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fontan surgery

Frank W DiPaola, Kurt R Schumacher, Caren S Goldberg, Joshua Friedland-Little, Aishwarya Parameswaran, Jonathan R Dillman
OBJECTIVES: Assess liver stiffness using ultrasound point shear wave elastography (US P-SWE) in children before and after the Fontan operation. METHODS: Eighteen children undergoing the Fontan operation were prospectively enrolled. Eight US P-SWE measurements were obtained from the right hepatic lobe before surgery, and at multiple postoperative time points. Temporally related inferior vena cava pressure (IVC) data was collected from medical records, when available...
October 17, 2016: European Radiology
Anitha Parthiban, Jami C Levine, Meena Nathan, Jennifer A Marshall, Girish S Shirali, Stephen D Simon, Steven D Colan, Jane W Newburger, Geetha Raghuveer
BACKGROUND: Postoperative echocardiography after congenital heart disease surgery is of prognostic importance, but variable image quality is problematic. We implemented a quality improvement bundle comprising of focused imaging protocols, procedural sedation, and sonographer education to improve the rate of optimal imaging (OI). METHODS: Predischarge echocardiograms were evaluated in 116 children (median age, 0.51 years; range, 0.01-5.6 years) from two centers after tetralogy of Fallot repair, arterial switch operation, and bidirectional Glenn and Fontan procedures...
October 11, 2016: Journal of the American Society of Echocardiography
Mineto Kamata, Corey Stiver, Aymen Naguib, Dmitry Tumin, Joseph D Tobias
OBJECTIVES: The objectives of this study were to evaluate the effect of ventricular morphology on perioperative outcomes during Fontan surgery. DESIGN: Retrospective cohort study. SETTING: Single standing, not-for-profit pediatric hospital. PARTICIPANTS: A total of 72 patients who underwent Fontan surgery using cardiopulmonary bypass without aortic cross-clamp between January 1, 2009 and December 31, 2014. INTERVENTIONS: None...
July 19, 2016: Journal of Cardiothoracic and Vascular Anesthesia
Sharon L Cheatham, Grace M Deyo
Hybrid stage I palliation combines cardiothoracic surgery and interventional transcatheter procedures for treatment of hypoplastic left heart syndrome. The approach is an alternative to the Norwood procedure, the traditional first stage of surgical palliation. Hybrid stage I palliation involves placing bilateral branch pulmonary artery bands and a patent ductus arteriosus stent through a median sternotomy, performed without cardiopulmonary bypass. The purpose of the bands is to control blood flow to the lungs and protect the pulmonary bed while the stent sustains systemic cardiac output...
October 2016: Critical Care Nurse
Eva Hlavackova, Martin Liska, Hana Jicinska, Jiri Navratil, Jiri Litzman
The Fontan operation or total cavopulmonal connection (TCPC) is a palliative surgical correction of rare and complex inborn cardiac malformations that are characterized by univentricular circulation. Protein-losing enteropathy (PLE) develops in 4-13% of patients after the Fontan procedure. Fontan-related PLE leads to secondary combined immunodeficiency marked by hypogammaglobulinemia and predominantly CD4+ lymphocytopenia. Here, we present 3 case reports of patients with secondary immunodeficiency after the Fontan operation...
September 30, 2016: International Archives of Allergy and Immunology
Jeremy P Moore, Blandine Mondésert, Michael S Lloyd, Stephen C Cook, Ali N Zaidi, Robert H Pass, Anitha S John, Frank A Fish, Kevin M Shannon, Jamil A Aboulhosn, Paul Khairy
BACKGROUND: Sudden cardiac death is a major contributor to mortality for adults with congenital heart disease. The subcutaneous implantable cardioverter-defibrillator (ICD) has emerged as a novel tool for prevention of sudden cardiac death, but clinical performance data for adults with congenital heart disease are limited. METHODS AND RESULTS: A retrospective study involving 7 centers over a 5-year period beginning in 2011 was performed. Twenty-one patients (median 33...
September 2016: Circulation. Arrhythmia and Electrophysiology
Sangeetha Viswanathan
This review attempts to answer the common questions faced by a clinician regarding thromboembolism and thromboprophylaxis in patients following Fontan surgery. The review is in an easy to understand question and answer format and discusses the currently available literature on the subject in an attempt to arrive at practical clinically relevant solutions. Patients who have undergone the Fontan operation are at a high risk for thromboembolism. Based on available evidence, there is a strong rationale for thromboprophylaxis...
September 2016: Annals of Pediatric Cardiology
Renee Margossian, Lynn A Sleeper, Gail D Pearson, Piers C Barker, Luc Mertens, Michael D Quartermain, Jason T Su, Girish Shirali, Shan Chen, Steven D Colan
BACKGROUND: Patients with functional single ventricles after the Fontan procedure have abnormal cardiac mechanics. The aims of this study were to determine factors that influence diastolic function and to describe associations of diastolic function with current clinical status. METHODS: Echocardiograms were obtained as part of the Pediatric Heart Network Fontan Cross-Sectional Study. Diastolic function grade (DFG) was assessed as normal (grade 0), impaired relaxation (grade 1), pseudonymization (grade 2), or restrictive (grade 3)...
September 10, 2016: Journal of the American Society of Echocardiography
Maryam Ghadimi Mahani, Prachi P Agarwal, Cynthia K Rigsby, Jimmy C Lu, Soudabeh Fazeli Dehkordy, Robyn A Wright, Adam L Dorfman, Rajesh Krishnamurthy
The total cavopulmonary connection (TCPC), or Fontan procedure, diverts systemic venous blood directly into the pulmonary arteries and is the palliative surgery of choice for patients with a wide variety of congenital heart diseases with single-ventricle physiologic characteristics. Pulmonary embolism and thrombosis are known complications and are among the major causes of morbidity and mortality in patients after TCPC. Magnetic resonance (MR) imaging is usually performed for postoperative evaluation of patients after single-ventricle repair; however, screening for thrombosis or embolism with MR imaging is not always feasible because of the emergent nature of the clinical presentation or because of artifacts from metallic devices or coils...
September 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Hiromu Miyake, Koji Fukumoto, Masaya Yamoto, Hiroshi Nouso, Masakatsu Kaneshiro, Mariko Koyama, Naoto Urushihara
Purpose Patients with asplenia syndrome (AS) are likely to have upper gastrointestinal tract malformations such as hiatal hernia. This report discusses the treatment of such conditions. Methods Seventy-five patients with AS underwent initial palliation in our institution between 1997 and 2013. Of these, 10 patients had hiatal hernia. Of the patients with hiatal hernia, 6 had brachyesophagus and 7 had microgastria. Results Of the 10 patients with hiatal hernia, 9 underwent surgery in infancy (7 before Glenn operation, 2 after Glenn operation)...
September 8, 2016: European Journal of Pediatric Surgery
Fred M Wu, Michael G Earing, Jamil A Aboulhosn, Melanie E Johncilla, Michael N Singh, Robert D Odze, Chinweike Ukomadu, Kimberlee Gauvreau, Michael J Landzberg, Anne Marie Valente
BACKGROUND: Hepatic fibrosis is highly prevalent in individuals with Fontan circulation. FibroSure (LabCorp, Burlington, NC) and hyaluronic acid (HA) have been validated for assessment of hepatic fibrosis in several forms of liver disease. We sought to determine whether these tests could identify Fontan patients with advanced hepatic fibrosis or cirrhosis. METHODS: Subjects who had liver biopsy and FibroSure or HA testing within 6 months of biopsy were identified from the Alliance for Adult Research in Congenital Cardiology Fontan Liver Health study...
July 27, 2016: Journal of Heart and Lung Transplantation
Stephen H Gregory, Madhav Swaminathan, Yasmin Maisonave, Kelly A Machovec
The number of patients reaching adulthood after undergoing Fontan palliation for the repair of a congenital heart defect continues to increase. In this case report, we present the anesthetic management of a patient with a history of tricuspid atresia treated with palliative Fontan repair who had developed clinical evidence of Fontan failure. He presented with septic shock secondary to streptococcal toxic shock syndrome complicated by a loculated pleural effusion. He underwent open thoracic decortication under 1-lung ventilation...
October 15, 2016: A & A Case Reports
José Soro García, Montserrat Blanco Ramos, Eva María García Fontán, Miguel Ángel Cańizares Carretero, Ana González Pińeiro
Primary pulmonary synovial sarcoma is an extremely rare tumor with an unknown cause. The diagnosis is established after other primary lung malignancies or metastatic extrathoracic sarcoma have been excluded. We report the case of a 69-year-old man who presented with a well-defined mass in the right upper lobe on a chest X-ray. A video-assisted thoracoscopic surgery (VATS) right upper lobectomy was performed. Immunohistochemically, neoplastic cells were positive for vimentin, CD56 and Bcl-2, and focally positive for CD99, epithelial membrane antigen and cytokeratin 7 and 19...
June 2016: Kardiochirurgia i Torakochirurgia Polska, Polish Journal of Cardio-Thoracic Surgery
Zheng Jinghao, Luo Kai, Huang Yanhui, Zhu Zhongqun, Gao Botao, Du Xinwei, Xu Zhiwei, Liu Jinfen
Objective Ebstein anomaly is a rare type of tricuspid malformation. The present surgical methods to resolve this anomaly include tricuspid valvuloplasty, palliative surgery, and tricuspid valve replacement. The purpose of this study was to evaluate the short- and midterm outcomes of different surgical treatments among children with Ebstein anomaly. Methods This was a retrospective study of 136 Ebstein anomaly patients undergoing surgery at our institution from January, 2006 to August, 2015. This cohort included 118 patients receiving tricuspid valvuloplasty, 14 patients receiving palliative surgery and 4 patients receiving tricuspid valve replacement...
August 10, 2016: Thoracic and Cardiovascular Surgeon
Ashish H Shah, Eric M Horlick, Andreas Eicken, Jeremy D Asnes, Martin L Bocks, Younes Boudjemline, Allison K Cabalka, Thomas E Fagan, Stephan Schubert, Vaikom S Mahadevan, Danny Dvir, Mark Osten, Doff B McElhinney
BACKGROUND: The most common reason for reintervention after a Björk modification of the Fontan procedure, in which the right ventricle (RV) is incorporated into the pulmonary circulation by connecting the right atrial (RA) appendage to the RV directly or with an extra-anatomic graft, is obstruction or regurgitation of the RA-RV connection. Transcatheter implantation of a valved stent is an appealing option for the treatment of RA-RV conduit dysfunction in these patients. In the present study, we assessed early and intermediate results after transcatheter valve implantation within an obstructed or regurgitant RA-RV modified Fontan pathway...
July 28, 2016: Catheterization and Cardiovascular Interventions
Xiao-Ke Shang, Rong Lu, Xi Zhang, Chang-Dong Zhang, Shu-Na Xiao, Mei Liu, Bin Wang, Nian-Guo Dong
Fontan surgery is a widely used palliative procedure that significantly improves the survival period of patients with complex congenital heart disease (CHD). However, it does not decrease postoperative complication rate. Previous studies suggested that elevated mean pulmonary artery pressure (mPAP) and vascular resistance lead to decreased exercise tolerance and myocardial dysfunction. Therapy with endothelial receptor antagonists (Bosentan) has been demonstrated to improve the patients' prognosis. A double-blind, randomized controlled trial was performed to explore the efficacy of Bosentan in treating patients who underwent the Fontan procedure...
August 2016: Journal of Huazhong University of Science and Technology. Medical Sciences
Hoi Lam She, Arno A W Roest, Emmeline E Calkoen, Pieter J van den Boogaard, Rob J van der Geest, Mark G Hazekamp, Albert de Roos, Jos J M Westenberg
PURPOSE: To evaluate the inflow pattern and flow quantification in patients with functional univentricular heart after Fontan's operation using 4D flow magnetic resonance imaging (MRI) with streamline visualization when compared with the conventional 2D flow approach. METHOD: Seven patients with functional univentricular heart after Fontan's operation and twenty-three healthy controls underwent 4D flow MRI. In two orthogonal two-chamber planes, streamline visualization was applied, and inflow angles with peak inflow velocity (PIV) were measured...
July 18, 2016: Congenital Heart Disease
Masaya Sugimoto, Hideharu Oka, Aya Kajihama, Kouichi Nakau, Seiko Kuwata, Clara Kurishima, Hiroshi Azuma
BACKGROUND: The incidence of late liver complications such as fibrosis or cirrhosis has increased among patients who have undergone the Fontan procedure. Magnetic resonance elastography (MRE) recently emerged as a technique to clinically evaluate liver fibrosis. However, few reports have described its use in evaluating liver fibrosis in children with congenital heart disease (CHD). METHODS AND RESULTS: Fifty-seven children were examined and divided into four groups: 27 with CHD who underwent intracardiac repair (ICR); 16 with CHD who underwent the Fontan procedure (Fontan); 14 in a control group (control); and two with cirrhosis (cirrhosis)...
September 2016: Journal of Cardiology
Luca Deorsola, Enrico Aidala, Maria Teresa Cascarano, Andrea Valori, Gabriella Agnoletti, Carlo Pace Napoleone
OBJECTIVES: Hepatic damage in Fontan circulation setting is well known. Mainly represented by fibrosis, it is still poorly understood. Transient hepatic elastography (Fibro-Scan) is a useful tool to test liver stiffness and is commonly used in the evaluation of liver fibrosis. Unfortunately, the increase of inferior vena cava pressure consequent to total cavopulmonary connection can probably interfere with Fibro-Scan evaluation altering the result evaluation with the conventional scale...
October 2016: Interactive Cardiovascular and Thoracic Surgery
Khaled Abdelhady, Anas Taqatqa, Carlos Miranda, Sawsan Awad
Pre-Fontan coil closure of aorto-pulmonary collaterals decreases single-ventricle volume load and improves outcome. Coiling of right and left internal mammary arteries may present a challenge to cardiothoracic surgery teams caring for these patients when future coronary artery bypass grafting is needed for SV patients. The goal of this study was to determine the frequency of internal mammary artery coil closure in SV patients in a single tertiary care center. A retrospective review of all pediatric single-ventricle patients who underwent cardiac catheterization between March 2009 and October 2015 at Rush Center for Congenital Heart Disease was performed...
October 2016: Pediatric Cardiology
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