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Fontan fenestration

Yujiro Ide, Masaya Murata, Hiroki Ito, Kisaburo Sakamoto
Right atrial isomerism and pulmonary atresia combined with major aortopulmonary collateral arteries are very rare. Surgical treatment becomes more challenging when an extracardiac total anomalous pulmonary venous connection (TAPVC) requiring surgical repair neonatally is also present. We describe a successful staged Fontan operation for a neonate with a single ventricle, right atrial isomerism, pulmonary atresia with major aortopulmonary collateral arteries, a small central pulmonary artery and a supracardiac TAPVC...
September 13, 2016: Interactive Cardiovascular and Thoracic Surgery
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
Gananjay G Salve, Shreepal A Jain, Arjun Adnaik, Krishnanaik Shivaprakash
Fontan operation and importance of fenestration in the treatment of unusual and complex forms of double outlet right ventricle (DORV) are well established. Nonetheless, rarely, the creation of fenestration becomes challenging in complex morphologies. We present one such child with situs solitus, dextrocardia, DORV, hypoplastic right ventricle, large ventricular septal defect, severe pulmonic stenosis, extremely small right atrium and left juxtaposed atrial appendages, who underwent Fontan operation. We created an unusual fenestration between left pulmonary artery and juxtaposed right atrial appendage on the left side, due to anatomic complexity...
October 2016: Interactive Cardiovascular and Thoracic Surgery
Luca Deorsola, Enrico Aidala, Maria Teresa Cascarano, Andrea Valori, Gabriella Agnoletti, Carlo Pace Napoleone
OBJECTIVES: Hepatic damage in Fontan circulation setting is well known. Mainly represented by fibrosis, it is still poorly understood. Transient hepatic elastography (Fibro-Scan) is a useful tool to test liver stiffness and is commonly used in the evaluation of liver fibrosis. Unfortunately, the increase of inferior vena cava pressure consequent to total cavopulmonary connection can probably interfere with Fibro-Scan evaluation altering the result evaluation with the conventional scale...
October 2016: Interactive Cardiovascular and Thoracic Surgery
Marc Gewillig, Stephen C Brown
The Fontan operation was first performed in 1968. Since then, this operation has been performed on thousands of patients worldwide. Results vary from very good for many decades to very bad with a pleiad of complications and early death. A good understanding of the physiology is necessary to further improve results. The Fontan connection creates a critical bottleneck with obligatory upstream congestion and downstream decreased flow; these two features are the basic cause of the majority of the physiologic impairments of this circulation...
July 15, 2016: Heart: Official Journal of the British Cardiac Society
Pradeepkumar Charlagorla, John P Breinholt
Plastic bronchitis is a rare life-threatening complication of the Fontan operation. Transcatheter Fontan fenestration can ameliorate symptoms by decompressing elevated venous pressures. Transcatheter creation of a fenestration can be technically challenging in cases with complex venous anatomy. We report a case of a 5-year-old boy with heterotaxy, dextrocardia with unbalanced atrioventricular canal (AVC), atrial and visceral situs inversus, left-sided superior vena cava (SVC), and left-sided interrupted inferior vena cava (IVC) with azygos continuation...
May 2016: Annals of Pediatric Cardiology
Shashi Raj, Eliot Rosenkranz, Barbara Sears, Sethuraman Swaminathan
The Fontan operation and its modifications are currently the standard palliation in patients with single ventricle physiology. The advantages of extracardiac conduit Fontan operation (ECF) have previously been described. We evaluated the outcome of patients who underwent ECF operation at our center since the year 2000 to assess morbidity, mortality and New York Heart Association (NYHA) functional class in the current era. Medical records of 97 patients who underwent ECF operation from November 2000 till October 2013 were reviewed to determine the diagnosis, age at surgery, surgical technique, postoperative complications, NYHA class at last follow-up, echocardiographic parameters, and most recent Holter monitor results...
August 2016: Pediatric Cardiology
Pei-Ni Jone, Michael M Ross, John A Bracken, Matthew J Mulvahill, Michael V Di Maria, Thomas E Fagan
BACKGROUND: Fused real-time three-dimensional transesophageal echocardiography and fluoroscopy has been used in adult patients during percutaneous mitral valve and aortic valve procedures. The use of fused echocardiographic/x-ray fluoroscopic imaging (FEX) in pediatric patients undergoing congenital heart disease catheterization has not been evaluated for feasibility and safety. The aims of this study were to assess the feasibility and safety of FEX for interventional guidance and to perform a comparison of atrial septal defect (ASD) device closure using this technology with traditional guidance methods...
June 2016: Journal of the American Society of Echocardiography
Osamah Aldoss, Benjamin E Reinking, Abhay Divekar
A 6-year-old male child born with hypoplastic left heart syndrome (HLHS) was palliated with an extracardiac nonfenestrated Fontan procedure (18-mm Gore-Tex tube graft). He developed low-pressure (mean Fontan pressure 10 mmHg) protein-losing enteropathy 6 months after Fontan palliation. After initially responding to medical therapy and transcatheter pulmonary artery stent implantation, he developed medically refractory protein-losing enteropathy. At this time, his transthoracic echocardiogram showed new restriction across his native atrial septum with an 8 mmHg mean gradient...
January 2016: Annals of Pediatric Cardiology
Han Ki Park, Hong Ju Shin, Young Hwan Park
OBJECTIVES: We investigated the results of a revision of a previous Fontan connection to total cavopulmonary connection (TCPC) in patients with failing Fontan circulation. METHODS: From July 1998 to April 2013, 21 patients who had failing Fontan circulation underwent revision of the previous Fontan operation to TCPC. The median age at TCPC conversion was 17.9 years (range, 4.6-38.1 years) and the median interval between initial Fontan operation and TCPC was 13.8 years (range, 2...
July 2016: Interactive Cardiovascular and Thoracic Surgery
Sachin Talwar, Anupam Das, Shiv Kumar Choudhary, Balram Airan
OBJECTIVE: Persistent pleural effusions are a major source of morbidity after univentricular repair. These are often refractory to conventional conservative therapy. We adopted a strategy of diaphragmatic fenestration (DF) in such patients and report the results. METHODS: Between January 2002 and 2014, we performed DF in 12 patients using an original technique that was first described by us. The medical records of all these patients were studied. Preoperative characteristics, amount and duration of effusions, and time to removal of chest tubes following DF were studied...
March 2016: World Journal for Pediatric & Congenital Heart Surgery
Jun Sasaki, John C Dykes, Lisa J Sosa, Jane L Salvaggio, Milagros D Tablante, Jorge Ojito, Danyal M Khan, Robert L Hannan, Anthony F Rossi, Redmond P Burke, Gil Wernovsky
OBJECTIVES: Children with functional single ventricle undergoing the Fontan operation consume considerable resources. The purpose of this study is to evaluate pre- and intraoperative risk factors for longer hospital stay and to describe the perioperative course at a single institution over a 15-year period. DESIGN: Retrospective cohort study. SETTING: A single pediatric cardiac ICU. PATIENTS: All consecutive patients undergoing a first-time Fontan operation from 2000 to 2014...
May 2016: Pediatric Critical Care Medicine
Jun-Yen Pan, Chu-Chuan Lin, Chieh-Jen Wu, Jen-Ping Chang
BACKGROUND/PURPOSE: Fontan operation has evolved from atriopulmonary connection to total cavopulmonary connection (TCPC) due to its advantages in terms of hemodynamics and reduction of atrium-related complications. We analyzed the early and intermediate-term results of extracardiac conduit TCPC (EC-TCPC) procedure in patients with functional single ventricle to investigate the risk factors of surgical mortality and intermediate failure. METHODS: Retrospective review of the medical records of 88 consecutive patients with functional single ventricle who underwent EC-TCPC from 2000 to 2013 was conducted...
May 2016: Journal of the Formosan Medical Association, Taiwan Yi Zhi
Marlo Ortiz-Vázquez, Osbaldo Espinoza-Blanco, Samuel Ramírez-Marroquín, Juan Calderón-Colmenero, Jose Antonio García-Montes, Jorge Cervantes-Salazar
UNLABELLED: Fontan operation is the final palliative stage of patients with univentricular hearts. Cardiopulmonary bypass (CPB) decreases ventricular performance and increases pulmonary artery pressures in the post operative recovery period. It seems that Fontan operation performed without CPB decreases short term morbidity and intra hospitalary length of stay. OBJETIVE: Compare outcome in Fontan patients who have undergone surgery with or without CPB. METHOD: This is a retrospective review of patients undergoing Fontan operation from january 2009 to december 2012...
January 2016: Archivos de Cardiología de México
Ronak J Naik, Neil R Patel, Ming Wang, Nishant C Shah
BACKGROUND: In 2007, the American Heart Association modified the infective endocarditis prophylaxis guidelines by limiting the use of antibiotics in patients with cardiac conditions associated with the highest risk of adverse outcomes after infective endocarditis. Our objective was to evaluate current practice for infective endocarditis prophylaxis among paediatric cardiologists. METHODS: A web-based survey focussing on current practice, describing the use of antibiotics for infective endocarditis prophylaxis in various congenital and acquired heart diseases, was distributed via e-mail to paediatric cardiologists...
August 2016: Cardiology in the Young
Masatoshi Shimada, Takahiko Sakamoto, Kentaro Umezu, Yorikazu Harada
We report a case of Fontan completion for a tricuspid atresia (TA) patient with left ventricular non-compaction (LVNC). The patient was diagnosed with TA (Ia) with LVNC by fetal echocardiography. Because the unfavourable prognosis of LVNC was anticipated, Imidapril as well as Carvedilol were administered to improve cardiac function, from the early stages of infancy. Staged Fontan completion with fenestration was successfully achieved with improvement of LV function.
March 2016: Interactive Cardiovascular and Thoracic Surgery
Monica Mattes, Jason Connor, Susan S Kelly, Matthew C Schwartz
BACKGROUND: Lymphopenia is common in patients with Fontan circulation and no history of protein-losing enteropathy, but this phenomenon has not been significantly described in the literature. METHODS: We retrospectively identified patients with Fontan circulation who underwent catheterization between January 2003 and January 2013 at our center. Patients who had complete blood count with differential drawn within 12 months of the catheterization were included. Patients were excluded if complete blood count with differential was drawn in setting of possible infection or if there was history of protein-losing enteropathy (PLE)...
May 2016: Congenital Heart Disease
Daniel De Wolf, Hugues Dessy, Nikolaus Haas
For some higher risk patients, fenestrations are still created in total cavopulmonary connection conduits completing the Fontan circulation for univentricular hearts. If these fenestrations remain patent and hemodynamics are favorable, they are closed to improve oxygenation and exercise tolerance and to decrease the risk of paradoxical emboli. Depending on the type of fenestration, different devices are used. Unfortunately, the search for the ideal device is still ongoing. We propose the first experience with the off-label use of the new Occlutech paravalvular leak device...
May 2016: Catheterization and Cardiovascular Interventions
Jaana I Pihkala, Merit Järvelä, Talvikki Boldt, Eero Jokinen, Ilkka Liikanen, Ilkka Mattila, Anneli Eerola
OBJECTIVE: To review the operative history and clinical and catheterization data on patients treated with total cavopulmonary connection (TCPC) with baffle fenestration and to study whether it is possible to predict the fate of fenestration. BACKGROUND: A baffle fenestration may improve postoperative outcomes after Fontan operation but is later associated with cyanosis and thromboembolic risk. Fenestration may close spontaneously or it can be closed percutaneously in patients with favorable hemodynamics...
May 2016: Catheterization and Cardiovascular Interventions
Rıza Türköz, Mehmet Çelik, Eda Palaoğlu, Ahu Baysal, Nimet Cındık, Gülendam Koçak
During an extracardiac conduit type of Fontan operation, fenestration is not always technically easy, particularly when the right atrium is small and located posteriorly. We describe here an easy technique for performing an anastomosis of a polytetrafluoroethylene ringed graft to the atrium for fenestration in the setting of an extracardiac conduit Fontan operation. The surgical technique starts with a purse string suture that is customized to fit the atrium and utilizes a ringed graft that is inserted into the right atrium through the purse string, which is then tied...
October 2015: World Journal for Pediatric & Congenital Heart Surgery
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