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https://www.readbyqxmd.com/read/28632651/imaging-in-adult-congenital-heart-disease
#1
Stephanie S Gaydos, Akos Varga-Szemes, Rochelle N Judd, Pal Suranyi, David Gregg
There has been tremendous growth in the population of adults with congenital heart disease (CHD) over the last few decades because of advances in medical care. Whereas some cases are cured during childhood, most patients instead undergo palliation, which leaves them at risk for late complications. Lifelong clinical follow-up involving serial multimodality imaging is helpful to monitor and guide the treatment of late complications. Imaging of these individuals is challenging because of their unique anatomy and therefore requires careful consideration on a case-by-case basis...
July 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/28631208/pulmonary-vein-doppler-patterns-in-infants-with-single-right-ventricle-anomalies-after-initial-staged-palliations
#2
Edward C Kirkpatrick, Jessica Steltzer, Pippa Simpson, Amy Pan, Andrea Dragulescu, Christine B Falkensammer, Sarah Gelehrter, Wyman W Lai, Jami Levine, Stephen Miller, Thomas A Miller, Jay Pruetz, Ritu Sachdeva, Deepika Thacker, Peter Frommelt
The aim of this study was to describe serial changes in echocardiographic Doppler pulmonary vein flow (PVF) patterns in infants with single right ventricle (RV) anomalies enrolled in the Single Ventricle Reconstruction trial. Measurement of PVF peak systolic (S) and diastolic (D) velocities, velocity time integrals (VTI), S/D peak velocity and VTI ratios, and frequency of atrial reversal (Ar) waves were made at three postoperative time points in 261 infants: early post-Norwood, pre-stage II surgery, and 14 months...
June 19, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28608149/exercise-capacity-and-self-efficacy-are-associated-with-moderate-to-vigorous-intensity-physical-activity-in-children-with-congenital-heart-disease
#3
Laura Banks, Shelly Rosenthal, Cedric Manlhiot, Chun-Po Steve Fan, Adam McKillop, Patricia E Longmuir, Brian W McCrindle
This study sought to determine whether exercise capacity, self-efficacy, and gross motor skills are associated with moderate-to-vigorous physical activity (MVPA) levels in children, and if these associations differ by congenital heart disease (CHD) type. Medical history was abstracted from chart review. We assessed MVPA levels (via accelerometry), percent-predicted peak oxygen consumption ([Formula: see text] cardiopulmonary exercise test), gross motor skill percentiles (test of gross motor development version-2), and self-efficacy [children's self-perceptions of adequacy and predilection for physical activity scale (CSAPPA scale)]...
June 12, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28608148/hypoplastic-left-heart-syndrome-sequencing-reveals-a-novel-notch1-mutation-in-a-family-with-single-ventricle-defects
#4
Matthew D Durbin, Adrian G Cadar, Charles H Williams, Yan Guo, David P Bichell, Yan Ru Su, Charles C Hong
Hypoplastic left heart syndrome (HLHS) has been associated with germline mutations in 12 candidate genes and a recurrent somatic mutation in HAND1 gene. Using targeted and whole exome sequencing (WES) of heart tissue samples from HLHS patients, we sought to estimate the prevalence of somatic and germline mutations associated with HLHS. We performed Sanger sequencing of the HAND1 gene on 14 ventricular (9 LV and 5 RV) samples obtained from HLHS patients, and WES of 4 LV, 2 aortic, and 4 matched PBMC samples, analyzing for sequence discrepancy...
June 12, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28599384/adolescent-and-parent-perspectives-prior-to-involvement-in-a-fontan-transition-program
#5
Karin du Plessis, Evelyn Culnane, Rebecca Peters, Yves d'Udekem
Background Successful transition to adult healthcare is particularly important for congenital heart disease (CHD) patients who have undergone palliative surgery, as they risk adverse events if not followed closely. This study examines young people at the worst end of the CHD spectrum who are born with a single ventricle (pumping heart chamber), and who undergo a series of operations that culminate in the Fontan surgical procedure. Purpose To explore adolescents with a Fontan circulation, and their parents', readiness for transition to adult care and pre-implementation of a transition program...
May 24, 2017: International Journal of Adolescent Medicine and Health
https://www.readbyqxmd.com/read/28597808/activation-delay-induced-mechanical-dyssynchrony-in-single-ventricle-heart-disease
#6
Daniel Forsha, Niels Risum, Piers Barker
We present the case of an infant with a single functional ventricle who developed ventricular dysfunction and heart failure due to an electrical activation delay and dyssynchrony. Earlier recognition of this potentially reversible aetiology may have changed her poor outcome.
June 9, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28593158/fontan-revision-with-y-graft-in-a-patient-with-unilateral-pulmonary-arteriovenous-malformation
#7
Jeong-Woo Lee, Jeong-Jun Park, Hyun Woo Goo, Jae Kon Ko
The extracardiac conduit Fontan procedure is the last surgical step in the treatment of patients with a functional single ventricle. An acquired pulmonary arteriovenous malformation may appear perioperatively or postoperatively due to an uneven hepatic flow distribution. Here we report a case of a bifurcated Y-graft Fontan operation in a 15-year-old male patient with a unilateral pulmonary arteriovenous malformation after an extracardiac conduit Fontan operation.
June 2017: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28592182/perioperative-and-anesthetic-considerations-in-atrioventricular-septal-defect
#8
Faith J Ross, Viviane G Nasr, Denise Joffe, Gregory J Latham
Atrioventricular septal defect results from a failure of normal endocardial cushion fusion during embryologic cardiac development. This developmental aberration results in defects in the atrial and/or ventricular septum and malformation of the atrioventricular valves. The pathophysiology of atrioventricular septal defect is variable, and ranges from mild left to right shunting similar to a simple atrial septal defect to complex single-ventricle heart disease. This review focuses on the spectrum of atrioventricular septal defect from partial to complete, without associated cardiac defects...
June 1, 2017: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/28583678/hospital-inpatient-costs-for-single-ventricle-patients-surviving-the-fontan-procedure
#9
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...
May 11, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28575209/clinical-standard-for-valve-area-after-common-atrioventricular-valve-plasty-for-a-single-ventricle
#10
Takashi Kido, Hiroaki Kawata, Shigemitsu Iwai, Kyoichi Nishigaki, Takayoshi Ueno, Hideto Ozawa, Yoshiki Sawa
OBJECTIVES: To determine a clinical standard for post-repair common atrioventricular valve orifice area based on mid- to long-term valve function in patients with a functional single ventricle. METHODS: The medical records of 19 single-ventricle patients who underwent common atrioventricular valve plasty from July 1988 to January 2013 were retrospectively reviewed. Bivalvation valvuloplasty was performed in 7 patients with relatively hypoplastic leaflets. The relationship between the orifice area of the repaired common atrioventricular valve measured intraoperatively and valve function and ventricular volume in the early postoperative period (median, 9...
May 31, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28572979/complex-effects-of-high-flow-nasal-cannula-therapy-on-hemodynamics-in-the-pediatric-patient-after-cardiac-surgery
#11
EDITORIAL
Yu Inata, Muneyuki Takeuchi
BACKGROUND: The high-flow nasal cannula (HFNC) system has been widely used for children in various clinical settings. However, the physiological and clinical impact of HFNC therapy on the pediatric patient with respiratory distress after cardiac surgery has not been thoroughly investigated. MAIN BODY OF THE ABSTRACT: It seems logical to use HFNC as a primary therapy for post-extubation respiratory failure after congenital heart surgery, in which low cardiac output syndrome and upper airway obstruction are commonly encountered; the HFNC therapy alleviates the work of breathing and large negative swings of intrathoracic pressure, which in turn helps to decrease the systemic ventricular afterload...
2017: Journal of Intensive Care
https://www.readbyqxmd.com/read/28571639/longitudinal-outcomes-of-patients%C3%A2-with%C3%A2-single-ventricle-after%C3%A2-the%C3%A2-fontan%C3%A2-procedure
#12
Andrew M Atz, Victor Zak, Lynn Mahony, Karen Uzark, Nicholas D'agincourt, David J Goldberg, Richard V Williams, Roger E Breitbart, Steven D Colan, Kristin M Burns, Renee Margossian, Heather T Henderson, Rosalind Korsin, Bradley S Marino, Kaitlyn Daniels, Brian W McCrindle
BACKGROUND: Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking. OBJECTIVES: This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. METHODS: Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9...
June 6, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28568112/factors-associated-with-early-extubation-after-superior-cavopulmonary-connection-analysis-from-single-ventricle-reconstruction-trial
#13
D Zakaria, M Rettiganti, J M Gossett, P Gupta
BACKGROUND: To evaluate the landscape of early extubation, and identify factors associated with early extubation (≤ 24 h) after superior cavopulmonary connection (stage 2 operation) among children with single ventricle anatomy. METHODS: Patients undergoing stage 2 operation after Norwood operation from the Pediatric Heart Network Single Ventricle Reconstruction (SVR) trial public-use dataset were included. Elastic net regularized logistic regression models were fitted to evaluate the factors associated with early extubation after stage 2 operation...
June 1, 2017: Acta Anaesthesiologica Scandinavica
https://www.readbyqxmd.com/read/28552079/clinical-epidemiology-and-centre-variation-in-chylothorax-rates-after-cardiac-surgery-in-children-a-report-from-the-pediatric-cardiac-critical-care-consortium
#14
Jason R Buckley, Eric M Graham, Michael Gaies, Jeffrey A Alten, David S Cooper, John M Costello, Yuliya Domnina, Darren Klugman, Sara K Pasquali, Janet E Donohue, Wenying Zhang, Mark A Scheurer
Introduction Chylothorax after paediatric cardiac surgery incurs significant morbidity; however, a detailed understanding that does not rely on single-centre or administrative data is lacking. We described the present clinical epidemiology of postoperative chylothorax and evaluated variation in rates among centres with a multicentre cohort of patients treated in cardiac ICU. METHODS: This was a retrospective cohort study using prospectively collected clinical data from the Pediatric Cardiac Critical Care Consortium registry...
May 29, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28549058/histone-deacetylase-adaptation-in-single-ventricle-heart-disease-and-a-young-animal-model-of-right-ventricular-hypertrophy
#15
Weston W Blakeslee, Kimberly M Demos-Davies, Douglas D Lemon, Katharina M Lutter, Maria A Cavasin, Sam Payne, Karin Nunley, Carlin S Long, Timothy A McKinsey, Shelley D Miyamoto
BACKGROUNDHistone deacetylase (HDAC) inhibitors are promising therapeutics for various forms of cardiac disease. The purpose of this study was to assess cardiac HDAC catalytic activity and expression in children with single ventricle heart disease of right ventricular morphology (SV), as well as in a rodent model of right ventricular hypertrophy (RVH).METHODSHomogenates of RV explants from non-failing controls and SV children were assayed for HDAC catalytic activity and HDAC isoform expression. Postnatal 1-day old rat pups were placed in hypoxic conditions and echocardiographic analysis, gene expression, HDAC catalytic activity and isoform expression studies of the RV were performed...
May 26, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28548989/imaging-adult-patients-with-fontan-circulation
#16
Salil Ginde, Benjamin H Goot, Peter C Frommelt
PURPOSE OF REVIEW: Survival after the Fontan procedure for palliation of single ventricle congenital heart disease has improved. However, adults with Fontan circulation are at risk for several complications including heart failure, thromboembolism, and protein-losing enteropathy. This review discusses the role of noninvasive imaging for surveillance and early detection of anatomic and functional abnormalities of the Fontan circulation that can impact the risk for Fontan failure over time...
May 25, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/28542047/inferior-vena-cava-thrombus-in-a-postpartum-patient-with-fontan-physiology-a-case-report
#17
Jessica A Tashjian, Hannah Fraint, James DiNardo, Kathryn Rouine-Rapp
Women with complex congenital heart disease, such as those with single-ventricle physiology, are surviving into adulthood and becoming pregnant. Because of their complex physiology, common peripartum complications pose unique risks. We describe a patient with a single ventricle who underwent an external vascular conduit, nonfenestrated Fontan procedure in childhood and then presented during the postpartum period with extensive thrombosis in her lower extremity deep venous system and inferior vena cava. In this article, we will discuss single-ventricle physiology and the implications of pregnancy, anesthetic considerations, and data for maternal and fetal outcomes in this population...
May 23, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28536530/the-total-artificial-heart-in-end-stage-congenital-heart-disease
#18
REVIEW
Chet R Villa, David L S Morales
The development of durable ventricular assist devices (VADs) has improved mortality rates and quality of life in patients with end stage heart failure. While the use of VADs has increased dramatically in recent years, there is limited experience with VAD implantation in patients with complex congenital heart disease (CHD), despite the fact that the number of patients with end stage CHD has grown due to improvements in surgical and medical care. VAD use has been limited in patients with CHD and end stage heart failure due to anatomic (systemic right ventricle, single ventricle, surgically altered anatomy, valve dysfunction, etc...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28532240/ventricular-assist-devices-for-the-failing-univentricular-circulation
#19
Edward Buratto, William Y Shi, Xin Tao Ye, Igor E Konstantinov
Improved survival following single ventricle palliation has led to a large population of patients with a univentricular circulation, many of whom develop heart failure. Increasing experience with ventricular assist devices (VAD) in children has paved the way for VAD support in those with failing univentricular circulation. Areas covered: The use of VADs to support the failing univentricular circulation is a relatively new concept. Most studies have focused on supporting patients with the failing systemic ventricle...
June 2017: Expert Review of Medical Devices
https://www.readbyqxmd.com/read/28528723/ventricular-pacing-in-single-ventricles-a-bad-combination
#20
Anica Bulic, Frank J Zimmerman, Scott R Ceresnak, Ira Shetty, Kara S Motonaga, Anne Freter, Anthony V Trela, Deb Hanisch, Lisa Russo, Kishor Avasarala, Anne M Dubin
BACKGROUND: Chronic ventricular pacing (VP) is associated with systolic dysfunction in a subset of pediatric patients with heart block and structurally normal hearts. The effect of chronic VP in congenital heart disease is less well understood, specifically in the single-ventricle (SV) population. OBJECTIVE: To determine the longitudinal effect of VP in SV patients. METHODS: SV patients with heart block and dual-chamber pacemakers requiring >50% VP were compared with nonpaced (controls) SV patients matched for age, sex, and SV morphology...
June 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
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