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Cardiology in the Young

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https://www.readbyqxmd.com/read/29463344/haemodynamic-consequences-following-closure-of-an-abernethy-malformation-in-a-patient-following-a-total-cavopulmonary-shunt
#1
Deepa Sasikumar, Bijulal Sasidharan, Anoop Ayyappan
A 17-year-old girl with situs ambiguous, hypoplastic right ventricle with a large ventricular septal defect, and severe pulmonary stenosis had undergone Kawashima operation 10 years back. She had significant desaturation because of a large Abernethy malformation, with reverse shunting from the inferior caval vein to the portal vein. It was closed with a vascular plug, with improvement in oxygen saturation. She developed extensive inferior caval vein thrombus following the procedure, which was managed conservatively by anti-coagulation...
February 21, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29455693/usefulness-and-safety-of-anakinra-in-refractory-kawasaki-disease-complicated-by-coronary-artery-aneurysm
#2
Marie-Paule Guillaume, Héloïse Reumaux, François Dubos
Kawasaki disease is an acute self-limited vasculitis of unknown aetiology. The prognosis depends mainly on coronary damage. There is no consensus regarding optimal adjunctive therapeutics for refractory forms to treatment by intravenous immunoglobulins and corticosteroids. We report the case of an 18-month-old infant with refractory Kawasaki disease complicated by diffuse aneurysms of coronary arteries and successfully treated by anakinra with partial regression of coronary aneurysms.
February 19, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29444731/isolated-aortic-dilation-without-osteoarthritis-a-case-of-smad3-mutation
#3
Jose Arroyave, Juan Manuel Carretero, Domenico Gruosso
Aneurysm-osteoarthritis syndrome is a recently discovered inherited autosomal dominant connective tissue disease caused by SMAD3 mutations. Aneurysm-osteoarthritis syndrome is responsible for 2% of familial thoracic aortic aneurysms and dissections and is characterised by aneurysms, dissections, and tortuosity throughout the arterial tree in combination with osteoarthritis. Early-onset osteoarthritis is present in almost all patients. We present the case of a non-syndromic young boy with SMAD3 mutation isolated from the dilated aortic root and ascending aorta without osteoarthritis...
February 15, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29444729/gemella-bergeri-infective-endocarditis-a-case-report-and-brief-review-of-literature
#4
S Javed Zaidi, Tarek Husayni, Mary A Collins
Gemella is a genus of Gram-positive bacteria found in the digestive tract of humans. They rarely cause systemic illness but have been recently implicated in several serious infections. We report infective endocarditis caused by Gemella bergeri in a 23-year-old with a bicuspid aortic valve status post-intervention in infancy.
February 15, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29444724/early-postoperative-remodelling-following-repair-of-tetralogy-of-fallot-utilising-unsedated-cardiac-magnetic-resonance-a-pilot-study
#5
Michael P DiLorenzo, Elizabeth Goldmuntz, Susan C Nicolson, Mark A Fogel, Laura Mercer-Rosa
Introduction The right ventricular adaptations early after surgery in infants with tetralogy of Fallot are important to understand the changes that occur later on in life; this physiology has not been fully delineated. We sought to assess early postoperative right ventricular remodelling in patients with tetralogy of Fallot by cardiac MRI. Materials and method Subjects with tetralogy of Fallot under 1 year of age were recruited following complete surgical repair for tetralogy of Fallot. Protocol-based cardiac MRI to assess anatomy, function, and flows was performed before hospital discharge using the feed and sleep technique, an unsedated imaging technique...
February 15, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29433601/successful-resection-of-giant-right-atrial-appendage-aneurysm-to-cure-chaotic-atrial-tachycardia-in-a-child-case-report
#6
Yi Zhang, Xiaomei Li, Yongqiang Jin
Right atrial appendage aneurysm is an extremely rare congenital malformation with unknown aetiology. The most common potential complication is atrial arrhythmias including atrial flutter, atrial fibrillation, and atrial tachycardia. These arrhythmias are usually refractory to medication therapy. Radiofrequency catheter ablation has poor efficacy with low success rate and high recurrence rate. Aneurysm resection is the recommended treatment with satisfactory efficacy. We report a child with chaotic atrial tachycardia due to giant right atrial appendage aneurysm who was successfully treated by aneurysm resection...
February 13, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29433600/development-and-preliminary-testing-of-the-brief-developmental-assessment-an-early-recognition-tool-for-children-with-heart-disease
#7
Jo Wray, Katherine L Brown, Deborah Ridout, Monica Lakhanpaul, Liz Smith, Angie Scarisbrick, Sara O'Curry, Aparna Hoskote
Introduction Neurodevelopmental abnormalities are common in children with CHD and are the highest-priority concerns for parents and professionals following cardiac surgery in childhood. There is no additional routine monitoring of development for children with CHD in the United Kingdom; hence, neurodevelopmental concerns may be detected late, precluding early referral and intervention. METHODS: An early recognition tool - the "Brief Developmental Assessment" - was developed using quality improvement methodology involving several iterations and rounds of pilot testing...
February 13, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29433591/paediatric-abstract-publication-rates-for-the-american-society-of-echocardiography-meeting
#8
Adam Morrison, John Kelly, Brian Rivera, Carl H Backes, Clifford L Cua
BACKGROUND: Abstract presentations of scientific information at meetings are important for broadcasting new information. Publication of these studies should be the final goal, but minimal data exist documenting publication rates, especially for paediatric sub-speciality meetings. The goal of this study was to document the manuscript publication rate for paediatric cardiac echocardiography abstracts and to determine whether there were differences between abstracts that were published versus not published...
February 13, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29428001/an-extremely-rare-clinical-entity-congenitally-corrected-transposition-with-situs-%C3%A4-nversus-and-single-coronary-artery-presented-with-complete-atrioventricular-block-in-a-young-man
#9
Omer Faruk Cirakoglu, Ali Bayraktar, Muhammet Rasit Sayin
Congenitally corrected transposition of the great arteries is a rare form of CHD. Situs inversus is a much less common variant of a congenitally corrected transposition of the great arteries. In rare cases, transposition events may be accompanied by various cardiac anomalies. However, situs inversus patients with congenitally corrected transposition, single coronary artery anomaly, and atrioventricular block together have not been reported previously. This combination of abnormalities is presented as a first in the literature...
February 12, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29422111/an-unusual-case-of-interrupted-cervical-aortic-arch-associated-with-long-segment-coarctation-of-the-descending-thoracic-aorta
#10
Mousam Dey, Naveen Garg, Sunil Kumar
Interrupted aortic arch is a rare congenital anomaly in newborns and infants and is commonly associated with other cardiovascular anomalies. Here, we report an unusual case of type A interrupted cervical aortic arch associated with long segment coarctation of the descending thoracic aorta. Patent ductus arteriosus reconstituted the descending thoracic aorta. Proximal segments of the left common carotid and left subclavian arteries were atretic. Echocardiography-gated multidetector CT angiography not only identified the type of aortic arch interruption in the neonate but also delineated the exact anatomical details...
February 9, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29415779/severe-bicuspid-aortic-stenosis-in-pregnancy-balancing-the-risk-of-prematurity-and-maternal-mortality
#11
Chinedu Nwabuobi, Megan McDowell, Sarah Običan
We report a case of combined severe aortic stenosis and regurgitation in a pregnant patient with a history of congenital bicuspid aortic valve. The patient presented at 22 weeks of gestation with angina and pre-syncopal symptoms. During her admission, she experienced intermittent episodes of non-sustained ventricular tachycardia and hypotension. A multi-disciplinary healthcare team was assembled to decide on the appropriate medical and surgical treatment options. At 28 weeks of gestation, the patient underwent a caesarean delivery immediately followed by a mechanical aortic valve replacement...
February 8, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29409567/percutaneous-retrieval-of-a-partially-flared-melody-valve
#12
Katarzyna Gendera, Peter Ewert, Andreas Eicken
We report on successful endovascular retrieval of an accidentally flared Melody valve in an adult patient with an indication for percutaneous pulmonary valve implantation. The Melody valve was removed through a 24 F sheath, introduced via the right jugular vein, and the urgent open-heart surgery was avoided.
February 7, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29409564/post-patent-ductus-arteriosus-ligation-syndrome-with-hypertension-and-masking-of-renal-artery-stenosis-in-an-infant
#13
Erica E ElSeed Peterson, Daniel Mauriello
Post-patent ductus arteriosus ligation syndrome is common, but rarely has hypertension been described following ductal ligation with an unclear mechanism. We report a case of an infant who exhibited features of post-patent ductus arteriosus ligation syndrome and hypertension, but was found to have bilateral renal artery stenosis. Increased systemic vascular resistance can be masked by the parallel circuit physiology of a patent ductus arteriosus.
February 7, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29409559/comparison-of-trace-element-levels-after-cardiopulmonary-bypass-between-cyanotic-and-acyanotic-patients
#14
Firat H Altin, Bahar Ozturk Kurt, Ibrahim C Tanidir, Mehmet Kaya, Okan Yildiz, Meliha Z Kahraman, Sinem B Celebi, Erkut Ozturk, Semra Ozdemir
Trace elements are essential micronutrients for the human body. In this study, we evaluated the alterations in copper, chromium, manganese, selenium, magnesium, zinc, iron, arsenic, boron, and silicon levels in children with cyanotic and acyanotic CHD who underwent cardiac surgery with cardiopulmonary bypass. Participants were divided into the following three groups: patients acyanotic CHDs (n=34), patients with cyanotic CHDs (n=30), and healthy controls (n=30). Blood samples were collected before the surgery and 1 hour after the sternum was closed...
February 7, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29409553/diagnostic-errors-in-paediatric-cardiac-intensive-care
#15
Priya N Bhat, John M Costello, Ranjit Aiyagari, Paul J Sharek, Claudia A Algaze, Mjaye L Mazwi, Stephen J Roth, Andrew Y Shin
Introduction Diagnostic errors cause significant patient harm and increase costs. Data characterising such errors in the paediatric cardiac intensive care population are limited. We sought to understand the perceived frequency and types of diagnostic errors in the paediatric cardiac ICU. METHODS: Paediatric cardiac ICU practitioners including attending and trainee physicians, nurse practitioners, physician assistants, and registered nurses at three North American tertiary cardiac centres were surveyed between October 2014 and January 2015...
February 7, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29409551/impact-of-prenatal-diagnosis-on-the-management-and-early-outcome-of-critical-duct-dependent-cardiac-lesions
#16
Varsha Thakur, Nathalie Dutil, Steven M Schwartz, Edgar Jaeggi
OBJECTIVE: The objective of this study was to compare the preoperative management and outcome of neonates with duct-dependent critical CHD with fetal versus postnatal diagnosis. METHODS: Patients referred with CHD to our centre from January 1, 2009 to December 31, 2010 were enrolled prospectively. Live births with a critical form of CHD, a gestational age ⩾36 weeks and a weight ⩾2 kg at birth, and the intention-to-treat were included in this sub-study. Excluded were neonates with lethal non-cardiac and/or genetic anomalies...
February 7, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29409546/suspected-necrotising-enterocolitis-after-surgery-for-chd-an-opportunity-to-improve-practice-and-outcomes
#17
Eleanor L Schuchardt, Jon Kaufman, Bridget Lucas, Kendra Tiernan, Suzanne Osorio Lujan, Cindy Barrett
Infants with CHD are at increased risk of necrotising enterocolitis, which can interfere with the achievement of adequate nutrition and, ultimately, growth and development. Necrotising enterocolitis is classified by severity as suspected, confirmed, and advanced. We sought to quantify the incidence of all types of necrotising enterocolitis among infants who underwent surgery, with a particular focus on suspected necrotising enterocolitis. This is a retrospective review of all infants <6 months of age who underwent cardiac surgery during 2012 and 2013 at Children's Hospital Colorado...
February 7, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29402340/whole-exome-sequencing-identifies-a-novel-scn5a-mutation-c335r-in-a-chinese-family-with-arrhythmia
#18
Hao Huang, Dong-Bo Ding, Liang-Liang Fan, Jie-Yuan Jin, Jing-Jing Li, Shuai Guo, Ya-Qin Chen, Rong Xiang
BACKGROUND: SCN5A encodes sodium-channel α-subunit Nav1.5. The mutations of SCN5A can lead to hereditary cardiac arrhythmias such as the long-QT syndrome type 3 and Brugada syndrome. Here we sought to identify novel mutations in a family with arrhythmia. METHODS: Genomic DNA was isolated from blood of the proband, who was diagnosed with atrial flutter. Illumina Hiseq 2000 whole-exome sequencing was performed and an arrhythmia-related gene-filtering strategy was used to analyse the pathogenic genes...
February 6, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29362005/a-rare-cause-of-cyanosis-in-newborns-arteriovenous-fistula-between-the-right-pulmonary-artery-and-the-left-atrium-and-its-treatment
#19
Kahraman Yakut, Birgul Varan, Murat Ozkan
The formation of a fistula between the right pulmonary artery and the left atrium via a sac is a very rare cyanotic congenital cardiopulmonary defect. A fistula between the pulmonary artery and left atrium may cause cardiac failure in utero. It can safely be treated surgically and in selected cases closure can be performed with transcatheter insertion of a device. In this article, we present a case with a fistula between the right pulmonary artery and the left atrium that was considered unsuitable for transcatheter closure and was safely treated surgically...
January 24, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29357959/a-rare-case-of-a-patient-with-aortic-root-aneurysm-bicuspid-aortic-valve-and-scimitar-syndrome-with-anomalous-venous-return-to-the-right-superior-pulmonary-vein
#20
Kamran Ahmadov, Catherine A Beigelman, Matthias Kirsch
A rare case of Scimitar Syndrome with an abnormal drainage of the right lower pulmonary vein into the right superior pulmonary vein associated with aortic root aneurism and bicuspid aortic valve is reported.
January 23, 2018: Cardiology in the Young
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