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https://www.readbyqxmd.com/read/28545820/extracardiac-conduit-fontan-outcome-data-in-early-adulthood
#1
Dominica Zentner, Caitlin Cheshire, Leeanne Grigg
BACKGROUND: To describe the survival and health outcome status of young adults with an extracardiac Fontan procedure performed either as a primary or conversion (secondary) Fontan surgery. METHODS: The database of the Adult Congenital Heart disease service at the Royal Melbourne Hospital was interrogated to identify all adults who had undergone a primary extracardiac conduit Fontan (n=29) or a Fontan conversion with this procedure (n=8). We then determined vital status, age, original anatomy and functional status in early adulthood in both groups...
May 3, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28534240/an-early-glenn-operation-may-be-associated-with-the-later-occurrence-of-protein-losing-enteropathy-in-fontan-patients-association-of-early-glenn-and-failing-fontan
#2
Bettina Unseld, Brigitte Stiller, Thomas Borth-Bruhns, Florian du Bois, Johannes Kroll, Jochen Grohmann, Thilo Fleck
Protein-losing enteropathy (PLE) and plastic bronchitis (PB) are major causes of long-term mortality after Fontan operation. The objective of this study was to determine early clinical risk factors before the onset of PLE and PB. In a cohort study, 106 Fontan patients between 2005 and 2013 were examined. A median of 5.3 (1.5-8.5) years later, follow-up questionnaires were used to group the patients in a PLE or PB group (n = 14) and a non-PLE/PB group (n = 92). Prevalence of PLE was 9.4% (n = 10) and of PB 3...
May 22, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28532983/towards-the-goal-of-achieving-a-normal-duration-and-quality-of-life-after-fontan-operation-creation-of-the-international-fontan-interest-group-i-fig-an-international-collaborative-initiative-dedicated-to-improving-outcomes
#3
Yves d'Udekem, Jack Rychik
A growing population of patients are now surviving the Fontan procedure and face an uncertain future and a clear burden of disease. The best medical therapy and follow-up modalities for these patients have not yet been identified, indications for transplantation are unclear and we have not been successful in mechanically supporting their circulation. There has only been a limited number of trials to address these issues. An international group has now been created to launch the large scale multi-centric research needed to provide the best future to this growing population: the International Fontan Interest Group (I-FIG)...
May 11, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28532389/the-use-of-macitentan-in-fontan-circulation-a-case-report
#4
Polyvios Demetriades, Amir Aziz, Robin Condliffe, Sarah E Bowater, Paul F Clift
BACKGROUND: The Fontan circulation, a result of a palliative procedure in patients with single systemic ventricles, is defined by chronically elevated pulmonary vascular resistance. When traditional heart failure therapies fail, pharmacological agents that reduce pulmonary artery pressures may be used. These include endothelial-receptor antagonists, prostanoids and phosphodiesterase type 5 inhibitors. We report the first use of macitentan, an endothelin-receptor antagonist, in a patient with a Fontan circulation...
May 22, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28527961/major-aortopulmonary-collateral-arteries-with-anatomy-other-than-pulmonary-atresia-ventricular-septal-defect
#5
William L Patrick, Richard D Mainwaring, Olaf Reinhartz, Rajesh Punn, Theresa Tacy, Frank L Hanley
BACKGROUND: Major aortopulmonary collateral arteries (MAPCAs) are frequently found in association with pulmonary atresia with ventricular septal defect (PA/VSD). However, some patients with MAPCAs do not have PA/VSD but have a variety of other "atypical" anatomic diagnoses. METHODS: This was a retrospective review of patients with MAPCAs and atypical anatomy. The 50 patients with MAPCAs could be divided into two subgroups: (1) single ventricle anatomy (n = 33) and (2) two ventricle anatomy (n = 17)...
May 17, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28501797/predictors-of-procedural-complications-in-adult-fontan-patients-undergoing-non-cardiac-procedures
#6
Alexander C Egbe, Arooj R Khan, Naser M Ammash, David W Barbara, William C Oliver, Sameh M Said, Emmanuel Akintoye, Carole A Warnes, Heidi M Connolly
OBJECTIVE: Limited data exist regarding the outcomes of non-cardiac procedures (NCPs) in adult patients after Fontan operations (Fontan patients). METHODS: To compare procedural outcomes after NCPs in Fontan patients with outcomes for two matched control groups: patients with repaired congenital heart disease and biventricular circulation (CHD-BiV) and patients with no heart disease (NHD). We defined cyanosis as oxygen saturation <90% and procedural hypoxia as saturation <80% or a decrease in saturation >10% from baseline...
May 13, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28497477/fontan-operation-with-bilateral-conduits-for-the-treatment-of-pulmonary-arteriovenous-fistulas-in-a-patient-with-polysplenia-after-reconstruction-of-a-non-confluent-pulmonary-artery
#7
Yuji Tominaga, Hiroaki Kawata, Shigemitsu Iwai, Sanae Yamauchi
We describe a case involving a successful Fontan procedure for the treatment of pulmonary arteriovenous fistulas in a patient with polysplenia. The patient was diagnosed with bilateral superior vena cavae, interrupted inferior vena cava with azygos connection, wide separate hepatic veins, and central pulmonary artery stenosis following repair of a non-confluent pulmonary artery. Bilateral extracardiac conduits were used to connect each hepatic vein to the pulmonary artery, opposite each superior vena cava. Clinical improvement in the pulmonary arteriovenous fistulas was observed within 4 months after surgery...
May 11, 2017: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/28480728/hepatocellular-carcinoma-in-a-non-failing-fontan-circulation
#8
Efrén Martínez-Quintana, Alberto Monescillo, Fayna Rodríguez-González
Patients after a Fontan operation exhibit some degree of fibrosis which are primarily located in a sinusoidal or centrilobular pattern. Cirrhosis may develop years later and the incidence of cancer is up to 5%. That is why some authors recommend that patients who are >10 years out from their Fontan operation should undergo cardiac assessment as well as the determination of alpha-fetoprotein level, the realization of liver imaging tests or even liver biopsy to stay ahead of neoplastic transformation.
May 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28462755/a-multi-institutional-study-of-factors-affecting-resource-utilisation-following-the-fontan-operation
#9
Kevin A Hinkle, Jacob Wilkes, Molly McFadden, Richard V Williams, LuAnn L Minich, Shaji C Menon
The few studies evaluating data on resource utilisation following the Fontan operation specifically are outdated. We sought to evaluate resource utilisation and factors associated with increased resource use after the Fontan operation in a contemporary, large, multi-institutional cohort. This retrospective cohort study of children who had the Fontan between January, 2004 and June, 2013 used the Pediatric Health Information Systems Database. Generalised linear regression analyses evaluated factors associated with resource use...
May 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28461021/pediatric-fontan-patients-are-at-risk-for-myocardial-fibrotic-remodeling-and-dysfunction
#10
Atsuko Kato, Eugénie Riesenkampff, Deane Yim, Shi-Joon Yoo, Mike Seed, Lars Grosse-Wortmann
BACKGROUND: Patients with single ventricle (SV) circulations are at risk for ventricular dysfunction. This study investigates whether there is evidence of increased myocardial fibrosis and myocardial dysfunction in children after the Fontan operation. METHODS: Consecutive children after the Fontan operation who underwent cardiac magnetic resonance (CMR) T1 relaxometry with a modified look-locker inversion recovery approach were included in this retrospective study...
April 22, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28456831/clinical-application-of-the-qrs-t-angle-for-the-prediction-of-ventricular-arrhythmias-in-patients-with-the-fontan-palliation
#11
Tuong-Vi Tran, Daniel Cortez
Fontan palliation patients are at risk for ventricular arrhythmias post-operatively. This study aimed to evaluate whether differences in the spatial QRS-T angle can reliably predict ventricular arrhythmias in patients who had undergone Fontan palliation. A total of 117 patients who had the Fontan palliation and post-Fontan catheterization were included. Ventricular arrhythmias were identified in nine patients. Measurements of ECG parameters including QRS vector magnitude, QRS duration, corrected QT interval, and spatial peaks QRS-T angles were performed, and compared between those with and without ventricular arrhythmias...
April 29, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28454933/is-it-time-for-some-new-ideas-for-patients-undergoing-the-fontan-operation-here-is-some-food-for-thought
#12
EDITORIAL
Osman O Al-Radi
No abstract text is available yet for this article.
April 7, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28454826/outcomes-in-adult-fontan-patients-with-atrial-tachyarrhythmias
#13
Alexander C Egbe, Heidi M Connolly, Arooj R Khan, Talha Niaz, Sameh S Said, Joseph A Dearani, Carole A Warnes, Abhishek J Deshmukh, Suraj Kapa, Christopher J McLeod
BACKGROUND: The optimal management strategy for atrial tachyarrhythmia in the Fontan population is unknown. METHODS: Retrospective review of 264 adult Fontan patients with atrial tachyarrhythmia evaluating 3 clinically adopted scenarios: antiarrhythmic drug (AAD) therapy, catheter ablation (CA), and Fontan conversion (FC). These patients were followed up at Mayo Clinic from 1994 to 2014. The study objective was to compare freedom from atrial tachyarrhythmia recurrence (AR) and occurrence of composite adverse events (stroke, heart failure hospitalization, death, or heart transplant) between treatment groups...
April 2017: American Heart Journal
https://www.readbyqxmd.com/read/28453807/attrition-in-patients-with-single-ventricle-and-trisomy-21-outcomes-after-a-total-cavopulmonary-connection
#14
Anastasios C Polimenakos, Sujata Subramanian, Chawki ElZein, Michel N Ilbawi
OBJECTIVES: Data are limited regarding the management of children with trisomy 21 (T21) syndrome and a functional single ventricle (FSV). We evaluated patients with T21 and a FSV who had a total cavopulmonary connection (TCPC). METHODS: From September 1999 to August 2012, 139 patients with a FSV underwent a TCPC. Sixty-five had unbalanced atrioventricular septal defect. Thirteen had T21. Three (of 13) had heterotaxy syndrome. The mean age at the Fontan operation was 27...
May 1, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28446492/hepatic-fibrosis-is-universal-following-fontan-operation-and-severity-is-associated-with-time-from-surgery-a-liver-biopsy-and-hemodynamic-study
#15
David J Goldberg, Lea F Surrey, Andrew C Glatz, Kathryn Dodds, Michael L O'Byrne, Henry C Lin, Mark Fogel, Jonathan J Rome, Elizabeth B Rand, Pierre Russo, Jack Rychik
BACKGROUND: Congestive hepatopathy is a recognized complication of Fontan physiology. Data regarding the incidence of hepatopathy and risk factors are lacking. METHODS AND RESULTS: Liver biopsies and cardiac catherizations were performed as part of an evaluation offered to all patients ≥10 years after Fontan. Quantitative determination of hepatic fibrosis was performed using Sirius red staining with automated calculation of collagen deposition per slide (%CD)...
April 26, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28431725/intraoperative-three-dimensional-mapping%C3%A2-of-supraventricular-tachycardia-in-a-young-fontan%C3%A2-patient
#16
James C Perry, Matthew R Williams, Suzanne Shepard, Andras Bratincsak, Cynthia Murphy, Raghav Murthy, Mohammad Ebrahim, John J Lamberti
Preoperative tachycardia is uncommon before an initial Fontan procedure. Catheter intervention can risk complications in small patients. A patient with fetal tachycardia and double inlet left ventricle underwent Norwood and bidirectional Glenn procedures. She had persistent tachycardia. A three-dimensional electroanatomic mapping system was modified for the operative procedure; it was used to identify the arrhythmia substrate and cryoablate an atrioventricular nodal tachycardia. Mapping added 20 minutes to the operation...
May 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28422842/the-role-of-renoscintigraphy-and-surgery-in-the-management-of-page-kidney-a-case-report
#17
Yu-Li Chiu, Chin Hu, Sin-Di Lee, Po-Yin Chen, Cheng-Pin Wang
RATIONALE: Page kidney is an uncommon condition that hypertension occurs secondary to microvascular ischemia and alternation of small-vessel hemodynamics due to external compression of renal parenchyma and activation of the renin-angiotensin-aldosterone system. There are no specific guidelines for the management of Page kidney in the literatures. PATIENT CONCERNS: A 17-year-old teenager who had Fontan procedure for tricuspid and pulmonary atresia in early childhood suffered from sudden onset of severe left flank pain during cardiac catheterization procedure...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28402400/unplanned-cardiac-reoperations-and-interventions-during-long-term-follow-up-after-the-norwood-procedure%C3%A2
#18
Eva Sames-Dolzer, Gregor Gierlinger, Michaela Kreuzer, Julia Schrempf, Roland Gitter, Christoph Prandstetter, Gerald Tulzer, Rudolf Mair
OBJECTIVES: Patients with hypoplastic left heart syndrome or related malformations are predominantly treated with a 3-stage palliation. Anatomic or physiologic problems can lead to unplanned additional surgical or catheter interventions during single ventricle palliation. Changes in operative technique may have an impact on the reoperation rate. METHODS: Between 1997 and 2014, 317 Norwood procedures were performed at our centre. A retrospective single centre investigation was carried out concerning incidence, timing, indication and type of unplanned interstage cardiac reoperations and catheter interventions during follow-up of Norwood patients...
June 1, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28386502/transumbilical-surgery-for-duodenal-stenosis-in-a-child-with-situs-inversus-the-first-report
#19
Isamu Saeki, Yu Ueno, Wataru Mukai, Reisuke Imaji, Takashi Akiyama
Background. Situs inversus is a rare congenital anomaly with a reported incidence of only 1 in 5,000 to 10,000 live births. Congenital duodenal stenosis complicated with situs inversus is an even rarer entity. Case Presentation. A 1-year-old girl with situs inversus who had undergone a hemi-Fontan procedure against a single ventricle in our hospital was referred to our department for vomiting and failure to thrive. An upper gastrointestinal contrast study and endoscopy revealed duodenal stenosis. A transumbilical radical operation as a minimally invasive surgery was successfully performed...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28385377/the-effects-of-modification-to-lateral-tunnel-fontan-procedure-for-prophylactic-arrhythmia-surgery
#20
Hong-Gook Lim, Jeong Ryul Lee, Yong Jin Kim
BACKGROUND: Refractory atrial arrhythmias and hemodynamic abnormalities are responsible for significant morbidity and mortality after the Fontan operation. We evaluated the long-term feasibility, safety, and efficacy of prophylactic atrial arrhythmia surgery performed concomitantly with the lateral tunnel Fontan operation. METHODS: From 1997 August to 2003 December, 27 patients underwent a initial lateral tunnel Fontan with an interventional atrial incision and cryoablation from the atriotomy to the coronary sinus and right atrioventricular valve annulus...
April 3, 2017: Annals of Thoracic Surgery
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