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Lorena Fontán, Qi Qiao, John M Hatcher, Gabriella Casalena, Ilkay Us, Matt Teater, Matthew Durant, Guangyan Du, Min Xia, Natalia Bilchuk, Spandan Chennamadhavuni, Giuseppe Palladino, Giorgio Inghirami, Ulrike Philippar, Hao Wu, David A Scott, Nathanael S Gray, Ari Melnick
The MALT1 paracaspase plays an essential role in Activated B-cell like Diffuse Large B cell Lymphoma (ABC DLBCL) downstream of B cell and Toll-like receptor pathway genes mutated in these tumors. Although MALT1 is considered to be a compelling therapeutic target, development of tractable and specific MALT1 protease inhibitors has thus far been elusive. Herein, we developed a target engagement assay that provides a quantitative readout for specific MALT1 inhibitory effects in living cells. This enabled a structure-guided medicinal chemistry effort culminating in the discovery of pharmacologically tractable irreversible substrate-mimetic compounds that bind the MALT1 active site...
July 19, 2018: Journal of Clinical Investigation
Weidan Chen, Ye Lu, Li Ma, Shengchun Yang, Yuansheng Xia, Minghui Zou, Xinxin Chen
OBJECTIVE: Apicocaval juxtaposition (ACJ) may complicate the selection of conduit route in patients with single ventricles when total cavopulmonary connection (TCPC) is performed. We reviewed our experience of pathway selection and evaluated the clinical results. METHODS: Of 128 patients who underwent TCPC at our hospital between January 2009 and April 2016, 31 with ACJ were included in this study. In 24 patients, the conduit was placed between the inferior vena cava (IVC) and the ipsilateral pulmonary artery...
July 12, 2018: Seminars in Thoracic and Cardiovascular Surgery
Alejandro López-Prieto, Lorena Rodríguez-López, Myriam Rincón-Fontán, Ana Belén Moldes, José Manuel Cruz
BACKGROUND: Recent studies have been proven that biosurfactants (BS) obtained in controlled fermentations have shown surfactant and antimicrobial properties. In this work a biosurfactant extract obtained from a raw agroindustrial stream of corn milling industry, was introduced in a drinkable probiotic yogurt containing Lactobacillus casei. RESULTS: The effect of the biosurfactant extract on the probiotic population was determined under different conditions of biosurfactant concentration, temperature and time...
July 12, 2018: Journal of the Science of Food and Agriculture
Jelle P G van der Ven, Eva van den Bosch, Ad J C C Bogers, Willem A Helbing
In patients with a functionally univentricular heart, the Fontan strategy achieves separation of the systemic and pulmonary circulation and reduction of ventricular volume overload. Contemporary modifications of surgical techniques have significantly improved survival. However, the resulting Fontan physiology is associated with high morbidity. In this review, we discuss the state of the art of the Fontan strategy by assessing survival and risk factors for mortality. Complications of the Fontan circulation, such as cardiac arrhythmia, thromboembolism, and protein-losing enteropathy, are discussed...
2018: F1000Research
Yajuan Zhang, Yabing Duan, Jun Yan, Qiang Wang, Shoujun Li, Haitao Xu
OBJECTIVE: The purpose of the study is to compare the effects of nesiritide on the discharge time and pleural effusion in children with total cavopulmonary connection (TCPC), and to provide a more reasonable clinical method for these children. METHODS: Forty-four who children underwent cavopulmonary connection between January 2016 and 2017 were retrospectively collected, and 5 children were excluded from analysis due to postoperative thrombosis or second Fontan surgery due to high pulmonary hypertension...
July 12, 2018: Pediatric Cardiology
Ghassen Cheniti, Xavier Iriart, Hubert Cochet, Nicolas Derval
No abstract text is available yet for this article.
July 9, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
Tatsunori Takahashi, Yumi Shiina, Michinobu Nagao, Kei Inai
The prognostic factors in patients with biventricular heart who underwent Fontan surgery remain unclear. This study wanted to assess the hypothesis that interventricular dyssynchrony evaluated by cardiac magnetic resonance imaging (MRI) can predict future cardiac events in patients with biventricular heart who have undergone Fontan surgery. We prospectively enrolled consecutive patients with biventricular Fontan circulation from 2003 to 2016, and performed protocolized cardiac MRI. We determined the stroke volume ratio (SVr) using the following formula to assess interventricular dyssynchrony: (stroke volume (SV) of the two whole ventricles)/(SV of the right ventricle + SV of the left ventricle), by tracing cine MRI data...
July 4, 2018: Heart and Vessels
Wuwan Wang, Xiankang Hu, Weiting Liao, Rutahoile Wh, David J Malenka, Xiaofang Zeng, Yunjing Yang, Panpan Feng, Li Wen, Wei Huang
No abstract text is available yet for this article.
January 1, 2018: Pulmonary Circulation
Anja Lehner, Ingram Schulze-Neick, Nikolaus A Haas
This report describes the first clinical experience with the new Occlutech Atrial Flow Regulator (AFR®) device for implementation of a late Fontan fenestration. The AFR® device secured a defined diameter of the fenestration without spontaneous re-occlusion of this extra-anatomic perforation. This ensured a permanent clinical improvement of our failing Fontan patient.
July 4, 2018: Cardiology in the Young
Eva R Hedlund, Henrik Ljungberg, Liselott Söderström, Bo Lundell, Gunnar Sjöberg
OBJECTIVES: The objective of this research was to study lung function, physical capacity, and effect of endurance training in children and adolescents after Fontan palliation compared with healthy matched controls. METHODS: Fontan patients (n=30) and healthy matched control patients (n=25) performed dynamic and static spirometry, and pulmonary diffusing capacity and maximal oxygen uptake tests, before and after a 12-week endurance training programme and at follow-up after 1 year...
July 4, 2018: Cardiology in the Young
Sunil J Ghelani, Steven D Colan, Nina Azcue, Ellen M Keenan, David M Harrild, Andrew J Powell, Tal Geva, Rahul H Rathod
BACKGROUND: Right ventricular (RV) morphology has been associated with adverse clinical outcomes in Fontan patients. The impact of RV versus left ventricular morphology on ventricular stress and strain in single ventricles is not well known. METHODS AND RESULTS: Cardiac magnetic resonance examinations in 193 patients with the Fontan circulation were retrospectively analyzed. Ventricular mass, volume, global circumferential and longitudinal strain, and global average end-systolic fiber stress were calculated using previously published methods...
July 2018: Circulation. Cardiovascular Imaging
Moon Sun Kim, Ja Kyoung Yoon, Seong Ho Kim, Ji Seok Bang, So Ick Jang, Sang Yoon Lee, Eun Young Choi, Su Jin Park, Hye Won Kwon
Purpose: The efficacy of percutaneous stent implantation for congenital heart disease (CHD) in Korea, where stent availability is limited, has not been determined. This study evaluated the acute and midterm results of stent implantation in different CHD subgroups. Methods: Stents were implanted in 75 patients with 81 lesions: (1) pulmonary artery stenosis (PAS) group, 56 lesions in 51 patients; (2) coarctation of the aorta (CoA) group, 5 lesions in 5 patients; (3) Fontan group, 13 lesions in 12 patients; (4) ductal stent group, 3 lesions in 3 patients; and (5) other CHD group, 4 lesions in 4 patients...
June 2018: Korean Journal of Pediatrics
R Vijayaraghavan, W Y Thay
Fontan and Baudet described the procedure in 1971 and was very useful to improve the quality of life in many complex cyanotic heart diseases. It has gone through various modifications since then to improve the outcome. The mortality was reported as 2.1% and survival rate of at 5 years, 10 years, 15 years and 20 years were 91%, 80%, 73%and 69% respectively. Though it was a useful palliative procedure to improve the quality of life, it has complications which may affect the morbidity and mortality like Protein loosing enteropathy ,reduced exercise capacity thromboembolism...
June 2018: Medical Journal of Malaysia
Kyle W Riggs, David L S Morales
No abstract text is available yet for this article.
June 2, 2018: Journal of Thoracic and Cardiovascular Surgery
Juan D Cano Sierra, Camilo F Mestra, Juan C Gelvez Nieto, Miguel Ronderos Dumit, Alberto García-Torres
The Fontan surgery involves the creation a conduit between the inferior vena cava and the right pulmonary artery. This conduit has a small fenestration that shunts the blood from right to left in case the pulmonary blood flow is limited; namely, if the pulmonary vascular resistance (PVR) is increased then the shunt is increased. Bacteria may bypass the pulmonary circulation and easily get access to the systemic circulation (bacteremia). We report the case of a patient that underwent Fontan surgery in 2010 and remained in a high-altitude city for 7 years, during this time he was asymptomatic until 2017 when he developed a brain abscess due to Streptococcus gordonii , a pathogen of dental plaque...
April 2018: Annals of Translational Medicine
Benjamin M Moore, Rachael L Cordina, Mark A McGuire, David S Celermajer
INTRODUCTION: Adults with congenital heart disease (CHD) are predisposed to arrhythmias, which can often be refractory to medical therapy. Sotalol is an attractive alternative antiarrhythmic to amiodarone in this younger patient population, given the latter's toxicity profile, but it may have proarrhythmic effects. We therefore aimed to assess the efficacy and safety of sotalol in adults with CHD. METHODS: We retrospectively assessed our adult CHD database for all patients ≥16 years old, with moderate to highly complex defects, who were prescribed sotalol between 2000 and 2017...
June 20, 2018: International Journal of Cardiology
Mercè Borràs Sans, Miguel Pérez-Fontán, Montserrat Martinez-Alonso, Auxiliadora Bajo, Àngels Betriu, José M Valdivielso, Elvira Fernández
[This corrects the article DOI: 10.1371/journal.pone.0186921.].
2018: PloS One
Geoffrey D Huntley, Abhishek J Deshmukh, Carole A Warnes, Suraj Kapa, Alexander C Egbe
Placement of an epicardial pacemaker system is often preferred over an endocardial system in patients who have undergone a Fontan operation, but data are limited on how these two systems perform over time in patients with Fontan palliation. We performed a retrospective review of adults with Fontan palliation who had pacemaker implantation and interrogation data at Mayo Clinic from 1994 to 2014. Lead parameters, pacing mode, and polarity were collected at the earliest device interrogation report. Clinic notes and device interrogation reports were reviewed at implantation, 6 months, and yearly after implantation to determine impedance, capture threshold (CT), and energy threshold (ET)...
June 14, 2018: Pediatric Cardiology
D E Segar, J R Maldonado, C G Brown, I H Law
Up to 10% of Fontan patients require pacemakers; an epicardial approach has historically been used. A transvenous approach can be used but carries risk of Fontan obstruction, thromboembolism, and can be technically challenging. The safety and efficacy of these approaches is not well described. The aim of this study was to compare epicardial and transvenous pacemaker outcomes in Fontan patients, specifically, device performance and adverse event rate. A retrospective review was performed on Fontan pacemaker patients followed at a single institution...
June 11, 2018: Pediatric Cardiology
Leigh C Reardon, Eugene C DePasquale, Jana Tarabay, Daniel Cruz, Hillel Laks, Reshma M Biniwale, Ronald W Busuttil, Fady M Kaldas, Sammy Saab, Robert S Venick, Jeannette P Lin, Ali Nsair, Mario C Deng, Abbas Ardehali, Martin Caderias, Amit Iygengar, Jamil A Aboulhosn
BACKGROUND: As the population of patients with a Fontan palliation grows so does, the number of patients with cardiac failure necessitating orthotopic heart transplant (OHT) and combined heart-liver transplant (CHLT). There is recent evidence that current era cardiac transplant in Fontan patients have improved outcomes, but most studies have a preponderance of pediatrics patients in their cohorts. We examine our institutional experience with adult OHT and CHLT transplantation for failed Fontan physiology...
June 26, 2018: Clinical Transplantation
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