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Congenital cardiac defect

Wasay M Shaikh Qureshi, Lianjie Miao, David Shieh, Jingjing Li, Yangyang Lu, Saiyang Hu, Margarida Barroso, Joseph Mazurkiewicz, Mingfu Wu
Single clonal tracing and analysis at the whole-heart level can determine cardiac progenitor cell behavior and differentiation during cardiac development, and allow for the study of the cellular and molecular basis of normal and abnormal cardiac morphogenesis. Recent emerging technologies of retrospective single clonal analyses make the study of cardiac morphogenesis at single cell resolution feasible. However, tissue opacity and light scattering of the heart as imaging depth is increased hinder whole-heart imaging at single cell resolution...
October 7, 2016: Journal of Visualized Experiments: JoVE
Rohit Bhoil, Shikha Sood, Sabina Bhoil, Anshul Chamail, R G Sood
Left coronary artery compression syndrome is an uncommon entity and characterized by compression of the LMCA in-between the aorta and an enlarged main pulmonary arterial trunk. It is usually associated with a congenital cardiac defect. Cardiac 64-slice MDCT provides a non-invasive and an accurate method for assessing the degree of dynamic LMCA compression throughout the cardiac cycle, its angulation relative to the left sinus of Valsalva and depiction of pulmonary pathology, making it a valuable tool in the workup of patients suspected of left coronary artery compression...
October 18, 2016: Acute Cardiac Care
Fabio de Araujo Motta, Libera Maria Dalla-Costa, Marisol Dominguez Muro, Andrea Lenzi, Gledson Luiz Picharski, Marion Burger
BACKGROUND: Candida species are the primary cause of invasive fungal infection in hospitalized children. There are few data on risk factors for postoperative candidemia in pediatric patients with congenital heart defects. This study aimed to identify risk factors for candidemia in patients with congenital heart defects who underwent cardiac surgery. METHODS: This was a case-control study conducted in patients admitted to a pediatric cardiology intensive care unit from January 2006 to December 2013...
November 2016: Pediatric Infectious Disease Journal
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
Aabid Hussain Mir, Zulfiqar Ali, Bashir Ahmad Dar, Imtiaz A Naqash, Samreena Bashir
Congenital heart defects are associated with various physiological disturbances. They pose anesthetic challenges for both cardiac and noncardiac surgeries. Atrioventricular septal defects are due to a developmental failure in the separation of atria and the ventricles into separate chambers and failure in the separation of mitral and tricuspid valves. We present a case of a child (1½ years), weighing 10 kg, diagnosed as congenital hydrocephalus who was planned for ventriculoperitoneal shunt. Child was having an oxygen saturation of 76% on room air...
September 2016: Anesthesia, Essays and Researches
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
Yi-Ju Ho, Hung-Chi Chen, Shirley H L Chang, Lung-Kung Yeh, David Hui-Kang Ma
BACKGROUND: Sclerocornea, a congenital corneal pathology characterized by bilateral scleralization of the cornea, which can be found in few cases with posterior fossa malformationshemangiomas-arterial anomalies-cardiac defects-eye abnormalities-sternal cleft and supraumbilical raphe (PHACES) syndrome. Presence of vascularization in peripheral cornea and smaller diameter of recipient cornea correlate to poor outcome of penetrating keratoplasty (PKP) in sclerocornea. Here we report a method to preserve limbus during PKP for small, irregular, and scleralized cornea...
October 2016: Medicine (Baltimore)
Anu K Kaskinen, Laura Martelius, Turkka Kirjavainen, Paula Rautiainen, Sture Andersson, Olli M Pitkänen
BACKGROUND: Lung ultrasounds show vertical artifacts known as B-lines in the presence of increased extravascular lung water (EVLW). We aimed to investigate whether lung ultrasound could estimate EVLW after congenital cardiac surgery. METHODS: This prospective observational study comprised 61 children (age range 3 days to 7.4 years) undergoing congenital cardiac surgery. We compared postoperative B-line scores from lung ultrasounds, early postoperative ultrasound as our primary interest, with corresponding postoperative chest radiography (CXR) lung edema scores, with static lung compliance, and with short-term clinical outcome interpreted as time on mechanical ventilation and length of pediatric intensive care unit (PICU) stay...
October 14, 2016: Pediatric Pulmonology
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
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
Katarzyna Iwanicka-Pronicka, Magdalena Socha, Maria Jędrzejowska, Małgorzata Krajewska-Walasek, Aleksander Jamsheer
Holt-Oram syndrome (HOS) features radial ray hypoplasia, heart defect and cardiac conduction impairment. Ulnar-mammary syndrome (UMS) characterizes congenital defects of the ulnar side of the upper limbs, underdevelopment of apocrine glands including hypoplasia and the dysfunction of mammary glands, hypogonadism and obesity. Inheritance of both conditions is autosomal dominant, mutations or deletions are found in the TBX5 and TBX3 gene, respectively. The Polish patient presented short stature, obesity, congenital malformation of the radial and ulnar side of the upper limbs, heart block, hypogonadism and dysmorphic features...
2016: SpringerPlus
Christopher E Slagle, Frank L Conlon
Congenital heart defects remain a leading cause of infant mortality in the western world, despite decades of research focusing on cardiovascular development and disease. With the recent emergence of several high-throughput technologies including RNA sequencing, chromatin-immunoprecipitation-coupled sequencing, mass-spectrometry-based proteomics analyses, and the numerous variations of these strategies, investigations into cardiac development have been transformed from candidate-based studies into whole-genome, -transcriptome, and -proteome undertakings...
November 2016: Trends in Genetics: TIG
Soichiro Funaki, Yasushi Shintani, Masato Minami, Takayoshi Ueno, Koichi Toda, Yoshiki Sawa, Meinoshin Okumura
Eisenmenger syndrome(ES) could occur at the end-stage of congenital heart disease and is one of the indications for lung transplantation. Survival following heart-lung transplantation is better than that following bilateral lung transplantation (LT). However, in Japan, because of limitations in regard to the numbers of donors available, LT with cardiac repair is usually chosen. Following LT with cardiac repair, careful management of the repaired heart is necessary, though there are few reports available regarding such postoperative cardiac management...
October 2016: Kyobu Geka. the Japanese Journal of Thoracic Surgery
Juan-Miguel Gil-Jaurena, Ramón Pérez-Caballero, Ana Pita-Fernández, María-Teresa González-López, Jairo Sánchez, Juan-Carlos De Agustín
BACKGROUND: Mid-line sternotomy is the commonest incision for cardiac surgery. Alternative approaches are becoming fashionable in many centres, amidst some reluctance because of learning curves and overall complexity. Our recent experience in starting a new program on minimally invasive pediatric cardiac surgery is presented. The rationale for a stepwise onset and the short-medium term results for a three-year span are displayed. METHODS: A three-step schedule is planned: First, an experienced surgeon (A) starts performing simple cases...
July 2016: Translational pediatrics
Zijo Begic, Edin Begic, Senka Mesihovic-Dinarevic, Izet Masic, Senad Pesto, Mirza Halimic, Almira Kadic, Amra Dobraca
OBJECTIVE: To show the place and role of continuous electrocardiographic twenty-four-hour ECG monitoring in daily clinical practice of pediatric cardiologists. METHODS: According to protocol, 2753 patients underwent dynamic continuous ECG Holter monitoring (data collected from the "Register of ECG Holter monitoring" of Pediatric Clinic, UCC Sarajevo in period April 2003- April 2015). RESULTS: There were 50,5% boys and 49,5% girls, aged from birth to 19 years (1,63% - neonates and infants, 2,6% - toddlers, 9,95% - preschool children, 35,5% - gradeschoolers and 50,3% children in puberty and adolescence)...
July 16, 2016: Acta Informatica Medica: AIM
Christophe P Teuwen, Tim I M Korevaar, Rosa L Coolen, Twan van der Wel, Charlotte A Houck, Reinder Evertz, Ameeta Yaksh, Jolien W Roos-Hesselink, Ad J J C Bogers, Natasja M S de Groot
AIMS: Atrial fibrillation (AF) is increasingly observed in patients with congenital heart defects (CHDs) who survive nowadays into adulthood. Yet, predictors of AF are scarce in this high-risk population. This study therefore examined the predictive ability of atrial extrasystole (AES) for development of AF in CHD patients. METHODS AND RESULTS: Adult CHD patients who had a 24 h Holter registration were followed to determine who developed AF. A total of 573 patients (49% male, mean age 35 ± 12 years) were included; they had a simple/complete repaired CHD (n = 279), complex repaired CHD (n = 251), or univentricular heart (UVH, n = 43)...
October 4, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
Wilson E Sadoh, Paul Ikhurionan, Charles Imarengiaye
BACKGROUND: A pre-anaesthestic echocardiogram (echo) is requested for most non-cardiac surgeries to identify possible cardiac structural anomalies. OBJECTIVE: To describe the prevalence and spectrum of structural cardiac abnormalities seen in various non-cardiac conditions. METHODS: We carried out a retrospective review of pre-anaesthetic echos performed over five years on children scheduled for non-cardiac surgery. The requests were categorised according to referring specialities, and the biodata and echo findings were noted...
September 9, 2016: Cardiovascular Journal of Africa
David R Busch, Craig G Rusin, Wanda Miller-Hance, Kathy Kibler, Wesley B Baker, Jeffrey S Heinle, Charles D Fraser, Arjun G Yodh, Daniel J Licht, Kenneth M Brady
While survival of children with complex congenital heart defects has improved in recent years, roughly half suffer neurological deficits suspected to be related to cerebral ischemia. Here we report the first demonstration of optical diffuse correlation spectroscopy (DCS) for continuous and non-invasive monitoring of cerebral microvascular blood flow during complex human neonatal or cardiac surgery. Comparison between DCS and Doppler ultrasound flow measurements during deep hypothermia, circulatory arrest, and rewarming were in good agreement...
September 1, 2016: Biomedical Optics Express
Tuncer Ahmet Ali, Karavelioğlu Afra, Baskin Embleton Didem, Elmas Muhsin
Holt-Oram syndrome (HOS) is a rare autosomal dominant disorder, characterized by upper limb dysplasia and congenital cardiac defect. We report two cases with HOS, first associated with renal agenesis, coronal hypospadias, urethral duplication and second associated with duodenal atresia and horseshoe kidney that have not been reported in English literature.
October 2016: Journal of Indian Association of Pediatric Surgeons
P Laborda-Vidal, B Pedro, M Baker, A R Gelzer, J Dukes-McEwan, T W Maddox
Pulmonic stenosis (PS) is the most common congenital cardiac disease in dogs. Boxers and English bulldogs are among the most commonly affected breeds and also commonly associated with an aberrant coronary artery (CA). If an aberrant CA is suspected and balloon valvuloplasty indicated, an intra-operative angiography is recommended prior to the procedure. ECG-gated computed tomography (CT) can be used to screen for CA anomalies in a quick and minimally-invasive way (preventing side effects associated with selective catheter angiography) and allowing early planning of the procedure...
September 29, 2016: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
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