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https://www.readbyqxmd.com/read/29779315/-improving-outcomes-of-patients-with-heterotaxy-and-functional-single-ventricle-a-10-year-follow-up-of-70-cases-in-a-single-institution
#1
F Cao, M H Zou, L Ma, Y S Xia, S C Yang, W D Chen, G D Huang, X X Chen
Objective: To review current-era palliation outcomes of patients with heterotaxy and functional single ventricle in a single institution. Methods: The clinical data of 70 consecutive patients with heterotaxy undergoing multistage single ventricle palliation in Guangzhou Women and Children's Medical Center from January 2008 to December 2017 were retrospectively reviewed, and the prognosis factors for mortality were analyzed. There were 53 male and 17 female patients.The median age was 13.3 months (range: 6 days to 150 months)...
May 1, 2018: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/29777280/primary-draining-vein-stenting-for-obstructive-total-anomalous-pulmonary-venous-connection-in-neonates-with-right-atrial-isomerism-and-functional-single-ventricle-improves-outcome
#2
Masataka Kitano, Takaya Hoashi, Takashi Kakuta, Kazuto Fujimoto, Akira Miyake, Ken-Ichi Kurosaki, Hazime Ichikawa, Isao Shiraishi
For neonates with right atrial isomerism (RAI), functional single ventricle (f-SV), and obstructive total anomalous pulmonary venous connection (TAPVC), primary TAPVC repair (TAPVCR) has a poor outcome. At our hospital, the survival rate at 1 year of such neonates undergoing primary TAPVCR between 1999 and 2010 (TAPVCR group) was 30% (3/10). Most deceased cases suffered from capillary leak syndrome and unstable pulmonary resistance after the surgeries. We sought to determine whether less invasive primary draining vein stenting (DVS) improved the outcome of these neonates...
May 18, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29755072/-primary-pulmonary-vein-stenosis-developed-after-bidirectional-glenn-procedure-report-of-two-cases
#3
Masatsugu Terada, Yukihiro Yoshimura, Yusuke Yamamoto, Akinori Hirano, Koichi Miyata, Naoya Fukushima, Hirotaka Oki, Masaru Miura
We experienced 2 cases of primary pulmonary vein stenosis(PVS),which developed after a bidirectional Glenn procedure was performed for complex heart defects with normal pulmonary venous return. Although the patients successfully underwent primary sutureless repair for left PVS, restenosis of the affected pulmonary veins occurred several months after surgery in both patients. Stent implantation followed by balloon angioplasty was performed for stent stenosis in 1 patient without effect. However, the patient later underwent a successful fenestrated Fontan procedure...
March 2018: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/29747936/pulmonary-screening-in-subjects-after-the-fontan-procedure
#4
Deborah R Liptzin, Michael V Di Maria, Adel Younoszai, Michael R Narkewicz, Sarah L Kelly, Kelly R Wolfe, Livia A Veress
OBJECTIVES: To review the pulmonary findings of the first 51 patients who presented to our interdisciplinary single-ventricle clinic after undergoing the Fontan procedure. STUDY DESIGN: We performed an Institutional Review Board-approved retrospective review of 51 patients evaluated following the Fontan procedure. Evaluation included history, physical examination, pulmonary function testing, and 6-minute walk. Descriptive statistics were used to describe the population and testing data...
May 7, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29736127/experimental-investigation-of-the-effect-of-non-newtonian-behavior-of-blood-flow-in-the-fontan-circulation
#5
Andrew L Cheng, Niema M Pahlevan, Derek G Rinderknecht, John C Wood, Morteza Gharib
The Fontan procedure for univentricular heart defects creates a unique circulation where all pulmonary blood flow is passively supplied directly from systemic veins. Computational simulations, aimed at optimizing the surgery, have assumed blood to be a Newtonian fluid without evaluating the potential error introduced by this assumption. We compared flow behavior between a non-Newtonian blood analog (0.04% xanthan gum) and a control Newtonian fluid (45% glycerol) in a simplified model of the Fontan circulation...
March 2018: European Journal of Mechanics. B, Fluids
https://www.readbyqxmd.com/read/29718154/early-extubation-is-associated-with-improved-early-outcome-after-extracardiac-total-cavopulmonary-connection-independently-of-duration-of-cardiopulmonary-bypass
#6
Stanislav Ovroutski, Peter Kramer, Sarah Nordmeyer, Mi-Young Cho, Matthias Redlin, Oliver Miera, Joachim Photiadis, Felix Berger
OBJECTIVES: Strict patient selection, short cardiopulmonary bypass (CPB) time and short mechanical ventilation are well-recognized aspects of optimizing the postoperative course after total cavopulmonary connection. In this study, we evaluated the influence of our early postoperative extubation strategy in our population of Fontan patients over the past 2 decades. METHODS: Early postoperative course was evaluated retrospectively in 211 consecutive patients, who were selected for total cavopulmonary connection in our institution between 1995 and 2015...
April 27, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29685556/pathophysiology-of-thrombosis-and-anticoagulation-post-fontan-surgery
#7
Chantal Attard, Joanna Huang, Paul Monagle, Vera Ignjatovic
The Fontan procedure has transformed the lives of children born with single-ventricle physiology, previously deemed inoperable. Worldwide, there are an increasing number of children with Fontan circulation, with the potential for survival into adulthood. Due to the abnormal circulation, Fontan patients have an increased risk of thromboembolic (TE) events, with up to 25% of events leading to death. Despite the importance of preventing TE events in this patient population, there is currently no clinical consensus on the optimal monitoring, thromboprophylaxis therapies, and treatment of these events...
April 14, 2018: Thrombosis Research
https://www.readbyqxmd.com/read/29672726/a-new-surgical-strategy-for-medically-uncontrolled-protein-losing-enteropathy-after-fontan-surgery
#8
Seul Gi Cha, Gi Beom Kim, Chung Noh, Woong Han Kim
Managing protein-losing enteropathy after Fontan surgery is challenging. We report a case of protein-losing enteropathy associated with Fontan circulation in a 14-year-old boy. He was treated medically for 2 years, without improvement until Fontan takedown and pulsatile bidirectional cavopulmonary shunting were performed.
April 16, 2018: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29666009/in-vitro-validation-of-a-self-driving-aortic-turbine-venous-assist-device-for-fontan-patients
#9
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
#10
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/29652434/the-columbus-of-cardiovascular-surgery-a-tribute-to-francis-fontan-1929-2018
#11
Magdalena Mazurak, Jacek Kusa, Szymon Orantek, Marian Zembala
No abstract text is available yet for this article.
2018: Kardiologia Polska
https://www.readbyqxmd.com/read/29627386/long-term-outcome-after-pulmonary-artery-banding-in-children-with-atrioventricular-septal-defects
#12
Edward Buratto, Brandon Khoo, Xin Tao Ye, Michael Daley, Christian P Brizard, Yves d'Udekem, Igor E Konstantinov
BACKGROUND: Patients with atrioventricular septal defect (AVSD) may require pulmonary artery banding (PAB), either as a part of a staged uni-ventricular palliation or to allow delayed bi-ventricular repair in those presenting with early heart failure. The long-term outcomes of PAB in children with AVSD have not been previously reported. METHODS: All children with AVSD who underwent PAB at a single institution were included in the study. Data were obtained from medical records and correspondence with general practitioners and cardiologists...
April 5, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29619223/respiratory-exacerbation-in-a-young-adult-with-cystic-fibrosis-and-tricuspid-atresia
#13
Jamie Wood, Abbey Sawyer, Siobhain Mulrennan, Andrew Bullock
Tricuspid atresia (TAt) is a complex congenital heart defect (CHD) characterized by the absence of the tricuspid valve and right ventricular hypoplasia requiring surgery in childhood, the Fontan procedure. We present a case of a 21-year-old male with TAt and cystic fibrosis (CF), who underwent a Fontan procedure in childhood, presenting to an adult CF clinic with severe deterioration in his respiratory status and multi-organ dysfunction associated with CF. This report describes problems associated with the management of a CF respiratory exacerbation and extrapulmonary manifestations of CF in the unique situation of a Fontan circulation, a circulation with absence of a subpulmonary ventricle and pulsatile pulmonary arterial blood flow where maintenance of systemic cardiac output is totally dependent on good respiratory function and low pulmonary artery pressures...
July 2018: Respirology Case Reports
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
#14
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/29521004/delayed-puberty-and-abnormal-anthropometry-and-its-associations-with-quality-of-life-in-young-fontan-survivors-a-multicenter-cross-sectional-study
#15
Shaji C Menon, Ragheed Al-Dulaimi, Brian W McCrindle, David J Goldberg, Ritu Sachdeva, Bryan H Goldstein, Thomas Seery, Karen C Uzark, Anjali Chelliah, Ryan Butts, Heather Henderson, Tiffanie Johnson, Richard V Williams
INTRODUCTION: We sought to evaluate the prevalence of delayed puberty and abnormal anthropometry and its association with quality of life (QoL) in young Fontan survivors. METHODS: This was a cross-sectional study at 11 Pediatric Heart Network centers. Demographic and clinical data, anthropomety, and Tanner stage were collected. Anthropometric measurements and pubertal stage were compared to US norms. QoL was assessed using Pediatric Quality of Life inventory (PedsQL)...
March 9, 2018: Congenital Heart Disease
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/29480841/evaluation-of-the-anatomic-and-hemodynamic-abnormalities-in-tricuspid-atresia-before-and-after-surgery-using-computational-fluid-dynamics
#17
Li-Jun Chen, Yu-Qi Zhang, Zhi-Rong Tong, Ai-Min Sun
Analysis of hemodynamics inside tricuspid atresia (TA) chamber is essential to the understanding of TA for optimal treatment. In this study, we introduced a combined computational fluid dynamics (CFD) to simulate blood flow in the left ventricle (LV) to study the diastolic flow changes in TA.Real-time 3-dimentional echocardiography loops (ECHO) were acquired in normal control group, in TA patients before surgery (pre-op group) and after surgery (post-op group). ECHO loops were reconstructed and simulated by CFD, the geometric, volumetric changes, and vortices in the LV were studies and compare among 3 groups...
January 2018: Medicine (Baltimore)
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/29469053/fontan-associated-liver-disease-a-review
#19
Luis Téllez, Enrique Rodríguez-Santiago, Agustín Albillos
Fontan-associated liver disease is a hepatic disorder arising from hemodynamic changes and systemic venous congestion following Fontan surgery. The histological changes produced in the liver are similar but not equivalent to those seen in other forms of cardiac liver disease. While the natural history of this form of liver disease is not well established, over time many Fontan patients develop portal hypertension-related complications such as ascites, variceal hemorrhage or encephalopathy. Fontan survivors also show an increased risk of hepatocellular carcinoma...
March 1, 2018: Annals of Hepatology
https://www.readbyqxmd.com/read/29457852/successful-management-of-living-donor-liver-transplantation-for-biliary-atresia-with-single-ventricle-physiology-from-peri-transplant-through-total-cavopulmonary-connection-a-case-report
#20
Yohei Yamada, Ken Hoshino, Takayuki Oyanagi, Ryohei Gatayama, Jun Maeda, Nobuyuki Katori, Yasushi Fuchimoto, Taizo Hibi, Masahiro Shinoda, Kentaro Matsubara, Hideaki Obara, Ryo Aeba, Yuko Kitagawa, Hiroyuki Yamagishi, Tatsuo Kuroda
Children with single ventricle physiology have complete mixing of the pulmonary and systemic circulations, requiring staged procedures to achieve a separation of these circulations, or Fontan circulation. The single ventricle physiology significantly increases the risk of mortality in children undergoing non-cardiac surgery. As liver transplantation for patients with single ventricle physiology is particularly challenging, only a few reports have been published. We herein report a case of successful LDLTx for an 8-month-old pediatric patient with biliary atresia, heterotaxy, and complex heart disease of single ventricle physiology...
May 2018: Pediatric Transplantation
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