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https://www.readbyqxmd.com/read/28904256/current-surgical-outcomes-of-congenital-heart-surgery-for-patients-with-down-syndrome-in-japan
#1
Takaya Hoashi, Norimichi Hirahara, Arata Murakami, Yasutaka Hirata, Hajime Ichikawa, Junjiro Kobayashi, Shinichi Takamoto
BACKGROUND: Current surgical outcomes of congenital heart surgery for patients with Down syndrome are unclear.Methods and Results:Of 29,087 operations between 2008 and 2012 registered in the Japan Congenital Cardiovascular Surgery Database (JCCVSD), 2,651 were carried out for patients with Down syndrome (9%). Of those, 5 major biventricular repair procedures [ventricular septal defect repair (n=752), atrioventricular septal defect repair (n=452), patent ductus arteriosus closure (n=184), atrial septal defect repair (n=167), tetralogy of Fallot (TOF) repair (n=108)], as well as 2 major single ventricular palliations [bidirectional Glenn (n=21) and Fontan operation (n=25)] were selected and their outcomes were compared...
September 12, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28901234/risk-factors-for-unfavorable-outcomes-after-bidirectional-cavopulmonary-anastomosis
#2
Nataliya R Nichay, Yuriy N Gorbatykh, Igor A Kornilov, Ilya A Soynov, Yuriy Y Kulyabin, Artem V Gorbatykh, Sergey M Ivantsov, Alexander V Bogachev-Prokophiev, Alexander M Karaskov
BACKGROUND: Bidirectional cavopulmonary anastomosis (BCPA) is an important preliminary step toward the Fontan procedure; thus, understanding of risk factors for morbidity and mortality after BCPA may ultimately promote improved rates of success with Fontan completion and general survival. This study evaluated survival and predictors of unfavorable outcomes in patients after BCPA. METHODS: Clinical data of 157 patients who underwent BCPA from 2003 to 2015 at a single center were retrospectively analyzed...
September 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28901223/the-lymphatic-system-the-achilles-heel-of-the-fontan-kreutzer-circulation
#3
Christian Kreutzer, Guillermo Kreutzer
In spite of excellent long term survival the Fontan Kreutzer procedure commonly presents late failure due to end-organ damage. Several advances have been described to refine single ventricle management and surgical techniques. However, very little research has been dedicated to the lymphatic circulation in the precarious Fontan hemodynamic state. The lymphatic circulation is clearly affected since there is increased lymph production, which requires to be drained at a similar or higher pressure than it is produced, commonly resulting in chronic lymphedema...
September 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28898966/employment-characteristics-of-a-complex-adult-congenital-heart-disease-cohort
#4
L Pickup, T Gaffey, P Clift, S Bowater, S Thorne, L Hudsmith
Background: Due to advances in surgical techniques and subsequent management, there have been remarkable improvements in the survival of patients with congenital heart disease. In particular, larger numbers of patients with complex disease are now living into adulthood and are entering the workforce. Aims: To establish the types of employment complex adult congenital heart disease (ACHD) patients are engaged in, based on the largest cohort of patients with a single-ventricle circulation in the UK...
August 1, 2017: Occupational Medicine
https://www.readbyqxmd.com/read/28893257/preoperative-single-ventricle-function-determines-early-outcome-after-second-stage-palliation-of-single-ventricle-heart
#5
Jacek Pająk, Michał Buczyński, Piotr Stanek, Grzegorz Zalewski, Marek Wites, Lesław Szydłowski, Bogusław Mazurek, Lidia Tomkiewicz-Pająk
BACKGROUND: Second-stage palliation with hemi-Fontan or bidirectional Glenn procedures has improved the outcomes of patients treated for single-ventricle heart disease. The aim of this study was to retrospectively analyze risk factors for death after second-stage palliation of single-ventricle heart and to compare therapeutic results achieved with the hemi-Fontan and bidirectional Glenn procedures. MATERIAL AND METHODS: We analyzed 60 patients who had undergone second-stage palliation for single-ventricle heart...
September 11, 2017: Cardiovascular Ultrasound
https://www.readbyqxmd.com/read/28889826/recombinant-factor-viia-as-a-rescue-therapy-in-severe-haemoptysis-in-a-patient-with-a-fontan-circulation
#6
Thomas W Mason, Andrew Retter, Gareth J Morgan
We describe the successful use of recombinant factor VIIa (rFVIIa) in the control of massive haemoptysis in a 17-year-old patient with a Fontan circulation. The patient was intubated and ventilated in the ICU with deteriorating gas exchange. Conventional methods to control the haemoptysis were ineffective, and rFVIIa was successfully administered as a rescue therapy. rFVIIa is a powerful pro-thrombotic agent, which is only licensed in haemophiliacs with acquired inhibitors to anticoagulation. It has been used off-license in the treatment of massive haemorrhage, although a Cochrane review did not show any significant benefit; however, it may have a role as a rescue therapy where alternatives options have been exhausted after careful risk-benefit analysis...
September 11, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28889811/cardiovascular-ct-for-evaluation-of-single-ventricle-heart-disease-risks-and-accuracy-compared-with-interventional-findings
#7
B Kelly Han, Marnie Huntley, David Overman, Dawn Witt, David Dassenko, Ross F Garberich, John R Lesser
OBJECTIVE: We sought to evaluate the risk and image quality from cardiovascular CT in patients across all stages of single-ventricle palliation, and to define accuracy by comparing findings with intervention and surgery. METHODS: Consecutive CT scans performed in patients with single-ventricle heart disease were retrospectively reviewed at a single institution. Diagnosis, sedation needs, estimated radiation dose, and adverse events were recorded. Anatomical findings, image quality (1-4, 1=optimal), and discrepancy compared with interventional findings were determined...
September 11, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28886927/suitability-of-cardiac-resynchronisation-therapy-in-patients-with-fontan-circulation-and-congenitally-corrected-transposition-of-the-great-arteries
#8
P Demetriades, A Bell, C Gubran, H Marshall, J de Bono, L Hudsmith
BACKGROUND: Cardiac resynchronisation therapy (CRT) is a well-recognised treatment in systolic heart failure. There is limited evidence in congenital patients with univentricular hearts or systemic right ventricles. In 2014 PACES/HRS published a consensus statement recommending CRT if ventricular ejection fraction (EF)≤35%, QRS duration≥150ms (with RBBB in systemic RV), NYHA II-IV and ventricular dilatation. The incidence of patients meeting these criteria in whom CRT is possible is not known...
September 2, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28886170/homoarginine-a-prognostic-indicator-in-adolescents-and-adults-with-complex-congenital-heart-disease
#9
Tanja Raedle-Hurst, Marieke Mueller, Andreas Meinitzer, Winfried Maerz, Thomas Dschietzig
BACKGROUND: Homoarginine (hArg) has been shown to be of prognostic value in patients with chronic left heart failure. The present study aims to assess the clinical utility and prognostic value of hArg levels in patients with complex congenital heart disease (CHD). METHODS: Plasma hArg levels were measured in 143 patients with complex CHD and compared to clinical status, echocardiographic and laboratory parameters as well as the occurrence of adverse cardiac events...
2017: PloS One
https://www.readbyqxmd.com/read/28884283/clinical-outcomes-of-early-scheduled-fontan-completion-following-kawashima-operation
#10
Takashi Kido, Takaya Hoashi, Masatoshi Shimada, Hideo Ohuchi, Kenichi Kurosaki, Hajime Ichikawa
OBJECTIVES: This study reviewed late clinical features after Kawashima operation to confirm the impact of scheduled subsequent early Fontan completion. METHODS: Of the 17 consecutive patients who underwent the Kawashima operation between 1987 and 2010, 11 underwent the procedure as inter-stage palliation (scheduled Fontan group). Ten of these patients underwent subsequent early Fontan completion after a median interval of 0.6 years. The remaining 6 patients underwent the Kawashima operation initially as definitive surgery (non-scheduled group)...
September 7, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28884212/routine-sildenafil-does-not-improve-clinical-outcomes-after-fontan-operation
#11
J Leslie Gaddis Collins, Mark A Law, Santiago Borasino, W Clinton Erwin, David C Cleveland, Jeffrey A Alten
Fontan operation can be complicated by persistent chest tube output (CTO) leading to prolonged hospital length of stay (LOS). Postoperative sildenafil administration has been shown to improve clinical outcomes in selected patients after Fontan. We initiated a practice change utilizing intravenous (IV) sildenafil in early postoperative period in all patients undergoing Fontan operation with aim to decrease LOS and CTO. Nineteen patients (February 2014-May 2016) received 0.35 mg/kg sildenafil IV (three doses) followed by enteral, 1 mg/kg every eight hours until hospital discharge...
September 7, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28881280/a-computational-study-of-the-fontan-circulation-with-fenestration-or-hepatic-vein-exclusion
#12
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...
August 25, 2017: Computers in Biology and Medicine
https://www.readbyqxmd.com/read/28879464/prevalence-of-subclinical-enteric-alpha-1-antitrypsin-loss-in-children-with-univentricular-circulation-following-total-cavopulmonary-connection
#13
Colm R Breatnach, Aoife Cleary, Terence Prendiville, Kathleen Crumlish, Helene Murchan, Colin J McMahon
Protein-Losing Enteropathy post Fontan palliation is associated with significant morbidity and mortality. To date, very little research has been carried out to improve early identification of enteric protein loss in these patients. We hypothesise that subclinical enteric protein loss may occur in patients post Fontan surgery. A cross-sectional study was performed on 43 patients post Fontan surgery. We collected specimens of stool and blood from patients with no symptoms of protein-losing enteropathy post Fontan...
September 6, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28877377/membrane-cholesterol-effect-on-the-5-ht2a-receptor-insights-into-the-lipid-induced-modulation-of-an-antipsychotic-drug-target
#14
Juan Manuel Ramírez-Anguita, Ismael Rodríguez-Espigares, Ramon Guixà-González, Agostino Bruno, Mariona Torrens-Fontanals, Alejandro Varela-Rial, Jana Selent
The serotonin 5-hydroxytryptamine 2A (5-HT2A ) receptor is a G-protein-coupled-receptor (GPCR) relevant for the treatment of CNS disorders. In this regard, neuronal membrane composition in the brain plays a crucial role in the modulation of the receptor functioning. Since cholesterol is an essential component of neuronal membranes, we have studied its effect on the 5-HT2A receptor dynamics through all-atom MD simulations. We find that the presence of cholesterol in the membrane increases receptor conformational variability in most receptor segments...
September 6, 2017: Biotechnology and Applied Biochemistry
https://www.readbyqxmd.com/read/28874285/international-collaborative-initiative-towards-improving-the-lives-of-patients-after-the-fontan-operation-a-call-for-action
#15
EDITORIAL
Margarita Brida, Michael A Gatzoulis
No abstract text is available yet for this article.
October 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28869334/percutaneous-thoracic-duct-intervention-to-treat-plastic-bronchitis-related-to-fontan-palliation
#16
Eric M DePopas, Livia A Veress, Farres Ahmed, Christopher M Rausch, Aparna Annam, Rajan Gupta
Plastic bronchitis is a life-threatening condition of airway obstructive cast formation. This pediatric case series from a high altitude center details the course of three patients after percutaneous thoracic duct interventions for refractory plastic bronchitis, with a follow-up interval of 3.5 years. In two patients, where cisterna chyli maceration (patients 2) or thoracic duct embolization (patient 3) was performed, a sustained clinical improvement is shown, with no subsequent cast recurrence. In patient 1, cisterna chyli maceration resulted in partial improvement...
September 4, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28868322/viability-and-tissue-quality-of-cartilage-flaps-from-patients-with-femoroacetabular-hip-impingement-a-matched-control-comparison
#17
Francisco Rodriguez-Fontan, Karin A Payne, Jorge Chahla, Omer Mei-Dan, Abigail Richards, Soshi Uchida, Cecilia Pascual-Garrido
BACKGROUND: Chondrolabral damage is commonly observed in patients with cam-type femoroacetabular impingement (FAI). Chondral flap reattachment has recently been proposed as a possible preservation technique. PURPOSE/HYPOTHESIS: The purpose of this study was to determine the viability and tissue quality of chondral flaps from patients with FAI at the time of arthroscopy. It was hypothesized that chondral flaps from patients with cam lesions of the hip would exhibit less viability and greater tissue degeneration than would those of a matched control group...
August 2017: Orthopaedic Journal of Sports Medicine
https://www.readbyqxmd.com/read/28862962/inspiratory-muscle-training-is-associated-with-improved-inspiratory-muscle-strength-resting-cardiac-output-and-the-ventilatory-efficiency-of-exercise-in-patients-with-a-fontan-circulation
#18
Karina Laohachai, David Winlaw, Hiran Selvadurai, Ganesh Kumar Gnanappa, Yves d'Udekem, David Celermajer, Julian Ayer
BACKGROUND: Patients with a Fontan circulation have reduced exercise capacity and respiratory muscle strength. Inspiratory muscle training (IMT) improves exercise capacity and quality of life in adults with heart failure. We assessed whether 6 weeks of a home-based program of IMT improves inspiratory muscle strength and the ventilatory efficiency of exercise in adolescent patients with a Fontan circulation. METHODS AND RESULTS: Twenty-three adolescent participants (aged 16±2 years) with a Fontan circulation underwent 6 weeks of IMT for 30 minutes daily...
August 21, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28851719/surgical-and-catheter-based-reinterventions-are-common-in-long-term-survivors-of-the-fontan-operation
#19
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
https://www.readbyqxmd.com/read/28845873/-late-complications-of-fontan-operation
#20
Alessandro Frigiola, Mauro Lo Rito
Complex univentricular heart defects are treated with the Fontan operation that consists in the diversion of the systemic venous return to the pulmonary arteries. Currently, the operation is achieved through a staged approach and with the use of prosthetic extracardiac conduits to connect the inferior vena cava to the pulmonary arteries. The improvement of early and late survival allowed these patients to reach adulthood, although long-term complications remain a relevant problem. Most of the complications are caused by chronic systemic venous hypertension due to the lack of a pumping sub-pulmonary ventricle...
September 2017: Giornale Italiano di Cardiologia
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