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https://www.readbyqxmd.com/read/29886663/-the-outcomes-of-operation-for-237-patients-with-ebstein-anomaly
#1
X Y Zhang, Q Y Wu, B Dong, H Y Li, M K Zhang, Y Q Jin
Objective: To evaluate the early and long-term outcomes cardiac surgery of patients with Ebstein anomaly. Methods: The clinic data of 237 patients with Ebstein anomaly received surgical procedures from March 2004 to December 2017 at Department of Cardiac Surgery, First Hospital of Tsinghua University was analyzed retrospectively. There were 105 male and 132 female patients with age of (19.4±16.7) years (ranging from 3 months to 64 years). The surgical procedures include anatomical repair in 188 patients, one and a half ventricle repair in 37 patients, tricuspid valve repair in 4 patients, tricuspid valve replacement in 10 patients, and Fontan procedure in 3 patients (total cavopulmonary connection in 2 patients; Glenn procedure in 1 patient)...
June 1, 2018: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/29868844/mid-term-result-of-atrioventricular-valve-replacement-in-patients-with-a-single-ventricle
#2
Koichi Sughimoto, Yasutaka Hirata, Norimichi Hirahara, Hiroaki Miyata, Takaaki Suzuki, Arata Murakami, Kagami Miyaji, Shinichi Takamoto
OBJECTIVES: Atrioventricular valve replacement is the last option to treat the atrioventricular valve regurgitation in single ventricle. This study investigates the mid-term outcomes of the atrioventricular valve replacement based on the Japan Cardiovascular Surgery Database registry. METHODS: From 2008 to 2014, 56 patients [34 males (61%) and 22 females (39%)] with a single ventricular circulation, underwent atrioventricular valve replacement. Questionnaires were collected to review operative data, mid-term mortality, morbidity and redo replacement...
June 2, 2018: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29861103/blunted-peripheral-blood-supply-and-underdeveloped-skeletal-muscle-in-fontan-patients-the-impact-on-functional-capacity
#3
Aida Luiza Ribeiro Turquetto, Marcelo Rodrigues Dos Santos, Ana Luiza Carrari Sayegh, Francis Ribeiro de Souza, Daniela Regina Agostinho, Patrícia Alves de Oliveira, Yarla Alves Dos Santos, Gabriela Liberato, Maria Angélica Binotto, Maria Concepcion Garcia Otaduy, Carlos Eduardo Negrão, Luiz Fernando Canêo, Fabio Biscegli Jatene, Marcelo Biscegli Jatene
BACKGROUND: Changes in circulatory physiology are common in Fontan patients due to suboptimal cardiac output, which may reduce the peripheral blood flow and impair the skeletal muscle. The objective of this study was to investigate the forearm blood flow (FBF), cross-sectional area (CSA) of the thigh and functional capacity in asymptomatic clinically stable patients undergoing Fontan surgery. METHODS: Thirty Fontan patients and 27 healthy subjects underwent venous occlusion plethysmography, magnetic resonance imaging of the thigh musculature and maximal cardiopulmonary exercise testing...
May 25, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29794380/successful-treatment-of-protein-losing-enteropathy-and-plastic-bronchitis-by-biphasic-cuirass-ventilation-in-a-patient-with-failing-fontan-circulation
#4
Seigo Okada, Jun Muneuchi, Yusaku Nagatomo, Kaori Nonaka, Chiaki Iida, Hiromitsu Shirouzu, Ryohei Matsuoka, Mamie Watanabe, Kunitaka Joo
We present a 16-year-old male patient with hypoplastic left heart syndrome who developed protein-losing enteropathy (PLE) and plastic bronchitis (PB) after a Fontan operation. He received medical therapies, including albumin infusion, unfractionated heparin, and high-dose anti-aldosterone therapy but could not obtain clinical relief. Biphasic cuirass ventilation (BCV) led to expectoration of bronchial casts and prompt resolution of PB. Notably, clinical symptoms related to PLE were dramatically improved after starting BCV...
May 23, 2018: International Heart Journal
https://www.readbyqxmd.com/read/29789917/comparison-of-clinical-profiles-in-patients-with-protein-losing-enteropathy-with-and-without-fontan-circulation
#5
Shin Ono, Hideo Ohuchi, Aya Miyazaki, Osamu Yamada
Protein-losing enteropathy (PLE) is a life-threatening complication in patients following the Fontan operation. However, PLE also develops in some patients with congenital heart disease (CHD) after biventricular repair (BVR). This study compared clinical profiles of PLE patients following the Fontan operation with those after BVR. We retrospectively reviewed clinical charts of postoperative CHD patients with PLE. The study population comprised 42 PLE patients (14BVR, 28Fontan). Postoperative follow-up period until onset was significantly shorter in the Fontan group than in the BVR group (14 ± 2 vs...
May 22, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29788322/outcomes-and-risk-factors-associated-with-tricuspid-valve-repair-in-children-with-hypoplastic-left-heart-syndrome
#6
Bahaaldin Alsoufi, Raina Sinha, Courtney McCracken, Janet Figueroa, Firat Altin, Kirk Kanter
OBJECTIVES: Tricuspid valve (TV) competence is important for successful palliation of hypoplastic left heart syndrome (HLHS). We report our experience with TV repair in HLHS patients with a focus on TV and right ventricular (RV) function and associated clinical outcomes. METHODS: From 2002 to 2012, 219 neonates with HLHS underwent the Norwood operation. Thirty patients who underwent TV repair at various stages comprised our current series cohort. Echocardiographic and clinical data were reviewed to determine the effectiveness of TV repair and outcomes of the patients...
May 19, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29780065/prediction-of-fontan-associated-liver-disease-using-a-novel-cine-magnetic-resonance-imaging-vortex-flow-map-in-the-right-atrium
#7
Umiko Ishizaki, Michinobu Nagao, Yumi Shiina, Kenji Fukushima, Tatsunori Takahashi, Yamato Shimomiya, Yuka Matsuo, Kei Inai, Shuji Sakai
BACKGROUND: Long-term hepatic dysfunction is an increasingly recognized complication of the Fontan operation for univentricular hearts. The purpose of this study was to determine whether Fontan-associated liver disease (FALD) could be predicted by flow dynamics in the right atrium (RA) of Fontan circulation.Methods and Results:Cardiac MRI and the serum levels of total bilirubin (TBil) and hyaluronic acid (HA) were analyzed in 36 patients who underwent an atriopulmonary connection type of Fontan operation...
May 18, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/29752389/pregnancy-in-women-with-a-fontan-circulation-a-systematic-review-of-the-literature
#8
Alvaro Garcia Ropero, Shankar Baskar, Jolien W Roos Hesselink, Andrea Girnius, Dominica Zentner, Lorna Swan, Magalie Ladouceur, Nicole Brown, Gruschen R Veldtman
BACKGROUND: The Fontan operation has provided life-saving palliation and adult survival for individuals born with single ventricle physiology. Many now seek advice about safe pregnancy. Little data are, however, available, consisting mainly of anecdotal experience and small series. This article seeks to review the published literature and identify lessons learnt from this collective experience. METHODS AND RESULTS: We conducted a systematic review to evaluate maternal and fetal outcomes of pregnancy in women with a Fontan circulation...
May 2018: Circulation. Cardiovascular Quality and Outcomes
https://www.readbyqxmd.com/read/29690942/deterioration-of-functional-abilities-in-children-surviving-the-fontan-operation
#9
M Florencia Ricci, Billie-Jean Martin, Ari R Joffe, Irina A Dinu, Gwen Y Alton, Gonzalo G Guerra, Charlene M T Robertson
Functional abilities are needed for activities of daily living. In general, these skills expand with age. We hypothesised that, in contrast to what is normally expected, children surviving the Fontan may have deterioration of functional abilities, and that peri-Fontan stroke is associated with this deterioration. All children registered in the Western Canadian Complex Pediatric Therapies Follow-up Program who survived a Fontan operation in the period 1999-2016 were eligible for inclusion. At the age of 2 years (pre-Fontan) and 4...
April 25, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29671004/impact-of-flow-differentials-according-to-cardiac-and-respiratory-cycles-on-three-types-of-fontan-operation
#10
Kee Soo Ha, Jae Young Choi, Jo Won Jung, Nam Kyun Kim
Few hemodynamic comparison studies on various types of Fontan operation have been reported. The objective of this study was to perform hemodynamic comparisons for flow size and volume in three types of Fontan operation: atriopulmonary connection (APC), lateral tunnel (LT), and extracardiac conduit (ECC). Forty patients with Fontan operation (8 with APC Fontan, 22 with LT Fontan, and 10 with ECC Fontan) were enrolled. Velocity time integral (VTI) and average peak velocity (APV) were assessed according to cardiac and respiratory cycles in SVC, IVC, hepatic vein, conduit, LPA, and RPA using direct intravenous Doppler echocardiography...
April 18, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29666009/in-vitro-validation-of-a-self-driving-aortic-turbine-venous-assist-device-for-fontan-patients
#11
Kerem Pekkan, Ibrahim Basar Aka, Ece Tutsak, Erhan Ermek, Haldun Balim, Ismail Lazoglu, Riza Turkoz
BACKGROUND: Palliative repair of single ventricle defects involve a series of open-heart surgeries where a single-ventricle (Fontan) circulation is established. As the patient ages, this paradoxical circulation gradually fails, because of its high venous pressure levels. Reversal of the Fontan paradox requires an extra subpulmonic energy that can be provided through mechanical assist devices. The objective of this study was to evaluate the hemodynamic performance of a totally implantable integrated aortic-turbine venous-assist (iATVA) system, which does not need an external drive power and maintains low venous pressure chronically, for the Fontan circulation...
March 11, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29662811/management-of-coronary-sinus-ostial-atresia-during-a-staged-operation-of-a-functional-single-ventricle
#12
Seung Ri Kang, Won Kyoun Park, Bo Sang Kwon, Jae Kon Ko, Hyun Woo Goo, Jeong-Jun Park
Coronary sinus ostial atresia (CSOA) with persistent left superior vena cava (LSVC) in the absence of an unroofed coronary sinus is a benign and rare anomaly that may be taken lightly in most instances. However, if overlooked in patients undergoing univentricular heart repair such as bidirectional Glenn or Fontan-type surgery, fatal surgical outcomes may occur due to coronary venous drainage failure. We report a case of CSOA with a persistent LSVC that was managed through coronary sinus rerouting during a total cavopulmonary connection, and provide a review of the literature regarding this rare anomaly...
April 2018: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29614908/two-cases-of-aortic-root-replacement-after-fontan-completion
#13
Kenji Suzuki, Takahiko Sakamoto, Narutoshi Hibino, Shigeyuki Aomi, Toshiharu Shin'oka, Kenji Yamazaki
Aortic root dilatation is a well-known complication in patients with congenital aortic valve malformation, tetralogy of Fallot, or a double outlet right ventricle. We report two rare patients who underwent composite graft replacement of the aortic root with a mechanical valve, the so-called Bentall-type operation, after Fontan completion. The pathological examination on the resected aortic wall revealed mucoid degeneration in tunica media and elastic fiber fragmentation. Our report emphasizes the need for close observation of these patients over a long-term period...
January 1, 2018: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29609888/impact-of-hemodynamics-and-fluid-energetics-on-liver-fibrosis-after-fontan-operation
#14
Phillip M Trusty, Zhenglun Wei, Jack Rychik, Pierre A Russo, Lea F Surrey, David J Goldberg, Mark A Fogel, Ajit P Yoganathan
OBJECTIVE: The staged Fontan procedure has shown promising short-term outcomes in patients with single ventricles. However, Fontan-associated liver disease is a marked problem as patients age. The purpose of this study is to investigate the relationship between hemodynamics and liver fibrosis in patients undergoing the Fontan. METHODS: A total of 33 patients undergoing the Fontan with liver fibrosis were included in this study. Cardiac magnetic resonance and phase-contrast cardiac magnetic resonance data, as well as catheterization measurements and liver biopsies, were obtained for each patient...
March 7, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29544424/association-of-habitual-activity-and-body-mass-index-in-survivors-of-congenital-heart-surgery-a-study-of-children-and-adolescents-with-tetralogy-of-fallot-transposition-of-the-great-arteries-and-fontan-palliation
#15
Michael L O'Byrne, Michael G McBride, Stephen Paridon, Elizabeth Goldmuntz
BACKGROUND: Obesity is associated with increased lifelong morbidity and reduced life span and is increasingly prevalent in the congenital heart disease population. Habitual exercise is an important aspect of a healthy lifestyle and primary prevention of obesity in the general population. The association between habitual activity and body mass index (BMI) has not been studied in children with congenital heart disease. METHODS: A cross-sectional analysis of two previously collected cohorts was performed, including participants 8 to 18 years old with tetralogy of Fallot, transposition of the great arteries, and single ventricle heart disease after a Fontan operation...
March 2018: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29492393/transplantation-in-the-single-ventricle-population
#16
Louise A Kenny, Fabrizio DeRita, Mohamed Nassar, John Dark, Louise Coats, Asif Hasan
The single ventricle patient population comprises the most complex cohort presenting to the cardiac transplant team, in terms of demographics, anatomic substrate, and unique physiology. It is also the most rapidly growing diagnostic group presenting for heart transplantation. In this manuscript, we aim to describe the changing landscape of transplantation in single ventricle conditions through reflection on our own institution's practice and experience, alongside contemporary literature review. Single ventricle patients are heterogeneous in terms of age, anatomic diagnosis and physiology according to surgical stage of repair...
January 2018: Annals of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/29492392/overview-of-adult-congenital-heart-transplants
#17
Roosevelt Bryant, David Morales
Transplantation for adult patients with congenital heart disease (ACHD) is a growing clinical endeavor in the transplant community. Understanding the results and defining potential high-risk patient subsets will allow optimization of patient outcomes. This review summarizes the scope of ACHD transplantation, the mechanisms of late ventricular dysfunction, the ACHD population at risk of developing heart failure, the indications and potential contraindications for transplant, surgical considerations, and post-transplant outcomes...
January 2018: Annals of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/29473337/acoustic-radiation-force-impulse-of-the-liver-after-fontan-operation-correlation-with-cardiopulmonary-exercise-test
#18
Michael V Di Maria, Lindsey Silverman, Adel K Younoszai, Mariana L Meyers, Kelley E Capocelli, Michael R Narkewicz
BACKGROUND: The current management paradigm for children with single ventricle heart disease involves a series of palliative surgeries, culminating in the Fontan operation. This physiology results in a high central venous pressure, and in the setting of single ventricle heart disease, results in hepatic injury and fibrosis over time. Fontan-associated liver disease (FALD) is universally present in this cohort, and the current gold standard for diagnosis remains biopsy. Noninvasive assessments of liver fibrosis, such as ultrasound with elastography or acoustic radiation force impulse (ARFI), has been utilized in this cohort...
February 23, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29470938/diagnostic-performance-of-mr-elastography-for-liver-fibrosis-in-children-and-young-adults-with-a-spectrum-of-liver-diseases
#19
Andrew T Trout, Rachel M Sheridan, Suraj D Serai, Stavra A Xanthakos, Weizhe Su, Bin Zhang, Daniel B Wallihan
Purpose To assess the diagnostic performance of magnetic resonance (MR) elastography-derived liver stiffness to detect liver fibrosis in a pediatric and young adult population with a spectrum of liver diseases. Materials and Methods This retrospective study included patients younger than 21 years of age who underwent MR elastography and liver biopsy within 3 months of one another between January 2012 and September 2016 for indications other than liver transplantation or Fontan palliation of congenital heart disease...
February 22, 2018: Radiology
https://www.readbyqxmd.com/read/29464511/computational-investigation-of-a-self-powered-fontan-circulation
#20
Marcus W Ni, Ray O Prather, Giovanna Rodriguez, Rachel Quinn, Eduardo Divo, Mark Fogel, Alain J Kassab, William M DeCampli
Children born with anatomic or functional "single ventricle" must progress through two or more major operations to sustain life. This management sequence culminates in the total cavopulmonary connection, or "Fontan" operation. A consequence of the "Fontan circulation", however, is elevated central venous pressure and inadequate ventricular preload, which contribute to continued morbidity. We propose a solution to these problems by increasing pulmonary blood flow using an "injection jet" (IJS) in which the source of blood flow and energy is the ventricle itself...
February 20, 2018: Cardiovascular Engineering and Technology
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