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Fontan fenestration

Koichi Sughimoto, Yuta Asakura, Christian P Brizard, Fuyou Liang, Takashi Fujiwara, Kagami Miyaji, Hao Liu
OBJECTIVES: There is no consensus or theoretical explanation regarding the optimal location for the fenestration during the Fontan operation. We investigated the impact of the location of the fenestration on Fontan haemodynamics using a three-dimensional Fontan model in various physiological conditions. METHODS: A three-dimensional Fontan model was constructed on the basis of CT images, and a 4-mm-diameter fenestration was located between the extracardiac Fontan conduit and the right atrium at three positions: superior, middle, and inferior part of the conduit...
April 5, 2017: Cardiology in the Young
Sebastian Góreczny, Paweł Dryżek, Gareth J Morgan, Anna Mazurek-Kula, Jacek J Moll, Jadwiga A Moll, Shakeel Qureshi, Tomasz Moszura
INTRODUCTION: Creation of a fenestration during completion of a total cavopulmonary connection (TCPC) has been associated with a reduction in early mortality and morbidity. However, the long-term benefits are negated by an associated limitation in exercise tolerance and the potential risks of thrombo-embolic complications. We sought to describe the safety and efficacy of an Amplatzer Duct Occluder II (ADO II) for transcatheter fenestration closure following TCPC. MATERIAL AND METHODS: Between January 2000 and July 2014, 102 patients underwent percutaneous closure of extra-cardiac TCPC fenestrations with a range of devices...
March 1, 2017: Archives of Medical Science: AMS
Katarzyna Januszewska, Anna Schuh, Anja Lehner, Robert Dalla-Pozza, Edward Malec
The goal of the study was to compare the early postoperative course after fenestrated lateral atrial tunnel (LT) and non-fenestrated extracardiac conduit (EC) Fontan operation (FO) in a single center where both techniques were parallelly used. Between 2004 and 2012, 56(32.7%) children underwent LT and 113(66.1%) EC FO. The mean age was 3.7 ± 2.9 years and mean weight was 14.6 ± 7.3 kg. The most common malformation was hypoplastic left heart syndrome (50.9%). The general approach was to perform LT in children after hemi-Fontan operation and EC in patients after Glenn anastomosis...
April 2017: Pediatric Cardiology
Fan Fan, Zhimin Liu, Shoujun Li, Tong Yi, Jun Yan, Fuxia Yan, Xu Wang, Qiang Wang
Although fenestration is used to improve the postoperative course of Fontan patients, the effect of fenestration on the extracardiac Fontan seems controversial especially at early postoperation. One hundred and eighty-three patients from January 2004 to June 2013 undergoing an extracardiac Fontan operation were retrospectively selected for this study. We divided the patients into low risk (93 patients) and high risk (90 patients) groups according to the risk factors recognized by previous studies and then compared the perioperative data between the nonfenestrated and fenestrated patients in each group...
January 23, 2017: Pediatric Cardiology
Ronald K Woods, Nancy S Ghanayem, Michael E Mitchell, Steven Kindel, Robert A Niebler
Because of the inadequacies inherent to a circulation supported by a single ventricle, many Fontan patients will experience failure of their circulation. To date, there is no medical regimen that reliably and consistently restores circulatory function in these patients. Because of the shortage of donor organs and the fact that many of these patients present with features that either preclude or render heart transplantation a high risk, there is an intense need to better understand how mechanical circulatory support (MCS) may benefit these patients...
January 2017: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
Matthew C Schwartz, David Nykanen, Craig Fleishman, Hamish M Munro, Jonathan Phillips, Raymond D Knapp, Donald Felix
STUDY DESIGN: Case series. OBJECTIVE: To describe transcatheter closure of the Fontan fenestration prior to posterior spinal fusion in two children to prevent paradoxical venous air embolism during the operation. SUMMARY OF BACKGROUND DATA: Scoliosis is common among patients with single-ventricle congenital heart disease who have undergone Fontan operation and spinal surgery can offer physiologic benefits. Venous air embolism is a rare, but important reported complication during spinal surgery performed in the prone position...
December 1, 2016: Spine
Ganesh K Gnanappa, David S Celermajer, Gary F Sholler, Tom Gentles, David Winlaw, Yves d'Udekem, Julian Ayer
Although long-term survival is now the norm, Fontan patients face significant morbidity and premature mortality. Wide variation exists in long-term Fontan management. With an aim of improving their long-term management, we conducted a systematic review to identify best available evidence and gaps in knowledge for future research focus. We also surveyed cardiologists in Australia and New Zealand managing Fontan patients, to determine the alignment of current local practice with best available evidence. A systematic review was conducted using strict search criteria (PRISMA guidelines), pertaining to long-term Fontan management...
January 2017: Pediatric Cardiology
S Ram Kumar, Grace Kung, Nathan Noh, Novel Castillo, Brian Fagan, Winfield J Wells, Vaughn A Starnes
BACKGROUND: We have previously shown that neonates in profound cardiogenic shock caused by a severe Ebstein anomaly can be successfully salvaged with fenestrated right ventricular exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single-ventricle management in these patients is not known. METHODS: We retrospectively reviewed the records of patients who underwent neonatal Starnes procedure between 1989 and 2015. Patient demographics, clinical variables, and outcome data were collected...
October 25, 2016: Circulation
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...
January 2017: 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
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