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https://www.readbyqxmd.com/read/28793902/30-year-experience-of-fontan-surgery-single-centre-s-data
#1
Laurynas Bezuska, Virgilijus Lebetkevicius, Rita Sudikiene, Daina Liekiene, Virgilijus Tarutis
BACKGROUND: The Fontan procedure has been modified several times since it was introduced into practice in 1968. As many patients now survive to adulthood, attention is directed towards their clinical status and late morbidity. We report our surgical experience of 30 years in Fontan procedures. METHODS: From January 1985 to January 2015, 80 patients underwent Fontan surgery. Twenty-one patients received an atrio-pulmonary Fontan (Group I), four patients underwent total cavopulmonary connection (TCPC) with an intra-atrial lateral tunnel (Group II), six patients received extra-cardiac TCPC with an aortic homograft (group III) and 49 patients received extra-cardiac TCPC with an expanded polytetrafluoroethylene conduit...
August 9, 2017: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/28783102/the-pulmonary-circulation-in-the-single-ventricle-patient
#2
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
#3
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/28730300/health-related-quality-of-life-in-paediatric-patients-with-congenital-heart-defects-association-with-the-type-of-heart-defect-and-the-surgical-technique
#4
A Heusch, H J Kahl, K O Hensel, G Calaminus
The aim of the study was to investigate the impact of a number of surgical interventions for a various congenital cardiac defects (CHDs) on self-reported HRQoL. METHODS: Patients who had received corrective surgery of several congenital heart defects (surgical VSD closure, Fallot, TGA after atrial or arterial switch or Fontan-type circulation for univentricular AV-connection) were interviewed in the office of their home peadiatric cardiologist. HRQoL in children along 7 dimensions was assessed using a standardised questionnaire (PEDQoL); information on the medical case history of each respondent was also collected...
July 20, 2017: Quality of Life Research
https://www.readbyqxmd.com/read/28702716/can-the-pulmonary-artery-wedge-pressure-be-used-reliably-as-a-surrogate-for-the-left-atrial-mean-pressure-in-pre-fontan-evaluation
#5
Bassel Mohammad Nijres, Ra-Id Abdulla, Sawsan Awad, Joshua Murphy
The correlation between mean pulmonary artery wedge pressure (PAWP) and left atrial mean pressure (LAMP) has been poorly studied in patients with single ventricle (SV) physiology (Bernstein et al. in Pediatr Cardiol 33: 15-20 2012). The aim of this study is to determine if the PAWP can be used safely as a surrogate to the LAMP to calculate the pulmonary vascular resistance (PVR) during the pre-Fontan evaluation. Also, we aimed to understand if the presence of significant systemic-to-pulmonary collaterals (SPCs) is a confounding factor for accurate estimation of the LAMP...
July 12, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28651677/influence-of-weight-at-the-time-of-first-palliation-on-survival-in-patients-with-a-single-ventricle
#6
Sushitha Surendran, T K Susheel Kumar, Ben Tansey, Jerry Allen, David Zurakowski, Umar Boston, Samir H Shah, Christopher J Knott-Craig
BACKGROUND: Numerous advances in surgical techniques and understanding of single-ventricle physiology have resulted in improved survival. We sought to determine the influence of various demographic, perioperative, and patient-specific factors on the survival of single-ventricle patients following stage 1 palliation at our institution. METHODS: We conducted a retrospective study of all single-ventricle patients who had undergone staged palliation at our institution over an 8-year period...
June 27, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28651669/gastrointestinal-haemorrhage-due-to-lymphangiectasia-caused-by-protein-losing-enteropathy-in-the-fontan-circulation
#7
Pauline Gras, Frédéric Gottrand, François Godart
We report the case of a 14-year-old boy with severe protein-losing enteropathy after Fontan surgery that led to lymphangiectasia, which caused gastrointestinal haemorrhage and required invasive treatment to stop the bleeding. Through this case and a review of the literature on protein-losing enteropathy after Fontan surgery, we highlight a rare and serious presentation of the disease and the difficulties of diagnosis and management.
June 27, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28649147/multiblock-high-order-large-eddy-simulation-of-powered-fontan-hemodynamics-towards-computational-surgery
#8
Yann T Delorme, Mark D Rodefeld, Steven H Frankel
Children born with only one functional ventricle must typically undergo a series of three surgeries to obtain the so-called Fontan circulation in which the blood coming from the body passively flows from the Vena Cavae (VCs) to the Pulmonary Arteries (PAs) through the Total Cavopulmonary Connection (TCPC). The circulation is inherently inefficient due to the lack of a subpulmonary ventricle. Survivors face the risk of circulatory sequelae and eventual failure for the duration of their lives. Current efforts are focused on improving the outcomes of Fontan palliation, either passively by optimizing the TCPC, or actively by using mechanical support...
January 17, 2017: Computers & Fluids
https://www.readbyqxmd.com/read/28599384/adolescent-and-parent-perspectives-prior-to-involvement-in-a-fontan-transition-program
#9
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/28583678/hospital-inpatient-costs-for-single-ventricle-patients-surviving-the-fontan-procedure
#10
MULTICENTER STUDY
Li Huang, Chris Schilling, Kim M Dalziel, Siyu Xie, David S Celermajer, John J McNeil, David Winlaw, Tim S Hornung, Dorothy J Radford, Leeanne E Grigg, Andrew Bullock, Gavin R Wheaton, Robert N Justo, Jayne Blake, Rachel Bishop, Karin Du Plessis, Yves d'Udekem
We estimated the inpatient resource use for a Fontan patient from birth to adulthood and explored factors that might induce cost differences (2014 US dollar). Inpatient costing records from 4 hospitals with greatest numbers of Fontan patients in Australia and New Zealand were linked with the Fontan registry database. Inpatient records between July 1995 and September 2014 for 420 Fontan patients were linked, and the most frequent primary diagnoses were hypoplastic left heart syndrome (20.7%), tricuspid atresia (19...
August 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28579257/pregnancy-after-fontan-kreutzer-surgery
#11
Marta Noris Mora, Silvia Escribà Bori, M Ángeles de la Fuente Sánchez, Fernando García Algas, Inmaculada Sánchez Pérez, Andrés Grau Sepúlveda
No abstract text is available yet for this article.
June 1, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28571639/longitudinal-outcomes-of-patients%C3%A2-with%C3%A2-single-ventricle-after%C3%A2-the%C3%A2-fontan%C3%A2-procedure
#12
MULTICENTER STUDY
Andrew M Atz, Victor Zak, Lynn Mahony, Karen Uzark, Nicholas D'agincourt, David J Goldberg, Richard V Williams, Roger E Breitbart, Steven D Colan, Kristin M Burns, Renee Margossian, Heather T Henderson, Rosalind Korsin, Bradley S Marino, Kaitlyn Daniels, Brian W McCrindle
BACKGROUND: Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking. OBJECTIVES: This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. METHODS: Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9...
June 6, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28571224/waldmann-s-disease-primary-intestinal-lymphangiectasia-with-atrial-septal-defect
#13
Shrikiran Aroor, Suneel Mundkur, Shravan Kanaparthi, Sandeep Kumar
Waldmann's disease or Primary Intestinal Lymphangiectasia (PIL) is a rare disorder of gastrointestinal tract characterized by dilated lymphatics and widened villi causing leakage of lymph into intestinal lumen. Loss of lymph leads to hypoalbuminemia, hyogammaglobulinemia and lymphopenia. Secondary lymphangiectasia occurs secondary to an elevated lymphatic pressure as in lymphoma, systemic lupus erythematosus, constrictive pericarditis, cardiac surgeries (Fontan's procedure), inflammatory bowel disease and malignancies...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28545820/extracardiac-conduit-fontan-outcome-data-in-early-adulthood
#14
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/28532389/the-use-of-macitentan-in-fontan-circulation-a-case-report
#15
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/28520542/hybrid-palliation-for-interrupted-aortic-arch-with-small-aortic-valve
#16
Yoshimasa Uno, Ayumu Masuoka, Kentarou Hotoda, Toshiyuki Katogi, Takaaki Suzuki
OBJECTIVES: Open heart surgery for interrupted aortic arch in the neonatal period is still a high-risk procedure related in part to patient factors such as low birth weight, other morphologic anomalies, and, especially, small aortic valve size. Recently, we performed hybrid palliation with bilateral pulmonary artery banding and ductal stenting as the first-stage palliation for such cases. In this study, the outcomes of this procedure were examined. METHODS: Six cases of interrupted aortic arch with a small aortic valve underwent the hybrid procedure in the neonatal period in our institute from 2010 to 2015 (mean age: 6...
May 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28500413/impaired-pulmonary-function-is-an-additional-potential-mechanism-for-the-reduction-of-functional-capacity-in-clinically-stable-fontan-patients
#17
Aída L R Turquetto, Luiz F Canêo, Daniela R Agostinho, Patrícia A Oliveira, Maria Isabel C S Lopes, Patrícia F Trevizan, Frederico L A Fernandes, Maria A Binotto, Gabriela Liberato, Glaucia M P Tavares, Rodolfo A Neirotti, Marcelo B Jatene
Central factors negatively affect the functional capacity of Fontan patients (FP), but "non-cardiac" factors, such as pulmonary function, may contribute to their exercise intolerance. We studied the pulmonary function in asymptomatic FP and its correlations with their functional capacity. Pulmonary function and cardiopulmonary exercise tests were performed in a prospective study of 27 FP and 27 healthy controls (HC). Cardiovascular magnetic resonance was used to evaluate the Fontan circulation. The mean age at tests, the mean age at surgery, and the median follow-up time of FP were 20(±6), 8(±3), and 11(8-17) years, respectively...
May 12, 2017: Pediatric Cardiology
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
#18
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/28472306/neuro-developmental-outcome-in-single-ventricle-patients-is-the-norwood-procedure-a-risk-factor
#19
Ina Rotermann, Jana Logoteta, Janine Falta, Philip Wegner, Olaf Jung, Peter Dütschke, Jens Scheewe, Hans-Heiner Kramer, Jan Hinnerk Hansen
OBJECTIVES: Complex neonatal surgery is considered a risk factor for neuro-developmental impairment in single-ventricle patients. Neuro-developmental outcome was compared between preschool-aged Fontan patients who underwent a Norwood procedure and single-ventricle patients not requiring neonatal surgery with cardiopulmonary bypass. METHODS: Verbal, performance and full-scale intelligence quotient (IQ) were evaluated with the Wechsler Preschool and Primary Scale of Intelligence...
May 2, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28470999/access-to-the-native-atria-following-conduit-total-cavopulmonary-anastomosis
#20
Robert L Petersen, Saar Danon, Saadeh Jureidini
OBJECTIVES: We describe the use of trans-thoracic and trans-conduit puncture to access the atria and perform interventional procedures in patients who have undergone conduit total cavopulmonary anastomosis. BACKGROUND: Catheter access to the atria following intra or extra-cardiac Fontan is desirable when there is a need for trans-atrial interventions. METHODS: Between 2009 and 2014, 5 patients ages 7 to 28 years underwent this approach; three trans-thoracic and 2 trans-conduit punctures...
May 4, 2017: Catheterization and Cardiovascular Interventions
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