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Systemic to Pulmonary Artery Shunting for Palliation

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Visual liver assessment using Gd-EOB-DTPA-enhanced magnetic resonance imaging of patients in the early post-Fontan period.

Kimiko Nakajima, Mitsuru Seki, Shinitsu Hatakeyama, Shuhei Arai, Yuji Asami, Kensuke Tanaka, Kentaro Ikeda, Shinya Shimoyama, Tomio Kobayashi, Takashi Miyamoto, Yasunori Okada, Hirokazu Arakawa, Takumi Takizawa,

No imaging modality can be used to evaluate Fontan-associated liver disease (FALD). We retrospectively reviewed hepatic gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging (EOB-MRI) characteristics of patients within 1 year post-Fontan procedure, and we evaluated the association between hepatic imaging abnormalities and clinical parameters, including follow-up cardiac catheterization and ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):4909]

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Flow Profile Characteristics in Fontan Circulation are Associated with the Single Ventricle Dilation and Function: Principal Component Analysis Study.

Michal Schäfer, Benjamin S Frank, Stephen M Humphries, Kendall S Hunter, Katherine L Carmody, Roni Jacobsen, Max B Mitchell, James Jaggers, Matthew L Stone, Gareth J Morgan, Alex J Barker, Lorna Browne, D Dunbar Ivy, Adel Younoszai, Michael V Di Maria,

The Fontan circulation is characterized as a non-pulsatile flow propagation without a pressure generating ventricle. However, flow through the Fontan circulation still exhibits oscillatory waves as a result of pressure changes generated by the systemic single ventricle. Identification of discrete flow patterns through the Fontan circuit may be important to ... Read more >>

Am. J. Physiol. Heart Circ. Physiol. (American journal of physiology. Heart and circulatory physiology)
[2020, :]

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Effectiveness of Bidirectional Glenn Shunt Placement for Palliation in Complex Congenitally Corrected Transposed Great Arteries.

Kai Ma, Lei Qi, Zhongdong Hua, Keming Yang, Hao Zhang, Shoujun Li, Sen Zhang, Fengpu He, Guanxi Wang,

Surgery for complex congenitally corrected transposed great arteries is one of the greatest challenges in cardiovascular surgery. We report our experience with bidirectional Glenn shunt placement as a palliative procedure for complex congenitally corrected transposition. We retrospectively identified 50 consecutive patients who had been diagnosed with congenitally corrected transposition accompanied ... Read more >>

Tex Heart Inst J (Texas Heart Institute journal)
[2020, 47(1):15-22]

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Dobutamine stress testing in patients with Fontan circulation augmented by biomechanical modeling.

Bram Ruijsink, Konrad Zugaj, James Wong, Kuberan Pushparajah, Tarique Hussain, Philippe Moireau, Reza Razavi, Dominique Chapelle, Radomír Chabiniok,

Understanding (patho)physiological phenomena and mechanisms of failure in patients with Fontan circulation-a surgically established circulation for patients born with a functionally single ventricle-remains challenging due to the complex hemodynamics and high inter-patient variations in anatomy and function. In this work, we present a biomechanical model of the heart and circulation ... Read more >>

PLoS ONE (PloS one)
[2020, 15(2):e0229015]

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The effect of a valved small conduit on systemic ventricle-pulmonary artery shunt in the Norwood-type palliation.

Shuhei Fujita, Masaaki Yamagishi, Yoshinobu Maeda, Keiichi Itatani, Satoshi Asada, Hisayuki Hongu, Eijiro Yamashita, Yuji Takayanagi, Hiroki Nakatsuji, Hitoshi Yaku,

OBJECTIVES:The aim of this study was to clarify the impact of valved systemic ventricle-pulmonary artery (SV-PA) shunt on outcomes after stage-1 Norwood-type palliation (NP) compared with the modified Blalock-Taussig shunt. METHODS:Consecutive patients who underwent NP between 2003 and 2019 were enrolled. SV-PA shunts using the expanded polytetrafluoroethylene valved conduit were ... Read more >>

Eur J Cardiothorac Surg (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery)
[2020, :]

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Diastolic Heart Failure in Patients With the Fontan Circulation: A Review.

Werner Budts, William J Ravekes, David A Danford, Shelby Kutty,

The Fontan circulation, accomplished by direct surgical connection of the vena cavae to the pulmonary arteries, can be an effective palliation for patients with a single ventricle. However, failure of the Fontan circulation can result from mechanical obstruction, cardiac arrhythmias, increasing pulmonary vascular resistance, or deteriorating ventricular performance. Although systolic ... Read more >>

JAMA Cardiol (JAMA cardiology)
[2020, :]

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Fontan Circulation of the Next Generation: Why It's Necessary, What it Might Look Like.

Shelby Kutty, Marshall L Jacobs, W Reid Thompson, David A Danford,

J Am Heart Assoc (Journal of the American Heart Association)
[2020, 9(1):e013691]

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Hemodynamic Effects of A Simplified Venturi Conduit for Fontan Circulation: A Pilot, In Silico Analysis.

Fang Zhu, Guocheng Shi, Chen Wen, Qian Zhang, Qihua Fu, Jinlong Liu, Zhongqun Zhu, Huiwen Chen,

OBJECTIVES:To study the effects of a self-powered Fontan circulation in both idealized Fontan models and patient-specific models. METHODS:In silico, a conduit with a nozzle was introduced from ascending aorta into the anastomosis of superior vena cava and pulmonary artery. Computational fluid dynamics (CFD) simulation was applied to calculate the fluid ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):817]

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Epidural anesthesia for emergency cesarean section in a woman with Fontan circulation: A case report.

Pin Wu, Sheng-Mei Zhu, Yong-Xing Yao,

RATIONALE:Anesthetic management of pregnant women with Fontan circulation remains challenging. There are few reports that describe the anesthetic management of cesarean section after Fontan surgery. Here, we present a case of successful epidural anesthesia in a woman with Fontan circulation who required emergency cesarean section. PATIENT CONCERNS:A 29-year-old woman at ... Read more >>

Medicine (Baltimore) (Medicine)
[2020, 99(4):e18986]

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Survival, Neurocognitive, and Functional Outcomes After Completion of Staged Surgical Palliation in a Cohort of Patients With Hypoplastic Left Heart Syndrome.

Joseph Atallah, Gonzalo Garcia Guerra, Ari R Joffe, Gwen Y Bond, Sunjidatul Islam, M Florencia Ricci, Mohammed AlAklabi, Ivan M Rebeyka, Charlene M T Robertson, ,

Background Management of patients with hypoplastic left heart syndrome has benefited from advancements in medical and surgical care. Outcomes have improved, although survival and long-term functional and cognitive deficits remain a concern. Methods and Results This is a cohort study of all consecutive patients with hypoplastic left heart syndrome undergoing ... Read more >>

J Am Heart Assoc (Journal of the American Heart Association)
[2020, 9(4):e013632]

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Exercise Performance at Increased Altitude After Fontan Operation: Comparison to Normal Controls and Correlation with Cavopulmonary Hemodynamics.

Michael V Di Maria, Sonali S Patel, Julie C Fernie, Christopher M Rausch,

Exercise performance declines as patients who have undergone Fontan operation enter adolescence. However, the effect of altitude on functional capacity after Fontan remains inadequately studied. Our aim was to describe exercise performance in a cohort of patients with Fontan physiology living at increased altitude and compare to a normal control ... Read more >>

Pediatr Cardiol (Pediatric cardiology)
[2020, :]

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The pulmonary vasculature: Achilles heel of the Fontan circulation.

Floris-Jan S Ridderbos, Tjark Ebels, Rolf M F Berger,

Heart (Heart (British Cardiac Society))
[2019, 105(24):1922-1923]

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[Early- and midterm outcomes of pulmonary artery band as an initial palliation in patients with single ventricle associated with unrestricted pulmonary blood flow].

M H Zou, F Cao, L Ma, Y S Xia, S C Yang, W D Chen, W L Li, X X Chen,

Objective: To examine the early- and midterm outcomes of pulmonary artery banding as an initial palliation in patients with single ventricle associated with unrestricted pulmonary blood flow. Methods: Between January 2008 and December 2017, 49 patients with single ventricle and unrestricted pulmonary blood flow underwent pulmonary artery banding at Department ... Read more >>

Zhonghua Wai Ke Za Zhi (Zhonghua wai ke za zhi [Chinese journal of surgery])
[2019, 57(12):939-943]

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Stenting of modified Blalock-Taussig shunt in adult with palliated pulmonary atresia and ventricular septal defect: a case report.

Julia Illner, Holger Reinecke, Helmut Baumgartner, Gerrit Kaleschke,

Background:Adults with complex congenital heart disease palliated with systemic-to-pulmonary artery shunts have become rare and represent a particularly challenging patient group for the cardiologist. One of the complications and causes of severe clinical deterioration during long-term follow-up are progressive obstruction or total occlusion of the shunt. The risk for surgical ... Read more >>

Eur Heart J Case Rep (European heart journal. Case reports)
[2019, 3(4):1-4]

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Differences in Pulmonary and Systemic Flow Measurements by Cardiac Magnetic Resonance vs Cardiac Catheterization and Relation to Collateral Flow in Single Ventricle Patients.

Michael R Hart, Wendy Whiteside, Sunkyung Yu, Ray Lowery, Adam L Dorfman, Maryam Ghadimi Mahani, Prachi P Agarwal, Jimmy C Lu,

Both cardiac magnetic resonance (CMR) and cardiac catheterization (cath) may assess patients with single ventricle physiology prior to stage II or Fontan palliation. However, development of significant aortopulmonary collaterals may invalidate assumptions of the Fick method. We compared CMR and cath flow measurements and evaluated the relation to collateral flow. ... Read more >>

Pediatr Cardiol (Pediatric cardiology)
[2020, :]

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Fontan-Associated Liver Disease: Spectrum of Disease in Children and Adolescents.

Steven L Rathgeber, Orlee R Guttman, Anna F Lee, Christine Voss, Nicole M Hemphill, Richard A Schreiber, Kevin C Harris,

Background Information is evolving on liver disease in pediatric patients with Fontan physiology. The purpose of this investigation is to evaluate the spectrum of liver disease in a pediatric population of patients with Fontan physiology and evaluate transient elastography (TE) as a noninvasive marker of liver disease. Methods and Results ... Read more >>

J Am Heart Assoc (Journal of the American Heart Association)
[2020, 9(1):e012529]

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Abdominal Skeletal Muscle Index as a Potential Novel Biomarker in Adult Fontan Patients.

Mathias Possner, Tarek Alsaied, Saira Siddiqui, David Morales, Andrew T Trout, Gruschen Veldtman,

Background:Fontan palliation results in a chronic multisystem disorder with diminished exercise capacity and increased risk of muscle wasting. The aims of this study were to assess the feasibility of skeletal muscle mass measurements in Fontan patients undergoing magnetic resonance imaging liver surveillance to compare muscle mass with a historic control ... Read more >>

CJC Open (CJC open)
[2020, 2(2):55-61]

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Rare Association of Absent Pulmonary Valve Syndrome with Double Outlet Right Ventricle and Hypoplastic Left Heart Complex.

Erik L Frandsen, Arpan R Doshi, Sujatha Buddhe, Bhawna Arya, Sathish M Chikkabyrappa,

Kans J Med (Kansas journal of medicine)
[2020, 13:21-22]

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Low pulmonary artery size might be associated with subclinical systemic ventricular dysfunction in the Fontan circulation.

Kenan Yalta, Orkide Palabiyik, Ertan Yetkin,

Heart (Heart (British Cardiac Society))
[2019, 105(24):1922]

Cited: 1 time

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Combination Therapy of Fenestrated-Fontan Procedure with Medication Improved Double-outlet Right Ventricle in Adulthood.

Tatsuro Sassa, Atsuko Nakayama, Akihito Saito, Katsura Soma, Ryo Inuzuka, Yasutaka Hirata, Issei Komuro,

J Cardiovasc Imaging (Journal of cardiovascular imaging)
[2019, 27(4):288-289]

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Transplantation of the failing Fontan.

Amanda D McCormick, Kurt R Schumacher,

Half a century has passed since the original Fontan palliation. In the interim, surgical and medical advancements have allowed more single ventricle patients to reach Fontan and long-term survival for Fontan patients to improve significantly. However, the risk for Fontan failure and need for heart transplantation remains. In this article ... Read more >>

Transl Pediatr (Translational pediatrics)
[2019, 8(4):290-301]

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Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand.

Kanthalas Lertsakulpiriya, Chodchanok Vijarnsorn, Prakul Chanthong, Paweena Chungsomprasong, Supaluck Kanjanauthai, Kritvikrom Durongpisitkul, Jarupim Soongswang, Thaworn Subtaweesin, Somchai Sriyoschati,

Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic congenital heart disease with a wide-range of presentations and treatment strategies, depending on the source of pulmonary circulation, anatomy of pulmonary arteries (PAs), and major aortopulmonary collateral arteries (MAPCAs). Data about the outcomes in developing countries is scarce. We ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):5165]

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A 4D flow MRI evaluation of the impact of shear-dependent fluid viscosity on in vitro Fontan circulation flow.

Andrew L Cheng, Choo Phei Wee, Niema M Pahlevan, John C Wood,

The Fontan procedure for univentricular heart defects creates a nonphysiologic circulation where systemic venous blood drains directly into the pulmonary arteries, leading to multiorgan dysfunction secondary to chronic low-shear nonpulsatile pulmonary blood flow and central venous hypertension. Although blood viscosity increases exponentially in this low-shear environment, the role of shear-dependent ... Read more >>

Am. J. Physiol. Heart Circ. Physiol. (American journal of physiology. Heart and circulatory physiology)
[2019, 317(6):H1243-H1253]

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Hepatic pathology in patients after Fontan operation: A computed tomography imaging study.

Yu-Chieh Chen, Ken-Pen Weng, Kuang-Jen Chien, Bo-Hau Chen, Kai-Sheng Hsieh, I-Hsin Tai, Shih-Hui Huang, Hsu-Hsia Peng, Jer-Shyung Huang, Ming-Ting Wu,

BACKGROUND:Hepatic dysfunction is an important long-term complication in Fontan patients. The purpose of this study was to evaluate the hepatic computed tomography (CT) findings after Fontan surgery and identify their association with clinical parameters. METHODS:This study recruited 43 patients (23 male and 20 female patients aged 15.3 ± 6.8 years), ... Read more >>

Crit. Care Med. (Critical care medicine)
[2019, 82(11):856-860]

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Congenital Heart Disease and the Liver.

Moira B Hilscher, Patrick S Kamath,

Clin Liver Dis (Hoboken) (Clinical liver disease)
[2019, 14(4):138-141]

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