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https://www.readbyqxmd.com/read/28532389/the-use-of-macitentan-in-fontan-circulation-a-case-report
#1
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/28520542/hybrid-palliation-for-interrupted-aortic-arch-with-small-aortic-valve
#2
Yoshimasa Uno, Ayumu Masuoka, Kentarou Hotoda, Toshiyuki Katogi, Takaaki Suzuki
OBJECTIVES: Open heart surgery for interrupted aortic arch in the neonatal period is still a high-risk procedure related in part to patient factors such as low birth weight, other morphologic anomalies, and, especially, small aortic valve size. Recently, we performed hybrid palliation with bilateral pulmonary artery banding and ductal stenting as the first-stage palliation for such cases. In this study, the outcomes of this procedure were examined. METHODS: Six cases of interrupted aortic arch with a small aortic valve underwent the hybrid procedure in the neonatal period in our institute from 2010 to 2015 (mean age: 6...
May 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28500413/impaired-pulmonary-function-is-an-additional-potential-mechanism-for-the-reduction-of-functional-capacity-in-clinically-stable-fontan-patients
#3
Aída L R Turquetto, Luiz F Canêo, Daniela R Agostinho, Patrícia A Oliveira, Maria Isabel C S Lopes, Patrícia F Trevizan, Frederico L A Fernandes, Maria A Binotto, Gabriela Liberato, Glaucia M P Tavares, Rodolfo A Neirotti, Marcelo B Jatene
Central factors negatively affect the functional capacity of Fontan patients (FP), but "non-cardiac" factors, such as pulmonary function, may contribute to their exercise intolerance. We studied the pulmonary function in asymptomatic FP and its correlations with their functional capacity. Pulmonary function and cardiopulmonary exercise tests were performed in a prospective study of 27 FP and 27 healthy controls (HC). Cardiovascular magnetic resonance was used to evaluate the Fontan circulation. The mean age at tests, the mean age at surgery, and the median follow-up time of FP were 20(±6), 8(±3), and 11(8-17) years, respectively...
May 12, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28497477/fontan-operation-with-bilateral-conduits-for-the-treatment-of-pulmonary-arteriovenous-fistulas-in-a-patient-with-polysplenia-after-reconstruction-of-a-non-confluent-pulmonary-artery
#4
Yuji Tominaga, Hiroaki Kawata, Shigemitsu Iwai, Sanae Yamauchi
We describe a case involving a successful Fontan procedure for the treatment of pulmonary arteriovenous fistulas in a patient with polysplenia. The patient was diagnosed with bilateral superior vena cavae, interrupted inferior vena cava with azygos connection, wide separate hepatic veins, and central pulmonary artery stenosis following repair of a non-confluent pulmonary artery. Bilateral extracardiac conduits were used to connect each hepatic vein to the pulmonary artery, opposite each superior vena cava. Clinical improvement in the pulmonary arteriovenous fistulas was observed within 4 months after surgery...
May 11, 2017: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/28472306/neuro-developmental-outcome-in-single-ventricle-patients-is-the-norwood-procedure-a-risk-factor
#5
Ina Rotermann, Jana Logoteta, Janine Falta, Philip Wegner, Olaf Jung, Peter Dütschke, Jens Scheewe, Hans-Heiner Kramer, Jan Hinnerk Hansen
OBJECTIVES: Complex neonatal surgery is considered a risk factor for neuro-developmental impairment in single-ventricle patients. Neuro-developmental outcome was compared between preschool-aged Fontan patients who underwent a Norwood procedure and single-ventricle patients not requiring neonatal surgery with cardiopulmonary bypass. METHODS: Verbal, performance and full-scale intelligence quotient (IQ) were evaluated with the Wechsler Preschool and Primary Scale of Intelligence...
May 2, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28470999/access-to-the-native-atria-following-conduit-total-cavopulmonary-anastomosis
#6
Robert L Petersen, Saar Danon, Saadeh Jureidini
OBJECTIVES: We describe the use of trans-thoracic and trans-conduit puncture to access the atria and perform interventional procedures in patients who have undergone conduit total cavopulmonary anastomosis. BACKGROUND: Catheter access to the atria following intra or extra-cardiac Fontan is desirable when there is a need for trans-atrial interventions. METHODS: Between 2009 and 2014, 5 patients ages 7 to 28 years underwent this approach; three trans-thoracic and 2 trans-conduit punctures...
May 4, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28457241/development-of-tachyarrhythmias-late-after-the-fontan-procedure-the-role-of-ablative-therapy
#7
REVIEW
Natasja M S de Groot, Ad J J C Bogers
Patients with a Fontan circulation are at a high risk of developing a variety of cardiac dysrhythmias after cardiac surgery. These dysrhythmias are most often supraventricular tachyarrhythmias (SVT), but ventricular tachyarrhythmias (VT) may also occur. Mechanisms underlying SVT are variable, including both ectopic activity and reentry. Over time, successive SVT may be caused by different mechanisms. The acute success rate of ablative therapy of atrial tachyarrhythmias is considerably high yet during long-term follow-up 'recurrences' frequently occur...
June 2017: Cardiac Electrophysiology Clinics
https://www.readbyqxmd.com/read/28456482/hospital-costs-and-cost-implications-of-co-morbid-conditions-for-patients-with-single-ventricle-in-the-period-through-to-fontan-completion
#8
Li Huang, Kim M Dalziel, Chris Schilling, David S Celermajer, John J McNeil, David Winlaw, Tom Gentles, Dorothy J Radford, Michael Cheung, Andrew Bullock, Gavin R Wheaton, Robert N Justo, Lisa A Selbie, Victoria Forsdick, Karin Du Plessis, Yves d'Udekem
BACKGROUND: Patients undergoing palliative surgeries for single-ventricle conditions are affected by multiple comorbidities or non-cardiac conditions. The prevalence, costs and the cost implications of these conditions have not been assessed. METHODS: Administrative costing records from four hospitals in Australia and New Zealand were linked with the Fontan registry database to analyze the inpatient resource use for co-morbid or non-cardiac conditions. Inpatient costing records from the birth year through to Fontan completion were available for 156 patients...
April 20, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28446492/hepatic-fibrosis-is-universal-following-fontan-operation-and-severity-is-associated-with-time-from-surgery-a-liver-biopsy-and-hemodynamic-study
#9
David J Goldberg, Lea F Surrey, Andrew C Glatz, Kathryn Dodds, Michael L O'Byrne, Henry C Lin, Mark Fogel, Jonathan J Rome, Elizabeth B Rand, Pierre Russo, Jack Rychik
BACKGROUND: Congestive hepatopathy is a recognized complication of Fontan physiology. Data regarding the incidence of hepatopathy and risk factors are lacking. METHODS AND RESULTS: Liver biopsies and cardiac catherizations were performed as part of an evaluation offered to all patients ≥10 years after Fontan. Quantitative determination of hepatic fibrosis was performed using Sirius red staining with automated calculation of collagen deposition per slide (%CD)...
April 26, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28439697/heart-transplantation-for-adults-with-congenital-heart-disease-current-status-and-future-prospects
#10
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...
April 24, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28422842/the-role-of-renoscintigraphy-and-surgery-in-the-management-of-page-kidney-a-case-report
#11
Yu-Li Chiu, Chin Hu, Sin-Di Lee, Po-Yin Chen, Cheng-Pin Wang
RATIONALE: Page kidney is an uncommon condition that hypertension occurs secondary to microvascular ischemia and alternation of small-vessel hemodynamics due to external compression of renal parenchyma and activation of the renin-angiotensin-aldosterone system. There are no specific guidelines for the management of Page kidney in the literatures. PATIENT CONCERNS: A 17-year-old teenager who had Fontan procedure for tricuspid and pulmonary atresia in early childhood suffered from sudden onset of severe left flank pain during cardiac catheterization procedure...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28410632/evolution-of-left-ventricular-size-in-late-survivors-of-surgery-for-hypoplastic-left-heart-syndrome
#12
Markus Bjurbom, Ajay J Iyengar, Florian Moenkemeyer, Igor E Konstantinov, Christian P Brizard, Yves d'Udekem
BACKGROUND: Aortic atresia and mitral atresia are predictors of adverse events in early life in patients with hypoplastic left heart syndrome, but it is unclear whether late outcomes are also affected, and whether this impact is related to a small hypertrophied left ventricle (LV) that impairs right ventricle (RV) function. METHODS: Thirty patients with hypoplastic left heart syndrome surviving with a Fontan procedure were identified. Follow-up echocardiograms were available at birth, before the Fontan procedure, and several years after...
April 12, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28402400/unplanned-cardiac-reoperations-and-interventions-during-long-term-follow-up-after-the-norwood-procedure%C3%A2
#13
Eva Sames-Dolzer, Gregor Gierlinger, Michaela Kreuzer, Julia Schrempf, Roland Gitter, Christoph Prandstetter, Gerald Tulzer, Rudolf Mair
OBJECTIVES: Patients with hypoplastic left heart syndrome or related malformations are predominantly treated with a 3-stage palliation. Anatomic or physiologic problems can lead to unplanned additional surgical or catheter interventions during single ventricle palliation. Changes in operative technique may have an impact on the reoperation rate. METHODS: Between 1997 and 2014, 317 Norwood procedures were performed at our centre. A retrospective single centre investigation was carried out concerning incidence, timing, indication and type of unplanned interstage cardiac reoperations and catheter interventions during follow-up of Norwood patients...
June 1, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28386502/transumbilical-surgery-for-duodenal-stenosis-in-a-child-with-situs-inversus-the-first-report
#14
Isamu Saeki, Yu Ueno, Wataru Mukai, Reisuke Imaji, Takashi Akiyama
Background. Situs inversus is a rare congenital anomaly with a reported incidence of only 1 in 5,000 to 10,000 live births. Congenital duodenal stenosis complicated with situs inversus is an even rarer entity. Case Presentation. A 1-year-old girl with situs inversus who had undergone a hemi-Fontan procedure against a single ventricle in our hospital was referred to our department for vomiting and failure to thrive. An upper gastrointestinal contrast study and endoscopy revealed duodenal stenosis. A transumbilical radical operation as a minimally invasive surgery was successfully performed...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28385377/the-effects-of-modification-to-lateral-tunnel-fontan-procedure-for-prophylactic-arrhythmia-surgery
#15
Hong-Gook Lim, Jeong Ryul Lee, Yong Jin Kim
BACKGROUND: Refractory atrial arrhythmias and hemodynamic abnormalities are responsible for significant morbidity and mortality after the Fontan operation. We evaluated the long-term feasibility, safety, and efficacy of prophylactic atrial arrhythmia surgery performed concomitantly with the lateral tunnel Fontan operation. METHODS: From 1997 August to 2003 December, 27 patients underwent a initial lateral tunnel Fontan with an interventional atrial incision and cryoablation from the atriotomy to the coronary sinus and right atrioventricular valve annulus...
April 3, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28359691/ablation-of-supraventricular-arrhythmias-in-adult-congenital-heart-disease-a-contemporary-review
#16
REVIEW
Nicolas Combes, Nicolas Derval, Sebastien Hascoët, Alexandre Zhao, Denis Amet, Mathieu Le Bloa, Alice Maltret, François Heitz, Jean-Benoit Thambo, Eloi Marijon
Supraventricular arrhythmias are an important and increasing cause of morbidity in adults with congenital heart disease, requiring specific management strategies. Pharmacological treatment has limited efficacy, and is often associated with some side-effects. Major improvements in catheter ablation techniques have opened new opportunities to better understand underlying mechanisms of supraventricular arrhythmias, offer better therapy, and eventually improve symptoms and quality of life in these patients. An array of tools and techniques are necessary to access relevant anatomical areas to address the arrhythmogenic substrate...
March 27, 2017: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/28347487/is-age-a-predisposing-factor-of-postoperative-complications-after-lung-resection-for-primary-pulmonary-neoplasms
#17
Miguel-Ángel Cañizares Carretero, Eva-María García Fontán, Montserrat Blanco Ramos, José Soro García, Rommel Carrasco Rodríguez, Emilio Peña González, Antonio Cueto Ladrón de Guevara
INTRODUCTION: Age has been classically considered as a determining factor for the development of postoperative complications related to lung resection for bronchogenic carcinoma. The Postoperative Complications Study Group of the Spanish Society of Thoracic Surgery has promoted a registry to analyze this factor. METHODS: A total of 3,307 patients who underwent any type of surgical resection for bronchogenic carcinoma have been systematically and prospectively recorded in any of the 24 units that are part of the group...
March 24, 2017: Cirugía Española
https://www.readbyqxmd.com/read/28341356/mortality-risk-stratification-in-fontan-patients-who-underwent-heart-transplantation
#18
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/28284501/cardiopulmonary-exercise-test-in-adults-with-prior-fontan-operation-the-prognostic-value-of-serial-testing
#19
Alexander C Egbe, David J Driscoll, Arooj R Khan, Sameh S Said, Emmanuel Akintoye, Fernando M Berganza, Heidi M Connolly
BACKGROUND: The purpose of the study was to determine the role of cardiopulmonary exercise test (CPET) indices in predicting cardiovascular adverse events (CAEs) in patients with Fontan palliation. CAE was defined as death or cardiac surgery. METHODS: Retrospective review of adult Fontan patients who had treadmill CPET at Mayo Clinic, 1994-2013. Patients with loss of follow-up defined as ≥2years without clinical follow-up were excluded. The results of serial CPETs were reviewed, and patients with CPETs meeting the following criteria were selected for analysis: maximum effort on serial CPETs, minimum of 3-year interval between CPETs, and absence of CAE between CPETs...
May 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28271152/arrhythmias-after-fontan-operation-with-intra-atrial-lateral-tunnel-versus-extra-cardiac-conduit-a-systematic-review-and-meta-analysis
#20
Dongxu Li, Qiang Fan, Yasutaka Hirata, Minoru Ono, Qi An
Current studies on the incidence of arrhythmias after the intra-atrial lateral tunnel (ILT) Fontan operation and the extra-cardiac conduit (ECC) Fontan operation are limited, with controversial results. This systematic review aimed to compare the prevalence of arrhythmias in patients who underwent ECC or ILT Fontan. Relevant studies comparing the incidence of arrhythmias and pacemaker implantation in ILT with ECC were identified through a literature search using MEDLINE, EMBASE, and the cochrane central register of controlled trials...
April 2017: Pediatric Cardiology
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