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Failed fontan

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https://www.readbyqxmd.com/read/29217440/endolymphatic-balloon-occluded-retrograde-abdominal-lymphangiography-boral-and-embolization-borale-for-the-diagnosis-and-treatment-of-chylous-ascites-technique-and-experience-in-three-patients
#1
Ravi N Srinivasa, Joseph J Gemmete, Matthew L Osher, Jeffrey Forris Beecham Chick
PURPOSE: To describe endolymphatic balloon-occluded retrograde abdominal lymphangiography (BORAL) and embolization (BORALE) for diagnosis and treatment of chylous ascites in patients with previously unidentifiable leakage site or failed lymphatic embolization. MATERIALS AND METHODS: Two (66%) men and one (33%) woman with mean age of 52 years (range: range: 14-79 years) presented with chylous ascites and underwent BORAL or BORALE between March 2016 and February 2017...
December 4, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/29177420/endothelin-1-may-play-an-important-role-in-the-fontan-circulation
#2
Masaya Aoki, Keiichi Hirono, Tomonori Higuma, Yoko Suzuki, Kazuhiko Nakayama, Fukiko Ichida, Hideki Origasa, Naoki Nishida, Johji Imura, Noriaki Emoto, Naoki Yoshimura
OBJECTIVES: Our goal was to evaluate whether endothlin-1 (ET-1) plays an important role in the Fontan circulation. METHODS: Thirteen patients with single-ventricle physiology (Glenn circulation, n = 7; Fontan circulation, n = 6) were evaluated using lung histopathological and immunohistochemical studies and then compared with the normal autopsied controls without congenital heart disease (n = 13). We evaluated the medial thickness of the small pulmonary arteries...
November 21, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29084001/assessing-the-borderline-ventricle-in-a-term-infant-combining-imaging-and-physiology-to-establish-the-right-course
#3
Meryl S Cohen
PURPOSE OF REVIEW: The purpose of this review is to describe the challenges associated with the diagnosis and treatment of children with borderline ventricles. A borderline ventricle is one in which there is concern that it will not be able to support its circulation. If a biventricular repair is attempted and fails, outcome is often poor. Thus, this early decision is important. RECENT FINDINGS: For the borderline right ventricle, options to add an additional source of pulmonary blood flow make the surgical strategy a bit more flexible than for patients with a borderline left ventricle...
January 2018: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/29050751/fibrosis-related-gene-expression-in-single-ventricle-heart-disease
#4
Stephanie J Nakano, Austine K Siomos, Anastacia M Garcia, Hieu Nguyen, Megan SooHoo, Csaba Galambos, Karin Nunley, Brian L Stauffer, Carmen C Sucharov, Shelley D Miyamoto
OBJECTIVE: To evaluate fibrosis and fibrosis-related gene expression in the myocardium of pediatric subjects with single ventricle with right ventricular failure. STUDY DESIGN: Real-time quantitative polymerase chain reaction was performed on explanted right ventricular myocardium of pediatric subjects with single ventricle disease and controls with nonfailing heart disease. Subjects were divided into 3 groups: single ventricle failing (right ventricular failure before or after stage I palliation), single ventricle nonfailing (infants listed for primary transplantation with normal right ventricular function), and stage III (Fontan or right ventricular failure after stage III)...
October 16, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29049683/ventricular-assist-device-in-a-failing-total-cavopulmonary-connection-a-new-step-by-step-approach
#5
Carlo Pace Napoleone, Maria Teresa Cascarano, Luca Deorsola, Andrea Valori
The management of failing Fontan patients with mechanical circulatory support has been reported with unsatisfactory results. A 5-year-old girl, listed for heart transplantation for failing Fontan, underwent rescue extracorporeal membrane oxygenation support. As it was necessary to move the patient to the Berlin Heart Excor, a step-by-step approach was designed to obtain a gradual adaptation to this form of support. A central extracorporeal membrane oxygenation, with a Berlin Heart apical and aortic cannulae, was implanted leaving the previously positioned femoral vein cannula in parallel with the outflow apical one...
September 11, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29035429/where-is-the-optimal-fontan-hemodynamics
#6
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/28941165/combined-heart-liver-transplantation-for-failing-fontan-circulation-in-a-late-survivor-with-single-ventricle-physiology
#7
Phuoc Duong, Louise Coats, John O'Sullivan, David Crossland, Beate Haugk, Sonya V Babu-Narayan, Jennifer Keegan, Mark Hudson, Gareth Parry, Derek Manas, Asif Hasan
Management of adults with failing Fontan physiology poses many challenges, especially as transplantation offers the only realistic alternative to palliative care. We present the first combined heart and liver transplant performed in Europe, for a late survivor of single ventricle palliation with the Fontan circulation. In addition to the conventional medical and surgical challenges posed, we highlight the management of the associated multi-organ failure with focus on the liver and novel strategies for assessment and optimization...
September 21, 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28799131/pulmonary-vascular-disease-in-a-failed-fontan-patient-with-down-s-syndrome
#8
Masaya Aoki, Keiichi Hirono, Tomonori Higuma, Yoko Suzuki, Kazuhiko Nakayama, Akio Yamashita, Kazuaki Fukahara, Fukiko Ichida, Noriaki Emoto, Naoki Yoshimura
It is well known that Down's syndrome is a strong risk factor for mortality after Fontan operations. We performed two lung biopsies in a Down's syndrome patient who underwent staged Fontan operations. The pathological findings revealed severe pulmonary arterial hypertrophy and a quantitative real-time polymerase chain reaction revealed the overexpression of endothelin and a decline in the eNOS level at the Fontan operation. Although the preoperative hemodynamic studies revealed that all of the criteria for Fontan had been fulfilled, the patient died of acute cardiac insufficiency, 35 days after the Fontan operation...
August 10, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28790279/-perioperative-management-of-fontan-operation
#9
Koichi Sughimoto, Kagami Miyaji
Surgical results of Fontan operation has been improved over the decades due to the introduction of the staged operations and some modifications of Fontan route from the classical atrio-pulmonary connection to total cavo-pulmonary connection. However, issues remain because of the single ventricular physiology of Fontan circulation. This article explains about the preoperative checklist for Fontan operation and tips on the postoperative management including an early extubation, use of inhaled nitric oxide after extubation, anticoagulation therapy, and efficacy of angiotensin converting enzyme inhibitor, supported by the cutting-edge evidence...
July 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28534240/an-early-glenn-operation-may-be-associated-with-the-later-occurrence-of-protein-losing-enteropathy-in-fontan-patients-association-of-early-glenn-and-failing-fontan
#10
Bettina Unseld, Brigitte Stiller, Thomas Borth-Bruhns, Florian du Bois, Johannes Kroll, Jochen Grohmann, Thilo Fleck
Protein-losing enteropathy (PLE) and plastic bronchitis (PB) are major causes of long-term mortality after Fontan operation. The objective of this study was to determine early clinical risk factors before the onset of PLE and PB. In a cohort study, 106 Fontan patients between 2005 and 2013 were examined. A median of 5.3 (1.5-8.5) years later, follow-up questionnaires were used to group the patients in a PLE or PB group (n = 14) and a non-PLE/PB group (n = 92). Prevalence of PLE was 9.4% (n = 10) and of PB 3...
August 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28532389/the-use-of-macitentan-in-fontan-circulation-a-case-report
#11
Polyvios Demetriades, Amir Aziz, Robin Condliffe, Sarah E Bowater, Paul F Clift
BACKGROUND: The Fontan circulation, a result of a palliative procedure in patients with single systemic ventricles, is defined by chronically elevated pulmonary vascular resistance. When traditional heart failure therapies fail, pharmacological agents that reduce pulmonary artery pressures may be used. These include endothelial-receptor antagonists, prostanoids and phosphodiesterase type 5 inhibitors. We report the first use of macitentan, an endothelin-receptor antagonist, in a patient with a Fontan circulation...
May 22, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28532240/ventricular-assist-devices-for-the-failing-univentricular-circulation
#12
Edward Buratto, William Y Shi, Xin Tao Ye, Igor E Konstantinov
Improved survival following single ventricle palliation has led to a large population of patients with a univentricular circulation, many of whom develop heart failure. Increasing experience with ventricular assist devices (VAD) in children has paved the way for VAD support in those with failing univentricular circulation. Areas covered: The use of VADs to support the failing univentricular circulation is a relatively new concept. Most studies have focused on supporting patients with the failing systemic ventricle...
June 2017: Expert Review of Medical Devices
https://www.readbyqxmd.com/read/28480728/hepatocellular-carcinoma-in-a-non-failing-fontan-circulation
#13
Efrén Martínez-Quintana, Alberto Monescillo, Fayna Rodríguez-González
Patients after a Fontan operation exhibit some degree of fibrosis which are primarily located in a sinusoidal or centrilobular pattern. Cirrhosis may develop years later and the incidence of cancer is up to 5%. That is why some authors recommend that patients who are >10 years out from their Fontan operation should undergo cardiac assessment as well as the determination of alpha-fetoprotein level, the realization of liver imaging tests or even liver biopsy to stay ahead of neoplastic transformation.
May 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28439697/heart-transplantation-for-adults-with-congenital-heart-disease-current-status-and-future-prospects
#14
REVIEW
Hikaru Matsuda, Hajime Ichikawa, Takayoshi Ueno, Yoshiki Sawa
Increased survival rates after corrective or palliative surgery for complex congenital heart disease (CHD) in infancy and childhood are now being coupled with increased numbers of patients who survive to adulthood with various residual lesions or sequelae. These patients are likely to deteriorate in cardiac function or end-organ function, eventually requiring lifesaving treatment including heart transplantation. Although early and late outcomes of heart transplantation have been improving for adult survivors of CHD, outcomes and pretransplant management could still be improved...
June 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28341356/mortality-risk-stratification-in-fontan-patients-who-underwent-heart-transplantation
#15
Christopher J Berg, Brenton S Bauer, Abbie Hageman, Jamil A Aboulhosn, Leigh C Reardon
The number of patients who require orthotopic heart transplantation (OHT) for failing Fontan physiology continues to grow; however, the methods and tools to evaluate risk of OHT are limited. This study aimed to identify a set of preoperative variables and characteristics that were associated with a greater risk of postoperative mortality in patients who received OHT for failing Fontan physiology. Thirty-six Fontan patients were identified as having undergone OHT at University of California-Los Angeles Medical Center from 1991 to 2014...
May 15, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28319522/a-numerical-simulation-comparing-a-cavopulmonary-assist-device-and-va-ecmo-for-failing-fontan-support
#16
Po-Lin Hsu, Dongfang Wang, Cherry Ballard-Croft, Dawei Xiao, Joseph B Zwischenberger
A cavopulmonary assist device (CPAD) has been developed for failing Fontan support. This CPAD pumps blood from superior/inferior vena cavae to pulmonary artery. In this study, we compared failing Fontan support with CPAD versus VA ECMO in silico. A numerical lumped-parameter circulatory system model was used to simulate Fontan circulation. Failing Fontan was simulated by increased pulmonary resistance. Central venous pressure (CVP), mean pulmonary arterial pressure (mPAP), left atrial pressure (LAP), and univentricular outflow (CO) were simulated and compared to published clinical data...
March 17, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28242080/can-fontan-conversion-for-patients-without-late-fontan-complications-be-justified
#17
Akihiko Higashida, Takaya Hoashi, Koji Kagisaki, Masatoshi Shimada, Hideo Ohuchi, Isao Shiraishi, Hajime Ichikawa
BACKGROUND: Fontan conversion from a classic Fontan operation such as atriopulmonary connection to total cavopulmonary connection with antiarrhythmia surgery is currently not indicated for patients without any late Fontan complications. METHODS: Thirty-two consecutive patients who underwent Fontan conversion between 1991 and 2012 were divided into 2 groups by the presence (group 1: n = 25, atrial tachyarrhythmia [AT] in 24 and protein-losing enteropathy in 4) or absence (group 2: n = 7) of late Fontan complications, and the surgical outcomes were retrospectively compared...
June 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28210768/the-lymphatic-circulation-in-adaptations-to-the-fontan-circulation
#18
REVIEW
Sabarinath Menon, Murthy Chennapragada, Shinya Ugaki, Gary F Sholler, Julian Ayer, David S Winlaw
Failing Fontan continues to be major problem for patients on the univentricular pathway. Failing Fontan is often complicated by chylothorax, plastic bronchitis and protein loosing enteropathy. The role of lymphatic circulation in Fontan circulation is still being researched. Newer imaging modalities give insight into the role of abnormal dilatation and retrograde flow in lymphatic channels post Fontan. Interventional strategies targeting abnormal lymphatic channels, provides an alternative management strategy for patients with failing Fontan...
June 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28118264/in-vitro-examination-of-the-heartware-circulite-ventricular-assist-device-in-the-fontan-connection
#19
Michael Tree, Phillip M Trusty, Timothy C Slesnick, Ajit Yoganathan, Shriprasad Deshpande, Kevin Maher
The failing Fontan physiology may benefit from ventricular assist device (VAD) mechanical circulatory support, although a subpulmonary VAD placed at the Fontan connection has never successfully supported the Fontan circulation long term. The HeartWare CircuLite continuous flow VAD was examined for Fontan circulatory support in an in vitro mock circulation. The VAD was tested in three different scenarios: VAD in parallel, baffle restricted VAD in parallel, and VAD in series. Successful support was defined as simultaneous decrease in inferior vena cava (IVC) pressure of 5 mm Hg or more and an increase in cardiac output (CO) to 4...
July 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28070902/a-modified-lvad-technique-to-augment-caval-and-pulmonary-arterial-blood-flow-in-the-failing-fontan-circulation
#20
Michael F Swartz, Louis DiVincenti, Karen Smith, Robin Westcott, Kevin Belmont, William Harris, Francisco Gensini, George M Alfieris
BACKGROUND: There are minimal circulatory support options for patients with a failing Fontan. The Heartmate II (HMII) left ventricular assist device (Thoratec, Bedford, MA) in its packaged state cannot augment caval/pulmonary arterial blood flow. AIM: We hypothesized that a modified HMII pump could augment caval and pulmonary arterial blood flow. METHODS: A bifurcated ringed Gore-Tex graft (W. L. Gore & Associates, Flagstaff, AZ) was sewn to the HMII inflow, and the outflow graft transected and tapered from 16 mm to 8 mm in diameter...
February 2017: Journal of Cardiac Surgery
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