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Congenital Heart Disease

Brian A Boe, Sharon L Cheatham, Aimee K Armstrong, Darren P Berman, Joanne L Chisolm, John P Cheatham
OBJECTIVE: We sought to describe the leaflet morphology variation in the Melody Transcatheter Pulmonary Valve (TPV) and evaluate associated outcomes. The Melody TPV is constructed from harvested bovine jugular venous valves which have been rigorously tested. Natural anatomic leaflet variations are seen in the Melody TPV but have not been evaluated. DESIGN: A Melody TPV leaflet morphology classification system was devised after reviewing a subset of photographed and implanted TPVs...
December 13, 2018: Congenital Heart Disease
Jennifer L Shepherd, Shahab Noori
OBJECTIVE: There is no consensus on the definition of a hemodynamically significant patent ductus arteriosus (hsPDA). In this review article, our objective is to discuss the main variables that one should consider when determining the hemodynamic significance of a PDA. RESULTS: We describe the various approaches that have been utilized over time to define an hsPDA and discuss the strengths and weaknesses of each echocardiographic index. Finally, we propose a comprehensive and individualized approach in determining the hemodynamic significance of the PDA...
December 12, 2018: Congenital Heart Disease
John M Ferguson
Even though up to 60% of premature infants less than 28 weeks gestation develop persistent patent ductus arteriosus (PDA), there remains controversy regarding if, when, and how to close the PDA. Failure to close the PDA has been associated with significant morbidity but no cause-and-effect has been proven for short-term or long-term outcomes in modern times. Surgical closure has the advantage of eliminating the PDA, but short-term complications and long-term adverse outcomes are worrisome. Intravenous indomethacin has been the "gold standard" for pharmacologic treatment over the past 40 years with high closure rates and decreased incidence of severe intraventricular hemorrhage (IVH) and pulmonary hemorrhage with early treatment but without improvement in long-term outcomes and with risk of renal toxicity...
December 11, 2018: Congenital Heart Disease
Anthony Merlocco
The ductus arteriosus (DA) has been studied since Galen. Initially after birth in neonates with obstruction to pulmonary blood flow, DA patency is integral to ensure output and oxygenation. While DA stenting dates back 25 years, there is emerging interest in better understanding how and when to utilize this strategy as an alternative to surgical shunt placement or ongoing prostaglandin administration. Understanding the normal fetal circulation and the perturbations that affect flow and oxygenation is integral to comprehending how normal DA anatomy and morphology may change and how this may influence technical and clinical considerations...
December 4, 2018: Congenital Heart Disease
Vanessa Marie Hormaza, Mark Conaway, Daniel Scott Schneider, Jeffrey Eric Vergales
OBJECTIVE: Limited information is known on how right ventricular function affects outcomes after stage 2 palliation. We evaluated the impact of different right ventricular indices prior to stage 2 palliation on morbidity and mortality. DESIGN: Retrospective study design. SETTING: Pediatric Heart Network Single Ventricle Reconstruction Trial Public Data Set. PATIENT: Any variant of stage 1 palliation and all anatomic hypoplastic left heart syndrome variants in the trial were evaluated...
December 2, 2018: Congenital Heart Disease
Soham Dasgupta, Ishaan Dave, Courtney E McCracken, Larry Mohl, Ritu Sachdeva, William Border
BACKGROUND: Physicians are exposed to workplace factors that may result in acute or chronic stress resulting in burnout. This may impact the productivity and result in suboptimal patient care practices. METHODS: We surveyed pediatric cardiology attending physicians at our institution to assess their perception of burnout and work-life balance using the Maslach Burnout Inventory and the Areas of Work-Life Survey. RESULTS: Forty-five out of the 50 pediatric cardiology attendings responded to the survey...
December 2, 2018: Congenital Heart Disease
Charlotte A Houck, Reinder Evertz, Christophe P Teuwen, Jolien W Roos-Hesselink, Janneke A E Kammeraad, Anthonie L Duijnhouwer, Natasja M S de Groot, Ad J J C Bogers
OBJECTIVE: Outcomes after surgical repair of complete atrioventricular septal defect (cAVSD) have improved. With advancing age, the risk of development of dysrhythmias may increase. The aims of this study were to (1) examine development of sinus node dysfunction (SND), atrial and ventricular tachyarrhythmias, and (2) study progression of atrioventricular conduction abnormalities in young adult patients with repaired cAVSD. STUDY DESIGN: In this retrospective multicenter study, 74 patients (68% female) with a cAVSD repaired in childhood were included...
November 28, 2018: Congenital Heart Disease
Ersin Çagrı Şimşek, Selcen Yakar Tülüce, Kamil Tülüce, Sadık Volkan Emren, Serap Çuhadar, Cem Nazlı
OBJECTIVE: The bicuspid aortic valve (BAV) is the most common congenital heart disease. The process of aortic dilatation is not completely clear in patients with the BAV. Apelin is a peptide found at high levels in vascular endothelial cells which has a role in vascular regulation and cardiovascular function. The aim of this study was to determine the relationship between serum apelin levels and ascending aortic dilatation in adult patients with BAV. DESIGN: This cross-sectional study included 62 patients with isolated BAV and to an age, gender, and body mass index-matched control group of 58 healthy volunteers with tricuspid aortic valve...
November 28, 2018: Congenital Heart Disease
Camden L Hebson, Michael E McConnell, David W Hannon
Dysautonomia is an increasingly recognized yet still poorly understood disease within the field of pediatrics. Symptoms, including dizziness, headaches, fatigue, joint pain, anxiety, and intolerance of heat or cold, are often significant and difficult to sort, especially in terms of their relation to each other. This often leads to referral to multiple subspecialists, who then proceed to treat seemingly familiar symptoms in kind. In the authors' experience, this leads to more frustration on the part of the patients and their physicians when symptom improvement does not follow (or can even worsen)...
November 28, 2018: Congenital Heart Disease
Stacey L Crockett, Courtney D Berger, Elaine L Shelton, Jeff Reese
Regulation of the ductus arteriosus, an essential fetal vessel connecting the pulmonary artery and aorta, is complex. Failure of this vessel to close after birth may result in a persistent left-to-right shunt through the patent ductus arteriosus, a condition associated with significant morbidities. Numerous factors contribute to the shift from fetal ductus patency to postnatal closure, requiring precise coordination of molecular cues with biomechanical forces and underlying genetic influences. Despite significant advances, questions remain regarding signaling dynamics and the natural time course of ductus closure, particularly in preterm neonates...
November 23, 2018: Congenital Heart Disease
Nayan T Srivastava, Roger Hurwitz, W Aaron Kay, George J Eckert, Alisha Kuhlenhoelter, Nicole DeGrave, Eric S Ebenroth
OBJECTIVE: For over 20 years, we have followed a cohort of patients who underwent the Mustard procedure for d-transposition of the great arteries. The current study follows the same cohort from our last study in 2007 to reassess their functional capacity and quality of life. PARTICIPANTS: Of the original 45 patients, six patients have required cardiac transplant and 10 patients have died, including two of the transplanted patients. Twenty-five of the remaining patients agreed to participate in this current study...
November 23, 2018: Congenital Heart Disease
Jennifer L Lapum, Suzanne Fredericks, Barbara Bailey, Terrence M Yau, Jennifer Graham, Ariane J Marelli
OBJECTIVE: The number of adults with congenital heart disease (CHD) has increased substantially because of medical advances that are extending life expectancy beyond childhood. A historical investigation is timely not only because of this significant demographic shift, but also because stakeholder engagement is increasingly important in shaping research directions. The study questions were: From the perspective of stakeholders, what is known about the medical treatment for the adult CHD cohort in Canada and how has it changed over time? DESIGN/METHODS: We harnessed the use of historical research methods and conducted interviews with 21 key informants in order to articulate a contextualized understanding of the evolution of adult CHD treatment in Canada...
November 19, 2018: Congenital Heart Disease
Joseph T Poterucha, Saraschandra Vallabhajosyula, Alexander C Egbe, Joseph S Krien, Devon O Aganga, Kimberly Holst, Adele W Golden, Joseph A Dearani, Sheri S Crow
BACKGROUND: High levels of vasoactive inotrope support (VIS) after congenital heart surgery are predictive of morbidity in pediatric patients. We sought to discern if this relationship applies to adults with congenital heart disease (ACHD). METHODS: We retrospectively studied adult patients (≥18 years old) admitted to the intensive care unit after cardiac surgery for congenital heart disease from 2002 to 2013 at Mayo Clinic. Vasoactive medication dose values within 96 hours of admission were examined to determine the relationship between VIS score and poor outcome of early mortality, early morbidity, or complication related morbidity...
November 19, 2018: Congenital Heart Disease
Matthew Lewis, William Whang, Angelo Biviano, Kathleen Hickey, Hasan Garan, Marlon Rosenbaum
BACKGROUND: Catheter ablation is commonly performed to treat atrial arrhythmias in adult congenital heart disease (ACHD). Despite the frequency of ablations in the ACHD population, predictors of recurrence remain poorly defined. OBJECTIVE: We sought to determine predictors of arrhythmia recurrence in ACHD patients following catheter ablation for atrial arrhythmias. METHODS: We performed a retrospective study of all catheter ablations for atrial arrhythmias performed in ACHD patients between January 12, 2005 and February 11, 2015 at our institution...
November 19, 2018: Congenital Heart Disease
Patrick D Evers, Tarek Alsaied, Jeffrey B Anderson, James F Cnota, Allison A Divanovic
OBJECTIVE: Maternal anti-Ro/SSA and anti-La/SSB antibodies can lead to fetal complete heart block (CHB). Current guidelines recommend weekly echocardiographic screening between 16 and 28 weeks gestation. Given the cost of screening and the rarity of conduction abnormalities in fetuses of mothers with low anti-Ro levels (<50 U/mL), we sought to identify a strategy that optimizes resource utilization. DESIGN: Decision analysis cost-utility modeling was performed for three screening paradigms: "standard screening" (SS) in which mid-gestation mothers are screened weekly, "limited screening" (LS) in which fetal echocardiograms are avoided unless the fetus develops bradycardia, and "targeted screening by maternal antibody level" (TS) in which only high anti-Ro values warrant weekly screening...
November 16, 2018: Congenital Heart Disease
Jun Oyamada, Chisato Shimizu, Jihoon Kim, Matthew R Williams, Eileen Png, Martin L Hibberd, Adriana H Tremoulet, James C Perry, Jane C Burns
BACKGROUND: We previously described the association of genetic variants in calcium channel genes and susceptibility to Kawasaki disease (KD), an acute, self-limited vasculitis, and the most common cause of acquired cardiac disease in children. Abnormal repolarization of cardiomyocytes and changes in T wave morphology have been reported in KD but have not been studied systematically. METHODS: We analyzed acute and convalescent ECG T wave morphology in two independent cohorts of KD subjects and studied the association between bifid T waves and genetic variants in previously reported genes with SNVs associated with cardiac repolarization...
November 5, 2018: Congenital Heart Disease
Adam B Christopher, Abraham Apfel, Tao Sun, Jackie Kreutzer, David S Ezon
OBJECTIVE: The most accurate noninvasive parameter to predict whether a patient with aortic coarctation will meet interventional criteria at catheterization remains elusive. We aim to determine the best independent echocardiographic predictors of a coarctation peak-to-peak pressure gradient ≥20 mm Hg at catheterization, the accepted threshold for intervention. DESIGN: Retrospective query of our catheterization database from 1/2007 to 7/2016 for the diagnostic code of aortic coarctation was performed...
November 5, 2018: Congenital Heart Disease
Alessia Callegari, Rhoia Neidenbach, Ornella Milanesi, Biagio Castaldi, Martin Christmann, Masamichi Ono, Jan Müller, Peter Ewert, Alfred Hager
AIM: The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and quality of life is unknown and objective of this study. METHODS: This multicenter retrospective/cross-sectional study included 232 patients (140 females, age 25.6 ± 10.8 years) after Fontan palliation (19.8% atrioventricular connection; 20...
October 30, 2018: Congenital Heart Disease
Neil C Zaki, Michael S Kelleman, W James Parks, Timothy C Slesnick, Michael E McConnell, Matthew E Oster
OBJECTIVE: Gated cardiac MRI offers the most detailed and accurate noninvasive method of assessing cardiac anatomy, particularly in patients with complex congenital heart disease. The proposed benefits of using cMRI as a routine screening tool in the Fontan population include early recognition of asymptomatic, postoperative anatomic and physiologic changes. In 2011, we therefore instituted at our center a recommended practice of cMRI screening in patients with Fontan physiology at 3 and 8 years post-Fontan operation...
October 30, 2018: Congenital Heart Disease
Masaki Sato, Kei Inai, Mikiko Shimizu, Hisashi Sugiyama, Toshio Nakanishi
OBJECTIVE: The recognition of fluid retention is critical in treating heart failure (HF). Bioelectrical impedance analysis (BIA) is a well-known noninvasive method; however, data on its role in managing patients with congenital heart disease (CHD) are limited. Here, we aimed to clarify the correlation between BIA and HF severity as well as the prognostic value of BIA in adult patients with CHD. DESIGN: This prospective single-center study included 170 patients with CHD admitted between 2013 and 2015...
October 23, 2018: Congenital Heart Disease
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