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congenital cardiac disease

Douglas Y Mah, Divya Shakti, Kimberlee Gauvreau, Steven D Colan, Mark E Alexander, Dominic J Abrams, David W Brown
Left atrial (LA) dilation has been shown to be associated with atrial fibrillation (AF) in the adult population, with some studies indicating that larger LAs are more prone to AF recurrence. The relation of LA size to AF in the pediatric and young adult population has not been investigated. In this study, all pediatric patients (aged ≤22 years) who presented to Boston Children's Hospital from January 2002 to December 2012 with AF were reviewed. Patients with significant congenital heart disease, cardiomyopathies, proven channelopathies, previous cardiac surgery, end-stage renal disease, or severe lung disease/cystic fibrosis were excluded...
September 30, 2016: American Journal of Cardiology
Masoumeh Ghafarzadeh, Mehrdad Namdari, Ali Eatemadi
Congenital heart disease (CHD) is the most prevalent congenital anomaly in newborn babies. Cardiac malformations have been induced in different animal model experiments, by perturbing some molecules that take part in the developmental pathways associated with myocyte differentiation, specification, or cardiac morphogenesis. The exact epigenetic, environmental, or genetic, basis for these molecules perturbations is yet to be understood. But, scientist have bridged this gap by introducing autologous stem cell into the defective hearts to treat CHD...
October 22, 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
Marco Astengo, Caroline Berntsson, Åse A Johnsson, Peter Eriksson, Mikael Dellborg
OBJECTIVE: Coarctation of the aorta (CoA) is a common condition. Adult patients with newly diagnosed CoA and patients with recurring or residual CoA require evaluation of the severity of aortic obstruction. Cardiac catheterization is considered the gold standard for the evaluation of hemodynamically significant CoA. The European Society of Cardiology (ESC) Guidelines for the management of grown-up congenital heart disease (GUCH) include noninvasive criteria for identifying significant CoA...
October 25, 2016: Congenital Heart Disease
Christopher F Tirotta, Tuan Nguyen, Steven Fishberger, Evelio Velis, Melissa Olen, Lourdes Lam, Danielle R Madril, Jessica Hughes, Richard G Lagueruela
BACKGROUND: Dexmedetomidine is a selective alpha-2 adrenergic agonist with sedative, analgesic, and anxiolytic properties. Dexmedetomidine has not been approved for use in pediatrics. Dexmedetomidine has been reported to depress sinus node and atrioventricular nodal function in pediatric patients; it has been suggested that the use of dexmedetomidine may not be desirable during electrophysiological studies. AIM: We hypothesize that the use of dexmedetomidine does not inhibit the induction of supraventricular tachyarrhythmias (SVT) during electrophysiological studies and does not inhibit the ablation of such arrhythmias...
October 25, 2016: Paediatric Anaesthesia
S Harikrishnan, G Sanjay, M Ashishkumar, Jaideep Menon, G Rajesh, R Krishna Kumar
BACKGROUND: Pulmonary hypertension (PH) is a disease associated with a high morbidity and mortality. There is paucity of data regarding PH from the developing countries including India. Idiopathic pulmonary arterial hypertension is the most important etiological factor in the western world, but PH secondary to rheumatic heart disease, chronic obstructive pulmonary disease and untreated congenital heart disease could well be the predominant causes in developing countries like India. The main objective of the PROKERALA study - Pulmonary hypertension Registry Of Kerala is to collect data regarding the etiology, practice patterns and one-year outcomes of patients diagnosed to have PH...
September 2016: Indian Heart Journal
K L Yew, Z Kang, A Anum
Coronary artery anomalies are often discovered incidentally during cardiac catheterization or computed tomography coronary angiography and may involve the affected coronary artery origin and its course. Coronary artery anomalies are associated with congenital heart disease. The affected coronary arteries may have an unusual high take off origin, origin from contralateral or non-coronary sinus, origin from the pulmonary artery, single coronary system or coronary artery fistula.
August 2016: Medical Journal of Malaysia
Amee M Bigelow, Brandon S Arnold, Gregory C Padrutt, John M Clark
In current practice, children with anatomically normal hearts routinely undergo fluoroscopy-free ablations. Infants and children with congenital heart disease (CHD) represent the most difficult population to perform catheter ablation without fluoroscopy. We report two neonatal patients with CHD in whom cardiac ablations were performed without fluoroscopy. The first infant had pulmonary atresia with intact ventricular septum with refractory supraventricular tachycardia, and the second infant presented with Ebstein's anomaly of the tricuspid valve along with persistent supraventricular tachycardia...
October 21, 2016: Cardiology in the Young
Jie Wu, Wei-Guang Ding, Minoru Horie
Long QT syndrome type 1 (LQT1) is a subtype of a congenital cardiac syndrome caused by mutation in the KCNQ1 gene, which encodes the α-subunit of the slow component of delayed rectifier K(+) current (IKs) channel. Arrhythmias in LQT1 are characterized by prolongation of the QT interval on ECG, as well as the occurrence of life-threatening cardiac events, frequently triggered by adrenergic stimuli (e.g., physical or emotional stress). During the past two decades, much advancement has been made in understanding the molecular pathogenesis underlying LQT1...
October 2016: Journal of Arrhythmia
Christoph Preuss, Melanie Capredon, Florian Wünnemann, Philippe Chetaille, Andrea Prince, Beatrice Godard, Severine Leclerc, Nara Sobreira, Hua Ling, Philip Awadalla, Maryse Thibeault, Paul Khairy, Mark E Samuels, Gregor Andelfinger
Left-ventricular outflow tract obstructions (LVOTO) encompass a wide spectrum of phenotypically heterogeneous heart malformations which frequently cluster in families. We performed family based whole-exome and targeted re-sequencing on 182 individuals from 51 families with multiple affected members. Central to our approach is the family unit which serves as a reference to identify causal genotype-phenotype correlations. Screening a multitude of 10 overlapping phenotypes revealed disease associated and co-segregating variants in 12 families...
October 2016: PLoS Genetics
Adriana Yock-Corrales, Flory Varela-Bulgarelli, Cary Barboza, Alfonso Gutierrez-Mata, Mark T Mackay, Franz Babl
OBJECTIVES: The aim was to describe clinical presentation, management, and outcomes of stroke in a tertiary emergency department (ED) of a developing country. METHODOLOGY: Retrospective case series of patients aged 1 month to 18 years presenting to an ED with radiological confirmed acute stroke during a 7-year period were studied. RESULTS: Ninety-five patients were identified. Twenty-five patients were excluded because of incomplete records (8) or not presenting via ED (17)...
October 4, 2016: Pediatric Emergency Care
Jessica H Rankin, Marsha Elkhunovich, Dina Seif, Mikaela Chilstrom
Neonatal respiratory distress is an emergent condition with a wide differential diagnosis. A 12-day-old newborn presented to the emergency department in respiratory distress. Point-of-care ultrasound allowed clinicians to rapidly exclude cardiac disease and pneumothorax as possible causes of the patient's respiratory distress, and expedited the identification of congenital diaphragmatic hernia. The ultrasound findings and technique, epidemiology, pathophysiology, and radiological diagnosis of congenital diaphragmatic hernia are reviewed...
October 2016: Pediatric Emergency Care
Ana L Neves, Tiago Henriques-Coelho, Adelino Leite-Moreira, José C Areias
OBJECTIVE: The aim of this article is to evaluate the clinical utility of brain natriuretic peptide in pediatric patients, examining the diagnostic value, management, and prognostic relevance, by critical assessment of the literature. DATA SOURCES: In December 2015, a literature search was performed (PubMed access to MEDLINE citations; and included these Medical Subject Headings and text terms for the key words: "brain natriuretic peptide," "amino-terminal pro-brain natriuretic peptide," "children," "neonate/s," "newborn/s," "infant/s," and "echocardiography...
September 30, 2016: Pediatric Critical Care Medicine
Kirsten C Odegard, Robert Vincent, Rahul G Baijal, Suanne M Daves, Robert G Gray, Alexander J Javois, Barry A Love, Philip Moore, David Nykanen, Lori Q Riegger, Scott G Walker, Elizabeth C Wilson
Current practice of sedation and anesthesia for patients undergoing pediatric and congenital cardiac catheterization laboratory (PCCCL) procedures is known to vary among institutions, a multi-society expert panel with representatives from the Congenital Heart Disease Council of the Society for Cardiovascular Angiography and Interventions, the Society for Pediatric Anesthesia and the Congenital Cardiac Anesthesia Society was convened to evaluate the types of sedation and personnel necessary for procedures performed in the PCCCL...
October 3, 2016: Anesthesia and Analgesia
Michael L Rigby
Major congenital or acquired heart disease in neonates presents with cyanosis, hypoxia, acute circulatory failure or cardiogenic shock. Antenatal diagnosis is made in up to 50% but heart disease is unanticipated in the remainder. The presence of significant heart disease in premature infants is also frequently not suspected at first; in general, whatever the underling cardiac anomaly, the clinical condition is worse, deteriorates more quickly and carries a poorer prognosis in premature and low birth weight infants...
October 13, 2016: Early Human Development
Elisabeth Leirgul, Kristoffer Brodwall, Gottfried Greve, Stein E Vollset, Henrik Holmstrøm, Grethe S Tell, Nina Øyen
OBJECTIVE: To investigate the association between pregestational or gestational diabetes and offspring risk of congenital heart defects and the association between large-for-gestational-age birth weight and risk of cardiac defects in offspring of diabetic women. METHODS: Information on pregestational and gestational diabetes, cardiac defects, and birth weight among all births in Norway in 1994-2009 was ascertained from the Medical Birth Registry of Norway, national health registries, and the Cardiovascular Disease in Norway project...
October 6, 2016: Obstetrics and Gynecology
Alessandro Giamberti, Francesca R Pluchinotta, Massimo Chessa, Alessandro Varrica, Raffaele Vitale, Alessandro Frigiola, Carlo Pappone, Marco Ranucci
AIMS: Supraventricular arrhythmias are a major cause of morbidity and mortality in adult patients with congenital heart disease (CHD). Intraoperative ablation offers an alternative for patients who failed ablation procedures or are requiring concomitant surgical intervention. We present our long-term results with the surgical treatment of arrhythmias in adults with CHD (ACHD) undergoing elective cardiac surgery and the clinical predictors for arrhythmia recurrence. METHODS AND RESULTS: Between 2002 and 2013, 80 consecutive patients with CHD, mean age of 39 years, underwent intraoperative ablation with monopolar irrigated radiofrequency during cardiac surgery procedures...
October 12, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
Erin A Fender, Ammar M Killu, Bryan C Cannon, Paul A Friedman, Christopher J Mcleod, David O Hodge, Craig S Broberg, Charles A Henrikson, Yong-Mei Cha
AIMS: Patients with congenital heart disease (CHD) are at increased risk for intracardiac device malfunction and infection that may necessitate extraction; however, the risk of extraction is poorly understood. This study addresses the safety of extraction in patients with structural heart disease and previous cardiac surgery. METHODS: This retrospective study included 40 CHD and 80 matched control patients, who underwent transvenous lead extractions between 2001 and 2014...
October 13, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
Candice Torres de Melo Bezerra Cavalcante, Nayana Maria Gomes de Souza, Valdester Cavalcante Pinto, Klébia Magalhães Pereira Castello Branco, Ronald Guedes Pompeu, Andreia Consuelo de Oliveira Teles, Rodrigo Cardoso Cavalcante, Giselle Viana de Andrade
Introduction: Risk Adjustment for Congenital Heart Surgery 1 (RACHS-1) score is a simple model that can be easily applied and has been widely used for mortality comparison among pediatric cardiovascular services. It is based on the categorization of several surgical palliative or corrective procedures, which have similar mortality in the treatment of congenital heart disease. Objective: To analyze the in-hospital mortality in pediatric patients (<18 years) submitted to cardiac surgery for congenital heart disease based on RACHS-1 score, during a 12-year period...
May 2016: Brazilian Journal of Cardiovascular Surgery
Jason Huang, Kristen K Patton, Jordan M Prutkin
BACKGROUND: The subcutaneous implantable cardioverter-defibrillator (S-ICD) is a novel implanted defibrillator for the prevention of sudden cardiac death that avoids intravascular access. Use of this device is limited by its inability to provide backup pacing. Combined use of the S-ICD with a permanent pacemaker may be the optimal choice in certain situations though experience with the use of both devices together remains limited. METHODS: We reviewed our single center experience with the S-ICD from March 2011 to November 2015...
October 13, 2016: Pacing and Clinical Electrophysiology: PACE
Youn Ju Rhee, Sung Joon Han, Yoo Young Chong, Min-Woong Kang, Shin Kwang Kang, Jae-Hyeon Yu
With advancements in complex repairs in neonates with complicated congenital heart diseases, extracorporeal membrane oxygenation (ECMO) has been increasingly used as cardiac support. ECMO has also been increasingly used for low birth weight (LBW) or very low birth weight (VLBW) neonates. However, since prematurity and LBW are risk factors for ECMO, the appropriate indications for neonates with LBW, especially VLBW, are under dispute. We report a case of ECMO performed in a 1,360-g premature infant with VLBW due to cardiopulmonary bypass weaning failure after repairing infracardiac total anomalous pulmonary venous return...
October 2016: Korean Journal of Thoracic and Cardiovascular Surgery
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