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Failed fontan

Loes Lambrecht, Bjorn Cools, Peter Witters
This case report describes intestinal angiodysplasias as a cause of persisting anaemia in a 16-year-old girl with a failing Fontan circulation. Although this is a very rare cause of anaemia, we highlight the importance of video capsule endoscopy in such patients to initiate a life-saving treatment.
September 20, 2016: Cardiology in the Young
Anil K Singhi, Sivakumar Kothandum
Failing Fontan physiology in univentricular hearts manifest with protein-losing enteropathy, plastic bronchitis, low cardiac output, and recurrent effusions. Transcatheter creation of fenestration in a failing Fontan may be useful in alleviating the symptoms by improving the cardiac output. It is traditionally achieved by puncturing through the conduit from femoral or jugular venous access. In the absence of good venous path, transhepatic access provides a direct route for needle puncture of the conduit. If marked intimal ingrowth into the conduit results in increasing rigidity and makes the conduit nonyielding, alternative approaches may be needed...
September 2016: Annals of Pediatric Cardiology
Masanori Mizuno, Hideo Ohuchi, Taka-Aki Matsuyama, Aya Miyazaki, Hatsue Ishibashi-Ueda, Osamu Yamada
We report multi-organ histopathological changes in a patient with protein-losing enteropathy (PLE) over 12 years after Fontan operation. A 14-year-old boy with right isomerism heart and single ventricle had undergone Fontan procedure at 19 months of age, and PLE was diagnosed at 28 months. He had several episodes of intestinal bleeding and pre-renal failure with elevated creatine, and eventually died of pneumonia. The intrapulmonary small arteries showed medial and intimal thickening resembling pulmonary hypertension...
September 11, 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Alper Guzeltas, Ibrahim Cansaran Tanidir, Murat Saygi
Six months after undergoing a Fontan operation, a 7-year-old boy with right atrial isomerism and a single functional ventricle was admitted to our emergency department with cyanosis. Emergency cardiac catheterization revealed a large veno-venous fistula that began in a left hepatic vein, connected to the left accessory hepatic veins, and drained into the common atrium, resulting in desaturation. The fistula was occluded proximally with an Amplatzer septal occluder, with satisfying results; the patient's systemic arterial saturation decreased during his hospital stay...
April 2016: Brazilian Journal of Cardiovascular Surgery
Stephen H Gregory, Madhav Swaminathan, Yasmin Maisonave, Kelly A Machovec
The number of patients reaching adulthood after undergoing Fontan palliation for the repair of a congenital heart defect continues to increase. In this case report, we present the anesthetic management of a patient with a history of tricuspid atresia treated with palliative Fontan repair who had developed clinical evidence of Fontan failure. He presented with septic shock secondary to streptococcal toxic shock syndrome complicated by a loculated pleural effusion. He underwent open thoracic decortication under 1-lung ventilation...
October 15, 2016: A & A Case Reports
Manchula Navaratnam, Ann Ng, Glyn D Williams, Katsuhide Maeda, Julianne M Mendoza, Waldo Concepcion, Seth A Hollander, Chandra Ramamoorthy
BACKGROUND: Combined heart and liver transplantation (CHLT) in the pediatric population involves a complex group of patients, many of whom have palliated congenital heart disease (CHD) involving single ventricle physiology. OBJECTIVE: The purpose of this study was to describe the perioperative management of pediatric patients undergoing CHLT at a single institution and to identify management strategies that may be used to optimize perioperative care. METHODS: We did a retrospective database review of all patients receiving CHLT at a children's hospital between 2006 and 2014...
October 2016: Paediatric Anaesthesia
Bari Murtuza, Antony Hermuzi, David S Crossland, Gareth Parry, Stephen Lord, Mark Hudson, Milind P Chaudhari, Simon Haynes, John J O'Sullivan, Asif Hasan
OBJECTIVES: Adults with failing Fontan circulation (FFC) represent a heterogeneous, high-risk group for cardiac transplantation with poor reported outcomes. We studied the impact of mode of Fontan failure (preserved versus impaired systolic ventricular function) and end-organ dysfunction on early survival in adults undergoing cardiac transplantation for FFC. METHODS: A single-centre, retrospective study of 26 adults (≥16 years) with FFC undergoing cardiac transplantation between 1990 and 2015...
July 11, 2016: European Journal of Cardio-thoracic Surgery
Kavitha N Pundi, Krishna Pundi, David J Driscoll, Joseph A Dearani, Zhuo Li, Sonja H Dahl, Bassem N Mora, Patrick W O'Leary, Richard C Daly, Frank Cetta, Jonathan N Johnson
We performed a retrospective review of outcomes after heart transplantation during long-term follow-up of a surgical cohort of 1138 Fontan patients who were followed at the Mayo Clinic. Follow-up information was obtained from medical records and a clinical questionnaire that was mailed to patients not known to be deceased at the initiation of the study. Forty-four of 1138 Fontan patients with initial or subsequent evaluation at Mayo had cardiac transplantation between 1988 and 2014 (mean age at transplantation was 23...
July 10, 2016: Pediatric Transplantation
Alexander C Egbe, Heidi M Connolly, Joseph A Dearani, Crystal R Bonnichsen, Talha Niaz, Thomas G Allison, Jonathan N Johnson, Joseph T Poterucha, Sameh M Said, Naser M Ammash
BACKGROUND: To determine if Fontan conversion (FC) resulted in improvement in exercise capacity (EC), and to determine the role of cardiopulmonary exercise test (CPET) in risk stratification of patients undergoing FC. METHODS: A retrospective review of patients who underwent CPET prior to FC at Mayo Clinic from 1994 to 2014. The patients who also underwent post-operative CPET were selected for the analysis of improvement in EC defined as 10% increase in baseline peak oxygen consumption (VO2)...
October 1, 2016: International Journal of Cardiology
Marta António, Andreia Gordo, Conceição Pereira, Fátima Pinto, Isabel Fragata, José Fragata
An infrequent but devastating late complication of Fontan circulation is protein-losing enteropathy (PLE), which results from unbalanced lymphatic homeostasis. Surgical decompression of the thoracic duct by redirecting its drainage to the pulmonary venous atrium has been introduced recently as a possible treatment. This report describes a single-institution experience with this innovative procedure in 2 patients with failing Fontan circulation with PLE refractory to optimized medical therapy.
June 2016: Annals of Thoracic Surgery
Brian A Boe, John E Rectenwald, Martin L Bocks
A 28-year-old male with single ventricular heart disease status post Fontan palliation and subsequent placement of left ventricle to ascending aorta (LV-AAo) valved conduit developed ascites and edema. Diagnostic catheterization revealed elevated ventricular end diastolic pressures (EDP) secondary to severe LV-AAo conduit regurgitation. Given the unique anatomy, surgical access via the right axillary artery provided optimal route for transcatheter valve implantation within the conduit. The procedure resulted in significant hemodynamic improvement with no complications...
May 17, 2016: Catheterization and Cardiovascular Interventions
Atsushi Kawaguchi, Qi Liu, Sean Coquet, Yutaka Yasui, Dominic Cave
While policy changes toward early extubation in the operating room (OR) have been commonly seen in palliative surgeries in single ventricle anatomy, no systematic assessment of their impact on patient outcome has been reported. All patients aged 0-17 years admitted to a PICU in a quaternary children's hospital for post-operative management following a primary Fontan procedure between 2005 and 2011 were included. Patients for revision of Fontan or patients admitted to adult Cardiovascular Intensive Care Unit were excluded...
August 2016: Pediatric Cardiology
İlker Kemal Yücel, Şevket Ballı, Emine Hekim Yılmaz, Ahmet Çelebi
A 5-year-old male with a double outlet right ventricle with noncommitted ventricular septal defect and pulmonary stenosis underwent a bidirectional Glenn operation at 2 years and a Fontan operation with ligation of the pulmonary trunk at 5 years. He presented with pleural effusion 3 months after the Fontan operation. Physical examination revealed a grade 3/6 systolic murmur in the pulmonary area. Echocardiographic evaluation revealed an antegrade pulmonary flow (APF) of gradient 80 mmHg across the ventriculopulmonary communication...
April 2016: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
Carl L Backer
No abstract text is available yet for this article.
July 15, 2016: Heart: Official Journal of the British Cardiac Society
Joost P van Melle, Djoeke Wolff, Jürgen Hörer, Emre Belli, Bart Meyns, Massimo Padalino, Harald Lindberg, Jeffrey P Jacobs, Ilkka P Mattila, Håkan Berggren, Rolf M F Berger, Rene Prêtre, Mark G Hazekamp, Morten Helvind, Matej Nosál, Tomas Tlaskal, Jean Rubay, Stojan Lazarov, Alexander Kadner, Viktor Hraska, José Fragata, Marco Pozzi, George Sarris, Guido Michielon, Duccio di Carlo, Tjark Ebels
OBJECTIVE: The objective of this European multicenter study was to report surgical outcomes of Fontan takedown, Fontan conversion and heart transplantation (HTX) for failing Fontan patients in terms of all-cause mortality and (re-)HTX. METHODS: A retrospective international study was conducted by the European Congenital Heart Surgeons Association among 22 member centres. Outcome of surgery to address failing Fontan was collected in 225 patients among which were patients with Fontan takedown (n=38; 17%), Fontan conversion (n=137; 61%) or HTX (n=50; 22%)...
July 15, 2016: Heart: Official Journal of the British Cardiac Society
Jacob R Miller, Kathleen E Simpson, Deirdre J Epstein, Timothy S Lancaster, Matthew C Henn, Richard B Schuessler, David T Balzer, Shabana Shahanavaz, Joshua J Murphy, Charles E Canter, Pirooz Eghtesady, Umar S Boston
BACKGROUND: Patients with a failing Fontan continue to have decreased survival after heart transplant (HT), particularly those with preserved ventricular function (PVF) compared with impaired ventricular function (IVF). In this study we evaluated the effect of institutional changes on post-HT outcomes. METHODS: Data were retrospectively collected for all Fontan patients who underwent HT. Mode of failure was defined by the last echocardiogram before HT, with mild or no dysfunction considered PVF and moderate or severe considered IVF...
July 2016: Journal of Heart and Lung Transplantation
Mirko Doss, Thomas Walther
Transcatheter aortic valve implantation (T-AVI) has been established as an alternative to conventional aortic valve replacement. The number of procedures is steadily rising and has reached comparable implant rates to conventional aortic valve replacement. The success of T-AVI is easily explained by their true minimally invasive nature, ease of use, and excellent hemodynamics. Whilst their use was initially limited to high-risk patients, current trends show that more and more intermediate-risk patients are now treated with theses prostheses...
2016: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
Robert D B Jaquiss, Hamza Aziz
For the child born with a functionally univentricular heart, multi-stage surgical palliation culminating in the Fontan operation is now well established as the standard of care. Though this is an effective approach, there are several inherent disadvantages to the Fontan circulation: subnormal resting cardiac output, impaired exercise capacity, increased risk of thromboembolism, and increased risk of arrhythmia. Perhaps most importantly, the long-term deleterious effects of substantial systemic venous hypertension, which is necessary to "drive" blood through the pulmonary vascular bed, include progressive dysfunction of other organ systems, particularly renal, lymphatic, gastrointestinal, and hepatic systems...
2016: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
Kirk R Kanter
Previous studies have reported that children with a prior Fontan procedure have decreased survival after heart transplantation. We examined 273 primary pediatric heart transplants. Since 1988, 33 (12.1%) of 273 children <18 years old undergoing primary heart transplantation had a Fontan procedure 3.7 ± 4.3 years before transplantation. Compared with 240 (87.9%) non-Fontan primary transplants, the Fontan patients were older (8.8 ± 5.2 vs 6.6 ± 5.9 years; P = .023), but were similar in presensitization and pre-transplant clinical status...
2016: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
Rainer Kozlik-Feldmann, Georg Hansmann, Damien Bonnet, Dietmar Schranz, Christian Apitz, Ina Michel-Behnke
Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a complex disease that presents with a broad spectrum of morphological and haemodynamic findings of varying severity. Recently, the aspect of paediatric pulmonary hypertensive vascular disease (PPHVD) has been introduced to expand the understanding of the full spectrum of pulmonary hypertension and increased pulmonary vascular resistance. Evaluation and treatment of PAH-CHD/PPHVD-CHD can be divided into in different topics...
May 2016: Heart: Official Journal of the British Cardiac Society
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