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https://www.readbyqxmd.com/read/29198937/cardiopulmonary-exercise-testing-in-the-evaluation-of-liver-disease-in-adults-who-have-had-the-fontan-operation
#1
Anushree Agarwal, Colin Cunnington, Aarthi Sabanayagam, Lucas Zier, Charles E McCulloch, Ian S Harris, Elyse Foster, Dougal Atkinson, Angela Bryan, Petra Jenkins, Jaspal Dua, Michael J Parker, Devinda Karunaratne, John A Moore, Jeffrey Meadows, Bernard Clarke, Johann Andreas Hoschtitzky, Vaikom Subramanian Mahadevan
BACKGROUND: Liver disease (LD) is a long-term complication in patients with a single ventricle who have had the Fontan operation. A decline in cardiopulmonary exercise testing (CPET) variables is associated with increased risk of hospitalization, but its association with LD is unknown. AIM: To determine the association between CPET variables and LD in adults who have had the Fontan operation. METHODS: We retrospectively reviewed the medical records from two tertiary institutions...
November 30, 2017: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/29198765/increased-pituitary-volumes-in-children-after-fontan-operation-congestion-in-the-other-portal-circulation
#2
Jun Muneuchi, Yusaku Nagatomo, Seigo Okada, Chiaki Iida, Hiromitsu Shirozu, Yuichiro Sugitani, Mamie Watanabe
We performed brain magnetic resonance imaging in 40 patients after the Fontan procedure and 40 control subjects. Pituitary volumes in patients after Fontan were significantly larger than those in the control subjects (472 [425-527] vs 257 [182-311]; P < .0001), and were significantly related to central venous pressure.
November 29, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29195575/twenty-five-year-outcomes-of-the-lateral-tunnel-fontan-procedure
#3
Thomas G Wilson, William Y Shi, Ajay J Iyengar, David S Winlaw, Rachael L Cordina, Gavin R Wheaton, Andrew Bullock, Thomas L Gentles, Robert G Weintraub, Robert N Justo, Leeanne E Grigg, Dorothy J Radford, Yves d'Udekem
The objective of this study was to characterize late outcomes of the lateral tunnel (LT) Fontan procedure. The outcomes of all patients who underwent an LT Fontan procedure in Australia and in New Zealand were analyzed. Original files were reviewed and outcomes data were obtained through a binational registry. Between 1980 and 2014, a total of 301 patients underwent an LT Fontan procedure across 6 major centers. There were 13 hospital mortalities, 21 late deaths, 8 Fontan conversions and revisions, 8 Fontan takedowns, and 4 heart transplantations...
2017: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29193758/haemodynamic-impact-of-pulmonary-vasodilators-on-single-ventricle-physiology
#4
Biagio Castaldi, Giulia Bordin, Massimo Padalino, Elena Cuppini, Vladimiro Vida, Ornella Milanesi
INTRODUCTION: The Fontan procedure is the palliative procedure for single ventricle physiology. Pulmonary resistance plays a key role in the success of this operation. There are conflicting data concerning the impact of pulmonary vasodilators on survival and functional capacity among Fontan patients. AIM: The aim of this retrospective, single-centre, non-randomised study was to investigate the potential effect of pulmonary vasodilators on pulmonary vasculature in Fontan patients...
November 28, 2017: Cardiovascular Therapeutics
https://www.readbyqxmd.com/read/29181796/risk-factors-for-an-elevated-ventricular-end-diastolic-pressure-prior-to-the-fontan-operation
#5
Matthew C Schwartz, Michael A Brock, David Nykanen, William DeCampli
Systemic ventricular end-diastolic pressure (SVEDP) is an important determinant of pulmonary artery pressure in those with a Fontan circulation. Predictors of an elevated SVEDP have been incompletely identified in this population. All who underwent the Fontan operation at our center between 1/2009 and 12/2013 were retrospectively identified. SVEDP at the pre-Fontan catheterization and other patient variables were extracted. We identified 61 patients. Pre-Fontan SVEDP was positively associated with systemic ventricular systolic pressure (β = 0...
November 27, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29178522/direct-hyperbilirubinemia-in-infants-with-congenital-heart-disease
#6
Jun Fujishiro, Masahiko Sugiyama, Tetsuya Ishimaru, Miho Watanabe, Kaori Sato, Noriko Hoshino, Chizue Uotani, Mai Kutsukake, Yoichiro Hirata, Akira Oka
BACKGROUND: An association between congenital heart disease (CHD) and infantile cholestasis, a key finding for the diagnosis of biliary atresia (BA), remains unknown. The aim of this study was to investigate the characteristics of direct hyperbilirubinemia (D-HB) in infants with CHD. METHODS: All neonates who were admitted to our hospital and diagnosed with CHD in 2015 and 2016 were included. D-HB (direct bilirubin≧ 2.0 mg/dL) within 60 days of age and other clinical parameters were retrospectively reviewed...
November 27, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29174423/posterior-spinal-fusion-for-scoliosis-after-fontan-procedure-a%C3%A2-case%C3%A2-series
#7
Masashi Uehara, Jun Takahashi, Yohei Akazawa, Shugo Kuraishi, Shota Ikegami, Toshimasa Futatsugi, Hiroki Oba, Hiroyuki Kato
BACKGROUND: As the surgical treatment of scoliosis after a Fontan procedure is very challenging due to the risk of various perioperative complications, case reports are scarce. We herein describe three patients who were successfully treated for scoliosis following a Fontan procedure and discuss their clinical and radiological outcomes. METHODS: We retrospectively reviewed three cases of scoliosis treated by posterior spinal fusion after a Fontan procedure. RESULTS: Mean preoperative major curve Cobb angle was 83...
November 23, 2017: Journal of Orthopaedic Science: Official Journal of the Japanese Orthopaedic Association
https://www.readbyqxmd.com/read/29168549/first-polish-analysis-of-the-treatment-of-advanced-heart-failure-in-children-with-the-use-of-berlinheart-excor-mechanical-circulatory-support
#8
Szymon Pawlak, Roman Przybylski, Janusz Skalski, Joanna Śliwka, Andrzej Kansy, Adam Grzybowski, Arkadiusz Wierzyk, Jacek Białkowski, Bohdan Maruszewski, Marian Zembala
BACKGROUND: The treatment of advanced heart failure in children and infants poses a serious management problem. Heart failure in that patient group is usually of congenital etiology. The treatment schedules for pediatric patients are in most cases adapted from the guidelines for treatment of adults. Up to 2009, the treatment of that extremely difficult group of patients was limited to pharmacological therapy and occasional heart transplantations. Constantly increasing problems with recruiting donors, especially for the pediatric group, contribute to the fact that mechanical support with the use of ventricular assist devices is for many children the only chance to survive the period of waiting for a heart donor...
November 23, 2017: Kardiologia Polska
https://www.readbyqxmd.com/read/29098349/vortex-flow-in-the-right-atrium-surrogates-supraventricular-arrhythmia-and-thrombus-after-atriopulmonary-connection-type-fontan-operation-vortex-flow-analysis-using-conventional-cine-magnetic-resonance-imaging
#9
Yumi Shiina, Kei Inai, Tatsunori Takahashi, Yamato Shimomiya, Umiko Ishizaki, Kenji Fukushima, Michinobu Nagao
We developed a novel imaging technique, designated as vortex flow (VF) mapping, which presents a vortex flow visually on conventional two-dimensional (2D) cine MRI. Using it, we assessed circumferential VF patterns and influences on RA thrombus and supraventricular tachycardia (SVT) in AP connection-type Fontan circulation. Retrospectively, we enrolled 27 consecutive patients (25.1 ± 9.2 years) and 7 age-matched controls who underwent cardiac MRI. Conventional cine images acquired using a 1.5-Tesla scanner were scanned for axial and coronal cross section of the RA...
November 2, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29096870/hypoplastic-left-heart-syndrome-is-not-a-predictor-of-worse-intermediate-mortality-post-fontan
#10
Billie-Jean Martin, Kandice Mah, Luke Eckersley, Joyce Harder, Charissa Pockett, Daryl Schantz, John Dyck, Mohammed Al Aklabi, Ivan M Rebeyka, David B Ross
BACKGROUND: An increasing proportion of those living with single ventricle physiology have hypoplastic left heart syndrome (HLHS). Our objective was to assess the association between HLHS and outcomes post Fontan operation. METHODS: All pediatric patients who underwent a Fontan procedure at the University of Alberta between 1996 and 2016 were included. Follow-up clinical data collected included early and late surgical or catheter reintervention, echocardiography, and long-term transplant-free survival...
October 30, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29084189/-multiorgan-malfunction-after-fontan-operation-in-adult-patients
#11
Monika Smaś-Suska, Paweł Iwaszczuk, Beata Róg, Piotr Weryński, Maria Olszowska, Piotr Podolec, Lidia Tomkiewicz-Pająk
Although 85% of patients with univentricular heart after Fontan procedure survive twenty years after operation, the procedure alone seems to be an inadequate treatment as a permanent clinical solution. Patients with a "Fontan physiology" additionally have to face a various extra-cardiac complications, including thyroid, liver and kidney dysfunction, which are not only potentially life-threatening, but also can potentiate the circulatory insufficiency. AIM: The aim of the study was to assess a multiorgan dysfunction in adult patients after Fontan operation in long term follow-up and compare to healthy controls...
October 23, 2017: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29064568/utility-of-preoperative-holter-monitoring-in-children-undergoing-extracardiac-conduit-fontan-operation
#12
Jun Sasaki, Michael M Lopez, Sherrie J Baysa, Ronald J Kanter, Anthony F Rossi
BACKGROUND: In patients undergoing extracardiac conduit Fontan (ECF) who require postoperative pacing, epicardial leads are usually required because of anatomical constraints. If indicated, these could be conveniently placed at the time of ECF. We have routinely performed ambulatory 24-hour Holter monitoring before ECF to determine the presence or absence of preoperative sinus node dysfunction, in attempt to avoid repeat sternotomy at a later time. METHODS: We performed a retrospective study of all patients undergoing ECF from January 2000 to December 2015...
October 24, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29054235/acquired-cor-triatriatum-sinister-after-the-fontan-operation-and-successful-resection-in-a-child
#13
S Javed Zaidi, Rishi R Adhikari, Chawki El-Zein, Saad Siddiqui
Cor triatriatum sinister is a rare congenital lesion encountered in children. It consists of a fibromuscular membrane that separates the left atrium into two chambers resulting in a triatrial heart and often occurs with other structural cardiac anomalies. The acquired form is uncommon and has only been reported after orthotopic heart transplantation or as a complication of infective endocarditis in adults. These cases were mostly because of hypertrophied atrial tissue or suture lines and torsion of the atrium...
November 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29049818/dor-procedure-for-pulmonary-atresia-with-intact-ventricular-septum-in-an-infant
#14
Yoonjin Kang, Jae Gun Kwak, Eung Rae Kim, Woong-Han Kim
Pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation confers a high risk for myocardial infarction and complications of ischaemia, including left ventricle aneurysms. We describe an infant with pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation and a left ventricle apical aneurysm who successfully underwent the Dor procedure and a subsequent Fontan operation. Surgery for postinfarction left ventricle aneurysm can be considered in infants...
September 2, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29035429/where-is-the-optimal-fontan-hemodynamics
#15
REVIEW
Hideo Ohuchi
Fontan circulation is generally characterized by high central venous pressure, low cardiac output, and slightly low arterial oxygen saturation, and it is quite different from normal biventricular physiology. Therefore, when a patient with congenital heart disease is selected as a candidate for this type of circulation, the ultimate goals of therapy consist of 2 components. One is a smooth adjustment to the new circulation, and the other is long-term circulatory stabilization after adjustment. When either of these goals is not achieved, the patient is categorized as having "failed" Fontan circulation, and the prognosis is dismal...
September 18, 2017: Korean Circulation Journal
https://www.readbyqxmd.com/read/29016830/a-stepwise-approach-to-conduit-puncture-for-electrophysiological-procedures-in-patients-with-fontan-circulation
#16
Jae-Sun Uhm, Nam Kyun Kim, Hee Tae Yu, Pil-Sung Yang, Jung Ok Kim, Tae-Hoon Kim, Mi Kyoung Song, Sang-Yun Lee, Boyoung Joung, Hui-Nam Pak, Jae Young Choi, Jo Won Jung, Moon-Hyoung Lee
Aims: In patients with Fontan circulation, the conduit may be punctured for electrophysiological procedures. We evaluated the feasibility and safety of a stepwise approach to conduit puncture in adults who have undergone Fontan operation. Methods and results: We included 13 consecutive patients with lateral tunnel or extracardiac conduit Fontan circulation [median age (interquartile range), 24.0 (16.0-25.0) years; seven men] who had undergone electrophysiological procedures...
June 14, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28988309/durable-benefit-of-particle-occlusion-of-systemic-to-pulmonary-collaterals-in-select-patients-after-superior-cavopulmonary-connection
#17
Michael L O'Byrne, David N Schidlow
Systemic to pulmonary arterial collaterals (SPC) are commonly found in patients undergoing staged operative palliation for single ventricle heart disease. Occlusion of SPC as part of pre-Fontan catheterization has been shown to improve hemodynamics acutely. Anecdotally, the effect of this intervention appears to be transient, and to our knowledge there is no data supporting its durability in these patients. Between 1/1/2016 and 5/1/2017, 24 children underwent Glenn operations at our institution. Of these, 3 patients had signs and symptoms deteriorating clinical status suggestive of volume overload in the period between their Glenn operation and Fontan completion, prompting heart catheterization...
October 7, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28976338/false-positive-perfusion-ventilation-spect-study-for-pulmonary-embolism-in-a-patient-with-fontan-circulation
#18
Emmanouil Panagiotidis
Fontan circulation is the consequence of an operation that results in the flow of systemic venous blood to the lungs without passing through a ventricle. An 18-year old man with a history of congenital heart disease surgically treated with Fontan circulation, presented with pleuritic chest pain and a raised D-dimer level. Perfusion/ventilation SPECT was performed to exclude the possibility of pulmonary embolism (PE) that showed unilateral reduced perfusion of the left lung with a mismatched right upper lobe defect, suspicious of PE...
October 3, 2017: Molecular Imaging and Radionuclide Therapy
https://www.readbyqxmd.com/read/28932977/hepatic-stiffness-using-shear-wave-elastography-and-the-related-factors-for-a-fontan-circulation
#19
Seong-Ook Kim, Sang-Yun Lee, So-Ick Jang, Soo-Jin Park, Hye-Won Kwon, Seong-Ho Kim, Chang-Ha Lee, Eun-Seok Choi, Seong-Kyu Cho, Sun-Hwa Hong, Yang-Min Kim
Hepatic problems related to a Fontan circulation have been highlighted and elastography using ultrasound is a non-invasive tool that can measure the severity of hepatic stiffness. We investigated the hepatic stiffness using shear wave elastography (SWE) and related factors in patients with a Fontan circulation. This study enrolled 64 patients with a Fontan circulation who underwent cardiac catheterization and abdominal ultrasound from 2011 to 2015. The correlation between the laboratory tests, hemodynamic factors by cardiac catheterization, and SWE was evaluated...
September 21, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28928608/intraocular-pressure-in-children-after-congenital-heart-surgery-a-single-center-study
#20
Sunali Goyal, Paul H Phillips, Lamonda A Corder, Michael J Robertson, Xiomara Garcia, Michael L Schmitz, Punkaj Gupta
BACKGROUND: The impact of varied cardiac physiologies on intraocular pressure (IOP) among children undergoing heart operations is unknown. AIM: The aim of this study was to determine the IOP among children with varying cardiovascular physiologies and varying hemodynamics after their heart operation. SETTING AND DESIGN: This was a prospective, observational study. MATERIALS AND METHODS: Patients ≤18 years undergoing congenital heart surgery were included in this study...
September 2017: Annals of Pediatric Cardiology
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