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https://www.readbyqxmd.com/read/28210768/the-lymphatic-circulation-in-adaptations-to-the-fontan-circulation
#1
REVIEW
Sabarinath Menon, Murthy Chennapragada, Shinya Ugaki, Gary F Sholler, Julian Ayer, David S Winlaw
Failing Fontan continues to be major problem for patients on the univentricular pathway. Failing Fontan is often complicated by chylothorax, plastic bronchitis and protein loosing enteropathy. The role of lymphatic circulation in Fontan circulation is still being researched. Newer imaging modalities give insight into the role of abnormal dilatation and retrograde flow in lymphatic channels post Fontan. Interventional strategies targeting abnormal lymphatic channels, provides an alternative management strategy for patients with failing Fontan...
February 16, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28196580/anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-with-renal-involvement-analysis-of-89-cases
#2
Fernando Caravaca-Fontán, Estefanía Yerovi, María Delgado-Yagu E, Cristina Galeano, Saúl Pampa-Saico, Maria Teresa Tenorio, Fernando Liaño
INTRODUCTION: The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis with renal involvement are associated with high morbi-mortality. In this study we analyse if the prognosis of these diseases have improved in recent years, and which factors influence the outcomes. MATERIAL AND METHODS: Retrospective single-centre observational study, which included all patients diagnosed with microscopic polyangiitis and granulomatosis with polyangiitis with renal involvement in the last 25 years...
January 6, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28184977/lateral-atrial-tunnel-fontan-operation-predisposes-to-the-junctional-rhythm
#3
Katarzyna Januszewska, Anna Schuh, Anja Lehner, Robert Dalla-Pozza, Edward Malec
The goal of the study was to compare the early postoperative course after fenestrated lateral atrial tunnel (LT) and non-fenestrated extracardiac conduit (EC) Fontan operation (FO) in a single center where both techniques were parallelly used. Between 2004 and 2012, 56(32.7%) children underwent LT and 113(66.1%) EC FO. The mean age was 3.7 ± 2.9 years and mean weight was 14.6 ± 7.3 kg. The most common malformation was hypoplastic left heart syndrome (50.9%). The general approach was to perform LT in children after hemi-Fontan operation and EC in patients after Glenn anastomosis...
February 10, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28184976/decreased-diastolic-ventricular-kinetic-energy-in-young-patients-with-fontan-circulation-demonstrated-by-four-dimensional-cardiac-magnetic-resonance-imaging
#4
Pia Sjöberg, Einar Heiberg, Pär Wingren, Jens Ramgren Johansson, Torsten Malm, Håkan Arheden, Petru Liuba, Marcus Carlsson
Four-dimensional (4D) flow magnetic resonance imaging (MRI) enables quantification of kinetic energy (KE) in intraventricular blood flow. This provides a novel way to understand the cardiovascular physiology of the Fontan circulation. In this study, we aimed to quantify the KE in functional single ventricles. 4D flow MRI was acquired in eleven patients with Fontan circulation (median age 12 years, range 3-29) and eight healthy volunteers (median age 26 years, range 23-36). Follow-up MRI after surgical or percutaneous intervention was performed in 3 patients...
February 10, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28184975/alkaline-phosphatase-a-biomarker-of-cardiac-function-in-pediatric-patients
#5
Elizabeth S Makil, Xinyu Tang, Elizabeth A Frazier, R Thomas Collins
Myocardial dysfunction and heart failure are common in pediatric patients with congenital and acquired heart disease. Alkaline phosphatase (AP) has been suggested as a biomarker for myocardial dysfunction after Fontan operation. We hypothesized that pediatric patients with myocardial dysfunction requiring orthotopic heart transplant (OHT) have diminished AP compared to normal. A retrospective review was performed in all patients who underwent OHT at Arkansas Children's Hospital between January 2007 and October 2012...
February 9, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28181900/an-alternative-technique-for-fontan-completion-in-patients-with-widely-separated-connections-of-the-inferior-systemic-veins-to-the-atrial-mass
#6
Giuseppe Pomè, Faker Ghedira, Eitan Keizman
Several surgical approaches for completion of a total cavopulmonary connection have been established for patients with anomalies of systemic venous connection in association with a functionally univentricular heart. We report an alternative technique designed to address the problem of widely separated inferior systemic veins, in which two extracardiac conduits were used. This technique was performed on three patients and yielded excellent short-term results, allowing us to operate on a beating heart with no need for cardioplegic arrest and aortic cross-clamping...
August 30, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/28157834/reduced-cortical-volume-and-thickness-and-their-relationship-to-medical-and-operative-features-in-post-fontan-children-and-adolescents
#7
Christopher G Watson, Christian Stopp, David Wypij, Jane W Newburger, Michael J Rivkin
BACKGROUND: We compared brain cortical and subcortical gray matter volumes and cortical thickness between post-Fontan patients and healthy controls, and examined brain anatomical associations with operative and medical history characteristics. METHODS: Post-Fontan (n = 128 volumes; n = 115 thickness) and control subjects (n = 48 volumes; n = 45 thickness) underwent brain MRI at ages 10-19 years. Subcortical and cortical volumes and cortical thicknesses were measured for intergroup comparison...
February 3, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28148729/psychiatric-disorders-in-adolescents-with-single-ventricle-congenital-heart-disease
#8
David R DeMaso, Johanna Calderon, George A Taylor, Jennifer E Holland, Christian Stopp, Matthew T White, David C Bellinger, Michael J Rivkin, David Wypij, Jane W Newburger
BACKGROUND AND OBJECTIVES: Mental health outcomes for survivors of critical congenital heart disease (CHD) remain under-investigated. We sought to examine psychiatric disorders and psychosocial functioning in adolescents with single ventricle CHD and to explore whether patient-related risk factors predict dysfunction. METHODS: This cohort study recruited 156 adolescents with single ventricle CHD who underwent the Fontan procedure and 111 healthy referents. Participants underwent comprehensive psychiatric evaluation including a clinician-rated psychiatric interview and parent- and self-report ratings of anxiety, disruptive behavior, including attention-deficit/hyperactivity disorder (ADHD), and depressive symptoms...
February 1, 2017: Pediatrics
https://www.readbyqxmd.com/read/28146206/case-1-2017-percutaneous-repair-of-right-atrioventricular-valve-insufficiency-and-blalock-taussig-shunt-after-fontan-operation-in-single-ventricle
#9
Edmar Atik, Renata Sá Cassar, Raul Arrieta
No abstract text is available yet for this article.
January 2017: Arquivos Brasileiros de Cardiologia
https://www.readbyqxmd.com/read/28140526/surveillance-for-liver-complications-after-the-fontan-procedure
#10
REVIEW
Moira B Hilscher, Jonathan N Johnson, Frank Cetta, David J Driscoll, John J Poterucha, William Sanchez, Heidi M Connolly, Patrick S Kamath
The physiological consequences of the Fontan circulation impose risk for hepatic dysfunction and may culminate in hepatic fibrosis, cirrhosis, and hepatocellular carcinoma. Consensus regarding appropriate surveillance modalities to diagnose liver disease in Fontan patients is lacking, in part due to the relative lack of strong evidence and prospective studies in this patient population. The goal of this paper is to critically review the current evidence and provide recommendations for the surveillance of hepatic complications in the post-Fontan patient population...
January 31, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28132780/early-mortality-and-concomitant-procedures-related-to-fontan-conversion-quantitative-analysis
#11
Margarita Brida, Helmut Baumgartner, Michael A Gatzoulis, Gerhard-Paul Diller
BACKGROUND: The Fontan palliation is associated with numerous complications during long-term. The Fontan conversion operation has been advocated as an option to avoid some of these problems by converting classical Fontan types to modern forms of the circulation. Early mortality of Fontan conversion, however, remains unclear as available reports include limited numbers of patients and the results are heterogeneous. METHODS: We reviewed all original articles from 1994 to 2016 reporting Fontan conversion operations...
January 25, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28132535/modified-total-cavopulmonary-shunt-as-a-staged-fontan-operation
#12
Hidetsugu Asai, Tsuyoshi Tachibana, Yasushige Shingu, Hiroki Kato, Satoru Wakasa, Yoshiro Matsui
The left superior vena cava became occluded in an infant with hypoplastic left heart syndrome. After a bidirectional Glenn procedure, he presented with severe oxygen desaturation and right ventricular dysfunction; the left superior vena cava drained into the inferior vena cava through collateral veins. As salvage therapy, we created a modified total cavopulmonary shunt using only autologous tissue in which the right hepatic vein and inferior vena cava drained into the pulmonary artery via a lateral tunnel in the right atrium...
January 1, 2017: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/28118264/in-vitro-examination-of-the-heartware-circulite-ventricular-assist-device-in-the-fontan-connection
#13
Michael Tree, Phillip M Trusty, Timothy C Slesnick, Ajit Yoganathan, Shriprasad Deshpande, Kevin Maher
The failing Fontan physiology may benefit from ventricular assist device (VAD) mechanical circulatory support, though a sub-pulmonary VAD placed at the Fontan connection has never successfully supported the Fontan circulation long term.The HeartWare CircuLite continuous flow VAD was examined for Fontan circulatory support in an in vitro mock circulation. The VAD was tested in three different scenarios: VAD in parallel, baffle restricted VAD in parallel, and VAD in series. Successful support was defined as simultaneous decrease in inferior vena cava (IVC) pressure of 5 mmHg or more and an increase in cardiac output (CO) to 4...
January 20, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28116475/effect-of-fenestration-on-early-postoperative-outcome-in-extracardiac-fontan-patients-with-different-risk-levels
#14
Fan Fan, Zhimin Liu, Shoujun Li, Tong Yi, Jun Yan, Fuxia Yan, Xu Wang, Qiang Wang
Although fenestration is used to improve the postoperative course of Fontan patients, the effect of fenestration on the extracardiac Fontan seems controversial especially at early postoperation. One hundred and eighty-three patients from January 2004 to June 2013 undergoing an extracardiac Fontan operation were retrospectively selected for this study. We divided the patients into low risk (93 patients) and high risk (90 patients) groups according to the risk factors recognized by previous studies and then compared the perioperative data between the nonfenestrated and fenestrated patients in each group...
January 23, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28112463/circulatory-support-using-the-impella-device-in-fontan-patients-with-systemic-ventricular-dysfunction-a-multicenter-experience
#15
Brian H Morray, Vivian V Dimas, Scott Lim, David T Balzer, Dhaval R Parekh, Nicolas M Van Mieghem, Peter Ewert, Dennis W Kim, Henri Justino, Doff B McElhinney, Thomas K Jones
BACKGROUND: There are limited mechanical circulatory support options for patients with single ventricle (SV) anatomy. This is a multicenter, retrospective study of the Impella pump to support the systemic ventricle in a cohort of SV patients with Fontan circulation. METHODS: Patients with SV anatomy supported with an Impella device from 2012 to 2015 were included. Demographic information, indication for support, adverse events and short-term outcome data were collected...
January 23, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28111407/determinants-of-aortic-size-and-stiffness-and-the-impact-on-exercise-physiology-in-patients-after-the-fontan-operation
#16
Hideo Ohuchi, Yosuke Hayama, Jun Negishi, Kanae Noritake, Aya Miyazaki, Osamu Yamada, Isao Shiraishi
The pathophysiology of congenital heart disease includes aortic dilation and increased stiffness. However, the clinical determinants and significance remain unclear in patients after the Fontan operation.Size and stiffness index (SI) of the ascending and descending aorta (aAO and dAO, respectively) were assessed using angiography in 130 consecutive Fontan patients and 30 age-matched controls. Compared with controls, Fontan patients showed a dilated aAO and smaller dAO (P < 0.0001) with greater SI (3.2 ± 0...
February 7, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28109386/an-unusual-cause-of-hemopericardium-22-years-after-a-fontan-procedure
#17
Billie-Jean Martin, Cameron Seaman, Jonathan D Windram, Ivan M Rebeyka
Late tamponade after cardiac operations is rare but reasonably well described. We report a case of exceedingly late tamponade secondary to a spontaneous coronary bleed 22 years after a Fontan operation, which was repaired with catheter intervention.
February 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28108065/assessment-of-central-venous-physiology-of-fontan-circulation-using-peripheral-venous-pressure
#18
Satoshi Masutani, Clara Kurishima, Akiko Yana, Seiko Kuwata, Yoichi Iwamoto, Hirofumi Saiki, Hirotaka Ishido, Hideaki Senzaki
OBJECTIVE: Elevated central venous pressure is a major cause of morbidity and mortality after the Fontan operation. The difference between mean circulatory filling pressure and central venous pressure, a driving force of venous return, is important in determining dynamic changes in central venous pressure in response to changes in ventricular properties or loading conditions. Thus, noninvasive central venous pressure and mean circulatory filling pressure estimation may contribute to optimal management in patients undergoing the Fontan operation...
December 19, 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28104198/patients-with-unbalanced-atrioventricular-canal-defects-can-undergo-the-fontan-operation-with-good-outcomes
#19
EDITORIAL
Sitaram M Emani
No abstract text is available yet for this article.
February 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28101662/strategies-to-prevent-cast-formation-in-patients-with-plastic-bronchitis-undergoing-heart-transplantation
#20
John J Parent, Robert K Darragh, Jeffrey G Gossett, Thomas D Ryan, Chet R Villa, Angela Lorts, John L Jefferies, Jeffrey A Towbin, Clifford Chin
Plastic bronchitis, a rare complication after Fontan palliation, carries a high morbidity and mortality risk. Heart transplantation is an effective treatment option, but casts may occur in the early post-operative period. We present a case series detailing peri-operative management strategies to minimize morbidity and mortality related to plastic bronchitis in patients undergoing heart transplantation. Patient 1 received no treatment pre-, intra-, or post-transplant for prevention of bronchial casts and developed severe respiratory acidosis 18 h following transplant...
January 19, 2017: Pediatric Cardiology
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