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Fontan fenestration

Sachin Talwar, Manikala Vinod Kumar, Vishnubhatla Sreenivas, Vishwa Prakash Gupta, Shiv Kumary Choudhary, Balram Airan
Background: The optimal timing, need for primary/staged procedure in patients undergoing univentricular palliation, is debatable. Aims: We performed this study to assess the exercise performance of patients undergoing various forms of univentricular palliation. Setting and Design: This was a retrospective, prospective comparative study conducted at a multispecialty tertiary referral center. Patients and Methods: Between January 2012 and June 2015, 117 patients undergoing either bidirectional Glenn (BDG) ( n = 43) or Fontan (total cavopulmonary connection [TCPC]) ( n = 74) underwent exercise testing...
January 2018: Annals of Pediatric Cardiology
William R Miranda, Alexander C Egbe, Donald J Hagler, Nathaniel W Taggart, Rick A Nishimura, Heidi M Connolly, Carole A Warnes
BACKGROUND: Pulmonary artery wedge pressure (PAWP) has been shown to correlate better with left atrial pressure (LAP) than ventricular end-diastolic pressure (VEDP) in acquired heart disease. The correlation between VEDP and PAWP and their performance as surrogates for LAP in Fontan patients is unknown. METHODS: Offline single-beat simultaneous measurement of PAWP and VEDP was performed in 50 adult Fontan patients and non-simultaneous hemodynamic data abstracted for calculation of pulmonary vascular resistance (PVR)...
December 28, 2017: International Journal of Cardiology
Mauro Lo Rito, Osman O Al-Radi, Arezou Saedi, Yasuhiro Kotani, V Ben Sivarajan, Jennifer L Russell, Christopher A Caldarone, Glen S Van Arsdell, Osami Honjo
BACKGROUND: We hypothesized that chylothorax could be a sign of intolerance to the Fontan physiology, and thus patients who develop chylothorax or pleural effusion have worse medium-term to long-term survival. METHODS: A total of 324 patients who underwent the Fontan operation between 2000 and 2013 were included. Chylothorax was defined as ≥5 mL/kg/day of chylomicron-positive chest drainage fluid no earlier than postoperative day 5 or drainage with >80% lymphocytes...
November 21, 2017: Journal of Thoracic and Cardiovascular Surgery
Mikael Laredo, Gilles Soulat, Magalie Ladouceur, Alexandre Zhao
No abstract text is available yet for this article.
November 22, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
Qiang Fan, Yunfei Ling, Yabo Wang, Qi An
The Fontan procedure is a commonly used final pathway of palliation for single-ventricle physiology. Performing it without fenestration can avoid fenestration-related drawbacks, including subnormal oxygen saturation, decreased exercise tolerance, paradoxical embolization and subsequent need for closure by catheter intervention. If emergency fenestration is required, however, it is not always technically easy to perform, particularly when the atrium is posteriorly located and difficult to expose. We describe an easy technique for creating an anastomosis using a ring-supported polytetrafluoroethylene graft to the atrium for fenestration, performed in 2 patients who underwent extracardiac conduit Fontan procedures without fenestration...
July 13, 2017: Interactive Cardiovascular and Thoracic Surgery
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...
February 1, 2018: Interactive Cardiovascular and Thoracic Surgery
Seong-Ook Kim, Sang-Yun Lee, So-Ick Jang, Soo-Jin Park, Hye-Won Kwon, Seong-Ho Kim, Chang-Ha Lee, Eun-Seok Choi, Seong-Kyu Cho, Sun-Hwa Hong, Yang-Min Kim
Hepatic problems related to a Fontan circulation have been highlighted and elastography using ultrasound is a non-invasive tool that can measure the severity of hepatic stiffness. We investigated the hepatic stiffness using shear wave elastography (SWE) and related factors in patients with a Fontan circulation. This study enrolled 64 patients with a Fontan circulation who underwent cardiac catheterization and abdominal ultrasound from 2011 to 2015. The correlation between the laboratory tests, hemodynamic factors by cardiac catheterization, and SWE was evaluated...
September 21, 2017: Pediatric Cardiology
Charles Puelz, Sebastián Acosta, Béatrice Rivière, Daniel J Penny, Ken M Brady, Craig G Rusin
Fontan patients may undergo additional surgical modifications to mitigate complications like protein-losing enteropathy, liver cirrhosis, and other issues in their splanchnic circulation. Recent case reports show promise for several types of modifications, but the subtle effects of these surgeries on the circulation are not well understood. In this paper, we employ mathematical modeling of blood flow to systematically quantify the impact of these surgical changes on extracardiac Fontan hemodynamics. We investigate two modifications: (1) the fenestrated Fontan and (2) the Fontan with hepatic vein exclusion...
October 1, 2017: Computers in Biology and Medicine
Tacy E Downing, Kiona Y Allen, David J Goldberg, Lindsay S Rogers, Chitra Ravishankar, Jack Rychik, Stephanie Fuller, Lisa M Montenegro, James M Steven, Matthew J Gillespie, Jonathan J Rome, Thomas L Spray, Susan C Nicolson, J William Gaynor, Andrew C Glatz
BACKGROUND: There are limited follow-up studies examining surgical and catheter-based reinterventions in long-term survivors of the Fontan operation. METHODS AND RESULTS: All 773 patients who underwent Fontan at our institution between 1992 and 2009 were retrospectively reviewed. Current information regarding post-Fontan intervention was available for 70%. By 20 years after Fontan, 65% of patients had experienced either surgical or transcatheter intervention. The median time to first reintervention was 9...
September 2017: Circulation. Cardiovascular Interventions
Sachin Talwar, Sukhjeet Singh, Vishnubhatla Sreenivas, Kulwant Singh Kapoor, Saurabh Kumar Gupta, Sivasubramanian Ramakrishnan, Shyam Sunder Kothari, Anita Saxena, Rajnish Juneja, Shiv Kumar Choudhary, Balram Airan
OBJECTIVES: Studies on older patients undergoing primary Fontan operation (FO) are limited, with conflicting results. We review our experience with these patients beyond the first decade of life. PATIENTS AND METHODS: Between January 2000 and December 2014, a total of 105 patients ≥10 years of age (mean 15.6 ± 4.9, range 10-31, median 15 years) underwent primary FO without a prior bidirectional superior cavopulmonary anastomosis (Bidirectional Glenn [BDG]). Mean preoperative New York Heart Association (NYHA) class was 2...
July 2017: World Journal for Pediatric & Congenital Heart Surgery
M Elisabeth Heal, Lanier B Jackson, Andrew M Atz, Ryan J Butts
Cardiopulmonary exercise testing (CPET) aids in clinical assessment of patients with Fontan circulation. Effects of persistent fenestration on CPET variables have not been clearly defined. Associations between fenestration and CPET variables at anaerobic threshold (AT) and peak exercise were explored in the Pediatric Heart Network Fontan Cross-Sectional Study cohort. Fenestration patency was associated with a greater decrease in oxygen saturation from rest to peak exercise (fenestration -4.9 ± 3.8 v. nonfenestration -3 ± 3...
July 2017: Congenital Heart Disease
Li Huang, Chris Schilling, Kim M Dalziel, Siyu Xie, David S Celermajer, John J McNeil, David Winlaw, Tim S Hornung, Dorothy J Radford, Leeanne E Grigg, Andrew Bullock, Gavin R Wheaton, Robert N Justo, Jayne Blake, Rachel Bishop, Karin Du Plessis, Yves d'Udekem
We estimated the inpatient resource use for a Fontan patient from birth to adulthood and explored factors that might induce cost differences (2014 US dollar). Inpatient costing records from 4 hospitals with greatest numbers of Fontan patients in Australia and New Zealand were linked with the Fontan registry database. Inpatient records between July 1995 and September 2014 for 420 Fontan patients were linked, and the most frequent primary diagnoses were hypoplastic left heart syndrome (20.7%), tricuspid atresia (19...
August 1, 2017: American Journal of Cardiology
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
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
Anastasios C Polimenakos, Sujata Subramanian, Chawki ElZein, Michel N Ilbawi
OBJECTIVES: Data are limited regarding the management of children with trisomy 21 (T21) syndrome and a functional single ventricle (FSV). We evaluated patients with T21 and a FSV who had a total cavopulmonary connection (TCPC). METHODS: From September 1999 to August 2012, 139 patients with a FSV underwent a TCPC. Sixty-five had unbalanced atrioventricular septal defect. Thirteen had T21. Three (of 13) had heterotaxy syndrome. The mean age at the Fontan operation was 27...
May 1, 2017: Interactive Cardiovascular and Thoracic Surgery
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...
September 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...
April 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
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