients presenting after 2009, when early extubation was always aimed regardless of the haemodynamic status...
October 18, 2016: Cardiology in the Young
Anitha Parthiban, Jami C Levine, Meena Nathan, Jennifer A Marshall, Girish S Shirali, Stephen D Simon, Steven D Colan, Jane W Newburger, Geetha Raghuveer
BACKGROUND: Postoperative echocardiography after congenital heart disease surgery is of prognostic importance, but variable image quality is problematic. We implemented a quality improvement bundle comprising of focused imaging protocols, procedural sedation, and sonographer education to improve the rate of optimal imaging (OI). METHODS: Predischarge echocardiograms were evaluated in 116 children (median age, 0.51 years; range, 0.01-5.6 years) from two centers after tetralogy of Fallot repair, arterial switch operation, and bidirectional Glenn and Fontan procedures...
October 11, 2016: Journal of the American Society of Echocardiography
Chaeyoun Oh, Joong Kee Youn, Ji-Won Han, Gi Beom Kim, Hyun-Young Kim, Sung-Eun Jung
INTRODUCTION: The Fontan procedure (FP) has become the standard operation for patients with single ventricle physiology. However, a long period of elevated systemic venous pressure and low cardiac output after the procedure result in chronic inflammation and liver cirrhosis, which may eventually lead to the occurrence of hepatocellular carcinoma (HCC). CLINICAL FINDINGS: We described the case of a 16-year-old female who developed HCC after the FP. At 21 months, the patient received a lateral tunnel FP, and 14 years later, she began complaining of abdominal distension, telangiectasia, and fatigue...
October 2016: Medicine (Baltimore)
Thomas Dejoie, Jill Corre, Helene Caillon, Cyrille Hulin, Aurore Perrot, Denis Caillot, Eileen Boyle, Marie-Lorraine Chretien, Jean Fontan, Karim Belhadj, Sabine Brechignac, Olivier Decaux, Laurent Voillat, Philippe Rodon, Olivier Fitoussi, Carla Araujo, Lotfi Benboubker, Charlotte Fontan, Mourad Tiab, Pascal Godmer, Odile Luycx, Olivier Allangba, Jean-Michel Pignon, Jean-Gabriel Fuzibet, Laurence Legros, Anne-Marie Stoppa, Mamoun Dib, Brigitte Pegourie, Frederique Orsini-Piocelle, Lionel Karlin, Bertrand Arnulf, Murielle Roussel, Laurent Garderet, Mohamad Mohty, Nathalie Meuleman, Chantal Doyen, Pascal Lenain, Margaret Macro, Xavier Leleu, Thierry Facon, Philippe Moreau, Michel Attal, Herve Avet-Loiseau
Guidelines for monitoring multiple myeloma patients expressing light chains only (light chain multiple myeloma; LCMM) rely on measurements of the monoclonal protein in urine. Alternatively serum free light chain (sFLC) measurements have better sensitivity over urine methods, however, demonstration that improved sensitivity provides any clinical benefit is lacking. Here we compared the performance of serum and urine measurements in 113 (72κ, 41λ) newly diagnosed LCMM patients enrolled onto the IFM-2009 trial...
October 11, 2016: Blood
R S Monteiro, D P Dob, M R Cauldwell, M A Gatzoulis
Women with a single ventricle circulation palliated with the Fontan operation require specialist multidisciplinary management. We report 14 such cases with successful pregnancies and detail the pathophysiology encountered. A combined obstetric and cardiac service between Chelsea and Westminster Hospital and Royal Brompton Hospital provides care for women with heart disease, and maintains a prospective database of referred women. We searched this database for women with a known Fontan circulation and reviewed the case notes and electronic patient records between January 1994 and December 2015...
September 7, 2016: International Journal of Obstetric Anesthesia
Malin Berghammer
No abstract text is available yet for this article.
November 2016: Acta Paediatrica
Bui Quoc Thang, Tatsuya Furugaki, Motoo Osaka, Yutaka Watanabe, Shinya Kanemoto, Fuminaga Suetsugu, Yuji Hiramatsu
PURPOSE: There is less certainty regarding the best strategy for treating neonates with functional single ventricle (SV) and hypoplastic aortic arch. We have applied a modified extended aortic arch anastomosis (EAAA) and main pulmonary artery banding (PAB) as an initial palliation in neonates with transverse arch hypoplasia and assessed the mid-term outcomes. METHODS: In total, 10 neonates with functional SV and extensive hypoplasia or interruption of the arch underwent a modified EAAA (extended arch anastomosis with a subclavian flap) concomitant with main PAB through a thoracotomy without cardiopulmonary bypass...
October 11, 2016: Annals of Thoracic and Cardiovascular Surgery
Mineto Kamata, Corey Stiver, Aymen Naguib, Dmitry Tumin, Joseph D Tobias
OBJECTIVES: The objectives of this study were to evaluate the effect of ventricular morphology on perioperative outcomes during Fontan surgery. DESIGN: Retrospective cohort study. SETTING: Single standing, not-for-profit pediatric hospital. PARTICIPANTS: A total of 72 patients who underwent Fontan surgery using cardiopulmonary bypass without aortic cross-clamp between January 1, 2009 and December 31, 2014. INTERVENTIONS: None...
July 19, 2016: Journal of Cardiothoracic and Vascular Anesthesia
Jun-Yen Pan, Chu-Chuan Lin, Jen-Ping Chang
Assessment of the pulmonary circulation status including pressure, resistance, size, and absence of anatomical distortion, is crucial to the successful Fontan operation. Most patients are found to have acceptable pulmonary arteries after previous palliation, although some degree of distortion is not uncommon. However, in rare instances, some patients have only one functioning lung with another pulmonary artery seriously hypoplastic or atretic. For theses patients, completion of a Fontan operation will be challenging...
September 2016: Acta Cardiol Sin
Inês C Mendes, Fernando Maymone-Martins, Rui Anjos
A 30-year-old female with tricuspid valve atresia, ventricular septal defect, and atrial septal defect had a neonatal modified Blalock Taussig shunt and a Fontan-Björk operation performed at five years of age. She did well initially but progressively developed signs of systemic congestion due to severe homograft stenosis and underwent successful percutaneous implantation of a Melody(®) pulmonary valve (Medtronic, Minneapolis, MN, USA) in the "tricuspid" position.
October 4, 2016: Journal of Cardiac Surgery
Yong-Hee Park, Da-Hye Yoo, Eun-Hee Kim, In-Kyung Song, Ji-Hyun Lee, Hee-Soo Kim, Woong-Han Kim, Jin-Tae Kim
This study aimed to investigate the optimal transducer level for accurate measurement of atrial and pulmonary arterial pressures in the supine position for patients with functional single ventricle. Contrast-enhanced chest computed tomographic images of 108 patients who underwent either the bidirectional cavopulmonary shunt (BCPS) placement or the Fontan procedure were reviewed. Vertical distances from the skin of the back to the uppermost levels of fluid in the single atrium or the pulmonary artery confluence and their ratios to the greatest anteroposterior (AP) diameter of the thoracic cage were determined...
October 1, 2016: Pediatric Cardiology
Sharon L Cheatham, Grace M Deyo
Hybrid stage I palliation combines cardiothoracic surgery and interventional transcatheter procedures for treatment of hypoplastic left heart syndrome. The approach is an alternative to the Norwood procedure, the traditional first stage of surgical palliation. Hybrid stage I palliation involves placing bilateral branch pulmonary artery bands and a patent ductus arteriosus stent through a median sternotomy, performed without cardiopulmonary bypass. The purpose of the bands is to control blood flow to the lungs and protect the pulmonary bed while the stent sustains systemic cardiac output...
October 2016: Critical Care Nurse
Johanna Calderon, Christian Stopp, David Wypij, David R DeMaso, Michael Rivkin, Jane W Newburger, David C Bellinger
OBJECTIVES: To investigate the long-term impact of early-term birth (37-38 weeks' gestation) relative to full-term birth (≥39 weeks' gestation) on neurodevelopmental and psychiatric outcomes in adolescents with single-ventricle congenital heart disease (CHD). STUDY DESIGN: This cross-sectional cohort study analyzed retrospective medical records from full term adolescents with single-ventricle CHD who underwent the Fontan procedure. Participants underwent neurodevelopmental and psychiatric evaluations, as well as structural brain magnetic resonance imaging...
September 27, 2016: Journal of Pediatrics
Eva Hlavackova, Martin Liska, Hana Jicinska, Jiri Navratil, Jiri Litzman
The Fontan operation or total cavopulmonal connection (TCPC) is a palliative surgical correction of rare and complex inborn cardiac malformations that are characterized by univentricular circulation. Protein-losing enteropathy (PLE) develops in 4-13% of patients after the Fontan procedure. Fontan-related PLE leads to secondary combined immunodeficiency marked by hypogammaglobulinemia and predominantly CD4+ lymphocytopenia. Here, we present 3 case reports of patients with secondary immunodeficiency after the Fontan operation...
September 30, 2016: International Archives of Allergy and Immunology
Travis J Wilder, Brian W McCrindle, Edward J Hickey, Gerhard Ziemer, Christo I Tchervenkov, Marshall L Jacobs, Peter J Gruber, Eugene H Blackstone, William G Williams, William M DeCampli, Christopher A Caldarone, Christian Pizarro
BACKGROUND: For neonates with critical left ventricular outflow tract obstruction (LVOTO), hybrid procedures are an alternative to the Norwood stage 1 procedure. Despite perceived advantages, however, outcomes are not well defined. Therefore, we compared outcomes after stage 1 hybrid and Norwood procedures. METHODS: In a critical LVOTO inception cohort (2005-2014; 20 institutions), a total of 564 neonates underwent stage 1 palliation with the Norwood operation with a modified Blalock-Taussig shunt (NW-BT; n = 232; 41%), Norwood operation with a right ventricle-to-pulmonary artery conduit (NW-RVPA; n = 222; 39%), or a hybrid procedure (n = 110; 20%)...
August 31, 2016: Journal of Thoracic and Cardiovascular Surgery
Alexander R Opotowsky, Fernando R Baraona, Finnian R Mc Causland, Brittani Loukas, Elizabeth Landzberg, Michael J Landzberg, Venkata Sabbisetti, Sushrut S Waikar
OBJECTIVES: To define whether adults with a Fontan circulation, who have lifelong venous congestion and limited cardiac output, have impaired glomerular filtration rate (GFR) or elevated urinary biomarkers of kidney injury. METHODS: We measured circulating cystatin C and creatinine (n=70) and urinary creatinine, albumin, kidney injury molecule-1 (KIM-1), neutrophil gelatinase-associated lipocalin (NGAL) and N-acetyl glucosaminidase (NAG) (n=59) in ambulatory adult Fontan patients and 20 age-matched and sex-matched controls...
September 26, 2016: Heart: Official Journal of the British Cardiac Society
Bryan H Goldstein, Elaine M Urbina, Philip R Khoury, Zhiqian Gao, Michelle A Amos, Wayne A Mays, Andrew N Redington, Bradley S Marino
BACKGROUND: Fontan survivors demonstrate diminished vascular function and functional outcomes, but the relationships between these measures have not been established. METHODS AND RESULTS: We performed a cross-sectional study of 60 Fontan survivors (52% male) with a mean age of 13.9±4.1 years and mean Fontan duration of 9.9±4.2 years. Multimodality assessment of endothelial function (reactive hyperemia index and flow-mediated dilation) and arterial stiffness (augmentation index and baseline pulse amplitude) was performed with peripheral arterial tonometry and brachial flow-mediated dilation...
2016: Journal of the American Heart Association
Hirofumi Saiki, Benjamin W Eidem, Tomohito Ohtani, Martha A Grogan, Margaret M Redfield
BACKGROUND: In adult Fontan patients, ventricular or arterial dysfunction may impact homeostasis of the Fontan circulation and predispose to heart failure. We sought to characterize ventricular-arterial (VA) properties in adult Fontan patients. METHODS AND RESULTS: Adult Fontan patients (n=170), including those with right (SRV, n=57) and left (SLV, n=113) dominant ventricular morphology, were compared to age, sex, and body size matched controls (n=170). Arterial function, load-insensitive measures of contractility, VA coupling, diastolic function, and ventricular efficiency were assessed...
2016: Journal of the American Heart Association
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