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fontan operation

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
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)
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
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
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
Daiji Takeuchi, Kei Inai, Tokuko Shinohara, Toshio Nakanishi, In-Sam Park
BACKGROUND: Coagulation abnormality is associated with a high incidence of intracardiac thrombus (ICT) and systemic thromboembolism in Fontan patients. The biomarker for detecting ICT is currently unknown. METHODS: We retrospectively investigated the underlying coagulation abnormality and useful biomarkers to screen for ICT in adult Fontan patients. We measured various biomarkers of blood coagulation, fibrinolysis, and platelet activity in 122 Fontan patients (Fontan group: median age [P25-P75]: 27 [20-34] years) and compared them to those in 50 patients with atrial septal defect (ASD group: 31 [24-40] years)...
September 12, 2016: International Journal of Cardiology
Jeremy P Moore, Blandine Mondésert, Michael S Lloyd, Stephen C Cook, Ali N Zaidi, Robert H Pass, Anitha S John, Frank A Fish, Kevin M Shannon, Jamil A Aboulhosn, Paul Khairy
BACKGROUND: Sudden cardiac death is a major contributor to mortality for adults with congenital heart disease. The subcutaneous implantable cardioverter-defibrillator (ICD) has emerged as a novel tool for prevention of sudden cardiac death, but clinical performance data for adults with congenital heart disease are limited. METHODS AND RESULTS: A retrospective study involving 7 centers over a 5-year period beginning in 2011 was performed. Twenty-one patients (median 33...
September 2016: Circulation. Arrhythmia and Electrophysiology
Alexander C Egbe, Heidi M Connolly, Christopher J McLeod, Naser M Ammash, Talha Niaz, Vidhushei Yogeswaran, Joseph T Poterucha, Muhammad Y Qureshi, David J Driscoll
BACKGROUND: There are limited data about the risk of thrombotic and embolic complication (TEC) in adults with atrial arrhythmia after Fontan operation. OBJECTIVES: This study sought to determine the risk of TEC in this population and the role of anticoagulation therapy in TEC prevention. METHODS: This was a retrospective review of adults with atrial arrhythmia after Fontan operation who were evaluated at the Mayo Clinic between 1994 to 2014...
September 20, 2016: Journal of the American College of Cardiology
Yujiro Ide, Masaya Murata, Hiroki Ito, Kisaburo Sakamoto
Right atrial isomerism and pulmonary atresia combined with major aortopulmonary collateral arteries are very rare. Surgical treatment becomes more challenging when an extracardiac total anomalous pulmonary venous connection (TAPVC) requiring surgical repair neonatally is also present. We describe a successful staged Fontan operation for a neonate with a single ventricle, right atrial isomerism, pulmonary atresia with major aortopulmonary collateral arteries, a small central pulmonary artery and a supracardiac TAPVC...
September 13, 2016: Interactive Cardiovascular and Thoracic Surgery
Sudip Dutta Baruah, Smita Mishra, Ashutosh Marwah, Rajesh Sharma
Fontan completion after prior Kawashima repair for single ventricle with interruption of the inferior vena cava can be accomplished by various methods. We describe a patient who underwent the connection of hepatic to hemiazygos vein that we believe would be superior to the conventional cavopulmonary connection in our patient.
September 2016: Annals of Pediatric Cardiology
Sangeetha Viswanathan
This review attempts to answer the common questions faced by a clinician regarding thromboembolism and thromboprophylaxis in patients following Fontan surgery. The review is in an easy to understand question and answer format and discusses the currently available literature on the subject in an attempt to arrive at practical clinically relevant solutions. Patients who have undergone the Fontan operation are at a high risk for thromboembolism. Based on available evidence, there is a strong rationale for thromboprophylaxis...
September 2016: Annals of Pediatric Cardiology
Masanori Mizuno, Hideo Ohuchi, Taka-Aki Matsuyama, Aya Miyazaki, Hatsue Ishibashi-Ueda, Osamu Yamada
We report multi-organ histopathological changes in a patient with protein-losing enteropathy (PLE) over 12 years after Fontan operation. A 14-year-old boy with right isomerism heart and single ventricle had undergone Fontan procedure at 19 months of age, and PLE was diagnosed at 28 months. He had several episodes of intestinal bleeding and pre-renal failure with elevated creatine, and eventually died of pneumonia. The intrapulmonary small arteries showed medial and intimal thickening resembling pulmonary hypertension...
September 11, 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Hiromu Miyake, Koji Fukumoto, Masaya Yamoto, Hiroshi Nouso, Masakatsu Kaneshiro, Mariko Koyama, Naoto Urushihara
Purpose Patients with asplenia syndrome (AS) are likely to have upper gastrointestinal tract malformations such as hiatal hernia. This report discusses the treatment of such conditions. Methods Seventy-five patients with AS underwent initial palliation in our institution between 1997 and 2013. Of these, 10 patients had hiatal hernia. Of the patients with hiatal hernia, 6 had brachyesophagus and 7 had microgastria. Results Of the 10 patients with hiatal hernia, 9 underwent surgery in infancy (7 before Glenn operation, 2 after Glenn operation)...
September 8, 2016: European Journal of Pediatric Surgery
David J Goldberg, Benjamin French, Anita L Szwast, Michael G McBride, Stephen M Paridon, Jack Rychik, Laura Mercer-Rosa
OBJECTIVE: Tricuspid annular plane systolic excursion (TAPSE) is a reproducible measure of right ventricular systolic function. We sought to determine the reliability of TAPSE measurements, to evaluate TAPSE in patients with hypoplastic left heart syndrome (HLHS) relative to normal values, and to correlate values of TAPSE with measures of exercise performance. DESIGN: Tricuspid annular plane systolic excursion measurements were performed off-line in the cohort of patients with single right ventricle anatomy who had been enrolled in the Sildenafil After Fontan Operation clinical trial...
September 5, 2016: Echocardiography
Bahaaldin Alsoufi, Courtney McCracken, Subhadra Shashidharan, Brian Kogon, William Border, Kirk Kanter
BACKGROUND: The two most common surgical strategies for the treatment of neonates born with single-ventricle anomalies associated with aortic arch obstruction are the Norwood operation and pulmonary artery banding plus coarctation repair (PAB+COA). We reviewed characteristics and outcomes of neonates who underwent those two surgical strategies at our institution. METHODS: Between 2002 and 2012, 94 neonates with a single ventricle and aortic arch obstruction (excluding hypoplastic left heart syndrome) underwent Norwood (n = 65) or PAB+COA (n = 29)...
September 1, 2016: Annals of Thoracic Surgery
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