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Congenital Heart Disease

Raffaele Giordano, Massimiliano Cantinotti, Giuseppe Comentale, Luigi Di Tommaso, Gabriele Iannelli, Emanuele Pilato, Gaetano Palma
BACKGROUND: In this study, we compared our experience about early and midterm follow-up outcomes for right anterolateral minithoracotomy (RAMT) vs full sternotomy (FS) in surgical aortic valve replacement (AVR) among adolescents with bicuspid aortic valve (BAV). METHODS: Patients were retrospectively enrolled from January 2008 to December 2017. Inclusion criteria were patients with BAV who had to undergo to AVR. They were divided in two groups: RAMT and FS. The choice of RAMT was based on individual surgeon's preferences or when expressly requested by patient that was informed of nonconventional approach...
October 9, 2018: Congenital Heart Disease
Soham Dasgupta, Megan Stark, Sonal Bhatt, Peter Fischbach, Shriprasad Deshpande
INTRODUCTION: A cardiac cause is an extremely rare etiology of pediatric chest pain. Despite its low sensitivity/specificity, exercise stress testing (EST) is widely used to determine the prognosis in patients with suspected/established coronary disease. We aimed to look at the utility of a combined cardiopulmonary EST in the evaluation of pediatric patients with chest pain. METHODS: After institutional review board approval, a retrospective chart review was performed of all pediatric patients who were referred for an EST for chest pain from January 2014 to 2017...
October 9, 2018: Congenital Heart Disease
Christian Paech, Franziska Wagner, Sebastian Mensch, Roman Antonin Gebauer
INTRODUCTION: Reflex vasovagal-or cardioinhibitory syncope is known to be a major cause of recurrent syncope in children. The mechanism of vasovagal syncope (VVS) is an interaction between a vagally mediated bradycardia or asystole and a more or less manifest vasodilatory component. Although pacing is not advisable as a standard approach in patients with VVS, it remains a treatment option of last resort in exceptionally severe cases, or patients with contraindication or refractoriness to drug therapy and life style changes...
October 9, 2018: Congenital Heart Disease
Shae Anderson, Courtney E McCracken, Ritu Sachdeva
OBJECTIVE: The educational intervention (EI) through the Pediatric Appropriate Use of Echocardiography (PAUSE) multicenter study resulted in improved appropriateness of transthoracic echocardiogram (TTE) orders at our center. The current study evaluated if this pattern persisted after cessation of EI and the potential physician characteristics influencing appropriateness. DESIGN: Outpatients (≤18 years old) seen for initial evaluation during the EI (July to October, 2015) and 6-month post-EI (May to August, 2016) phases were included...
October 8, 2018: Congenital Heart Disease
Mohd Nizam Mat Bah, Mohd Hanafi Sapian, Mohammad Tamim Jamil, Nisah Abdullah, Emieliyuza Yusnita Alias, Norazah Zahari
OBJECTIVES: There is limited data on congenital heart disease (CHD) from the lower- and middle-income country. We aim to study the epidemiology of CHD with the specific objective to estimate the birth prevalence, severity, and its trend over time. DESIGN: A population-based study with data retrieved from the Pediatric Cardiology Clinical Information System, a clinical registry of acquired and congenital heart disease for children. SETTING: State of Johor, Malaysia...
October 5, 2018: Congenital Heart Disease
Nurdan Ozturk Tasar, Pelin Kosger, Nevzat Uzuner, Birsen Ucar
OBJECTIVE: We aimed to investigate the effect of ventricular septal defect (VSD) and heart failure on cerebral blood flow (CBF) in children, whether heart failure treatment improves CBF, and if there is any relationship between CBF and serum N-terminal pro-brain natriuretic peptide (NT-proBNP) level. METHOD: Forty children with VSD (13 with heart failure) aged between 1 and 36 months were studied. The control group comprised 25 healthy children in the same age group...
October 2, 2018: Congenital Heart Disease
Martin A Chacon-Portillo, Rodrigo Zea-Vera, Huirong Zhu, Heather A Dickerson, Iki Adachi, Jeffrey S Heinle, Charles D Fraser, Carlos M Mery
OBJECTIVE: There are limited studies analyzing pulsatile Glenn as a long-term palliation strategy for single ventricle patients. This study sought to determine their outcomes at a single institution. DESIGN: A retrospective review was performed. SETTING: Study performed at a single pediatric hospital. PATIENTS: All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included. OUTCOME MEASURES: Pulsatile Glenn failure was defined as takedown, transplant, or death...
October 2, 2018: Congenital Heart Disease
Carlos E B Branco, Roney O Sampaio, Flavio Tarasoutchi, Justin P Zachariah
In the 2017, "Cairo Accord on Rheumatic Heart Disease-From Molecules to The Global Community" experts from endemic areas enumerated an approach to reduce the population burden of rheumatic heart disease. The 10 key recommendations include immediate logistical objectives as well as domains for further study. Echocardiographic population screening programs were relegated to research alone. Given the large body of supporting data, relegating echo screening to an investigational modality is an opportunity lost...
October 2, 2018: Congenital Heart Disease
Shahnawaz M Amdani, Robert D Ross, Paul A Webster, Daniel R Turner, Thomas J Forbes, Daisuke Kobayashi
INTRODUCTION: Reduction of radiation dosage in the pediatric cardiac catheterization laboratory (PCL) is important to reduce the risk of its stochastic effect in children with congenital heart disease. Lowering the frame rate would reduce radiation dosage possibly at the expense of image quality, potentially resulting in higher fluoroscopic time and procedural complication rate. METHODS: The data were retrospectively analyzed in three eras: era 1 (n = 234), cineangiography 30 frames/sec (f/s) and fluoroscopy 15 pulse/sec (p/s); era 2 (n = 381), cineangiography 30 f/s and fluoroscopy 6 p/s; and era 3 (n = 328), cineangiography 15 f/s and fluoroscopy 6 p/s...
October 2, 2018: Congenital Heart Disease
Nikki M Singh, Rohit S Loomba, Todd M Gudausky, Michael E Mitchell
INTRODUCTION: In patients with tetralogy of Fallot (TOF), use of transannular patch (TAP) may be required in order to relieve significant right ventricular outflow tract obstruction, subsequently resulting in pulmonary insufficiency (PI). The monocusp valve has been used to temporarily reduce insufficiency in hopes to improve short and midterm outcomes. The purpose of this study was to assess for potential benefits of the monocusp valve in this subset of patients. DESIGN: Between 2005 and 2016, 119 patients with TOF with pulmonary stenosis who underwent repair with TAP were analyzed, 43 (36...
September 27, 2018: Congenital Heart Disease
Xin Li, Chengxin Zhang, Di Dai, Haiyuan Liu, Shenglin Ge
OBJECTIVE: We conducted a meta-analysis to evaluate the effects of prophylactic perioperative dexmedetomidine administration on postoperative junctional ectopic tachycardia (JET) and acute kidney injury (AKI) in pediatric patients having undergone cardiac surgery. DESIGN: This systematic review was registered with PROSPERO (CRD42017083880). Databases including PubMed, Cochrane Central Register of Controlled Trials, and Web of Science were searched for randomized controlled trials (RCTs) and observational cohort studies from its inception to March 2018...
September 27, 2018: Congenital Heart Disease
Lidia R Bons, Lucas Uchoa de Assis, Silvy Dekker, Robert M Kauling, Judith A A E Cuypers, Hence J M Verhagen, Ricardo P J Budde, Jolien W Roos-Hesselink
OBJECTIVE: The aim is to present current clinical practice of thoracic aorta screening in a tertiary referral center. We identified how often imaging techniques were used for screening and established the value of transthoracic echocardiography (TTE) in comparison with computed tomography (CT) to detect aortic dilation. We also investigated which additional abnormalities of the heart, aorta or smaller arteries were discovered. DESIGN: All patients ≥15 years who visited our tertiary center in 2012-2016 for first thoracic aortic screening were retrospectively included...
September 27, 2018: Congenital Heart Disease
Joshua Saef, Sandeep Sodhi, Kristen M Tecson, Vanessa Al Rashida, Jong Mi Ko, Kamila S White, Philip A Ludbrook, Ari M Cedars
OBJECTIVE: Growth in the adults with congenital heart disease (ACHD) population represents a challenge to the health care infrastructure. As patients with chronic disease are increasingly held accountable for their own care, contributors to disease-specific health knowledge, which are known to correlate with patients' participation in care, merit investigation to design patient-focused interventions. DESIGN: We conducted a single-site, cross-sectional study of ACHD patients...
September 27, 2018: Congenital Heart Disease
Phong Teck Lee, Felix Maverick Uy, Jie Sheng Foo, Ju Le Tan
BACKGROUND: Ventricular septal defect (VSD) is one of the most common congenital heart anomalies in childhood and there is an increasing prevalence of VSDs in the adult population. The long-term risk of infective endocarditis (IE) is of concern. The aim of this study was to clarify and compare the incidence of IE in adults with repaired and unrepaired VSDs. METHODS: Patients with VSDs were identified using the Adult Congenital Heart Disease registry at the National Heart Centre Singapore...
September 27, 2018: Congenital Heart Disease
Rohit S Loomba, Seth B Gray, Saul Flores
INTRODUCTION: Ketamine is a drug often used for procedural sedation or as adjunct agent for general sedation in children with congenital heart disease. In the clinical realm, there is often confusion regarding the effects of ketamine on hemodynamics, particularly pulmonary vascular resistance and systemic vascular resistance. We performed a meta-analysis of studies investigating the effects of ketamine on hemodynamics. METHODS: A systematic review was conducted to identify studies characterizing the hemodynamic effects of ketamine in children with congenital heart disease...
September 27, 2018: Congenital Heart Disease
Martina Nassif, Reindert P van Steenwijk, Jacqueline M Hogenhout, Huangling Lu, Rianne H A C M de Bruin-Bon, Alexander Hirsch, Peter J Sterk, Berto J Bouma, Bart Straver, Jan G P Tijssen, Barbara J M Mulder, Robbert J de Winter
OBJECTIVE: The association between secundum atrial septal defects (ASD) and asthma-like dyspnea with consequent long-term pulmonary inhalant use, is poorly understood in adult ASD patients. Airway hyperresponsiveness is suggested to be the underlying mechanism of cardiac asthma from mitral valve disease and ischemic cardiomyopathy. We hypothesized that airway hyperresponsiveness may also be found in adult ASD patients. Our aim was to study airway responsiveness in adult ASD patients before percutaneous closure and at short-and long-term postprocedural follow-up...
September 21, 2018: Congenital Heart Disease
Eliana Al Haddad, Damien J LaPar, Jeffrey Dayton, Elizabeth H Stephens, Emile Bacha
BACKGROUND: Congenital heart defects affect nearly 1% of all children born per year in the United States, and complete atrioventricular canal (CAVC) accounts for 2%-9%. While several patch materials have been used for septal defect closure during CAVC repair, clear superiority of one material over another has yet to be established. METHODS: A retrospective review of clinical outcomes following CAVC repair at Morgan Stanley Children's Hospital/Columbia University was performed on operations conducted from March 2010 to September 2017...
September 21, 2018: Congenital Heart Disease
Benjamin M Moore, Rachael L Cordina, Mark A McGuire, David S Celermajer
OBJECTIVE: Amiodarone is a highly effective antiarrhythmic therapy, however its toxicity profile often limits treatment. This is particularly relevant in adults with congenital heart disease (CHD), who are often young and in whom other antiarrhythmic agents commonly fail or are contraindicated. We sought to determine incidence and predictors of adverse effects caused by amiodarone in adult CHD (ACHD). DESIGN: A retrospective review of patients with moderate to complex ACHD treated with amiodarone at our center between 2000 and 2017 was performed...
September 21, 2018: Congenital Heart Disease
Shiraz A Maskatia, Christopher J Petit, Curtis D Travers, David J Goldberg, Lindsay S Rogers, Andrew C Glatz, Athar M Qureshi, Bryan H Goldstein, Jingning Ao, Ritu Sachdeva
BACKGROUND: In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echocardiographic parameters associated with 2V circulation, (2) identify echocardiographic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions. METHODS: We performed a retrospective analysis of patients who underwent RV decompression for PA/IVS at four centers...
September 21, 2018: Congenital Heart Disease
Shankar Baskar, Seth B Gray, Erica L Del Grippo, Onyekachukwu Osakwe, Adam W Powell, Justin T Tretter
Cardiology fellows-in-training, both in adult and pediatric hospitals, need structured education in regards to congenital heart disease (CHD) nomenclature. With improved survival of patients with CHD, it is not uncommon for these patients to seek care in multiple adult and pediatric hospitals. A deep understanding of CHD nomenclature would aid in providing accurate medical and surgical care for these patients. In this forum, we share our experience with such structured education and also comment on recent advances in morphologic imaging that would aid in understanding the nomenclature...
September 21, 2018: Congenital Heart Disease
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