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https://www.readbyqxmd.com/read/28799708/acute-and-mid-term-outcomes-of-stent-implantation-for-recurrent-coarctation-of-the-aorta-between-the-norwood-operation-and-fontan-completion-a-multi-center-pediatric-interventional-cardiology-early-career-society-investigation
#1
Osamah Aldoss, Bryan H Goldstein, Saar Danon, Sebastian Goreczny, Robert G Gray, Shyam Sathanandam, Wendy Whiteside, Derek A Williams, Jeffrey D Zampi
OBJECTIVES: We sought to evaluate outcomes of stent implantation (SI) for recurrent coarctation of the aorta (RC) following the Norwood operation. BACKGROUND: RC is common following the Norwood operation. Balloon angioplasty (BA) is standard treatment but may result in unsatisfactory relief of RC. SI may improve RC, but outcome data are limited. METHODS: We performed a multi-center retrospective study of patients who underwent SI for RC between the Norwood operation and Fontan completion...
August 11, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28799131/pulmonary-vascular-disease-in-a-failed-fontan-patient-with-down-s-syndrome
#2
Masaya Aoki, Keiichi Hirono, Tomonori Higuma, Yoko Suzuki, Kazuhiko Nakayama, Akio Yamashita, Kazuaki Fukahara, Fukiko Ichida, Noriaki Emoto, Naoki Yoshimura
It is well known that Down's syndrome is a strong risk factor for mortality after Fontan operations. We performed two lung biopsies in a Down's syndrome patient who underwent staged Fontan operations. The pathological findings revealed severe pulmonary arterial hypertrophy and a quantitative real-time polymerase chain reaction revealed the overexpression of endothelin and a decline in the eNOS level at the Fontan operation. Although the preoperative hemodynamic studies revealed that all of the criteria for Fontan had been fulfilled, the patient died of acute cardiac insufficiency, 35 days after the Fontan operation...
August 10, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28795517/cardiovascular-adaptation-to-the-fontan-circulation
#3
REVIEW
Gruschen R Veldtman, Alexander R Opotowsky, Samuel G Wittekind, Jack Rychik, Daniel J Penny, Mark Fogel, Bradley S Marino, Marc Gewillig
Although medium-term survival following Fontan operations in the modern era has improved dramatically, late cardiovascular and extracardiac morbidity are common and are associated with impaired quality of life and premature late mortality. This serves as a reminder of the extraordinary adaptations required of the cardiovascular system when the systemic arterial, systemic venous and pulmonary circulations are placed in series coupled to a single ventricular pump. This article reviews the key features and principles that govern interactions between the ventricle, systemic arterial circulation, the systemic venous and pulmonary circulatory compartments, the microcirculation, and lymphatic circulations...
August 10, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28790279/-perioperative-management-of-fontan-operation
#4
Koichi Sughimoto, Kagami Miyaji
Surgical results of Fontan operation has been improved over the decades due to the introduction of the staged operations and some modifications of Fontan route from the classical atrio-pulmonary connection to total cavo-pulmonary connection. However, issues remain because of the single ventricular physiology of Fontan circulation. This article explains about the preoperative checklist for Fontan operation and tips on the postoperative management including an early extubation, use of inhaled nitric oxide after extubation, anticoagulation therapy, and efficacy of angiotensin converting enzyme inhibitor, supported by the cutting-edge evidence...
July 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28790278/-perioperative-care-for-patients-with-hypoplastic-left-heart-syndrome
#5
Yujiro Ide, Kisaburo Sakamoto
Hypoplastic Left Heart Syndrome (HLHS) is one of the most challenging congenital heart defects which require surgical interventions during neonatal period. In normal risk cohort, its surgical outcome has been improved dramatically since Dr. William Norwood reported the 1st successful case more than 30 years ago. The introduction of routine bilateral pulmonary artery banding with subsequent Norwood operation during early infantile( we call it as "rapid 2 stage Norwood operation") has brought an earlier hemodynamic stabilization after Norwood operation...
July 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28783102/the-pulmonary-circulation-in-the-single-ventricle-patient
#6
REVIEW
Amanda Hauck, Nicolas Porta, Steven Lestrud, Stuart Berger
In recent decades, survival of children with complex congenital heart disease has improved considerably. Specifically, children with a variety of congenital heart defects resulting in 'single ventricle' physiology can now undergo palliative surgery that allows survival beyond the neonatal period, and in many cases into adulthood, despite having a single functional ventricular pumping chamber supplying both the pulmonary and systemic circulation. Our growing understanding of the functionally univentricular heart has resulted in freedom from Fontan failure of >50% at 25 years post-Fontan...
August 7, 2017: Children
https://www.readbyqxmd.com/read/28761995/intermediate-term-thrombotic-risk-in-contemporary-total-cavo-pulmonary-connection-for-single-ventricle-circulations
#7
Jenna M Faircloth, Olivia Roe, Tarek Alsaied, Joseph S Palumbo, Alexander Vinks, Gruschen R Veldtman
Despite the common occurrence of thrombosis in Fontan circulations, the mid-term thrombotic risk beyond the first two postoperative years is poorly defined especially in total cavo pulmonary Fontan. This study examines the thrombotic incidence and risk beyond the first 2 years after contemporary Fontan surgery. Using a retrospective cohort study design, 89 Fontan patients, 50 male, were included and evaluated with a median of 8.3 years (IQR 6.8-11.4) follow-up. Hospital records were reviewed for known risk factors of thrombosis, thrombotic events, antiplatelet and anticoagulation management, and basic characteristics...
July 31, 2017: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/28734438/extracardiac-fontan-operation-through-a-right-thoracotomy
#8
Suvro S Sett, Rocco J Lafaro
An extracardiac Fontan operation through a right thoracotomy incision is an alternative approach in children who have an ascending aorta that is in close proximity to the sternum, as in aortic atresia or transposed great vessels, and in those who have had prior mediastinitis.
August 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28716702/how-to-perform-transconduit-and-transbaffle-puncture-in-patients-who-have-previously-undergone-the-fontan-or-mustard-operation
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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)]...
August 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28599384/adolescent-and-parent-perspectives-prior-to-involvement-in-a-fontan-transition-program
#15
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
#16
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
#17
RANDOMIZED CONTROLLED TRIAL
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
#18
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
#19
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
#20
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
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