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Pediatric Cardiology

Nazmi Narin, Ozge Pamukcu, Aydin Tuncay, Ali Baykan, Suleyman Sunkak, Onur Tasci, Kazim Uzum, Levent Saltık
Untreated ventricular septal defect (VSD) is an important cause of congestive heart failure in early infancy. Growth is impaired in this population, and surgical closure is challenging because of congestion in the lungs, making infants prone to respiratory infection, and because of their poor nutritional status. The aim of this study is to share our experience with percutaneous VSD closure in patients under 1 year of age. Patients with hemodynamically significant left-to-right shunt, less than 1 year of age, and with VSD diameter ≤ 6 mm were retrospectively included in the study between December 2014 and January 2017...
March 15, 2018: Pediatric Cardiology
Johannes Krämer, Felix Kreuzer, Michael Kaestner, Peter Bride, Fabian von Scheidt, Jannos Siaplaouras, Heiner Latus, Dietmar Schranz, Christian Apitz
Right ventricular (RV) hypertrophy is regarded as the adaptation on chronic RV pressure load in pulmonary hypertension. As the RV Sokolow-Lyon index (RVSLI) is an electrocardiographic marker of RV hypertrophy, we hypothesized that RVSLI might be able to reflect RV pressure load. Therefore, the purpose of this study was to characterize the diagnostic impact of the RVSLI in children with idiopathic pulmonary arterial hypertension (IPAH) in order to assess disease severity and to evaluate its value for the prediction of worse outcome...
March 14, 2018: Pediatric Cardiology
Ibtessam R Hussein, Rima S Bader, Adeel G Chaudhary, Randa Bassiouni, Maha Alquaiti, Fai Ashgan, Hans-Juergen Schulten, Mohammad H Al Qahtani
Congenital heart defects (CHDs) are the most common birth defects in neonatal life. CHDs could be presented as isolated defects or associated with developmental delay (DD) and/or other congenital malformations. A small proportion of cardiac defects are caused by chromosomal abnormalities or single gene defects; however, in a large proportion of cases no genetic diagnosis could be achieved by clinical examination and conventional genetic analysis. The development of genome wide array-Comparative Genomic Hybridization technique (array-CGH) allowed for the detection of cryptic chromosomal imbalances and pathogenic copy number variants (CNVs) not detected by conventional techniques...
March 14, 2018: Pediatric Cardiology
Wen Lin, Deqiang Li
Ventricular myocardial development is a well-orchestrated process involving different cardiac structures, multiple signal pathways, and myriad proteins. Dysregulation of this important developmental event can result in cardiomyopathies, such as left ventricle non-compaction, which affect the pediatric population and the adults. Human and mouse studies have shed light upon the etiology of some cardiomyopathy cases and highlighted the contribution of both genetic and environmental factors. However, the regulation of ventricular myocardial development remains incompletely understood...
March 13, 2018: Pediatric Cardiology
Arisa Kojima, Tadayoshi Hata, Tsuneaki Sadanaga, Yuri Mizutani, Hidetoshi Uchida, Yuri Kawai, Masahiko Manabe, Masayuki Fujino, Yoshihiko Eryu, Hiroko Boda, Masafumi Miyata, Tetsushi Yoshikawa
Reduced heart rate (HR) variability in preterm infants compared with full-term infants suggests that autonomic cardiac control is developmentally delayed. However, the association between developmental changes in myocardial repolarization and gestational age remains unknown. This study investigated the association between the myocardial repolarization lability index, namely the QT variability index (QTVI) = log10 [(QTv/QTm2 )/(HRv/HRm2 )], and the perinatal profile of healthy 1-month-old infants. We included 209 infants (143 boys and 87 girls; mean gestational weeks at birth, 38...
March 12, 2018: Pediatric Cardiology
Luke Lamers, Erick E Jimenez, Catherine Allen, Derreck Hoyme, Entela Bua Lushaj, Petros V Anagnostopoulos
The purpose of this study was to assess the diagnostic capabilities of transesophageal echocardiography (TEE) compared to completion angiography for detection of residual post-operative pulmonary artery lesions. This is a retrospective review of 19 consecutive surgical cases involving the pulmonary arteries that had post-operative TEE and completion angiography from 2014 to 2017. The echocardiograms were reviewed by 2 blinded examiners and categorized as adequate or inadequate visualization of the surgical repair...
March 10, 2018: Pediatric Cardiology
Hari Rajagopal, Santosh C Uppu, Justin Weigand, Simon Lee, Ruchika Karnik, Helen Ko, Puneet Bhatla, James Nielsen, John Doucette, Ira Parness, Shubhika Srivastava
Right atrial (RA) size is a prognostic indicator for heart failure and cardiovascular death in adults. Data regarding use of RA area (RAA) by two-dimensional echocardiography as a surrogate for RA size and allometric modeling to define appropriate indexing of the RAA are lacking. Our objective was to validate RAA as a reliable measure of RA size and to define normal reference values by transthoracic echocardiography (TTE) in a large population of healthy children and develop Z-scores using a validated allometric model for indexing RAA independent of age, sex, and body size...
March 9, 2018: Pediatric Cardiology
Samuel Wittekind, Wayne Mays, Yvette Gerdes, Sandra Knecht, John Hambrook, William Border, John Lynn Jefferies
Patients with a Fontan circulation have impaired exercise capacity. Cardiac rehabilitation (CR) has shown promise in enhancing peak exercise parameters in this population, but an improvement in submaximal exercise has not been consistently demonstrated. We assessed the hypothesis that participation in CR will be associated with more efficient oxygen extraction and ventilation during submaximal exercise. In this prospective study, pediatric Fontans completed two 60 min CR sessions per week for 12 weeks. Cardiopulmonary exercise testing and stress echocardiography were performed at baseline and last CR session, and then compared with a paired sample t test...
March 9, 2018: Pediatric Cardiology
Takashi Kido, Takaya Hoashi, Masataka Kitano, Masatoshi Shimada, Kenichi Kurosaki, Hatsue Ishibashi-Ueda, Hajime Ichikawa
The purpose of the study is to analyze the impact of hybrid stage 1 palliation on right ventricular myocardial pathology in hypoplastic left heart syndrome. Sufficient amount of right ventricular biopsies could be obtained from 16 of 32 patients who underwent Norwood operation between 2007 and 2013. Histopathological findings of right ventricle in patients who underwent primary Norwood operation (primary group, n = 5), patients with aortic atresia (HS1P AA group, n = 6) or aortic stenosis (HS1P AS group, n = 5) who underwent staged Norwood palliation following hybrid stage 1 palliation were compared...
March 9, 2018: Pediatric Cardiology
Gabriel Altit, Shazia Bhombal, Krisa Van Meurs, Theresa A Tacy
INTRODUCTION: Newborns with congenital diaphragmatic hernia (CDH) have varying degrees of pulmonary hypoplasia and pulmonary hypertension (PH), and there is limited evidence that cardiac dysfunction is present. We sought to study early neonatal biventricular function and performance in these patients by reviewing early post-natal echocardiography (ECHO) measurements and comparing them to normal term newborns. METHODS: Retrospective case-control study reviewing clinical and ECHO data on term newborns with CDH and normal controls born between 2009 and 2016...
March 9, 2018: Pediatric Cardiology
Shinya Iwasawa, Tomomi Uyeda, Mika Saito, Taku Ishii, Akio Inage, Yuji Hamamichi, Satoshi Yazaki, Tadahiro Yoshikawa
Amiodarone (AMD) is a class III anti-arrhythmic drug that is highly effective for tachyarrhythmia treatment. AMD is widely used in adults with congenital heart disease (CHD); however, higher doses of AMD (> 200 mg/day) can cause various non-cardiac side effects. The purpose of this study was to assess the efficacy, safety, and adverse events of low-dose AMD (≤ 200 mg/day) for tachyarrhythmia in patients with CHD. We retrospectively studied 80 patients with CHD and tachyarrhythmia who received oral low-dose AMD (≤ 200 mg/day) from January 2004 to March 2016...
March 9, 2018: Pediatric Cardiology
Shai Tejman-Yarden, Eyal Nof, Roy Beinart, Nadav Ovadia, Yuval Goldshmit, Jonathan Buber, Hagith Yonath, Eitan Keizman, Michael Glikson
Permanent cardiac pacing is the only effective solution for patients with symptomatic bradycardia and heart block. About 10% of patients undergoing implantation of the conventional pacing system develop complications related to the subcutaneous pocket or the leads and in pediatric patients lead problems may rise in up to 30% of the patients. The leadless pacemaker devices were developed in order to minimize some of those complications. We present a case of an 11-year-old patient who presented after the sudden death of his older brother, with recurrent episodes of syncope and documented prolonged sinus pauses...
March 8, 2018: Pediatric Cardiology
Emilie Jean-St-Michel, James M Meza, Jonathon Maguire, John Coles, Brian W McCrindle
Ventricular dysfunction affects survival in patients with single right ventricle (RV), and remains one of the primary indications for heart transplantation. Since it is challenging to predict the capacity of patients with ventricular dysfunction to proceed to the stage II procedure, we sought to identify factors that would be associated with death or heart transplantation without achieving stage II for single RV patients with ventricular dysfunction after Norwood procedure. The Single Ventricle Reconstruction (SVR) trial public-use database was used...
March 8, 2018: Pediatric Cardiology
Dai Asada, Kenichi Okumura, Kazuyuki Ikeda, Toshiyuki Itoi
As the important role of longitudinal shortening in ventricular function has been well recognized over the past decade, evaluation of longitudinal systolic function of the left ventricle has become a subject of growing interest. Tissue motion annular displacement of the mitral valve (TMAD) is a new parameter of longitudinal systolic function. Although some studies have reported that this new parameter correlates with left ventricular ejection fraction (LVEF) in adults, little is known about TMAD in normal children...
March 8, 2018: Pediatric Cardiology
Laura Mercer-Rosa, Okan U Elci, Nelangi M Pinto, Ronn E Tanel, Elizabeth Goldmuntz
Deletion of 22q11.2 (del22q11) is associated with adverse outcomes in patients with tetralogy of Fallot (TOF). We sought to investigate its contribution to perioperative outcome in patients with a severe form of TOF characterized by pulmonary atresia (PA) or severe pulmonary stenosis (PS) and major aortopulmonary collateral arteries (MAPCAS). We conducted a retrospective review of patients with TOF/MAPCAS who underwent staged surgical reconstruction between 1995 and 2006. Groups were compared according to 22q11...
March 8, 2018: Pediatric Cardiology
Marzena Barczuk-Falęcka, Łukasz A Małek, Hubert Krysztofiak, Danuta Roik, Michał Brzewski
Physical training is associated with changes in cardiac morphology called the "athlete's heart", which has not been sufficiently studied in children. The aim of the study was to analyze cardiac adaptation to exercise in pre-adolescent soccer players. Thirty-six soccer players (mean age 10.1 ± 1.4 years) and 24 non-athlete male controls (10.4 ± 1.7 years) underwent cardiac magnetic resonance. Measurements of myocardial mass, end-diastolic and end-systolic volume, stroke volume and ejection fraction for left and right ventricle (LV, RV) were performed...
March 8, 2018: Pediatric Cardiology
Renuka E Peterson, Grace Freire, Cynthia J Marino, Saadeh B Jureidini
Right ventricular-dependent coronary circulation (RVDCC) is associated with pulmonary atresia with intact ventricular septum and is defined by two or more epicardial coronary arteries with atresia or severe stenosis resulting in the dependency of coronary supply by retrograde flow. The hypothesis of this study is that coronary Doppler flow patterns on echocardiography can be used to distinguish patients with RVDCC. Between 2007 and 2016, we reviewed 16 patients with pulmonary atresia or critical pulmonary stenosis...
March 8, 2018: Pediatric Cardiology
Lv Liu, Rong Yu
No abstract text is available yet for this article.
March 8, 2018: Pediatric Cardiology
Aamisha Gupta, Dennis VanLoozen, Anastasios C Polimenakos, Kenneth A Murdison
Anomalous origin of one pulmonary artery from the ascending aorta is a rare congenital anomaly. Even more rarely reported is its presence in conjunction with persistent pulmonary hypertension of the newborn (PPHN). We present a case of a full-term infant, initially thought to have PPHN and later found to have anomalous origin of the right pulmonary artery from the ascending aorta. We discuss our management concept which included use of PgE1 infusion to restore fetal circulation prior to surgical treatment in this unique clinical scenario...
March 2, 2018: Pediatric Cardiology
Emily F Moore, Jennifer Pak, Christa Jefferis-Kirk, Arlene Armatage, Richard A Kronmal, Jack C Salerno, Matthew D Files
Heparin is used to decrease the risk of thromboembolic complications during electrophysiology studies (EPS); however, there is wide practice variation and minimal evidence to guide heparin dosing, particularly in pediatric patients. This study retrospectively analyzed heparin dosing and response, measured via activated clotting time (ACT), in patients undergoing EPS and used these data (pre-protocol cohort, n = 40), as well as guidance from available literature to implement a standardized heparin protocol (phase 1, n = 43)...
March 2, 2018: Pediatric Cardiology
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