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fontan surgery

Michael L O'Byrne, Michael G McBride, Stephen Paridon, Elizabeth Goldmuntz
BACKGROUND: Obesity is associated with increased lifelong morbidity and reduced life span and is increasingly prevalent in the congenital heart disease population. Habitual exercise is an important aspect of a healthy lifestyle and primary prevention of obesity in the general population. The association between habitual activity and body mass index (BMI) has not been studied in children with congenital heart disease. METHODS: A cross-sectional analysis of two previously collected cohorts was performed, including participants 8 to 18 years old with tetralogy of Fallot, transposition of the great arteries, and single ventricle heart disease after a Fontan operation...
March 2018: World Journal for Pediatric & Congenital Heart Surgery
Shaji C Menon, Ragheed Al-Dulaimi, Brian W McCrindle, David J Goldberg, Ritu Sachdeva, Bryan H Goldstein, Thomas Seery, Karen C Uzark, Anjali Chelliah, Ryan Butts, Heather Henderson, Tiffanie Johnson, Richard V Williams
INTRODUCTION: We sought to evaluate the prevalence of delayed puberty and abnormal anthropometry and its association with quality of life (QoL) in young Fontan survivors. METHODS: This was a cross-sectional study at 11 Pediatric Heart Network centers. Demographic and clinical data, anthropomety, and Tanner stage were collected. Anthropometric measurements and pubertal stage were compared to US norms. QoL was assessed using Pediatric Quality of Life inventory (PedsQL)...
March 9, 2018: Congenital Heart Disease
Louise A Kenny, Fabrizio DeRita, Mohamed Nassar, John Dark, Louise Coats, Asif Hasan
The single ventricle patient population comprises the most complex cohort presenting to the cardiac transplant team, in terms of demographics, anatomic substrate, and unique physiology. It is also the most rapidly growing diagnostic group presenting for heart transplantation. In this manuscript, we aim to describe the changing landscape of transplantation in single ventricle conditions through reflection on our own institution's practice and experience, alongside contemporary literature review. Single ventricle patients are heterogeneous in terms of age, anatomic diagnosis and physiology according to surgical stage of repair...
January 2018: Annals of Cardiothoracic Surgery
Li-Jun Chen, Yu-Qi Zhang, Zhi-Rong Tong, Ai-Min Sun
Analysis of hemodynamics inside tricuspid atresia (TA) chamber is essential to the understanding of TA for optimal treatment. In this study, we introduced a combined computational fluid dynamics (CFD) to simulate blood flow in the left ventricle (LV) to study the diastolic flow changes in TA.Real-time 3-dimentional echocardiography loops (ECHO) were acquired in normal control group, in TA patients before surgery (pre-op group) and after surgery (post-op group). ECHO loops were reconstructed and simulated by CFD, the geometric, volumetric changes, and vortices in the LV were studies and compare among 3 groups...
January 2018: Medicine (Baltimore)
Michael V Di Maria, Lindsey Silverman, Adel K Younoszai, Mariana L Meyers, Kelley E Capocelli, Michael R Narkewicz
BACKGROUND: The current management paradigm for children with single ventricle heart disease involves a series of palliative surgeries, culminating in the Fontan operation. This physiology results in a high central venous pressure, and in the setting of single ventricle heart disease, results in hepatic injury and fibrosis over time. Fontan-associated liver disease (FALD) is universally present in this cohort, and the current gold standard for diagnosis remains biopsy. Noninvasive assessments of liver fibrosis, such as ultrasound with elastography or acoustic radiation force impulse (ARFI), has been utilized in this cohort...
February 23, 2018: Congenital Heart Disease
Luis Téllez, Enrique Rodríguez-Santiago, Agustín Albillos
Fontan-associated liver disease is a hepatic disorder arising from hemodynamic changes and systemic venous congestion following Fontan surgery. The histological changes produced in the liver are similar but not equivalent to those seen in other forms of cardiac liver disease. While the natural history of this form of liver disease is not well established, over time many Fontan patients develop portal hypertension-related complications such as ascites, variceal hemorrhage or encephalopathy. Fontan survivors also show an increased risk of hepatocellular carcinoma...
March 1, 2018: Annals of Hepatology
Yohei Yamada, Ken Hoshino, Takayuki Oyanagi, Ryohei Gatayama, Jun Maeda, Nobuyuki Katori, Yasushi Fuchimoto, Taizo Hibi, Masahiro Shinoda, Kentaro Matsubara, Hideaki Obara, Ryo Aeba, Yuko Kitagawa, Hiroyuki Yamagishi, Tatsuo Kuroda
Children with single ventricle physiology have complete mixing of the pulmonary and systemic circulations, requiring staged procedures to achieve a separation of these circulations, or Fontan circulation. The single ventricle physiology significantly increases the risk of mortality in children undergoing non-cardiac surgery. As liver transplantation for patients with single ventricle physiology is particularly challenging, only a few reports have been published. We herein report a case of successful LDLTx for an 8-month-old pediatric patient with biliary atresia, heterotaxy, and complex heart disease of single ventricle physiology...
February 19, 2018: Pediatric Transplantation
Precylia Fernandes, Lito Mantagou, Ram Ramaraj, Umber Agarwal, Joyce Su Ling Lim
Introduction: A functionally single cardiac ventricle seen on foetal ultrasound scan carries a guarded prognosis. The antenatal diagnosis of anomalous pulmonary venous connection (APVC) remains challenging, if there is no associated structural cardiac abnormality. Antenatally, a combination of complex cardiac anomaly with suspected isomerism should raise the possibility of associated total anomalous pulmonary venous connection (TAPVC). There needs to be a high index of suspicion for TAPVC, in functional single ventricle and suspected isomerism, as this carries a very grim outcome postnatally...
February 2018: Ultrasound: Journal of the British Medical Ultrasound Society
Masanari Kuwabara, Koichiro Niwa, Tomohiko Toyoda, Takeaki Shirai, Shigeru Tateno, Hideo Ohuchi, Yasuhiko Tanaka, Fukiko Ichida, Tomoo Fujisawa, Teiji Akagi, Yoshiki Mori
BACKGROUND: Fontan-associated liver disease (FALD) is an important late complication involving liver dysfunction, such as liver cirrhosis (LC) and hepatocellular carcinoma (HCC), in patients undergoing the Fontan procedure. However, the prevalence, clinical manifestation, and methods of diagnosis of FALD are still not well established.Methods and Results:This study comprised 2 nationwide surveys in Japan. First, the prevalence of LC and/or HCC in patients undergoing the Fontan procedure was determined...
February 14, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
Luis Téllez, Enrique Rodríguez de Santiago, Agustín Albillos
Fontan-associated liver disease refers to the disturbance in the liver secondary to hemodynamic changes and systemic venous congestion following Fontan surgery. Although the natural history of this disease has not yet been established, patients with more advanced liver injury develop the complications of portal hypertension, such as ascites, variceal haemorrhage, or encephalopathy. Moreover, patients with Fontan surgery may have an increased risk of hepatocellular carcinoma. Periodic liver monitoring is essential to prevent this disease and provide early treatment of liver complications...
February 9, 2018: Revista Española de Cardiología
C L Poh, Y d'Udekem
AIM: We now know that 20-40% of patients with a single ventricle will develop heart failure after the second decade post-Fontan surgery. However, we remain unable to risk-stratify the cohort to identify patients at highest risk of late failure and death. We conducted a systematic review of all reported late outcomes for patients with a Fontan circulation to identify predictors of late death. METHODS: We searched MEDLINE, Embase and PubMed with subject terms ("single ventricle", "Hypoplastic left heart syndrome", "congenital heart defects" or "Fontan procedure") AND ("heart failure", "post-operative complications", "death", "cause of death", "transplantation" or "follow-up studies") for relevant studies between January 1990 and December 2015...
December 20, 2017: Heart, Lung & Circulation
Margarita Brida, Gerhard-Paul Diller, Michael A Gatzoulis
The systemic right ventricle (SRV) is commonly encountered in congenital heart disease representing a distinctly different model in terms of its anatomic spectrum, adaptation, clinical phenotype, and variable, but overall guarded prognosis. The most common clinical scenarios where an SRV is encountered are complete transposition of the great arteries with previous atrial switch repair, congenitally corrected transposition of the great arteries, double inlet right ventricle mostly with previous Fontan palliation, and hypoplastic left heart syndrome palliated with the Norwood-Fontan protocol...
January 30, 2018: Circulation
Laura S Kverneland, Peter Kramer, Stanislav Ovroutski
Almost fifty years after its first clinical application, the modified Fontan operation is among the most frequently performed procedures in congenital heart disease surgery in children today. The objective of this review is to systematically summarize the international evolution of outcomes in regard to morbidity and mortality of patients with Fontan palliation. All studies published over the past five decades with more than 100 Fontan patients included were screened. In eligible studies, information concerning preoperative patients' characteristics, Fontan modifications employed, early mortality, long-term survival and frequency of relevant complications was extracted...
January 25, 2018: Congenital Heart Disease
Juan Pedro-Botet, Elisenda Climent, David Benaiges, Juana A Flores-Le-Roux, Jose M Ramon, Montserrat Villatoro, Laia Fontané, Juan J Chillarón, Albert Goday
No abstract text is available yet for this article.
August 2017: Atherosclerosis
Dominik Siallagan, Yue-Hin Loke, Laura Olivieri, Justin Opfermann, Chin Siang Ong, Diane de Zélicourt, Anastasios Petrou, Marianne Schmid Daners, Vartan Kurtcuoglu, Mirko Meboldt, Kevin Nelson, Luca Vricella, Jed Johnson, Narutoshi Hibino, Axel Krieger
BACKGROUND: Despite advances in the Fontan procedure, there is an unmet clinical need for patient-specific graft designs that are optimized for variations in patient anatomy. The objective of this study is to design and produce patient-specific Fontan geometries, with the goal of improving hepatic flow distribution (HFD) and reducing power loss (Ploss), and manufacturing these designs by electrospinning. METHODS: Cardiac magnetic resonance imaging data from patients who previously underwent a Fontan procedure (n = 2) was used to create 3-dimensional models of their native Fontan geometry using standard image segmentation and geometry reconstruction software...
December 5, 2017: Journal of Thoracic and Cardiovascular Surgery
Jeffrey P Jacobs, John E Mayer, Sara K Pasquali, Kevin D Hill, David M Overman, James D St Louis, S Ram Kumar, Carl L Backer, Charles D Fraser, James S Tweddell, Marshall L Jacobs
The Society of Thoracic Surgeons Congenital Heart Surgery Database is a comprehensive clinical outcomes registry which captures almost all pediatric cardiac surgical operations in the United States. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This article summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency...
January 11, 2018: Annals of Thoracic Surgery
Luiz Fernando Caneo, Aida L R Turquetto, Rodolfo A Neirotti, Maria A Binotto, Leonardo A Miana, Carla Tanamati, Juliano G Penha, João B D Silveira, Thais M Alexandre E Silva, Fabio B Jatene, Marcelo B Jatene
BACKGROUND: The Fontan operation (FO) has evolved and many centers have demonstrated improved results relative to those from earlier eras. We report a single-institution experience over three decades, describing the outcomes and evaluating risk factors. METHODS: Successive patients undergoing primary FO were divided into era I (1984-1994), era II (1995-2004), and era III (2005-2014). Clinical and operative notes were reviewed for demographic, anatomic, and procedure details...
May 2017: World Journal for Pediatric & Congenital Heart Surgery
Ivan A Yurlov, Vladimir P Podzolkov, Dmitry V Kovalev, Mikhail M Zelenikin, Victor B Samsonov, Nikolay S Chikin, Lyudmila A Yurpol'skaya, Tatyana V Mataeva
OBJECTIVES: This study aimed to investigate the indications for and the results of axillary arterio-venous fistula in patients with a functionally single ventricle at mid-term follow-up after bidirectional cavopulmonary connection. METHODS: From 2012 to 2017, 20 patients aged 21 ± 8.7 years underwent axillary arterio-venous fistula after bidirectional cavopulmonary connection in our institution. The interval between bidirectional cavopulmonary connection and axillary arterio-venous fistula was 13...
January 5, 2018: European Journal of Cardio-thoracic Surgery
Jun Negishi, Hideo Ohuchi, Aya Miyazaki, Etsuko Tsuda, Isao Shiraishi, Kenichi Kurosaki
BACKGROUND: Heart failure (HF) is an important complication in adults with congenital heart disease (CHD), but because only a few studies have focused on acute HF hospitalization in adults with CHD, we study aimed to define the clinical characteristics of such patients and examine the differences in acute HF between adults with CHD and acquired heart disease.Methods and Results:We retrospectively evaluated 50 adults with CHD admitted for treatment of acute HF and compared their data with those from Japanese HF registries...
December 29, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
Kristina N Heye, Walter Knirsch, Beatrice Latal, Ianina Scheer, Kristina Wetterling, Andreas Hahn, Hakan Akintürk, Dietmar Schranz, Ingrid Beck, Ruth O Gorman Tuura, Bettina Reich
BackgroundLittle is known about the relationship between brain volumes and neurodevelopmental outcome at 2 years of age in children with single-ventricle congenital heart disease (CHD). We hypothesized that reduced brain volumes may be associated with adverse neurodevelopmental outcome.MethodsVolumetric segmentation of cerebral magnetic resonance imaging (MRI) scans was carried out in 44 patients without genetic comorbidities and in 8 controls. Neurodevelopmental outcome was assessed with the Bayley-III scales...
January 2018: Pediatric Research
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