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https://www.readbyqxmd.com/read/29035429/where-is-the-optimal-fontan-hemodynamics
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
https://www.readbyqxmd.com/read/28066626/update-in-the-extraction-of-airway-foreign-bodies-in-adults
#16
REVIEW
Montserrat Blanco Ramos, Maribel Botana-Rial, Eva García-Fontán, Alberto Fernández-Villar, Mercedes Gallas Torreira
Foreign body (FB) aspiration into the airway is lees common in adults than children. Nevertheless its incidence does not decrease through time. We present clinical relevant aspects of airway FBs on the basis of a selective review of pertinent literature retrieved by a search in the PubMed database. The most common aspirated FBs by adults are organics, especially fragments of bones and seeds. Symptoms usually are cough, chocking and dyspnea. Right localization, especially bronchus intermedius and right lower lobe, is more frequent...
November 2016: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28007062/rescuing-the-late-failing-fontan-focus-on-surgical-treatment-of-dysrhythmias
#17
REVIEW
Carl L Backer
Patients with a failing Fontan frequently have dysrhythmias. This review will focus on the treatment of those dysrhythmias based on the 140 patients who have had a Fontan conversion with arrhythmia surgery at Ann & Robert H. Lurie Children's Hospital of Chicago (Chicago, IL). Important technical points to be emphasized are the following:• Atrial fibrillation is extremely common in patients with a failing Fontan and strong consideration should be given to a Cox-maze III during any Fontan conversion• Sinus node dysfunction is common post-conversion and all patients should receive a dual-chamber epicardial pacemaker• Careful preoperative imaging with either computed tomographic or magnetic resonance imaging is needed to predict re-entry problems at the time of repeat sternotomy• If the patient has protein-losing enteropathy, ascites, or a right or indeterminate ventricular morphology, strong consideration should be given to heart transplantation rather than a Fontan conversion and arrhythmia surgery...
January 2017: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
https://www.readbyqxmd.com/read/27988989/single-center-outcomes-of-combined-heart-and-liver-transplantation-in-the-failing-fontan
#18
Benjamin A D'Souza, Stephanie Fuller, Lacey P Gleason, Nicole Hornsby, Joyce Wald, Karen Krok, Abraham Shaked, Lee R Goldberg, Alberto Pochettino, Kim M Olthoff, Yuli Y Kim
Long-term outcomes of the Fontan operation include Fontan failure and liver disease. Combined heart-liver transplantation (CHLT) is an option for select patients although limited data exist on this strategy. A retrospective review of Fontan patients 18 years or older referred for cardiac transplant evaluation between 2000 and 2013 at the Hospital of the University of Pennsylvania was performed. All patients were considered for potential CHLT. Clinical variables such as demographics, perioperative factors, and short-term outcomes were reviewed...
March 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/27878634/relationship-between-habitual-exercise-and-performance-on-cardiopulmonary-exercise-testing-differs-between-children-with-single-and-biventricular-circulations
#19
Michael L O'Byrne, Sanyukta Desai, Megan Lane, Michael McBride, Stephen Paridon, Elizabeth Goldmuntz
Increasing habitual exercise has been associated with improved cardiopulmonary exercise testing (CPET) performance, specifically maximal oxygen consumption in children with operatively corrected congenital heart disease. This has not been studied in children following Fontan palliation, a population in whom CPET performance is dramatically diminished. A single-center cross-sectional study with prospective and retrospective data collection was performed that assessed habitual exercise preceding a clinically indicated CPET in children and adolescents with Fontan palliation, transposition of the great arteries following arterial switch operation (TGA), and normal cardiac anatomy without prior operation...
March 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/27834759/current-expectations-for-cardiac-transplantation-in-patients-with-congenital-heart-disease
#20
REVIEW
James K Kirklin, Waldemar F Carlo, F Bennett Pearce
Congenital heart disease accounts for 40% of pediatric heart transplants and presents unique challenges to the transplant team. Suitability for transplantation is defined in part by degree of sensitization, pulmonary vascular resistance, and hepatic reserves. The incremental transplant risk for patients with congenital heart disease occurs within the first 3 months, after which survival is equivalent to transplantation for cardiomyopathy. Single ventricle with prior palliation, and especially the failing Fontan, carry the highest risk for transplantation and are least amenable to bridging with mechanical circulatory support...
November 2016: World Journal for Pediatric & Congenital Heart Surgery
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