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https://www.readbyqxmd.com/read/28716702/how-to-perform-transconduit-and-transbaffle-puncture-in-patients-who-have-previously-undergone-the-fontan-or-mustard-operation
#1
Jae-Sun Uhm, Nam Kyun Kim, Tae-Hoon Kim, Boyoung Joung, Hui-Nam Pak, Moon-Hyoung Lee
No abstract text is available yet for this article.
July 14, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28696879/outcomes-of-patients-undergoing-primary-fontan-operation-beyond-first-decade-of-life
#2
Sachin Talwar, Sukhjeet Singh, Vishnubhatla Sreenivas, Kulwant Singh Kapoor, Saurabh Kumar Gupta, Sivasubramanian Ramakrishnan, Shyam Sunder Kothari, Anita Saxena, Rajnish Juneja, Shiv Kumar Choudhary, Balram Airan
OBJECTIVES: Studies on older patients undergoing primary Fontan operation (FO) are limited, with conflicting results. We review our experience with these patients beyond the first decade of life. PATIENTS AND METHODS: Between January 2000 and December 2014, a total of 105 patients ≥10 years of age (mean 15.6 ± 4.9, range 10-31, median 15 years) underwent primary FO without a prior bidirectional superior cavopulmonary anastomosis (Bidirectional Glenn [BDG]). Mean preoperative New York Heart Association (NYHA) class was 2...
July 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28625386/hemodynamic-determinants-of-mortality-after-fontan-operation
#3
Hideo Ohuchi, Aya Miyazaki, Jun Negishi, Yosuke Hayama, Michikazu Nakai, Kunihiro Nishimura, Hajime Ichikawa, Isao Shiraishi, Osamu Yamada
BACKGROUND: Elevated central venous pressure (CVP), low cardiac output, and mild hypoxia are common early and late after Fontan operations. However, the association of these characteristics with late mortality is unclear. We aimed to elucidate the hemodynamic determinants of mortality after Fontan operation. METHOD: We evaluated early (group early; 0.5-5years postoperatively, n=387) and late (group late; ≥15years postoperatively, n=161) Fontan hemodynamics that included CVP (mm Hg), cardiac index (CI; L/min per m(2)), systemic ventricular end-diastolic volume index (mL/m(2)), ejection fraction (EF; %), and arterial blood oxygen saturation (%)...
July 2017: American Heart Journal
https://www.readbyqxmd.com/read/28624095/fontan-circulation-over-time
#4
Djoeke Wolff, Joost P van Melle, Beatrijs Bartelds, Floris-Jan S Ridderbos, Graziella Eshuis, Elisabeth B H J van Stratum, Salvador J Recinos, Brigitte W M Willemse, Hans Hillege, Tineke P Willems, Tjark Ebels, Rolf M F Berger
The unique, unphysiological Fontan circulation is associated with an impaired functional status of the patients that is suggested to deteriorate over time. Unfortunately, previous studies did not integrate both pulmonary and cardiac determinants of functional status. In addition, a comparison with the natural decrease in exercise capacity in healthy subjects (in both children and adults) is lacking. This single-center study aims to investigate the functional status in a cohort of Fontan patients in relation to time since Fontan completion and to identify its determinants, including cardiac characteristics and pulmonary characteristics...
August 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28610439/congenitally-corrected-transposition-complex-anatomic-repair-or-fontan-pathway
#5
Supreet P Marathe, Matthew I Jones, Julian Ayer, Jessica Sun, Yishay Orr, Charlotte Verrall, Ian A Nicholson, Richard B Chard, Gary F Sholler, David S Winlaw
Background Successful anatomic repair of congenitally corrected transposition of the great arteries achieves excellent outcomes. Several centers report excellent long-term survival with the Fontan pathway as well. We have selectively applied both approaches depending on individual patient morphology, with anatomic repair preferred but utilizing the Fontan pathway when high technical complexity or operative risk is anticipated. Methods Hospital records over an 18-year period (1998-2016) were reviewed to identify patients with congenitally corrected transposition of the great arteries who underwent surgical management...
January 1, 2017: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/28608149/exercise-capacity-and-self-efficacy-are-associated-with-moderate-to-vigorous-intensity-physical-activity-in-children-with-congenital-heart-disease
#6
Laura Banks, Shelly Rosenthal, Cedric Manlhiot, Chun-Po Steve Fan, Adam McKillop, Patricia E Longmuir, Brian W McCrindle
This study sought to determine whether exercise capacity, self-efficacy, and gross motor skills are associated with moderate-to-vigorous physical activity (MVPA) levels in children, and if these associations differ by congenital heart disease (CHD) type. Medical history was abstracted from chart review. We assessed MVPA levels (via accelerometry), percent-predicted peak oxygen consumption ([Formula: see text] cardiopulmonary exercise test), gross motor skill percentiles (test of gross motor development version-2), and self-efficacy [children's self-perceptions of adequacy and predilection for physical activity scale (CSAPPA scale)]...
June 12, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28599384/adolescent-and-parent-perspectives-prior-to-involvement-in-a-fontan-transition-program
#7
Karin du Plessis, Evelyn Culnane, Rebecca Peters, Yves d'Udekem
Background Successful transition to adult healthcare is particularly important for congenital heart disease (CHD) patients who have undergone palliative surgery, as they risk adverse events if not followed closely. This study examines young people at the worst end of the CHD spectrum who are born with a single ventricle (pumping heart chamber), and who undergo a series of operations that culminate in the Fontan surgical procedure. Purpose To explore adolescents with a Fontan circulation, and their parents', readiness for transition to adult care and pre-implementation of a transition program...
May 24, 2017: International Journal of Adolescent Medicine and Health
https://www.readbyqxmd.com/read/28593158/fontan-revision-with-y-graft-in-a-patient-with-unilateral-pulmonary-arteriovenous-malformation
#8
Jeong-Woo Lee, Jeong-Jun Park, Hyun Woo Goo, Jae Kon Ko
The extracardiac conduit Fontan procedure is the last surgical step in the treatment of patients with a functional single ventricle. An acquired pulmonary arteriovenous malformation may appear perioperatively or postoperatively due to an uneven hepatic flow distribution. Here we report a case of a bifurcated Y-graft Fontan operation in a 15-year-old male patient with a unilateral pulmonary arteriovenous malformation after an extracardiac conduit Fontan operation.
June 2017: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28577680/results-of-a-phase-i-ii-multi-center-investigation-of-udenafil-in-adolescents-after-fontan-palliation
#9
David J Goldberg, Victor Zak, Bryan H Goldstein, Shan Chen, Michelle S Hamstra, Elizabeth A Radojewski, Eileen Maunsell, Seema Mital, Shaji C Menon, Kurt R Schumacher, R Mark Payne, Mario Stylianou, Jonathan R Kaltman, Tina M deVries, James L Yeager, Stephen M Paridon
BACKGROUND: The Fontan operation results in a circulation that is dependent on low pulmonary vascular resistance to maintain an adequate cardiac output. Medical therapies that lower pulmonary vascular resistance may augment cardiac output and improve long-term outcomes. OBJECTIVES: This phase I/II clinical trial conducted by the Pediatric Heart Network was designed to evaluate short-term safety, pharmacokinetics (PK), and preliminary efficacy of udenafil in adolescents following Fontan...
June 2017: American Heart Journal
https://www.readbyqxmd.com/read/28573700/long-term-follow-up-of-fontan-completion-in-adults-and-adolescents
#10
Tai Fuchigami, Mitsugi Nagashima, Takeshi Hiramatsu, Goki Matsumura, Minori Tateishi, Noriyasu Masuda, Kenji Yamazaki
BACKGROUND: The Fontan procedure is rarely performed in adults and adolescents in the present era. We review our results with the Fontan procedure in adolescents and young adults. METHODS: Between 1974 and 2010, 79 consecutive patients underwent the Fontan procedure at an age ≥ 15 years (mean age at Fontan operation, 20.3 years ± 4.5 years). Forty-five patients underwent atriopulmonary connection, 11 underwent the Bjork procedure, and 23 underwent total cavopulmonary connection...
July 2017: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/28545820/extracardiac-conduit-fontan-outcome-data-in-early-adulthood
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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